Surgery Department Book

CHAPTER 1 Surgical Ethics

INTRODUCTION

Ethics - Definition

- The word ethics is derived from the Greek word ―ethos‖, which means ―character‖. - Formally speaking, ethics is a branch of philosophy that defines things that are beneficial to individuals and society, and establishes the nature of obligations, or duties that people owe themselves and one another

Ethics - History

- The Greek healers in the 4th century BC drafted the Hippocratic Oath and promised to ―prescribe regimens for the good of my patients according to my ability and my judgment and never do harm to anyone‖.

Surgical Ethics

- Ethical study investigates what should be our character and conduct (behavior). - Morality is subject to re-examination and improvement. - The concepts of justice and fairness require critical evaluation and improvement. - Ethical argument should remain relevant.

BASIC PRINCIPLES OF MEDICAL ETHICS

Autonomy

- When making decisions about healthcare procedures patients must have freedom of thought, intention, and action. - Patients must be informed of the consequences of surgery that may adversely affect them.

Beneficence

- It means act of charity, mercy, and kindness - The surgeon should act in the best interest of the patient.

- Surgeons depend on different technology, ranging from diathermy to the lighting in the operation room. - Therefore, a conscientious (wishing to do one's work or duty well and thoroughly) surgeon should ensure that the equipment functions properly and reliably. - Equipment failure can compromise patient care and increase the likelihood of surgical complications.

Non-maleficence

- It means non-harming or inflicting the least harm possible - Most importantly, you should make sure not to do any harm. - Ensure that the procedure doesn‘t harm the patient or any other individuals in society

Justice

- The allocation of scarce health resources and deciding who gets which resources. - The four main areas that the surgeon must consider when assessing justice: 1. Fair distribution of scarce resources 2. Competing needs 3. Rights and obligations 4. Potential conflicts with established legislations

ISSUES – SURGICAL ETHICS

Autonomy

- You should respect the autonomy of patients and their ability to choose treatment options. - Recognize the patient‘s right to self-determination. - Patients have the right to choose their surgical treatments and surgical care. - Respecting the autonomy of the patient is the basis for the ability of the patient to make informed consent.

Informed Consent and Difficulties

- Individuals have the right to obtain all available medical information and the ability to make autonomous decisions regarding their health care. - Information that should be provided to the patient: 1. Explanation of the patient‘s illness. 2. Explanation of untreated natural history. 3. Suggestion of the most suitable surgery. 4. Discussion of the risks and the benefits. 5. Alternative treatments and the expected outcome (Prognosis)

Consent Principles - Venue: the venue should be a quiet and calm place. - Consent form: the consent form should be in the patient‘s language. - Time: you should take your own decision regarding the time. - Principal person: you- the surgeon- are the principal person. - Entry: case record. - The information, which you provide to the patient, should be accurate and reasonably complete. 1. You should avoid using technical language, to ensure that the patient understand what you say. 2. Translators should be provided. 3. You should clarify any doubts that the patient has.

Practical Difficulties 1. Refusal or waiver by patient. 2. Temporarily unconscious patients. 3. Children that are under the age of 18 are considered minors and are legally incompetent. 4. Incompetence of other kinds.

End-of-Life Issues - In unusual (near to death) circumstances, where there is no evidence that a specific treatment that the patient desires is beneficial from any perspective, then the physician need not provide such treatment. - If there is no treatment options, that is, the patient‘s brain is dead but the patient‘s family still insists on treatment, given that the physician is powerless (there is nothing that the physician can do), then the treatment must be stopped. - Noted in case sheet along with the senior clinician‘s agreement.

Confidentiality

- The principle of confidentiality is that the patient‘s personal information, that is revealed to the surgeon by the patient is private, and has limits and restrictions on how and when it can be disclosed to a third party. - The patient (and the treating doctor i.e. the surgeon), both have dignity. - Breaking confidentiality (situations where breaking the confidentiality of the patient is acceptable): 1. If the patient poses a threat to himself or to others in society. 2. Sharing the information of the patient with other team members, to improve the treatment options. 3. Public interest. 4. Court order

Surgical Research

- Surgeons have the auxiliary (other) task to improve operative techniques to assure their patients that the care provided is best. - The management of such regulation is conducted through research ethics committees, and surgeons are not allowed to participate in surgical research without the approval of such bodies.

Good Standards

- In order to successfully maintain life and health to an acceptable standard, surgeons must only provide specialized treatments, in which they have been properly trained. - Thus, the surgeon must continue receiving education, throughout his entire career, in case of new surgical procedures. - Not doing so is placing the interests of the surgeon higher than that of the patient, which is not acceptable, neither morally nor professionally.

CHAPTER 2 Shock in Surgery

Shock is a state of acute cardiovascular or circulatory failure. It leads to decreased delivery of oxygenated blood to the body's organs and tissues or impaired oxygen utilization by peripheral tissues, resulting in end-organ dysfunction

PATHOPHYSIOLOGY OF SHOCK

Changes in preload, stroke volume, system vascular resistance, and cardiac output can result in impaired tissue and organ perfusion. The impaired delivery of oxygen to peripheral cells that occurs in shock results in a transition from aerobic to anaerobic cellular metabolism. Anaerobic metabolism generates lactate via metabolism of glucose to pyruvate, and lactate can be used as a surrogate marker for tissue hypoxemia and the severity of shock. Cells can engage in anaerobic metabolism for a limited time, but persistent cellular hypoxia results in cell death and tissue necrosis, leading to multi-organ system dysfunction and failure. The saturation of venous oxygen measured from central vessels (e.g. SVC), is another biochemical marker of peripheral O2 uptake and can be used diagnostically to help in assessment of prognosis.

TYPES OF SHOCK

Hypovolemic Shock

It occurs due to inappropriately low intravascular volume leading to a decrease of preload, stroke volume, and cardiac output. Hypovolemic shock can be due to decreased intravascular fluid or decreased blood volume from hemorrhage for example.

Cardiogenic Shock

Myocardial infarction is the most common cause of cardiogenic shock, which results from failure of the left ventricle (LV) to generate adequate arterial flow to deliver oxygenated blood to peripheral tissues. Cardiogenic shock may be due to disruptions in stroke volume and/or heart rate. Failure of the LV may be due to right ventricle failure or valvular disease. Processes that can negatively affect stroke volume include aberrations in preload, afterload, and myocardial contractility.

Obstructive Shock

It results from either a critical decrease in preload or an increase in left ventricle outflow obstruction. Extra-cardiac processes that increase intra-thoracic pressure can result in obstructive shock by decreasing cardiac compliance and interrupting venous return by compressing the inferior or superior vena cava. Tension pneumothorax, herniation of abdominal contents into the thorax, and positive pressure ventilation are processes that result in decreased cardiac compliance and obstruction of the vena cava, decreased preload, and decreased cardiac output. Extra-cardiac processes that cause right ventricle outflow obstruction include severe pulmonary hypertension and massive pulmonary embolism. Increased right ventricle obstruction causes a decrease in right ventricle stroke volume, pulmonary arterial flow, left ventricle preload, and left ventricle cardiac output, as well as a decrease in delivery of oxygenated blood to peripheral tissue.

Anaphylactic Shock

Anaphylaxis can result in shock due to a mixed distributive and hypovolemic pathophysiology. Anaphylaxis results from activation of mast cells and basophils through immunoglobulin-E binding a specific allergen, resulting in the release of immuno-stimulatory and vasoactive proteins, with profound systemic vasodilation and diffuse vascular leak. Vasodilatation results in decreased systemic vascular resistance and mean arterial pressure (distributive pathophysiology). Vascular leak results in extravasation of intra-vascular fluid and decreased preload (hypovolemic pathophysiology). Volume resuscitation is appropriate in anaphylactic shock, but the mainstay of treatment is rapid administration of epinephrine, which should be given immediately if anaphylaxis is suspected. Histamine receptor antagonists (H1- and H2-blockers) and glucocorticoids are also recommended for patients with anaphylactic shock.

Septic Shock

It is defined as life-threatening organ dysfunction caused by a dys-regulated host response to infection. It is a subset of sepsis with circulatory and cellular/metabolic dysfunction associated with a higher risk of mortality. This can be caused by infections such as bacterial (Gram +ve and Gram –ve), fungal, viral and protozoal infections

CLINICAL FEATURES OF SHOCK

Manifestations of the cause of shock and features of shock depend on the degree of loss of volume and on duration of shock and can be classified into mild, moderate and severe as follows:

Mild Shock

- Collapse of subcutaneous veins of extremities esp. the feet, which become pale and cool - Sweat on forehead, hand and feet - Urine output normal. - Pulse rate normal. - Blood pressure normal. - Patient feels thirsty and cold

Moderate Shock

- Mild shock features + - Drowsy and confused - Oliguria - Pulse rate increased usually less then 100/min. - Blood pressure normal initially then falls in later stage

Severe Shock

- Unconscious - Gasping respiration - Anuria - Rapid pulse - Profound hypotension

STAGES OF SHOCK

1. Initial stage: The cells become leaky and switch to anaerobic metabolism. 2. Non-progressive (compensated) stage: Attempt to correct the metabolic upset of shock. 3. Progressive (decompensated) stage: Eventually the compensation will begin to fail. 4. Refractory stage: Organs fail and the shock can no longer be reversed

MANAGEMENT OF SHOCK

Aim of Treatment

- Treat the cause - Improve cardiac function - Improve tissue perfusion

Principles of Resuscitation

A: Airway: patent upper airway B: Breathing: adequate ventilation and oxygenation C: Circulation: placement of adequate intravenous (IV) access

Emergency Measures and Monitoring

- Patients may need to be immediately intubated - Central pulses should be palpated upon arrival - The chest should be auscultated - If the patient‘s blood pressure is unknown. Patients should be immediately placed on a cardiac monitor, and an automated blood - Pressure cuff should be used to frequently monitor their mean arterial pressure. - Intravenous (IV) access should be obtained, preferably with 2 large-bore IV lines. - Occasionally, patients in shock will require an urgent central venous line placement for vascular access to deliver medications or for volume resuscitation. Intra-osseous line placement is a fast and viable central circulation access option in patients with difficult intravenous access. - Oxygen saturation should be checked immediately and continuously monitored. - An estimation of jugular venous pressure has been advocated to evaluate for right atrial pressures - A 12-lead electrocardiogram (ECG) may help identify the etiology of shock; in addition to demonstrating evidence of myocardial infarction - Urine output has to be assessed and catheterization is needed

Laboratory Investigations

- Complete blood count (CBC) - Basic serum chemistries (including renal function), and other tests (liver function tests, lipase/amylase, cardiac biomarkers, etc) - Arterial blood gas test. Venous blood gas test provides information of patient‘s acid/base status, but does not provide accurate information regarding oxygenation. - Relevant cultures should be obtained early in the diagnostic work-up. - Blood cultures are appropriate in most patients with suspected infection - Urine, cerebral spinal fluid (CSF), pleural, ascitic, and/or other fluid compartment cultures should be sent, as clinically indicated

Imaging Studies

- X-ray chest - Echocardiography - Other studies related to the cause (ultrasound abdomen, computed tomography scan abdomen, etc)

Fluid Therapy in Shock

- Crystalloid Solutions (Normal saline - Ringers Lactate solution - Hartmann‘s solution): Crystalloids are typically indicated for the initial treatment of undifferentiated shock, although there is an emerging body of evidence that this may not be the best selection for trauma patients or patients with traumatic hemorrhagic shock

- Colloid Solutions: Hydroxy-ethyl starch should not be used for volume resuscitation in patients in shock, especially septic shock - Blood transfusion RBCs contribute to oxygen carrying capacity (hemoglobin). Replacement with all other solutions will support volume and improve end-organ perfusion, but will not provide additional oxygen carrying capacity

Dynamic Fluid Response Infusing 250-500 ml of fluids rapidly over 5-10 minutes - Responders: Improvement - Transient responders: revert back - Non-responders

Vasopressors

Once a patient is determined to be euvolemic, but there is still ineffective oxygen delivery, vasoactive, various vasopressor medications may be used to support the mean arterial pressure by increasing systemic vascular resistance and/or cardiac output. - Nor-epinephrine is a strong alpha agonist with some beta-1 activity, and it is a recommended. Initial choice for most categories of shock, particularly when the etiology of shock is unknown. - Inotropes (Dobutamine) may be of value in cardiogenic shock / severe septic shock to increase the cardiac output, although their use has been linked in recent studies with higher incidence of arrhythmias

Antibiotic Therapy in Septic Shock

- Administration of IV anti-microbials should be initiated as soon as possible after recognition and within one hour for both sepsis and septic shock. - Empiric broad-spectrum therapy with one or more anti-microbials is recommended for patients presenting with sepsis or septic shock to cover all likely pathogens. - Empiric anti-microbial therapy should be narrowed once pathogen identification and sensitivities are established and/or adequate clinical improvement is noted. - Systemic anti-microbial prophylaxis in patients with severe inflammatory states of non- infectious origin is not recommended. - If combination therapy is initially used for septic shock, de-escalation is recommended with discontinuation of combination therapy within the first few days in response to clinical improvement and/or evidence of infection resolution

End-points of Resuscitation in Shock

Classic / Traditional - Restoration of blood pressure - Normalization of heart rate and urine output - Appropriate mental status

Improved / Global - All of the above plus - Normalization of serum lactate levels - Resolution of base deficit

Goal Directed Approach - Urine output > 0.5 mL/kg/h

- Central venous pressure (CVP): 5 -10 cm H2O - Mean arterial pressure (MAP): 65-90 mmHg - Central venous oxygen concentration > 70%

CHAPTER 3 Hemostasis and Blood Transfusion

HEMOSTASIS

- Hemostasis is the act of restricting or stopping bleeding from a damaged vessel or injured organ during surgery or other invasive procedures. - Bleeding may be of arterial or venous origin. Arterial bleeding is characterized by being pulsatile, whereas venous bleeding usually oozes without any pulsation. - During surgery, balance should be maintained between bleeding and coagulation such that blood should continue to flow and supply tissues at the operative site while at the same time excessive blood loss is prevented/controlled

Factors that may Contribute to Bleeding in Surgery

Procedural Factors Patient Factors - Surgical incisions - Variant anatomical considerations - Exposed bone - Medications (e.g. anticoagulants) - Large surfaces of exposed capillaries - Coagulopathies (hepatic decompensation). - Vascular adhesions stripped during surgery - Platelet dysfunction or deficiency - Friable tissues not amenable for suturing - Fibrinolytic activity - Poor nutritional status

Consequences of Uncontrolled Bleeding during Surgery

On the Patient On the Surgeon/Procedure - Anemia and hypovolemic shock. - Prolongation of the procedure time - Reduction in core temperature. - Obstruction of the surgical visual field. - Thrombocytopenia. - The need for ICU admission - Longer postoperative hospital-stay - Hazards of blood transfusion and massive replacement.

Different Methods to Achieve Hemostasis

Methods used to achieve hemostasis are listed in the Table below1. They may mechanical, thermal, or chemical.

Table 1: Methods used to achieve hemostasis

Method Examples

Mechanical Methods 1. Direct pressure 2. Fabric pads/gauze sponges/sponges 3. Sutures/staples/ligating clips Thermal/Energy-Based Methods 1. Electro-surgery - Monopolar diathermy - Bipolar diathermy - Bipolar vessel sealing device - Argon enhanced coagulation 2. Ultrasonic device 3. Laser Chemical Methods Pharmacological Agents 1. Epinephrine 2. Vitamin K 3. Protamine 4. Desmopressin 5. Lysine analogues (e.g. aminocaproic acid, transemic acid)

Topical Hemostatic Agents 1. Passive (ie, mechanical) agents - Collagen-based products

- Cellulose - Gelatin - Polysaccharide spheres 2. Active agents: - Thrombin products 3. Flowables 4. Sealants - Fibrin sealants - Polyethylene glycol (PEG) polymers - Albumin and glutaraldehyde

Mechanical Methods

Direct Pressure - The use of direct pressure or compression with one or more fingers at a bleeding site is typically a surgeon‘s first choice to attempt to control bleeding as is the simplest and fastest method (Figure 1). Arterial bleeding is more easily controlled with direct pressure than venous bleeding, which may not always be controlled with direct pressure, and in some cases, direct pressure may even increase the vascular injury and bleeding owing to its thin wall.

Figure 1. Hemostasis by direct pressure - In general, maintaining pressure for 15 to 20 seconds will cause small clots to form at the end of blood vessels. If a major artery or vein has been injured, direct pressure should only be used as an initial temporary method to halt bleeding until the proximal and distal ends of the vessel become controlled or ligated using any of the methods mentioned below.

Fabric Pads/Gauzes/Sponge - Sponge sticks are often used to apply pressure in deep body cavity recesses; care should be taken when removing the sponge stick to avoid dislodging fresh clots. - Packing an area of venous bleeding can help to reduce blood loss when direct pressure control is not an option or when there is generalized bleeding from systemic coagulopathy that has occurred because of infection, trauma, massive blood loss, or platelet dysfunction. Counting the number of sponges used in packing of a cavity should be performed by the surgical team member placing them to ensure all items used will be retrieved before wound closure to avoid missed or retained surgical pack

Figure 2. Hemostasis by packing the bleeding area

Sutures/Staples/Ligating Clips - Sutures and ties are used during operative procedures as ligatures to tie off blood vessels and control bleeding (Figure 3). Considerations when using suture include the type of tissue it will be used on, its tensile strength, and whether it is absorbable or not. Figure 3: Hemostasis by sutures intra-operatively - Staples are applied by using sterile, disposable stapling devices that place staggered rows of titanium staples and concurrently divide the tissue located between the rows of staples to achieve hemostasis (Figure 4). - Ligating clips or hemostatic clips are used to control bleeding blood vessels in an easy and quick way to achieve hemostasis efficiently and reduce the risk of foreign body reaction that may occur with suture material. Ligating clips are available in various sizes and must be used with the Figure 4: Hemostasis by applying staples corresponding size clip applier (Figure 4). or clips

Thermal/Energy-Based Methods

Electro-surgery Electrocautery was developed in the 1930s and it entails the use of high-frequency alternating current for cutting, coagulating, and vaporizing tissues in both open and laparoscopic surgical procedures. Electrosurgical energy is delivered in either monopolar or bipolar mode. A complete electrical circuit is required for current to flow when using either of these modes. The potential risks of electro-surgery use include patient injuries, operating surgeon(s) injuries, fires, and electromagnetic interference with other medical equipment or in-vitro electronic devices. Accordingly, to use such devices, safety is heightened by adhering to good clinical practices, and awareness of adverse events that may occur with patient burns.

1) Monopolar electro-surgery It is the most frequently used electrosurgical method of hemostasis. In a monopolar circuit, electrical current flows from the generator through an active electrode to the patient where controlled heat is produced, which results in either cutting or coagulation of the tissue. The completion of the circuit requires the current to flow from the patient to the dispersive electrode placed on the patient‘s body before it returns to the generator.

2) Bipolar electro-surgery It is another mode where the electrical circuit is completed between the two blades of a bipolar forceps without the need to flow through the patient. Accordingly, a dispersive electrode is not needed. Heat is generated in the tissue as the current flows from one bipolar tip

through the tissue and back to the other tip (Figure 5). Figure 5: Hemostasis by bipolar electrosurgery

3) Bipolar vessel sealing technology It is an advanced electrosurgical modality in which the intimal layers of a vessel are fused together creating a permanent seal (Figure 6). Such devices apply heat over time with high compression, which makes them capable of simultaneously sealing and transecting vessels up to 7 mm in diameter, large tissue pedicles, or Figure 6: Hemostasis by bipolar vessel-sealing vascular bundles.

4) Argon-enhanced coagulation Argon-enhanced coagulation technology uses a stream of inert, non- combustible argon gas to conduct the electrosurgical current (Figure 7). Argon gas is heavier than air and displaces nitrogen and oxygen. The electrosurgical current ionizes the argon gas, which makes it more conductive to heat than air and helps create a bridge between the electrode and the tissue.

Figure 7: Hemostasis by Argon plasma coagulation

Lasers Laser the acronym for light amplification by stimulated emission of radiation. They are common heat-generating devices used by surgeons to provide hemostasis.

Ultrasonic Devices Ultrasonic devices represent another example of vessel-sealing devices that convert electrical energy into mechanical energy that oscillates longitudinally at the point of contact of both device tips enabling simultaneously coagulation and cutting of the vessel ends (Figure 8). These devices can seal vessels up to 5 mm in diameter and offer an alternative to electrosurgical devices mentioned above. Figure 8. Hemostasis by ultrasonic device

Chemical Methods/Topical Hemostatic Products

Pharmacological Agents - Epinephrine causes direct vasoconstriction of blood vessels making it useful during surgery, because it can be applied topically to reduce bleeding. - Vitamin K also plays a role in the coagulation process and may be administered preoperatively as a pro-coagulant to reverse the effects of warfarin. It should be given orally or IV rather than subcutaneous or intramuscular injection route. - Protamine is the only current agent to reverse heparin anticoagulation. (But not low- molecular-weight heparin). - Desmopressin is a synthetic analogue of vasopressin. It stimulates the release of von Willebrand factor (vWF) from endothelial cells to enhance primary hemostasis. While it assists in reducing perioperative bleeding, the effect of desmopressin is too small to influence other, more clinically relevant outcomes, such as the need for blood transfusion and repeat procedures. - Synthetic lysine analogues are anti-fibrinolytic agents that competitively inhibit activation of plasminogen, thereby reducing its conversion to plasmin, and thus reducing the degradation of fibrin clots.

Topical Hemostatic Products A wide range of topical hemostatic products are available for use in the operating room. Such topical agents act to achieve hemostasis either passively or actively.

Passive hemostatic agents - These hemostatic agents achieve control of bleeding via acceleration of the coagulation cascade by forming a physical, lattice-like matrix that adheres to the bleeding site which thereby activates the extrinsic clotting pathway and provide a platform around which platelets can adhere and aggregate to form a fibrin clot. Accordingly, these agents can only be used among patients with intact coagulation profile. They are generally used as first-line hemostatic agents since they are usually readily available, require no special storage or preparation, and relatively inexpensive.

- Available passive hemostatic agents include: 1. Collagen-based products as Microfibrillar® and Ultra-foam® (non-absorbable), or Helistat® (absorbable). 2. Oxidized regenerated cellulose such as Surgicel® and Surgicel Nu-Knit®. 3. Porcine gelatins such as Gelfoam®.

Active hemostatic agents - Active hemostatic agents, in contrast, have biological activity and directly participate at the end of the coagulation cascade to stimulate conversion of fibrinogen at the bleeding site to fibrin to create a fibrin. Active hemostatic agents include topical thrombins (e.g. bovine, pooled human plasma, or recombinant). Since thrombin acts at the end of the clotting cascade, its action is less affected by coagulopathies from clotting factor deficiencies or platelet malfunction, and thus useful for patients receiving antiplatelet and/or anticoagulation medications. - However, topical thrombin requires the presence of fibrinogen in the patient‘s blood to be able to act which makes it ineffective in patients having afibrinogenemia. Hemostasis typically occurs within 10 minutes in most patients and the control of local bleeding via active hemostatic agents is more effective than passive agents, which makes them more expensive.

Flowable hemostatic agents - Flowable hemostatic agents combine passive and active hemostatic agents into a single application product (e.g., FloSeal®).

Sealants - Sealants work by forming a barrier that is impervious to the flow of most liquids. - There are four types of materials approved for use as sealants for surgical hemostasis: 1. Fibrin sealants 2. Polyethylene glycol (PEG) polymers 3. Albumin with glutaraldehyde 4. The new cyanoacrylate sealant.

BLOOD TRANSFUSION

- Blood transfusion is an essential therapeutic intervention that has been widely used in medical practice to manage anemia or hemorrhage since the early 20th century. - Blood transfusion aims at providing recipients with blood component(s) to improve their physiological status.

Indications of Blood Transfusion

Acute Anemia - Surgical hemorrhage (Hgb < 8 g/dl or presence of symptoms - Traumatic hemorrhage complicated by shock with inadequate tissue perfusion

- Acute non-surgical/non-traumatic hemorrhage as bleeding peptic ulcer or bleeding diverticular disease (Hgb <7 g/dl or presence of symptoms). - Critical illness with Hgb <7 g/dl or presence of symptoms. - Septic shock with Hgb <7 g/dl.

Chronic anemia - Chronic blood loss (hepatic disorders, bleeding disorders).

Blood Groups

There are four types of blood groups, O, A, B, and AB. Blood typing test is used to find out the blood group among patients. The type of blood group is determined by the presence of certain type of antigens on the red blood cells (RBCs). - Group A has "A" antigen - Group B has B antigen - Group AB has both A and B antigens - Group O has core H antigen (universal donor). Our serum naturally has antibodies against the red cell antigen that is not present on our own RBCs. Accordingly: - Group A has anti-B antibodies - Group B has anti-A antibodies - Group AB has no anti-A or –B antibodies (universal recipient) - Group O has both anti-A and anti-B antibodies This creates the basis for blood typing and cross-matching of donor‘s and recipient‘s blood prior to transfusion (Table).

Blood Groups (AB, A, B, O)

Blood Antigens on RBCs Antibodies in Can donate RBCs Can receive RBCs Group serum to to: from:

AB A and B None AB A, B, AB, O A A Anti B A, AB A, O B B Anti A B, AB B, O Anti A and O None O, A, B, AB O anti B

Available Types of Blood Products

1. Whole Blood - It is the unseparated blood collected into a container containing an anticoagulant- preservative solution. - Indicated for RBC replacement associated with hypovolemia as in hemorrhagic shock.

2. Red Cell Concentrate / Packed RBCs - It comprises RBCs after centrifugation of whole blood and removal of most of plasma. - Indicated for replacement of red cells in anemic patients without hypovolemia. 3. Platelet Concentrate - Indicated for prevention or treatment of bleeding due to thrombocytopenia or platelet dysfunction. 4. Fresh Frozen Plasma (FFP) - It is plasma that has been separated from a unit of whole blood. - It contains water, electrolytes, clotting factors and other proteins (mostly albumin). - Indicated for enhancing coagulation by restoring plasma clotting factors as in chronic severe liver diseases, DIC, and anti-thrombin III deficiency. 5. Cryoprecipitate - It is the cold insoluble precipitate component of blood having factor VIII, vWF, fibrinogen and factor XIII as its major constituents. - Indicated for treating factor VIII Deficiency (Hemophilia A), Von - Willebrand‘s Disease, Hypo-fibrinogenemia (as in disseminated intra-vascular coagulation [DIC], dysfibrinogenemia, or afibrinogenemia). 6. Plasma Derivatives - Sterile, concentrated specific proteins obtained from large pools of donor plasma. - They include albumin and immunoglobulins. - They are indicated in treating patients with specific protein deficiencies or to provide them with passive immunity

Complications of Blood Transfusion

- Acute hemolytic reaction due to mis-matched transfusion. - Urticarial rash. - Anaphylaxis. - Non-hemolytic febrile reaction due to cytokines from the transfused blood unit released into the recipient‘s circulation. - Transfusion-related acute lung injury. - Sepsis due to infected blood product. - Circulatory overload. - Air embolism. - Iron overload.

CHAPTER 4 Wounds and Wound Healing

Definition

- A wound is an injury, especially one in which the skin or another external surface is torn, pierced, cut, or otherwise broken.

OPEN WOUNDS

Etiology (Types)

1. Incisions or Incised Wounds These are caused by a clean, sharp-edged object such as a scalpel (surgical wound), a razor or glass-splinter. 2. Lacerations Irregular tear-like wounds caused by some blunt trauma. Lacerations & incisions may appear linear (regular) or stellate (irregular). The term laceration is commonly misused in reference to incisions. 3. Abrasions (Grazes) Superficial wounds in which the top most layer of the skin (epidermis) is scraped off. Abrasions are often caused by a sliding fall onto a rough surface. 4. Puncture Wounds These are caused by an object puncturing the skin (e.g. nail or needle). 5. Penetration wounds These are caused by an object such as a knife entering and coming out from the skin, or reaching a body cavity. 6. Gunshot wounds Gunshot wounds are caused by a bullet or similar projectile driving into or through the body. There may be two wounds, one at the site of entry & one at the site of exit, generally referred to as a "through-and-through."

Classification of Surgical Wounds

As shown in Table 1, wounds may be considered as ―clean‖, ―clean- contaminated‖, ―contaminated‖, or ―dirty‖.

Table 1: Classification of Surgical Wounds

Type of Wound Characteristic Features

1. Clean - No hollow viscus entered - Primary wound closure - No inflammation - No breaks in aseptic technique - Elective procedure 2. Clean-contaminated - Hollow viscus entered, but controlled - Primary wound closure - No inflammation - Minor breaks in aseptic technique - Mechanical drain used - Bowel preparation pre-operatively 3. Contaminated - Uncontrolled spillage from viscus - Inflammation apparent - Major breaks in aseptic technique 4. Dirty - Untreated and uncontrolled spillage from viscus - Pus in the operative wound - Open suppurative wound, severe inflammation

CLOSED WOUNDS

Etiology

The types of closed wounds are: 1. Contusions They are more commonly known as bruises. They are caused by a blunt force trauma that damages the tissues under the skin. 2. Hematomas They are caused by damage to a blood vessel that in turn causes blood to collect under the skin. 3. Crush injury They are caused by a great amount of force applied for a long time.

PHYSIOLOGY OF WOUND HEALING

A surgeon‘s role in wound management is to create an environment in which the healing process can proceed in an optimal fashion. As noted by John Hunter, ―injury alone has in all cases a tendency to produce the disposition & the means of a cure.‖

History

- The earliest accounts of wound healing date back to about 2000 B.C. - Galen emphasized the importance of maintaining a moist environment to ensure adequate healing. - Ambriose Paré found that simply dressed gunshot wounds heal faster & are less painful than when treated with boiling oil, the previously accepted method. - Ignaz Philip Semmelweis advocated need for washing hands - Joseph Lister began soaking his instruments in phenol & spraying the operating rooms, reducing the mortality rates (MR) from 50% to 15%.

Processes of Wound Healing

Repair of tissue damage is separated into two processes, regeneration and healing. 1. Regeneration refers to growth of cells and tissues to replace lost structures. 2. Healing is the effort of tissues to restore normal function and structure after injury - To reform barriers to fluid loss and infection - To limit further entry of foreign organisms and material. - To re-establish normal blood and lymphatic flow patterns. - To restore the mechanical integrity of the injured system.

Primary Mediators of Wound Healing

- Wound healing process is orchestrated by the carefully regulated release of growth factors (GFs) and cytokines - GFs bind to specific receptors on cells that deliver signals that have 2 main effects 1. Stimulation of transcription 2. Regulation of cell entry into the cell cycle

Growth Factors (GFs) - These are polypeptides produced in normal and wounded tissues that stimulate cellular migration, proliferation and function (Table 2). - Often named for the cells from which they were first derived. However, names are often misleading, because GFs have been shown to have multiple functions. - Most GFs are very potent and cause significant effects in nanomolar concentrations (Table 2).

Table 2: Growth factors: cell origin and biological effects

Cytokines - These are small proteins or glycoproteins secreted for the purpose of altering the function of target cells in an endocrine, paracrine, or autocrine fashion. - They are pleiotropic i.e. have multiple effects from a single gene (Table 3)

Table 3: Cytokines: cell origin and biological effects

Cytokine Wound Cell Origin Cellular and Biological Effects

TNF-∞ Macrophages - PMN migration and cytotoxicity - Collagen synthesis - Provides metabolic substrate IL-1 Macrophages - Fibroblast and keratinocyte chemotaxis Keratinocytes - Collagen synthesis IL-2 T-Lymphocytes - Increase fibroblast infiltration and metabolism IL-6 Macrophages - Fibroblast proliferation PMNs - Hepatic acute-phase protein synthesis Fibroblasts IL-8 Macrophages - Fibroblast, PMN and keratinocyte Fibroblasts chemotaxis IFN-ᵞ T-Lymphocytes - Macrophage and PMN activation Macrophages - Retards collagen synthesis and cross- linking - Stimulates collagenase activity Anti-Inflammatory Cytokines IL-4 T-Lymphocytes - Inhibition of TNF, IL-1 and IL-6 Basophils production; fibroblast proliferation, Mast cells collagen synthesis IL-10 T-Lymphocytes - Inhibition of TNF, IL-1 and IL-6 Macrocytes production. Keratinocytes - Inhibits macrophage and PMN activation

TNF: tumor necrosis factor, IL: interleukin, IFN: interferon, PMN: Polymorphonuclear neutrophil

Phases of Wound Healing

Normal wound healing follows a predictable pattern that can be divided into overlapping phases defined by characteristic cellular populations and biochemical activities (Figure 1). 1. Hemostasis and inflammation 2. Proliferation 3. Maturation and remodeling

Figure 1. Three phases of wound healing

Inflammatory Phase It represents the tissue‘s attempt to limit damage and is closely related with healing process. Healing is impossible without inflammation. The events can be divided into vascular events and cellular events

Vascular Events - The vascular events include: 1. Earliest manifestation is vasodilatation (VD), which follows a transient constriction of arterioles lasting a few seconds. 2. Wounding disrupts tissue integrity and direct exposure of extra-cellular matrix to platelets. 3. Initial contact between platelets and collagen requires vonWillebrand factor (vWF). 4. The clotting cascade is initiated through both intrinsic and extrinsic pathways. - Vasodilatation is followed by increased permeability of micro-vasculature, followed by stasis, which → accumulation of leucocytes along the vascular endothelium, which then migrate through the vascular wall into interstitial tissue. - Permeability increases due to 1. Formation of endothelial gaps in venules, 2. Direct endothelial injury, 3. Delayed prolonged leakage, 4. Leucocyte mediated endothelial injury, 5. Increased transcytosis and leakage from new vessels - The combination of intense VD and increased vascular permeability → clinical findings of inflammation; rubor (redness), tumor (swelling), calor (heat) and dolor (pain).

Cellular Events - Cellular infiltration after injury follows a characteristic, predetermined sequence as shown in Figure 2. - Inflammatory Granulocytes • These are attracted by inflammatory mediators in the first 48 hours • The produce oxygen-derived free radicals • Non-specific

Figure 2. Cellular events after wounding

- Inflammatory Macrophages Monocytes • Attracted to the area by complement. Activated by: fibrin, foreign body (FB) material and exposure to hypoxic & acidotic environment • Reached maximum after 24 hours • Remain for weeks Activated Macrophages • Essential for progression onto the proliferative phase • Mediate (1) Angiogenesis (FGF, PDGF, TGF-a&b and TNF-a) and (2) Fibroplasia (IL‘s, EGF & TNF) • Synthesize NO • Secrete collagenases - T-Lymphocytes • Population of inflammatory/immune cells that routinely invades the wound. • Less numerous than macrophages, numbers peak at about 1 week post injury • Bridge the transition from inflammatory to proliferative phase of healing • Depletion of most wound T-lymphocytes reduces wound strength and collagen content • Also exert a down regulating effect on fibroblast collagen synthesis by cell- associated interferon-γ, TNF-α & IL-1.

Proliferative Phase - It is the second phase of wound healing and roughly spans days 4 through 12 - It is during this phase that tissue continuity is re-established - Fibroblasts and endothelial cells are the last cell populations to infiltrate the healing wound & the strongest chemotactic factor for fibroblasts is PDGF. - Recruited fibroblasts first need to proliferate & then become activated, to carry out their 1ry function of matrix synthesis remodeling. - The 4 major events are: fibroplasia, angiogenesis, epithelialization and contraction 1. Fibroplasia • The proliferative phase begins with degradation of the initial fibrin-platelet provisional matrix. • During fibroplasia, fibroblasts synthesize and deposit the replacement ECM at the wound site • As fibroblasts proliferate, they become predominant cell types by 3-5 days in clean, non-infected wounds. • The initial fibrin matrix is replaced by a provisional matrix of fibronectin & hyaluron, which facilitates fibroblast migration. • Fibrous structural proteins such as collagen and elastins

Collagen - It is the most common protein in the animal world providing the extracellular framework for all multicellular organisms - Its deposition, maturation, and subsequent remodeling are essential to the functional integrity of the wound. - At least 27 types of collagen encoded by 41 genes dispersed on at least 14 chromosomes, - Type I collagen is the major component of extracellular matrix in skin. - Type III, normally present in skin, becomes more prominent and important during the repair process - Type IV is non-fibrillar, main component of basement membrane together with laminin. - Each chain of collagen is composed of a glycine residue in every 3rd position. The 2nd position in the triplet is made up of Proline or Lysine Elastin, Fibrillin and Elastic Fibers - Provide the resilience to allow for recoil after transient stretch. - Elastin is composed of hydrophobic & alanine- and lysine-rich α- helical segments that alternate along the polypeptide chain - Elastic fibers consist of an elastin core covered with a sheath of microfibrils, which are composed of several distinct glycoproteins, such as fibrillin. - Microfibrils appear before elastin in developing tissues and seem to form a scaffold on which the secreted elastin molecules are deposited.

2. Angiogenesis - It is the process of new blood vessel (BV) formation - Macrophages regulate angiogenesis during the inflammatory phase. - Angiogenesis occurs is by a. Degradation of the basement membrane of post-capillary venules b. Migration of cells through this gap is promoted by FGF, PDGF and TGF-β. PECAM-1, also found on endothelial cells, modulates their interaction with each other as they migrate into the wound c. Tubule or lumen formation involving cell-cell and cell-matrix interactions. New capillaries differentiate into arterioles and venules, whereas others undergo involution and apoptosis, with ingestion by macrophages. d. Deposition of the basement membrane resulting in capillary maturation.

3. Epithelization - New epithelial cells for wound closure are provided by fixed basal cells in a zone near the edge of the wound - The epidermal cell layer thickens and the marginal basal cells migrate over the wound defect. Once these keratinocytes begin migrating they do not divide until epidermal continuity is restored. - Daughter cells flatten and migrate over the wound as a sheet, moving in a leap-frog and tumbling fashion (epiboly). - Migration of keratinocytes over the wound is guided by cell adhesion glycoproteins, such as tenascin & fibronectin, which are their ―railroad tracks‖. - After re-establishment of the epithelial layer, keratinocytes and fibroblast secrete laminin and type IV collagen to form basement membrane - Keratinocytes become columnar and divide to restore the layering of the epidermis and reform a barrier. 4. Contraction - It is the process in which the surrounding skin is pulled circumferentially toward an open wound. - It does not occur with closed surgical incisions. - It reduces the size of the wound dramatically without new tissue formation. - Speeds wound closure compared to epithelization and scar formation alone - The amount of contraction is related to both the size of the wound and the mobility of the skin. - In humans contractions is greatest in the trunk and perineum, least on the extremities and intermediate on the head and neck.

Remodeling Phase - The ECM is dynamic and is constantly undergoing remodeling - Collagen cross-linking ↓ its degradation and improves wound tensile strength - Lysyl oxidase is the major intermolecular cross linking enzyme. - Degradation is by collagenases, gelatinases and matrix metallo-proteinases

- Scar formation is the final outcome of wound repair in children and adults - The ultimate pattern of collagen in scar is one of densely packed fibers and not the reticular pattern found in unwounded dermis - Scar has no epidermal appendages (hair follicles & sebaceous glands) and it has a collagen pattern that is distinctly different from the unwounded skin - Remodeling occurs during months to years to form a mature scar. - The early scar appearance is red due to its dense capillary network induced at the injury site - Scars are usually hypo-pigmented after full maturation. - However, it can become hyper-pigmented in darker pigmented patients and in those lighter pigmented patients who receive sun exposure. - During remodeling, wounds gradually become stronger with time. - Wound tensile strength increases rapidly from 1-8 weeks after wounding and correlates with collagen cross-linkage. - It increases at a slower pace till 1 year. - Tensile strength of the wound best reaches only 80% that of unwounded skin.

FACTORS AFFECTING WOUND HEALING

Factors affecting wound healing include age, infection, nutrition, hypoxia, anemia, hypo-perfusion, metabolic disorders, steroids, chemotherapeutic drugs and ionizing radiation

Age

- Aging produces intrinsic physiological changes that lead to delayed or impaired wound healing. - With aging, collagen undergoes qualitative and quantitative changes. - Dermal collagen content decreases with aging and aging collagen fibers show distorted architecture & organization. - The increased incidence of cardiovascular disease, metabolic diseases (diabetes mellitus, malnutrition and vitamin deficiencies), cancer all contribute to the higher incidence of wound problems in the elderly

Infection

- It is the most common cause of healing delays - If the bacterial count in the wound exceeds 105 organisms per gram of tissue, or if any β- hemolytic streptococci are present, the wound will not heal by any means. - Bacteria prolong the inflammatory phase and interfere with epithelialization, contraction, and collagen deposition. - Endotoxins stimulate phagocytosis and release of collagenase - Bacteria may accelerate expression or increase concentrations of MMPs, GFs and cytokines in chronic-type wounds.

- Inactive precursors of MMPs are activated by bacterial proteinases of the Thermolysin family (Pseudomonas, Vibrio and Serratia). - Bacterial phospholipase C can disrupt normal re-epithelialization by decreasing cell-cell contact and increasing cell migration, possibly by altering integrin expression and by upregulating MMP-9.

Nutrition

- Precise caloric requirements for optimal healing have not been determined. - Malnourished patients have diminished hydroxyproline accumulation (an index of collagen deposition) into subcutaneously implanted polytetrafluoroethylene tubes when compared to normally nourished patients. - Malnutrition correlates clinically with enhanced rates of wound complications and increased wound failure after diverse surgical procedures.

Nutrition - Arginine - Arginine deficiency results in decreased wound-breaking strength and wound collagen - The main effect of Arginine on wound healing is to enhance wound collagen deposition. - Reduction in breaking strength during the first weeks of healing are directly related to new collagen synthesis - Arginine supplementation may lead to improvement in wound strength as a consequence of enhanced collagen deposition

Nutrition - Vitamin A - Deficiency impairs wound healing, whereas supplemental vitamin A benefits wound healing in non-deficient humans and animals. - Vitamin A increases the inflammatory response in wound healing, probably by increasing the lability of lysosomal membranes. - There is increased influx of macrophages, with increase in their activation and increased collagen synthesis. - Directly increases collagen production and epidermal GF receptors when it is added in- vitro to cultured fibroblasts. - Supplemental vitamin A can reverse the inhibitory effects of corticosteroids on wound healing. Nutrition – Scurvy (Vitamin C deficiency) - It causes a defect in wound healing, particularly via a failure in collagen synthesis & cross-linking. - Vitamin C is required for the conversion of Proline and Lysine to Hydroxyproline and Hydroxylysine, respectively. - Vitamin C deficiency is also associated with increased incidence of wound infection

Nutrition – Zinc - In deficiency states, there is reduced fibroblast proliferation, reduced collagen synthesis, impaired overall wound strength and delayed epithelialization.

Hypoxia, Anemia and Hypoperfusion

- Low O2 tension has a deleterious effect on all aspects of wound healing. - Fibroplasia is significantly impaired by local hypoxia.

- Optimal collagen synthesis requires O2 as a co-factor - Factors affecting local O2 delivery are either systemic (low volume or cardiac failure) or local (arterial insufficiency, local vasoconstriction, or excessive tension on tissues). - The level of vasoconstriction of the subcutaneous capillary bed is exquisitely responsive to fluid status, temperature and hyper-active sympathetic tone as is often induced by post-operative pain.

Steroids and Chemotherapeutic Drugs

- Large doses or chronic usage of glucocorticoids reduce collagen synthesis and wound strength. - Major effect is to inhibit the inflammatory phase of wound healing and the release of lysosomal enzymes - Steroids also inhibit epithelialization and contraction and contribute to increased rates of wound infection, regardless of the time of administration - All chemotherapeutic anti-metabolite drugs adversely affect wound healing by inhibiting early cell proliferation and wound DNA and protein synthesis

Metabolic Disorders

Diabetes Mellitus (DM) - Uncontrolled DM results in reduced inflammation, angiogenesis and collagen synthesis. - The accompanying large and small vessel disease contributes to local hypoxemia. - Defects in granulocyte function, capillary ingrowth and fibroblast proliferation all have been described in Diabetes. - Obesity, insulin resistance, hyperglycemia and diabetic renal failure contribute significantly and independently to the impaired wound healing observed in diabetics. - Reduced expression of growth factors like VEGF, IGF 1, FGF 1, KGF and PDGF - Diabetic fibroblasts and keratinocytes have diminished proliferation rates and collagen production.

Uremia - It is associated with disordered wound healing. - Experimentally, uremic animals demonstrate reduced wound collagen synthesis and breaking strength

Ionizing Radiation

- Ionizing radiation causes endothelial cell injury with endarteritis resulting in atrophy, fibrosis and delayed tissue repair

- Angiogenesis is not initiated - Rapidly dividing cell populations like keratinocytes and fibroblasts are the most sensitive to radiation.

ABNORMAL WOUND HEALING

1. Inadequate regeneration e.g. CNS, corneal ulcers and non-union of bone. 2. Inadequate scar formation e.g. diabetic foot ulcers (DFU), pressure sores and stasis ulcers. 3. Excessive regeneration e.g. neuromas, hyperkeratosis in psoriasis and colonic polypi. 4. Excessive fibrosis e.g. hyper-trophic scars and keloids.

CHAPTER 5 Surgical Infections

Definition - Infections that should be treated by surgical intervention - Infections following surgical procedure occurring at the wound site (surgical site infection [SSI] or at a distant site)

CLASSIFICATION

General Classification

According to duration - Acute infection (<3 weeks) - Sub-acute infection (3weeks - 2 months) - Chronic infection (>2 months)

According to phase - Local phase - Systemic phase

Classifications of Soft Tissue Infections

The different types of soft tissue infections are summarized in Table 1.

Table 1: Soft tissue infections Skin Subcutaneous Tissue Fascia

- Imptigo - Wound infection - Necrotising fasciitis - Erysipelas - Synergistic (Fournier gangrene) - Lymphangitis necrotizing cellulites - Subfascial: - Folliculitis (Meleney's ulcer) - Non-clostridial - Furuncle/carbuncle - Non-clostridial gas- myositis/myonecrosis - Hidradenitis suppurativa producing infection - Streptococcal myositis - Pyoderma gangrenosum - Clostridial cellulites - Clostridial (gas gangrene) myositis/myonecrosis

Classification of Hospital Acquired Infection (ICU)

- Sinusitis - Neurological: meningitis - ventriculitis - Chest: pneumonia - lung abscess - empyema - Gastrointestinal: Enterocolitis - Urinary tract infection (UTI) - Catheter-related infections

ETIOLOGY

Protective Physiological Mechanisms

1. Intact epithelial surfaces 2. Chemical: Low gastric pH 3. Humoral: antibodies, complement and opsonins 4. Cellular: phagocytic cells, macrophages, polymorphonuclear cells and killer lymphocytes

Causes of Reduced Host Resistance

1. Metabolic malnutrition (including obesity), diabetes, uraemia, jaundice 2. Disseminated diseases: cancer and acquired immunodeficiency syndrome (AIDS) 3. Iatrogenic: radiotherapy, chemotherapy, steroids, surgical trauma.

Risk Factors of Wound Infection

1. Metabolic malnutrition (including obesity), diabetes mellites (DM), uraemia, jaundice 2. Disseminated diseases: cancer and AIDS 3. Iatrogenic: radiotherapy, chemotherapy, steroids, surgical trauma. 4. Immune suppression 5. Poor tissue perfusion 6. Foreign body 7. Poor surgical and disinfection techniques 8. Colonization and translocation in the gastrointestinal tract (GIT)

Organisms Involved in Wound Infection

- Staphylococcus - Streptococci - Clostridia - E-Coli - Klebsiella - Psudomonas - Fungi

SURGICAL WOUNDS

Classification of Wound Infection

The type of wound infections and related infection rate are listed in Table 2.

Table 2: Classification of Wound Infection

Type Example Infection Rate - Clean wounds Thyroidectomy 1-2% - Clean contaminated Cholecystectomy 2-5% - Contaminated Colon surgery 5-30% - Dirty Peritonitis

Surgical Site Infection Prophylaxis

Pre-operative preparation - Short hospital stay - Control risk factors - Bathing before surgery - Shaving - Bowel prep - Antiseptic measures - Antibiotics

Prophylactic measures - Sterilization of equipment - Shaving the surgical site - Skin disinfection - Sterile draping of operative field - Scrubbing, gowning and gloving - Irrigation of the wound with antibiotic solution - Prophylactic systemic antibiotics peri-operatively

Clinical Picture

- It usually appears between the 5 and 10 days post-operatively - Symptoms: fever, pain in the wound - Signs: the wound (surgical site) becomes swollen, tender, red, hot, and fluctuant

Differential Diagnosis

Wound infection should be differentiated from other causes of post-operative fever, which include the following:

- Chest infection - Deep vein thrombosis (DVT) - Urinary tract infection (UTI) - Seroma - Hematoma

SYSTEMIC INFECTIONS

Types - Bacteremia: presence of pathogenic organisms in the blood stream without clinical picture of infection - Septicemia: presence of toxic manifestation of infection - Pyemia: pus in the blood (systemic or portal)

Classification - Sepsis: SIRS with documented infection - Severe sepsis: SIRS with evidence of one or more organ failure - MODS: multiple organ dysfunction - MSOF: multiple systemic organ failure

ACUTE INFECTIONS

Classification (Types)

Table 3 summarizes the different types of acute infections; non-specific or specific.

Table 3: Types of Acute Infections Non-Specific Acute Infections Specific Acute Infections

- Post-operative wound infection - Tetanus - Cellulitis - Gas Gangrene - Erysipelas - Necrotizing Fasciitis - Boil (furuncle) - Carbuncle - Hydradenitis suppurativa - Acute abscess - Acute lymphangitis and lymphadenitis - Bacteremia and septicemia

A. Non-Specific Acute Infections

Cellulitis

Definition: It is an invasive non-suppurative infection of the loose connective tissue Organisms: streptococci [common], staphylococci [occasionally], mixture

Clinical picture - The affected area is red, indurated, hot and painful - It spreads rapidly with ill-defined edge - The skin may be the seat of blisters - Fever - Lymphangitis in the form of red streaks - No suppuration - In severe cases: patches of skin necrosis with sloughing of subcutaneous tissues Differential Diagnosis 1. Contact allergy 2. Chemical inflammation 3. DVT Treatment 1. Rest and elevation of the affected part 2. Antibiotic: penicillin

Erysipelas

Definition - It is a rapidly spreading non-suppurative inflammation of the lymphatics of the skin caused by hemolytic streptococci. Clinical Picture - Systemically: Toxemia - Locally: similar to cellulitis, but there are the following differences: 1. The color of the skin is rose-pink 2. The edge is well-defined 3. There may be islets of inflammation beyond the spreading margin Complications - Facial erysipelas may lead to cavernous sinus thrombosis - Septicemia - Recurrent erysipelas may block the lymphatics leading to elephantiasis. Treatment - Isolation - Similar to cellulitis

Boil (Furuncle)

- Definition: It is a staphylococcal infection of a hair follicle or a sebaceous gland. - Common sites: Face, neck and axilla. - Clinical Picture: It is common in patients with diabetes. It presents as a small painful indurated swelling, which is red, hot and very tender - Treatment: Antibiotics and antiseptics.

Carbuncle

Definition - It is infective gangrene of the subcutaneous tissues usually by Staphylococcus aureus. Pathology - Infection usually starts in a hair follicle - It extends to the subcutaneous fat where other hair follicles get the infection. - Multiple areas of necrosis and thrombosis of blood vessels occur. - Patches of skin undergo sloughing and separate from the underlying granulation tissue Clinical Picture - It is common in immuno-compromised patients as in diabetics. - The common sites: face, nape of the neck, and the back - There is usually severe toxemia. - It starts as a painful induration of the skin and subcutaneous tissues. - The skin is red. - The central part of the swelling becomes soft. - Multiple areas of skin thin out and separate forming multiple sinuses. Complications - Local spread of infection. - Pyemia and septicemia. - Cavernous sinus thrombosis - Epidural abscess or meningitis Treatment - Antibiotics (culture and sensitivity of the discharge). - Control of diabetes. - Surgical excision of sloughs

Hydradenitis Suppurativa

Definition - Mixed staphylococcal and streptococcal infection of the apocrine sweat glands in the perineum or the axilla. Clinical Picture - It produces multiple abscesses and sinuses. Treatment - Surgical drainage of abscesses. - Antiseptic and antifungal applications. - Surgical excision of apocrine sweat-bearing skin followed by skin graft is essential.

Acute Abscess

Definition - It is a localized suppurative inflammation. Etiology - Pyogenic organisms; the commonest are staphylococci (produce coagulase enzyme).

Pathogenesis The organism reaches the tissues by: - Direct access through wounds, scratches and abrasions. - Local extension from an adjacent focus - Lymphatic spread. - Blood spread. Pathology: An abscess consists of three zones: 1- A central zone of coagulative necrosis 2- An intermediate zone of granulation tissue. 3- A peripheral zone of acute inflammation. Sequel - Resolution. - Pointing and rupture. - Spread infection locally, lymphatics or blood - Chronicity. Clinical Picture - Locally: painful tender mass, skin is red, and edematous. Draining lymph nodes are usually enlarged and tender - Systemically: fever, malaise, Headache, Tachycardia, Anorexia - When pus forms: 1- Fever becomes hectic. 2- Skin shows pitting edema. 3- Pain becomes throbbing. 4- The inflammatory reaction becomes localized 5- Fluctuation test becomes positive. 6- There is shooting leucocytosis Treatment - Before suppuration: antibiotic, rest, hot fomentation and supportive general measures. - After suppuration: surgical drainage. Culture and sensitivity and antibiotic (if there are systemic manifestations).

Acute Lymphangitis and Lymphadenitis

Acute lymphangitis is due to infection of lymph vessels by organisms usually streptococci. Acute lymphadenitis is due to spread of infection along lymphatics from a septic focus in the drainage area to the lymph- nodes. Treatment - Antibiotics. - Hot applications. - Surgical drainage if suppuration occurs

B. Specific Acute Infections

Tetanus

Definition - It is a specific anaerobic infection that is mediated by neurotoxin of Clostridium tetani and leads to nervous irritability and tetanic muscular contractions. Etiology - Organism: Clostridiuam tetani is gram positive anaerobic bacillus with a terminal spore giving the characteristic drum-stick appearance. - Mode of Infection 1. Wounds: -hypoxic, containing devitalized tissue or a foreign body. 2. Umbilical stump: tetanus neonatorum Pathogenesis - The neuro-toxin is an exotoxin produced locally and reaches the central nervous system (CNS) along the blood stream, the motor nerves, or both. - When the toxin reaches the nervous system, it is fixed by the motor cells and cannot be detected in the blood or cerebrospinal fluid (CSF). - The anti-toxin can only neutralize the toxin before it gets fixed to the nervous tissue. - The toxin increases the excitability of the motor cells of the medulla and spinal cord, so slightest stimuli produce violent spasm. - Death results from exhaustion, hyperpyrexia, heart failure, asphyxia or pneumonia. Clinical Picture Incubation Period - Symptoms during incubation period are vague such as tenderness, rigidity of the muscles, swelling at the site of wound, local twitches, restlessness, and anxiety. Tonic Stage - Pain and tingling in the area of injury. - Limitation of movements of the jaw. - Spasm of the facial muscles. - Stiffness of the neck. - Dysphagia. - Laryngospasm. - Hesitancy in micturition. Clonic Stage - Reflex paroxysms of violent muscular contraction. - Relaxation is incomplete during the intervals between clonic contractions. - Spasm of the intercostal muscles and diaphragm leads to long period of apnea. - Temperature is elevated with profuse sweating. - Marked tachycardia - Laboratory finding: polymorph-nuclear leucocytosis Prevention - Immunization (active) every 7-10 years. - Those previously immunized within 10 years: need a booster dose of tetanus toxoid.

- Those who received less than three doses: need a booster dose of tetanus toxoid and tetanus immunoglobulin (TIG) (passive). - Individuals not previously immunized: need full immunization with tetanus toxoid and tetanus immunoglobulin Treatment - Neutralize toxin with TIG. - Wound debridement. - Avoid sudden stimuli. - Muscle relaxant with mechanical ventilation may require tracheostomy. - Aqueous penicillin G - Nursing.

Gas Gangrene

Definition - It is an acute spreading infection associated with gas formation and profound toxemia caused by anaerobic spore-bearing bacilli of the clostridium group. Pathology - Clostridia proliferate and produce toxins that diffuse into the surrounding tissue. - The toxins destroy local circulation. - This allows further invasion. - Factors predisposing to gas gangrene: 1. Lacerated wounds involving bulky muscles. 2. Presence of foreign bodies or devitalized tissues. 3. Ischemia of muscles. 4. Infection by anaerobic bacteria Bacteriology - Organisms falls into two groups: 1. Saccharolytic organisms: Cl. Welchii, Cl. Septicum, and Cl. edematiens 2. Proteolytic organisms: Cl. Sporogenes, Cl. Histolyticum and Cl. tertium Clinical Picture - Incubation period varies from few hours to few days. - Generally 1. The patient is pale, anxious, and apprehensive. 2. Temperature may be raised and there is marked tachycardia. 3. Hands are cold and clammy. 4. An icteric tinge may be present and there is oliguria. 5. In severe case there is shock. - Locally 1. Pain and numbness in the affected area. 2. Swelling and there may be crepitus with gas bubbles. 3. A sanguineous discharge of a characteristic odor. 4. The affected muscles brick red then greenish and finally black discoloration, do not contract, do not bleed if cut, the skin black.

Prevention - Adequate debridement of wounds. - Antibiotics. - Avoid tissue hypoxia. Treatment - Wound management. - Hyperbaric oxygenation. - Antibiotics: penicillin

Necrotizing Fasciitis

Definition - It is an invasive infection usually caused by a mixed microbial flora including microphilic streptococci, staphylococci, Gram-negative bacteria and anaerobes, especially streptococci, and bacteroids. Pathogenesis - The infectious process spreads along the fascial planes and results infectious thrombosis of the vessels passing between the skin and deep circulation. - Superficial skin necrosis follows - Hemorrhagic bullae appear as the first sign of skin death. Fascial and subcutaneous fat necrosis involves wider area than the skin Clinical Picture - There are manifestations of toxemia with fever and tachycardia. - The skin shows hemorrhagic bullae and necrosis surrounded by oedema and inflammation. - Crepitus is occasionally present. Investigations - Swab for culture and sensitivity - At surgery: edematous, dull grey fascia and subcutaneous tissue with visible thrombi in penetrating vessels Prevention - Adequate debridement of wounds - Antibiotics Treatment - Surgical - Antibiotics - Blood transfusion

PRINCIPLES OF ANTOBIOTIC MANAGEMENT

Cultures from: - Exudate: The most reliable diagnosis for treatment. Both aerobic and anaerobic culture

- Blood: Diagnostic step for unknown source. Fail to capture causative organisms in bacteremia and unnecessary to diagnose sepsis - Sputum - Urine

Proper Choice of Antibiotics - Bacteriostatic agents: Prevent growth of bacteria - Bactericidal agents: Actually kill bacteria - Effective agent against the infecting organism - Adequate contact between agent and organism - Absence of toxic side effect of the agent - Augmentation of host defences to maximize antibacterial effects - Administer antibiotics on empiric basis before the laboratory reports - A combination of antibiotics for probable polymicrobic infection - Culture and sensitivity test before antibiotic therapy - Hazards 1. Colonization 2. The quantitative appearance of changes in the microflora that are induced by antibiotic therapy 3. Super-infection 4. A new microbial disease introduced or potentiated by antibiotic therapy 5. Super-infection is frequently the result of colonization. - For potentially contaminated wounds - Only an adjunct and not a substitute to good surgical technique

Type of Wound - Clean procedure: No antibiotics are necessary - Clean contaminated procedure: Contact of the interior of respiratory, urinary, GIT - Contaminated procedure: Complicated by gross spillage of intestinal contents or wounds secondary to trauma - Dirty wounds: In contact with intra-abdominal or peri-rectal abscess

Other Principles - Early and enough for adequate tissue and body fluid levels - Being necessary to maintain adequate tissue levels intra-operatively - Length of operation and serum half-life of antibiotics

Infection and the Surgeon - Full face mask - Water-proof gowns - Boot - Double gloving - Only essential personnel in the operating room - No unnecessary movement - Slow, meticulous, operative techniques 44

CHAPTER 6 Principles of Surgical Oncology

PRINCIPLES OF SURGICAL ONCOLOGY

Definition

Surgical oncology is the use of surgery in diagnosis and treatment of cancer. Surgery can be used by itself to treat the cancer or it can be done with other methods.

Role of Surgery

- Role of Surgery is to diagnose cancer, stage cancer, provide radical cure or plan other treatment techniques - Not all cancers can be treated with surgery. The tumor may not be safely removed without causing damage for the patient. - Surgery in cancer is used to remove the whole cancer (radical curative), remove some, but not all the tumor (debulking or palliative). A surgeon may not be able to safely remove all of the cancer because it sometimes invades other important organs. - Another role of surgery is to reform the body‘s condition after a surgery. This is called reconstructive or restorative surgery. Examples are breast reconstruction or reversal of colostomy.

Role of Surgery with Other Therapies

- Surgery may be the only treatment for some patients. For many people, surgery is used along with other treatments. These can be chemotherapy, biological, targeted or immune therapies, radiation therapy, and hormone therapy. Other surgeries, such as those used in interventional specialties, may be used too. - Neoadjuvant therapy: This means the surgery is done after some other form of treatment. - Adjuvant therapy: This means the surgery is done before the other form of treatments. - Despite the advances in medical and radiation oncology, surgery is still the only modality with the potential to cure most solid cancers. Surgeons have a pivotal role in cancer treatments and research, leading the diagnostic and treatment pathways for most cancers from counselling patients about their diagnosis through to surgery and aftercare. They have also led many of the great advances in cancer research. - However, cancer care has evolved very rapidly over the last few decades and therefore a new type of surgeon is needed to keep pace with these changes. No longer is surgery alone the only treatment for most solid malignancies but a combination of surgery and

multi-modal therapies (with highly focused radiotherapy, targeted molecular therapies and poly-chemotherapy) becoming the modern standard of care. - As a result, the surgeon, can no longer work in isolation but must lead a multidisciplinary team. The surgeon must be more than just a technician and must understand the biology and natural history of the disease as well as the contributions made by other disciplines to the cancer patients‘ treatment. It is at this point that the surgeon becomes a surgical oncologist. Examples include: collaboration with radiation and medical oncologists on the use of neoadjuvant chemotherapy or radiotherapy to enhance or permit surgery possible and on the indications for adjuvant therapy after surgery. Collaboration with radiologists to plan surgery or optimize resection margins.

Disadvantages of Surgery

1. Pain 2. Bleeding 3. Infection 4. Delayed wound healing. Bleeding.

NEOPLASMS

Definition Cell population characterized by: - Excessive, non-useful proliferation - Unresponsive to normal control mechanisms - Harmful to adjacent tissue.

Histological Cascade - Normal cells - Hyperplasia - Atypia - Dysplasia - Carcinoma in-situ - Invasive malignancy

Tumour Grading - G1: Well differentiated - G2: Moderated differentiated - G3: Poorly differentiated - G4: Undifferentiated

Types of Neoplasms - Benign - Locally-malignant - Malignant: Carcinoma (95%), or sarcoma (5%)

Signs of Changing from Benign into Malignant - Rapid growth - Bleeding - New vasculature - Hard consistency - Fixation - Infiltrating nearby structure - Dissemination: lymph nodes or remote secondaries

Characteristics of Sarcoma versus Carcinoma - Younger age - Rapid rate of growth - Rapid local invasion - More blood-born metastasis - Worse prognosis - Less sensitive to radiotherapy and chemotherapy

Premalignant Lesions - Barrett‘s esophagus: cancer esophagus - Bowen disease of skin: squamous cell carcinoma (SCC) (epithelioma) - Ulcerative colitis: cancer of the colon - Chronic atrophic gastritis: gastric cancer - Familial intestinal polyposis: cancer of the colon - Xeroderma pigmentosa: epithelioma

Techniques for Obtaining Tissue Biopsy - Needle biopsy: fine-needle aspiration cytology (FNAC), Core tissue biopsy - Incisional biopsy - Excisional biopsy - Frozen-section biopsy - Brush cytology

Most Common Sites of Metastasis - Bone - Brain - Lung - Liver

Routes of Spread 1. Direct extension: to nearby organs 2. Lymphatic spread: common in epithelial neoplasms (carcinoma) 3. Vascular spread: more common in sarcomas 4. Spread through serous cavities: peritoneal seeding (cancer of the stomach)

Clinical Staging - Stage I: cancer confined to its primary site - Stage II: spread to regional lymph nodes (LNs) - Stage III: more locoregional advanced disease - Stage IV: metastasis to distant sites

Prognosis - Best 5-year survival in patients with cancer of skin, cervix, uterus and bladder; while lowest survival is with pancreatic and gastric cancer - Females tend to have a higher 5-year survival than males.

Principles of Cancer Surgery - Complete excision of tumor mass with nearby extension and LN involvement (radical operation) - Prevention of tumor cell implantation during surgery - Surgery for palliation: to improve the quality of life e.g. relief of intestinal obstruction, removal of mass causing pain

Follow-up - Response to treatment - Relapse - Diagnosis of new tumour

Psychological Aspects - On diagnosis: shock, denial, sadness - On treatment: depression, loneliness - On follow up: worry, emotionless

CHAPTER 7 Groin Hernias

HERNIA: INTRODUCTION

Definitions - Hernia means ―To bud‘ or ‗to protrude‘, ‗off shoot‘ (Greek) - ‗rupture‘ (Latin). - A hernia is defined as "an area of weakness or disruption of the fibro-muscular tissues of the body wall". - Often hernia is also defined as "an actual anatomical weakness or defect". - Hernia is also defined as "an abnormal protrusion of a viscous or a part of a viscous through an opening, artificial or natural with a sac covering it".

Incidence - About 15% of males AND 5% of females will develop groin hernia. - Approximately, 75-85% of abdominal wall hernias are groin hernias (inguinal and femoral) - Inguinal hernia is the most common hernia (73%) because of weakness of the muscular anatomy in the inguinal region and presence of natural weakness like the deep inguinal ring and cord structures. Incisional hernia is next to inguinal hernia in occurrence. Incidence of femoral hernia is 17%; umbilical 8.5% and others 1.5% (excluding incisional hernia).

Common and Rare Sites of Abdominal Wall Hernia (Figure 1)

Figure 1. Common sites of abdominal wall hernia (1. Inguinal, 2. Incisional, 3. Femoral, 4. Epigastric, 5. Umbilical). Rare sites (6. Obturator, 7. Spigelian, 8. Lumbar, 9. Gluteal)

Etiology

- Straining o Lifting of heavy weights. o Chronic cough (tuberculosis, chronic bronchitis, bronchial asthma, emphysema). o Chronic constipation (habitual, rectal stricture). o Urinary causes: Benign prostatic hyperplasia (BPH), carcinoma prostate (old age), stricture urethra (young age), and phimosis and meatal stenosis (very young age). - Obesity. - Pregnancy and weakness of pelvic musculature (especially femoral hernia in females). - Smoking. - Ascites. - Appendectomy through McBurney‘s incision may injure the ilio-inguinal nerve causing right sided direct inguinal hernia (DIH). - An indirect inguinal hernia (IIH) occurs in a congenital, preformed sac (remains of processus vaginalis). Chance of presence of bilateral preformed sac is 60%. - Familial collagen disorder - Prune Belly syndrome. - Acquired herniation is also probably due to collagen deficiency called metastatic emphysema of Read.

Parts of Hernia

1. Sac - It is a diverticulum of peritoneum with mouth, neck, body and fundus. - The "neck" is narrow in indirect sac but wide in direct sac. - Body of the sac is thin in infants, children & in indirect sac but is thick in direct & long-standing hernia. - Hernia without neck: Those hernias with larger mouth lack neck, e.g. direct hernia, incisional hernia. - Hernia without sac: Fatty hernia of linea alba = protrusion of extra-peritoneal pad of fat. 2. Coverings - Coverings of the sac are the layers of the abdominal wall through which the sac passes. 3. Contents of Sac - Omentum = (Omentocele). Initially it can be reduced easily, but difficult later. - Intestine = Enterocele (Commonly small bowel, but sometimes large bowel). It is difficult to reduce the contents initially. - A portion of circumference of bowel = Richter’s hernia. - UB may be the content or part of the posterior wall of the sac = cystocele. - Ovary, often with Fallopian tube. - Meckel‘s diverticulum = Littre’s hernia. - Fluid: Fluid is secreted from congested bowel or omentum. It may be an infected fluid or ascitic fluid or blood from the strangulated sac.

Classification of Hernia

Classification I: Clinical 1. Reducible Hernia: Hernia gets reduced on its own or by the patient or by the surgeon. Intestine reduces with gurgling and is difficult to reduce the first portion. Omentum is doughy and it is difficult to reduce the last portion. Expansile impulse on cough is present. 2. Irreducible Hernia: Here contents cannot be returned to the abdomen due to narrow neck, adhesions, over-crowding. Irreducibility predisposes to strangulation. 3. Obstructed Hernia: It is an irreducible hernia with obstruction, but blood supply to the bowel is not interfered. It eventually leads to strangulation. 4. Inflamed Hernia It is due to inflammation of the contents of the sac, e.g. appendicitis, salpingitis. Here, the hernia is tender but not tense; overlying skin is red and edematous. 5. Strangulated Hernia: It is an irreversible hernia with obstruction to blood flow. The swelling is tense, tender, with no impulse on cough and with features of intestinal obstruction, which may be absent in case of omentocele, Richter‘s or Littre‘s hernia.

Classification II: Etiological 1. Congenital (Common): It occurs in a preformed sac/defect. Clinically may present at a later period due to any of the precipitating causes like in indirect inguinal hernia (IIH). 2. Acquired: It is 2ry to any causes which raise the intra-abdominal pressure leading into weakening of the area like in direct inguinal hernia (DIH).

Classification III: According to Contents 1. Omentocele (omentum) 2. Enterocele (intestine) 3. Cystocele (Urinary bladder). 4. Littre‘s hernia—Meckel‘s diverticulum (NB: Littre described Meckel‘s diverticulum in a hernial sac 81 years before Meckel was born). 5. Maydl‘s hernia. 6. Sliding hernia. 7. Richter‘s hernia—part of the bowel wall.

Classification IV: According to Site 1. Inguinal hernia (in the inguinal canal). 2. Femoral hernia (in the femoral canal). 3. Obturator hernia. 4. Diaphragmatic hernia. 5. Lumbar hernia. 6. Spigelian hernia. 7. Umbilical hernia. 8. Epigastric hernia.

INGUINAL HERNIA

Surgical Anatomy of Inguinal Canal

- Superficial inguinal Ring (SIR): It is a triangular opening in the external oblique aponeurosis (EOA) and is 1.25 cm above the pubic tubercle. The ring is bounded by a supero-medial & infero-lateral crus. Normally, the ring does not admit the tip of little finger. - Deep Inguinal Ring (DIR): It is a U-shaped condensation of the transversalis fascia, lies 1.25 cm above the inguinal ligament midway between the symphysis pubis & anterior superior iliac spine (ASIS). - Inguinal (Poupart’s) Ligament: It is formed by the lower border of the EOA, which is thickened and folded backwards on itself, extending from the ASIS to the pubic tubercle. - Inguinal Canal: It is an oblique passage in lower part of abdominal wall, 4 cm long, situated above the medial ½ of inguinal ligament, extending from the DIR to the SIR. In infants, both rings are superimposed without obliquity of the inguinal canal. In females, the inguinal canal is called the "canal of Nuck". - Coverings of spermatic cord 1. Internal spermatic fascia from the fascia transversalis 2. Cremasteric fascia. 3. External spermatic fascia from EOA is seen below the SIR, in the scrotum. - Boundaries of inguinal canal (Figure 2) 1. Anteriorly: EOA 2. Laterally: conjoint muscle (Arched fibers of internal oblique). 3. Posteriorly: Inferior epigastric artery (IEA), fascia transversalis & conjoined tendon medially. 4. Above: Conjoined muscle - Below: Inguinal ligament.

Figure 2. Boundaries (+ contents) of inguinal canal

Classification of Inguinal Hernia

Classification I Anatomical Classification of Inguinal Hernia (Figure 3) 1. Indirect inguinal hernia (IIH): It comes out through the DIR (deep internal ring) along with the cord. It lies lateral to the IEA. 2. Direct inguinal hernia (DIH): It occurs through the posterior wall of the inguinal canal through ‗Hesselbach‘s triangle‘ (bounded medially by the lateral border of rectus muscle, laterally by the IEA & inferiorly, by the inguinal ligament).

Figure 3. Anatomical location of IIH and DIR (and femoral hernia)

Classification II According to the Extent 1. Incomplete - Bubonocele: The sac is confined to the inguinal canal. - Funicular: The sac crosses SIR, but does not reach the bottom of the scrotum. 2. Complete: - The sac descends to bottom of the scrotum N.B. Saddle-bag or pantaloon hernia has both IIH and DIH

Newer Classifications of Hernia I. Gilbert Classification

Type I - Hernia has got snug the DIR through which a peritoneal sac passes out as indirect sac. Type II - Hernia has a moderately enlarged DIR, which admits one finger but < 2-finger breadth. Once reduced, it protrude during coughing or straining Type III - Hernia has got large DIR with a defect >2-finger breadth. Hernia descends into the scrotum or with sliding hernia. Once reduced, it immediately protrudes out without any straining Type IV - It is direct hernia with large full blow out of the posterior wall of the inguinal canal. The DIR is intact Type V - It is a direct hernia protruding out through punched out hole/defect in the Transversalis fascia. The DIR is intact Type VI - Pantaloon / double hernia Type VII - Femoral hernia N.B. Types VI & VII are Robbin’s Modification. II. Nyhus Classification

Type I - Indirect inguinal hernia (IIH) with normal DIR. Type II - IIH with dilated DIR Type III - Posterior wall defect a. Direct inguinal hernia (DIH). b. Pantaloon hernia. c. Femoral hernia Type IV - Recurrent hernia

A. INDIRECT (OBLIQUE) INGUINAL HERNIA (IIH)

Incidence

- General incidence: It is the commonest type of hernia (65%) in males. - Age: It is more common in younger age group as compared to DIH, which is more common in the elderly. - Side: It is more common on right side in the first decade of life, but incidence is equal on both sides in the 2nd decade. It is bilateral in 30% of cases.

Pathology

- The sac is thin in IIH. The neck is narrow and lies lateral to the inferior epigastric vessels. - Coverings of IIH from inside out: 1. Extra-peritoneal tissue 2. Internal spermatic fascia 3. Cremasteric muscle 4. External spermatic fascia 5. Skin.

Types (Figure 4)

1. Bubonocele The hernia in bubonocele is limited to the inguinal canal. 2. Funicular The processus vaginalis is closed just above the epididymis. Contents of the sac can be felt separately from testis, which lies below the hernia.

3. Complete (Scrotal) The testis appears to lie within the lower part of hernia. It can occur in any age group. It occurs in a congenital preformed sac (processus vaginalis). More commonly, contents descend into the pre-existing sac only when there are precipitating causes, which force the content down.

Figure 4. Types of indirect inguinal hernia (IIH): bubonocele – funicular – complete (scrotal)

Clinical Features

- Gender: It is more common in males (M:F ratio = 20:1). - Patient presents with dragging pain and swelling in the groin, which is better seen while coughing and standing; and felt together with an expansile impulse. - The swelling is confined to the inguinal region in bubonocele (Figure 5), reaches down to the middle of the scrotum in the funicular type (Figure 6), and descends down to the scrotum completely in the scrotal (complete) type, which may reach the thigh (giant) (Figure 7).

Figure 5. Left bubonocele Figure 6. Left Funicular IIH Figure 7. Complete

- Contents are small bowel, large bowel, omentum or combination of all these. Sometimes, in females, the ovary and tubes may be the content. In infants, the swelling appears when the child cries and is often translucent. Differences between enterocele and omentocele are:

Enterocele Omentocele (Epiplocele)

- The first part is difficult to reduce but the - The first part is easier to reduce but last part is easier. the last part is difficult. - Gurgling sound on reduction of contents. - It has a doughy feeling - Peristalsis may be seen (inspection). - No peristalsis seen - Resonant on percussion. - Dull on percussion - Bowel sounds may be heard - Bowel sounds not heard

- It is usually reducible, but can be complicated with irreducibility, inflammation, obstruction or strangulation. - Internal ring (DIR) occlusion test The DIR ring is located half an inch above the mid-inguinal point (center point between ASIS and symphysis pubis). After reducing the contents, in lying down position, the internal ring is occluded using the thumb. The patient is asked to stand up and cough. If a swelling appears medial to the thumb, then it is a DIH (Figure 8). If the swelling does not appear and on releasing the thumb, it appears during coughing, then it is an IIH. - Ring invagination test (obsolete): After reduction of contents, the little finger/index finger of the examiner is invaginated from the bottom of the scrotum, pushed up & rotated to enter the SIR. The impulse on coughing is felt at the tip of the invaginated finger. - Zieman’s test: The examiner places his index finger on the DIR & the middle finger on the SIR and the ring finger over the saphenous opening. The patient is asked to cough or to hold the nose and blow. If the impulse is felt on the index finger, it is an IIH. - Head or leg rising test: To look for abdominal wall muscle tone and Malgaigne bulgings. - Valsalva maneuver: It is also used to check the tone of abdominal wall muscles. - General examination: Abdominal, chest and urological examination is done to look for any precipitating factor such as chronic bronchitis, bronchial asthma, ascites, stricture urethra, or benign prostatic hyperplasia. PR examination is a must.

Figure 8. DIR test: Appearance of the swelling = DIH

- Differences between IIH and DIH

Criteria DIH IIH

Incidence Less common More common - Occurrence Old Any age (young & middle age) - Age More likely to be bilateral Usually unilateral - Side Etiology Usually acquired weakness Mainly congenital defect Pathology - Sac Posterior to the cord Within the layers of the cord - Neck of the sac Medial to the IEA, wide. Lateral to the IEA, narrow - Coverings EOA. Cord layers + EOA - Parietal defect Weak fascia transversalis DIR Clinical Picture - Shape Hemispherical Oblong - Descent to scrotum Negative or very rare Common - Reduction Backwards Upward, backward & laterally - Descent Forwards Downward, forward & medially - SIR size Normal Wide - SIR test Impulse felt on finger pulp Impulse felt on finger tip - DIR test Negative Positive Complications Less common due to wide neck More common (narrow neck) Surgery Herniotomy: usually unnecessary Usually necessary (long sac)

Investigations

- Laboratory investigations: CBC - coagulation profile - - Chest X-ray to rule out chronic bronchitis. - Ultrasound (US) of the abdomen. - Tests relevant to the precipitating causes.

Treatment

- Treatment is always surgery. Surgical repair reconstructs the inguinal canal as it is physiologically, with 2 rings, one abdominal, the other subcutaneous; and with 2 walls, one posterior and the other anterior, between which the spermatic cord passes obliquely.

- Precipitating causes should be treated first, like TURP for BPH, dilatation of stricture urethra, treatment of chronic bronchitis. Patient is advised to avoid smoking. - In infants: Whether it is hernia or hydrocele, only herniotomy is done through inguinal approach (Michaelis plank operation). - In adults: It includes herniotomy (excision of hernial sac) and herniorrhaphy or hernioplasty (ideal) (strengthening of the posterior wall of inguinal canal either by repair or mesh, respectively). Hernioplasty is the present choice (ideal) for all groin hernias. A mesh is placed either onlay/underlay (over the conjoint tendon to inguinal ligament) or inlay (in the pre-peritoneal space). Polypropylene mesh is used. Herniotomy is done prior to mesh placement. TEP (Totally extra-peritoneal laparoscopic pre-peritoneal mesh repair) is the preferred method. (For detail refer hernioplasty in later part of this chapter).

Herniotomy - Anesthesia: Spinal, general or local anesthesia. - Incision: 1.25 cm above and parallel to the medial 2/3 of the inguinal ligament - Procedure o The 2 layers of superficial fascia (outer Camper‘s fascia, inner Scarpa‘s fascia) are incised. o The EOA is incised. The upper leaf is reflected above and the lower leaf is reflected downwards to expose the inguinal ligament. The ilio-inguinal nerve is safeguarded. o The cremasteric muscle is opened. Cord structures are dissected. The sac, which is pearly white in color and lies anterior and lateral to the spermatic cord is identified. o Dissection is usually started from the fundus and extended towards the neck, which is identified by extra-peritoneal fat. The neck is narrow and is lateral to the IEA. o The sac is opened at the fundus, contents, if any, are reduced and the sac is twisted so as to prevent the content from coming back. It is transfixed using absorbable suture material (Vicryl 2-0) and is excised distally.

Herniorrhaphy Modified Edwardo Bassini‘s (1887) Herniorrhaphy (Figure 9) - It is strengthening of the posterior wall of the inguinal canal by approximation of the conjoint tendon and inguinal ligament using interrupted non-absorbable mono-filament sutures {polypropylene}. The medial most stitch is taken from the periosteum of pubic tubercle (called as key or Bassini‘s stitch). The EOA and other layers are closed. - Multifilament suture material like silk is not used as it may precipitate infection & the tensile strength is not as good as monofilament suture material. - Continuous suture is not used as it compromises the blood supply and interferes with proper healing; and strength will not be as adequate as interrupted sutures. - Earlier, the commonest surgery done for inguinal hernia was modified Bassini‘s repair. Now hernioplasty is the commonly done procedure for both DIH and IIH. In direct hernia, the sac is usually not opened in contrast to indirect hernia.

Figure 9. Modified Bassini's repair: Approximation of the conjoint tendon to the inguinal ligament using interrupted non- absorbable mono- filament sutures.

Lytle‘s Repair - Often, the internal ring (DIR) is narrowed by placing interrupted sutures over the medial side of the ring to the transversalis fascia using interrupted Vicryl sutures in order to narrow the ring and push the cord laterally (Figure 10)

Figure 10. Lyttle repair: Narrowing of the internal ring (DIR) using interrupted sutures

Shouldice Repair (Toronto) - The transversalis fascia is thin, but tough and so double-breasting of this fascia using continuous non-absorbable sutures strengthens the posterior wall of the inguinal wall. - It is a multi-layered repair. After herniotomy, the transversalis fascia is incised along the line of the wound from the DIR to the pubic tubercle. - The lower flap of fascia is sutured to posterior part of the upper flap. Upper flap is sutured to the inguinal ligament. It causes double breasting of the transversalis fascia. Then conjoint tendon and inguinal ligament is further approximated by 2 layers of continuous sutures. - The EOA is sutured in 2 layers (double-breasting) in front of the cord. Hence the original Shouldice repair is a 6-layered procedure; first 2 layers of transversalis fascia, next 2 layers of conjoint tendon and last 2 layers of EOA. Suture material used here is fine steel wire of 34 gauge (in original Shouldice repair) or polypropylene or polyethylene. - Recurrence rate (RR) is 1%.

Berliner Modified Shouldice Repair - It involves double-breasting of the transversalis fascia like in Shouldice repair and a single- layer closure of the EOA without any additional two-layered repair of the conjoint tendon to the inguinal ligament.

McVay Operation—1940 (Cooper‘s Ligament Repair) - It is done by placing interrupted sutures between the transversalis fascia to Copper‘s ligament (superior pubic ligament) starting from the pubic tubercle medially towards the femoral sheath and later continued as suture repair between the transversalis fascia and iliopubic tract laterally up until entrance of the cord is reached. - It is a pure tissue repair. It requires relaxing vertical incision at the lateral border of the anterior rectus sheath, from the pubic tubercle extending superiorly for 4 cm. - It covers all three groin defects; DIR, IIH and femoral hernia.

Hernioplasty - A prosthetic mesh (e.g. Prolene, Vipro, ultrapro) is used to strengthen the posterior wall of the inguinal canal. - Onlay: e.g. Lichtenstein repair (Figure 11). - Underlay: e.g. PHS repair (Gilbert’s), Stoppas, totally extra-peritoneal pre-peritoneal (TEP), trans-abdominal pre-peritoneal (TAPP) mesh repair. - RR is significantly reduced by prosthetic mesh repair. But incidence of mesh inguinodynia increases due to entrapment of ilio-inguinal or ilio-hypogastric nerves in the mesh. Even though, it is still the commonly done repair for inguinal hernia. - Not used in children, in strangulated hernia or sepsis; only tissue repair is done.

Figure 11. Hernioplasty Lichtenstein repair: The mesh is placed between the conjoint tendon & the inguinal ligament (tension-free)

2. DIRECT INGUINAL HERNIA (DIH)

Incidence

- 10-15% of the hernias are direct and 35% of inguinal hernias are direct. - 50% of direct hernias occur bilateral. - It is uncommon in females and children. Surgical Anatomy

- It is always acquired, due to weakening of posterior wall of inguinal canal. - Hernia is medial to the IEA with a wide neck. - The sac is thick & often the medial wall or content may be the urinary bladder (UB). - Coverings of DIH from inside out: (1) extra-peritoneal tissue, (2) internal spermatic fascia, (3) cremasteric muscle, (4) external spermatic fascia & (5) skin. - Direct hernia occurs through Hesselbach’s triangle, which is bounded by the inferior epigastric artery (IEA) laterally, lateral border of the rectus abdominis medially and the inguinal ligament below. It is divided into medial and lateral halves by the obliterated umbilical artery (medial umbilical ligament) (Figure 12). Thus, DIH is classified as medial or lateral depending on which part of the Hesselbach‘s triangle, is arising from.

Figure 12. Hesselbach’s triangle: It is bounded by the IEA laterally, lateral border of rectus medially and inguinal ligament (medial half) below.

Clinical Examination

- Internal ring test (refer back) - Malgaigne bulgings are often seen in these patients on clinical examination more than in IIH. They are protrusions of abdominal wall muscle during leg-raising test as weak, soft, supple, swellings, which signify poor abdominal muscle tone. - The DIH rarely descends into the scrotum and strangulation is not as common as in indirect IIH. - However, in long-standing cases, it can descend down to the scrotum and strangulation can occur.

Treatment

Surgical Repair - Usually the direct sac is not opened. - Care should be taken at the medial aspect due to the presence of UB (the UB should be emptied before surgery). - Ideally, hernioplasty (mesh repair) is done. - In case of bilateral hernia, mesh repair can be done on both sides together. - Laparoscopic approach or supra-pubic approach may be better in bilateral cases.

Funicular Direct Hernia (Pre-vesical Hernia) = Ogilvie’s hernia - It is a type of DIH, which is prone for strangulation. - It is a narrow-necked hernia with pre-vesical fat and portion of the urinary bladder and/or intestine that herniate through a small defect in the medial part of the conjoined tendon just above the pubic tubercle. - It occurs in elderly males.

3. RECURRENT INGUINAL HERNIA

Incidence is 10%. Recurrence within 3 years it is called ―early‖; after 3 years ―late‖.

Predisposing Factors

Pre-operative Operative Post-operative

- Smoking. - Tension on the - Infection (50%). - Straining (e.g. chronic cough, sutures. - Hematoma chronic constipation or difficult - Inadequate technique formation. micturition). (e.g. improper suture - Retained sac in - ↑ intra-abdominal pressure material used) pantaloon hernia. (straining, tumor, ascites) - Weakness of the - Post-operative - General factors (e.g. old age, anterior abdominal straining. anemia, and hypo-proteinemia). wall (AAW)

Recurrence Rate

- Bassini‘s repair (10%), Shouldice repair (1%), Hernioplasty (1-3%), other types (5%.). - True or false recurrences? If hernia occurs in the inguinal region after inguinal hernia repair it is called true recurrence, but if another groin hernia (e.g. femoral) appears, it is called false recurrence. Currently, any groin hernias that reappears, it is a true recurrence. Clinical Features

- Similar to inguinal hernia. Defect is usually narrow and more likely to strangulate. - It can be medial or lateral recurrence depending on the location of the sac. - Medial recurrence is common (tension on suture line is greatest near pubic tubercle).

Treatment

- Treatment of the cause of recurrence is followed by hernioplasty. - Laparoscopic (TEP/TAPP) approach is better for recurrent hernia. - In open repair, pre-peritoneal mesh repair is ideal. - In elderly people, Koontz orchidectomy or cord excision at the inguinal canal may be added after proper prior consent.

Hernioplasty Definition and mechanism - It is strengthening of posterior inguinal wall in case of indirect hernia or in any large hernia with weak abdominal wall using a supportive material. - This allows and supports good fibroblast proliferation, which in turn strengthens the weak posterior wall of inguinal canal or abdominal wall. Material Used - Synthetic: Non-absorbable e.g. Prolene mesh, Dacron mesh, Marlex mesh, Mersilene mesh, or absorbable e.g. Vipro mesh and Ultrapro mesh. Indications - Direct hernia. - Recurrent / re-recurrent hernia - incisional hernia. - Old age. - Hernia with weak abdominal muscle tone. - Sliding hernia. Principles - Size of the mesh should be bigger than the size of the defect. - Mesh should be fixed above to the conjoint tendon & below to the inguinal ligament using interrupted, non-absorbable sutures. - Absolute hemostasis and prevention of infection are important. Complications - Infection. - Mesh extrusion (external). - Foreign body (FB) reaction. - Mesh erosion into the UB, bowel or vessels may occur (rare). - Mesh inguinodynia causing hyperesthesia & pain along the distribution of the ilio-inguinal or ilio-hypogastric nerves.

4. SLIDING HERNIA (HERNIA-EN-GLISSADE)

Definition

- The posterior wall of the sac is not only formed by the parietal peritoneum, but also by sigmoid colon with its mesentery on left side; cecum on right side and often with portion of the urinary bladder (Both sides) (1:2000). Rarely, small bowel sliding hernia or sacless sliding hernia can occur (1:8000). Content of the sac is usually small bowel or omentum.

Clinical Features

- Gender: Sliding hernia occurs exclusively in males; mainly on the left side (85%). - Age: It is seen commonly in adults and elderly patients. - It commonly occurs in indirect sac, but femoral & direct sliding hernias may occur. - Bilaterality is extremely rare. - Large globular swelling in the inguinal region descending into the scrotum, often irreducible or does not get reduced completely.

Treatment

- The posterior wall of the sac should not be separated from the large bowel or bladder. If tried, injury may result to these organs leading to fecal or urinary fistulas. - The partially excised sac is pushed into the peritoneal cavity with posterior wall & repair is done usually using prolene mesh (Hernioplasty). - In LaRoque repair, the DIR is incised above and below; reefing of the sigmoid colon mesentery is done, which forms the posterior wall of the inguinal canal so that the entire sigmoid is replaced into the peritoneal cavity. - Right-sided sliding hernia will have the cecum and appendix in its posterior wall; the cecum should not be separated from posterior wall of the sac, which may otherwise → fecal fistula. The appendix should not be removed as it may precipitate sepsis. - Appendices epiploicae of the sigmoid colon should not be removed since they may contain small colonic diverticula which may get opened to contaminate the field. - The urinary bladder (UB) may be present on medial side of the sac, which should not be separated; if UB injury occurs it should be sutured in two layers with Vicryl. Inside-out purse-string suture on the opened sac is done and the sac with its posterior wall is pushed into the abdominal cavity. - Urinary catheterization is a must and a truss should be completely avoided.

5. PANTALOON HERNIA (DOUBLE HERNIA, SADDLE HERNIA, ROMBERG HERNIA)

- Here both direct & indirect inguinal sacs are present & straddle the IEA. - It clinically presents as a direct hernia. - During surgery, the indirect sac may be missed, which leads to recurrent hernia.

6. INFANTILE HERNIA

- The processus vaginalis is closed at DIR and the hernial sac either invaginates the processus vaginalis as ―inverted umbrella‘ or comes behind processus vaginalis. - It is difficult to diagnose clinically, and is often diagnosed in the operating room.

Complications of Inguinal Hernia

Incarcerated Hernia - The lumen of portion of the colon occupying a hernial sac is blocked with feces. Here the content of the bowel could be indented with the finger, like putty. - In incarcerated hernia, sac and contents are densely adherent to each other (contents are fixed to the sac). It is always irreducible; often obstructed but may not be strangulated.

Strangulated Hernia Pathology - It occurs when blood supply of the contents of hernia is seriously impaired leading to formation of gangrene (Figure 13). - Initially venous return is impaired → congestion of the bowel → further dilatation of the bowel, which becomes purple colored → fluid collects in the sac → eventually, arterial blood supply is impaired → bowel becomes dark, brownish black with flabby and friable wall → bacteria migrate trans-serosally & multiply in fluid of the sac → perforation occurs at the site of constriction ring → Peritonitis occurs. - Common bacteria in strangulated hernia include E. coli, anaerobic streptococci, anaerobic bacteria and Klebsiella - Strangulation commonly occurs in the small bowel and also in the large bowel. Occasionally, strangulated omentocele also can occur without any intestinal obstruction. - Strangulation can occur in inguinal, femoral, obturator, umbilical or any hernia. The IIH is more prone for strangulation than DIH due to narrow neck, adhesions and arrow SIR.

Table 13. Strangulated hernia with gangrenous bowel and toxic fluid

- Part of circumference of the bowel when strangulated, is called Richter’s hernia wherein the patient presents with diarrhea, toxicity mimicking gastroenteritis. Richter‘s hernia is more common with femoral and obturator hernias.

- Maydl‘s hernia (Hernia-in-W): In this case, a loop of bowel in the form of ‗W‘ lies in the hernial sac and the center portion of the ‗W‘ loop is strangulated & lies within the abdominal cavity (Figure 14). Thus local tenderness over the hernia is not marked & the hernia gets reduced with the strangulated loop in the center of the “W”. Strangulation in this case is often missed during surgery & may lead to peritonitis due to retained gangrenous loop.

Figure 14. Maydl‘s hernia (Hernia-in-W) Clinical Features - Symptoms: Sudden severe pain, initially over a pre-existing hernia, which later becomes generalized over the abdomen. Vomiting, constipation & distension of the abdomen. - Local examination: The hernia is (1) tense, (2) severely tender, (3) irreducible and (4) without any expansile impulse on coughing. Rebound tenderness is diagnostic. - General features: Toxicity + dehydration & oliguria + electrolyte imbalance.

Investigations - Laboratory tests: Serum electrolytes, serum urea and creatinine, CBC (leucocytosis) - Imaging: Plain X-ray abdomen (erect) shows multiple air-fluid levels + US abdomen.

Treatment - Hospitalization: The patient must be admitted to hospital. - Pre-operative medical treatment o Naso-gastric aspiration + IV fluids to correct dehydration and electrolyte imbalance. o Prophylactic antibiotics. o Catheterization to maintain an adequate urine output. - Emergency surgery o Groin incision is made with incision extending into the most prominent area of the swelling. The sac is exposed & the constriction ring and SIR are released (cut). o The sac is opened carefully without allowing the spillage of fluid (usually spillage occurs extra-peritoneally) and the fluid is sucked with the bowel being held with fingers to prevent it from getting reduced. o The viability of the bowel is checked by color, peristalsis, pulsation, and bleeding. If gangrenous, resection and anastomosis is done and a drain is placed. o Bassini‘s repair is done by placing interrupted non-absorbable sutures. Antibiotics, IV fluids are continued. The drain is removed in 4-5 days. Once bowel movement begins, oral diet is started (in 5 days). Usually a mesh is not used in strangulated hernia repair - Post-operative Problems: infection, leak with fistula, septicemia

FEMORAL HERNIA

Surgical Anatomy of the Femoral Canal

- It is the medial, most compartment of the femoral sheath, which extends from femoral ring above to saphenous opening below (Figure 15). - It contains fat, lymphatics and LN of Cloquet. - It is 1.25 cm long and 1.25 cm wide at the base. Below, it is closed by cribriform fascia. - The femoral ring is bounded anteriorly by inguinal ligament; posteriorly by ilio-pectineal ligament of Cooper, pubic bone & fascia covering the pectineus muscle; medially, by the concave, sharp lacunar (Gimbernat’s) ligament; and laterally, by a thin septum separating from femoral vein (Figure 16).

Figure 15. Anatomy of the femoral Figure 16. Anatomy of the boundaries of the sheath femoral ring

Etiology (Predisposing Factors)

1. Wide femoral canal. 2. Multiple pregnancies.

Pathology in Femoral Hernia

- Through femoral canal, hernial sac descends down vertically up to saphenous opening and then escapes out into the loose areolar tissue to expand out like a retort. Because of its irregular pathway and narrow neck, it is more prone for obstruction and strangulation. - During surgery, precaution should be taken about the femoral vein and pubic branch of obturator artery (or accessory obturator artery), which often may get injured leading to torrential hemorrhage.

Clinical Features

- It is common in females (2:1 ratio) and in multipara. It is rare before puberty. - 20% occurs bilateral; however, more common on right side. - Patients Present with a swelling (Figure 17) in the groin below and lateral to the pubic tubercle (inguinal hernia is above & medial to the pubic tubercle) (Figure 18). - Swelling, impulse on coughing, reducibility, gurgling sound during reduction, dragging pain, are the usual features.

Figure 17. Right Femoral hernia presenting Figure 18. Femoral hernia, below and with a swelling in the groin lateral to the pubic tubercle (unlike inguinal hernia)

- Obstruction or strangulation → intestinal obstruction and a painful, tender, inflamed, irreducible swelling without any impulse + features of toxicity. - Often, a femoral hernia can be associated with inguinal hernia. Moreover, 40% of femoral hernias present as emergency hernia with obstruction/strangulation. - Gaur’s sign: In femoral hernia, distention of superficial epigastric and/or circumflex iliac veins occurs due to the pressure by the hernial sac. - Hydrocele of femoral hernia occurs when adherent omentum secretes fluid into the sac. - Herniation through a gap in the lacunar ligament (medial) is always strangulated is called Laugier’s femoral hernia. In congenital dislocation of hip, femoral hernia occurs behind the femoral vessels = Narath’s femoral hernia. - If the sac lies under the pectineal fascia, it is called Cloquet’s hernia. - Often on the medial side, a portion of the urinary bladder forms the wall of the femoral hernial sac (sliding femoral hernia).

Treatment (Figures 19 and 20)

1. Lockwood-low Operation: - The sac is approached below the inguinal ligament through a groin crease incision (or over the swelling) so that the fundus of the sac is dissected by direct vision and repair is done from below. - The inguinal ligament is sutured to Cooper‘s ligament (standard and ideal (Cooper‘s ligament repair).

2. McEvedy-high Operation - An incision is made over the femoral canal extending vertically above the inguinal ligament. The sac is dissected from below, the neck from above and repair is done from above. - It gives a very good exposure of the sac and repair is also easier. It is done in strangulated femoral hernia.

3. Lotheissen’s Repair - It is done through an inguinal canal approach (like inguinal hernia). The transversalis fascia is opened & the neck of the sac is identified in the femoral ring. The sac is dissected from above, the neck is ligated and repair is done. After herniotomy, the conjoined tendon is sutured to the ilio-pectineal ligament by interrupted sutures (2 or 3), using non- absorbable mono-filament sutures. Care should be taken to avoid injury of the femoral vein, pubic branch of obturator artery or UB. - It is not as strong as Cooper‘s ligament repair. Complications, in general, include bleeding hematoma or abscess.

Figure 20. Femoral hernia repair (inguinal Figure 19. Different approaches for surgical repair of ligament to Cooper's ligament). In Lotheissen‘s femoral hernia repair, conjoint tendon is sutured to Cooper's.

4. AK Henry’s Approach - Repair of bilateral femoral hernia via a lower abdominal incision.

5. Polypropylene mesh can be buttressed over the femoral canal to close the defect.

6. Laparoscopic Mesh Repair — TEP/TAPP.

CHAPTER 8 Ventral Hernias

INTRODUCTION

Definition Any protrusion through the abdominal wall with the exception of hernia through the inguino- femoral region is defined as ventral hernia. Incisional hernia (80%) & primary defects in the abdominal fascia (Figure 21), which can cause umbilical hernia, epigastric hernia (Figure 22), para-umbilical hernia (PUH) or Spigelian hernia (Figure 23) are grouped under ventral hernia

Figure 21. Fascial defect causing Figure 22. Epigastric AND Figure 23. Classical site of ventral hernia umbilical hernias. Spigelian hernia

Clinical Presentation Ventral hernia can be: - Non-complicated (reducible) or complicated (irreducible, obstructed, strangulated). - Single or multiple small defects (Swiss-cheese hernia).

Causes - Congenital defect. - Acquired causes e.g. Obesity, smoking, or chronic cough (straining).

Management Pre-operative preparation - Proper skin hygiene, respiratory care, control of obesity & antibiotics - Relevant investigations Surgical repair - Open mesh repair: inlay; onlay; sublay, underlay - Laparoscopic mesh placement: underlay (directly in front of contents) using dual mesh or four-layered mesh. 71

INCISIONAL HERNIA

Definition

- It is herniation through a weak abdominal scar (scar of previous surgery other than hernia repair, otherwise, it is called recurrent hernia).

Incidence

- It is common in old age and obese individuals. - It occurs in 10% of abdominal surgeries; 70% in the first 5 ys and 30% within 5-10 ys.

Predisposing Factors

- Abdominal scars may injure nerves of abdominal muscles e.g. scar of major surgeries (biliary, pancreatic) and scar of emergency surgeries (peritonitis, acute abdomen). - Faulty technique of closure. - ↑ intra-abdominal pressure (straining, cough, BPH, straining, stricture urethra, ascites). - Poor nutritional status of the patient, anemia and hypo-proteinemia - Debilitating disease (e.g. uremia, TB, jaundice or .malignancy) - Immunosuppression. - Smoking in postoperative period.

Clinical Features

History-Taking - Details of previous surgery and duration of the incisional hernia itself should be recorded. - History of wound infection, wound dehiscence, whether surgery done was an emergency or elective, and tension sutures placed or not. - History of pain, irreducibility and details of precipitating factors should be recorded. - Other precipitating factors are similar to inguinal hernia like smoking, urinary / respiratory / abdominal symptoms.

Clinical Examination - Swelling in the scar region. It may be small or large; huge or massive (diffuse) (Figure 24), more common in the lower abdomen. - Impulse on coughing. - Gurgling sound ± visible bowel peristalsis - Eventually features of irreducibility, obstruction, strangulation (complications) - Scar, its site and extent, whether healed primarily or secondarily, skin over the scar and swelling is noted. - Details of the swelling with expansile impulse on coughing and examination (supine/standing) are done. Figure 24. Huge incisional - The gap cannot be assessed in irreducible hernia. hernia. Note scar of previous operation

Investigations

The precipitating factors must be looked for: - Imaging: Chest X-ray - US abdomen. - Tests relevant for causes.

Pre-operative Preparation

- Reduction of weight and control of obesity. - Correction of nutritional deficiencies and anemia. - Treatment of diabetes mellitus (DM), hypertension, cardiac and respiratory problems or other precipitating causes. - Massive incisional hernia after reduction might cause IVC compression, paralytic ileus and diaphragmatic elevation with respiratory embarrassment (abdominal compartment syndrome). It is prevented by prior increasing the capacity of peritoneal cavity by creating

pneumo-peritoneum using CO2 so as to increase the peritoneal pressure by 12-15 cm of H2O, daily for 3-6 weeks. Later, definitive surgery is done.

Treatment Strategy

- Mesh repair of the incisional hernia defect is always better and ideal with less recurrence. - Adequate-sized mesh is placed either outer to the peritoneum (sublay), or outer to the musculo-aponeurotic abdominal layer (onlay/overlay), or occasionally combined sublay and onlay mesh placement, both deeper and outer to the musculo-aponeurotic layer. - Rive’s Stoppa’s mesh placement for incisional hernia is placing the mesh between the posterior rectus sheath & rectus muscle (retro-rectus mesh placement is easier for dissection and mesh placement). - Commonly Polypropylene mesh is used. Other materials used are Dacron, Polytetrafl uoroethylene (PTFE) mesh, Polyglycolic mesh (Vicryl mesh) or combined Polypropylene and Polyglycolic acid mesh (Vipro mesh). Prevention of infection/hemostasis and drainage are important. A drain (suction drain) must be placed after surgery. - Laparoscopic mesh repair is done for incisional hernia by placing a mesh under the defect laparoscopically in the intra-peritoneal plane. The problem of this underlay placement is chances of adhesion and GI fistula formation, but still it is found to be safer. Laparoscopic pre-peritoneal mesh-placement is also done for smaller defects. Currently, dual mesh (PTFE) or 4-layered mesh is available. Here, the mesh is placed under the peritoneum deep to the defect after reducing the contents. The mesh is fixed with sutures and tacks. In four-layered mesh, the deepest 1st layer is mad of absorbable cellulose, which allows new peritoneum to creep underneath. The 2nd layer is PDS/PTFE mesh, the 3rd layer is Polypropylene mesh and the last 4th layer is again PDS/PTFE mesh. It is ideal but costly. - Cattell’s operation: When the defect is <3 cm and if the patient is having adequate abdominal muscle tone, layer-by-layer anatomical repair is done using monofilament non-absorbable suture material like Polypropylene / Polyethylene, ideally with interrupted sutures. The sac should be dissected, ligated & excised prior to repair. The peritoneum and posterior rectus sheath are opposed as 1st layer & the anterior rectus sheath as 2nd layer.

- Double breasting of the rectus sheath using interrupted non-absorbable sutures using mono-filament suture. It is overlapping the rectus sheath in 2 layers with 2 rows of sutures. - Keel operation is done in large defects. The scar is excised and dissected beyond the margin of the defect. The sac is never opened unless there is obstruction of the content. The sac is inverted using continuous/interrupted inverting non-absorbable sutures, layer- by-layer until the defect margins are opposed together, which is then again sutured with interrupted sutures. (N.B. Keel is inverted beam of the ship). - Nuttall’s operation is done for lower midline incisional hernia. Attachments of the recti muscles are detached from the pubic bones and are crossed over to fix to opposite pubic bones so as to create a firm abdominal wall support by crossed recti muscles.

Different Types of Mesh Repair for Incisional Hernia

- Sublay (outer to the peritoneum) is the ideal method. Large-sized mesh is placed pre- peritoneally. It needs not be fixed as abdominal pressure keeps it in position (according to Pascal‘s law). - Underlay (under the peritoneum, directly over the content): Now it is accepted, but there are chances of adhesions/fistula formation. It is used in laparoscopic repair. Dual mesh/four-layered mesh is used. - Overlay mesh (outer to the musculo-aponeurotic layer). Here, the mesh is placed under the SC tissue. It carries a high recurrence rate (RR) (30%). Thus, it is not recommended. - Combined inlay and overlay with 2 layers of mesh. - Rive‘s Stoppa‘s method of placing the mesh between the posterior rectus sheath & the rectus muscle widely. Dissection is done at least 10 cm beyond the defect under the rectus muscle in front of the posterior rectus sheath; the mesh should cover 5 cm beyond the defect all around. Recurrence rate (RR) with this method is <10%. - Components separation technique is a better method in large defects. Here, the skin and SC fat are dissected of the anterior rectus sheath → EOA is incised 2 cm lateral to the rectus abdominis → the EOA is separated from the internal oblique until posterior axillary line → additional relaxing incisions are made over the rectus sheath, internal oblique, transverse abdominis → it is repeated on opposite side → 20 cm mobilization is achieved → also reinforced with a large mesh. Component separation technique causes often lateral bulges on both sides. If it is done without mesh support and RR is high. Its advantage is that a defect up to 20 cm can be easily brought together. The technique is also called as Autologenous repair by vascularized innervated muscle flaps) (Ramirez, 1990). - Inlay (bridging at myo-aponeurotic level outer to peritoneum): Adequate-sized mesh is placed in the defect level bridging the gap to cover the pre-peritoneum. It is better by principle but still shows a high RR.

Surgeries Recommended for Repair of Incisional Hernia

- Mesh repair — commonly done and ideal - Component separation technique - Layer-by-layer closure — Cattell‘s operation - Double-breasting of the rectus sheath 73

Post-operative Care

- Analgesics and antibiotics. - Nasogastric aspiration and prevention of paralytic ileus + fluid management - Abdominal binder for support of the abdominal wall during recovery period - Control of obesity & other precipitating factors. - Stopping smoking and treating other associated causes. - Early ambulation. - The drain should be kept until drainage becomes minimal.

Complications of Incisional Hernia Surgery

- Wound infection, sinus or seroma formation - Paralytic ileus - Abdominal compartment syndrome in large hernias - Entero-cutaneous fistula - Infection of the mesh - Recurrence

Additional Problems in Large Incisional Hernia

- While reducing the bulky contents like bowel and omentum, inadequate intra-abdominal capacity results in increased intra-abdominal pressure causing inferior vena cava (IVC) compression, mesenteric edema followed by stasis of splanchnic bed, paralytic ileus, diaphragmatic elevation and respiratory distress (abdominal compartment syndrome), urinary and bowel disturbances. Abdominal capacity can be raised by creating regular pneumo-peritoneum over the period of 3-6 weeks. - Lordosis and back pain may be the presenting feature. - The sac and contents may get adherent to the thin skin over the summit of the hernia leading to skin ulceration and occasionally, fistula formation. - It may often require resection of the adherent bowel segment. - Large mesh-placement is required.

Important Notes

- It is now universally accepted that prosthetic repair is the gold standard for all incisional hernia. - Non-prosthetic repair has got only historical value as the recurrence rate is very high (as high as 55%).

UMBILICAL HERNIA

Anatomy of Umbilical Region

- The umbilical ring is a complex structure, which is related to the linea alba, falciform ligament, obliterated urachus (median umbilical ligament) and umbilical fascia (Richet’s fascia). - It is the meeting point of 4 folds of embryonic plate and three systems; GIT (vitello- intestinal), urinary (urachus) and vascular (umbilical vessels). - It is located at the level of L4 and L5 vertebral discs; at a lower position in infants. - It is a water-shed area for venous and lymphatic drainage; above the umbilicus, it drains to axillary vein or lymphatics; and below to the inguinal area. - Umbilical skin is supplied by T10 spinal cord. - Umbilical hernia develops due to either absence of umbilical fascia or incomplete closure of umbilical defect. The weakest part in the umbilical cicatrix is the upper part where hernia begins.

Etiology

- Umbilical hernia is herniation through a weak umbilical scar (cicatrix). - It can be congenital in newborns and infants (common in males, 2:1) or acquired in adults (common in females). - Congenital umbilical hernia is common in Africa or in people of African origin (8 times), while the acquired type is similar to any ventral hernia. - It is common in infants and children, occurs commonly due to neonatal sepsis. - It is seen in 20% of newborn infants. - Umbilical hernia is common in Down‘s syndrome, Beckwith-Weidman syndrome.

Clinical Features

- It presents with a swelling in the umbilical region (Figure 25) within the first few months after birth, the size ↑ during crying. It is hemi-spherical in shape. - The defect can be felt with the finger during crying. - It can undergo irreducibility and obstruction, which presents with pain, distension, vomiting.

Treatment

- Initially, conservative. In 93-95% of cases, it disappears spontaneously in few months after birth (masterly inactivity). It can be hastened by adhesive strapping across the abdomen. Figure 25. Umbilical hernia

Indications for Surgery 1. If it persists; even after the age of two years. 2. If the defect is > 2 cm in size. 3. Acquired/adult umbilical hernia.

Surgical Procedures 1. Primary closure of the defect - An infra-umbilical incision (Figure 26) is made encircling its lower half (smiling incision). - The sac is dissected circumferentially and is released off from the umbilicus and SC tissue. It is opened; contents are reduced; excess part is excised up to the umbilical ring. The defect is closed with interrupted non- Figure 26. Infra (sub)-umbilical incision for surgical absorbable Prolene sutures. treatment of umbilical hernia 2. Mayo‘s operation - The horizontally-opened rectus sheath is approximated as double-breasting with the upper flap overlapping the lower flap in front. It is rarely done today. 3. Sub-lay mesh repair - In a large umbilical hernia (defect >3 cm) with a degenerated skin on its surface, it is often difficult to retain the umbilicus. When it is attempted to save the umbilicus, the infra-umbilical incision should extend laterally about 2 cm on each side at 3 and 9 O‘clock positions. The sac is dissected and is excised after excision of redundant part. - Currently, it is standard to use polypropylene mesh as sub-lay or in the retro-rectus position and then the rectus sheath is closed. 4. Umbilectomy - Unhealthy thin skin over a large umbilical hernia is a real problem. It is better to do umbilectomy in these patients (excision of umbilical cicatrix). - It is done only in adults with large umbilical hernia with thinning of the umbilical skin. Prior consent and repair and creation of the umbilicus (umbilicoplasty) is needed. 5. Open dual PTFE and Polypropylene mesh placement - Umbilical hernia is dissected similarly through a sub-umbilical incision. Redundant sac is excised and the peritoneum is not closed. A special composite mesh containing wider PTFE on the inner side with little smaller Polypropylene mesh on the outer aspect is used. It has also got two additional straps of the mesh attached to the outer part of the main composite mesh which are used to hold and later to fix into the defect anteriorly. - This technique yields excellent results with a low recurrence rate. 6. Laparoscopic umbilical hernia repair - It is similar to any ventral hernia repair through laparoscopy, which is done under general anesthesia. It is useful only in large umbilical hernia.

PARA-UMBILICAL HERNIA (PUH)

Etiology / Pathology

- It is a protrusion or herniation through the linea alba, just above or below the umbilicus. - It enlarges ovally, often attaining a large size and sags downwards. - The neck of the sac is relatively narrow. - Contents are usually omentum, small bowel and sometimes, large bowel. - It has a tendency to undergo adhesion formation, irreducibility & obstruction.

Clinical Features

- Age: It occurs commonly in adults - Gender: Common in females > males (5:1). - It presents as a swelling (Figure 27), which has a smooth surface, distinct edges, soft consistency, resonant on percussion (bowel) with dragging pain and impulse on cough. Large hernias can present with intestinal colic due to subacute intestinal obstruction. Eventually, obstruction and strangulation can occur. Figure 27. Para-umbilical hernia (PUH) in an adult gentleman. Treatment

Treatment is Always Surgical - Dissection of the hernial sac and placement of mesh in the retro-rectus plane and under the umbilicus is the ideal treatment. - Often, umbilectomy is required and mesh placement is useful when the defect is large (> 4 cm). - If there is strangulation, resection of bowel segment and anastomosis is done followed by repair of the hernia. - Mayo’s operation o Via a transverse elliptical incision, the sac is identified and dissected. o Herniotomy is done. o Double-breasting of the defect in the rectus is done by interrupted non-absorbable sutures. - Additional lipectomy (panniculectomy) may be done in case of obese patients with pendulous abdomen. - Post-operative weight reduction. - The use of abdominal binder is required for these patients.

FATTY HERNIA OF THE LINEA ALBA / EPIGASTRIC HERNIA

Pathology

- It occurs usually through a defect in the decussation of the fibers of the linea alba anywhere between the xiphoid process and the umbilicus. - Extra-peritoneal fat protrudes through the defect as fatty hernia of the linea alba presenting like a swelling in the upper midline with an impulse on cough (Figure 28) - It is a sacless hernia. Later, protrusion enlarges and drags a pouch of peritoneum, presenting as a true epigastric hernia. - If the defect is <1.5 cm, the lateral margin of the defect is formed by only anterior and posterior lamina of the rectus sheath; if the defect is >1.5 cm, then lateral margin is formed also by the rectus muscle. - Content of true epigastric hernia is omentum, but sometimes it may be small bowel.

Clinical Features

- Common in muscular men / manual laborers. - 20% of epigastric hernias are multiple (Swiss-cheese-like). - It is often asymptomatic. - Swelling in the epigastric region, which is tender. - Pain in epigastric region; often associated with peptic ulcer (may be the only cause of pain), which should be ruled out by gastroscopy. - Impulse on cough and defect in the epigastric region are also positive. - Irreducibility, obstruction, strangulation as seen in any other hernia.

Figure 28. Fatty hernia of the linea alba (FHLA). Just protrusion of the extra-peritoneal pads of fat occur i.e. NO sac.

Treatment

- Through a vertical incision, the sac is dealt with and the defect is closed as usual. - A large defect is supported with a pre-peritoneal mesh. - Complete reconstruction of the linea alba is needed from the xiphi-sternum to the umbilicus in Swiss-cheese type using different methods such as interrupted 1ry closure using - Polypropylene sutures or the modified shoelace technique (after removing strip of the medial margins of the linea alba; double-breasting of the linea alba is under-taken).

SPIGELIAN HERNIA

Definition and Site

- It is a type of inter-parietal hernia occurring at the level of the arcuate line through the Spigelian point. - The hernial sac lies either deep to the internal oblique muscle or between the external and internal oblique muscles.

Surgical Anatomy and Pathology

- It is a lateral ventral hernia through Spigelian fascia at any point along its line. - Semilunar line of Spigel is a line from pubic tubercle to tip of 9th costal cartilage. It marks the lateral margin of the rectus sheath. - Semicircular arcuate line (fold) of Douglas is the lower end of posterior lamina of rectus sheath below the umbilicus & above the pubis. - Spigelian fascia is the area between the lateral border of the rectus muscle and the external and internal oblique and transversus abdominis muscle. - Spigelian hernia can occur above (10%) or below (90%) the umbilicus. Below the umbilicus, it occurs at the junction of linea semilunaris and the linea semicircularis (wider and weaker point). - In Spigelian hernia, the defect is formed by the internal oblique & transversus abdominis muscle. The external oblique is outer to the hernial sac.

Clinical Features

- Age and gender: It is common in females >50 y. - It presents as a soft, reducible mass lateral to the rectus muscle and below the umbilicus, with impulse on cough. - Strangulation is common. - Presence of precipitating factors (obesity, chronic cough, old age, multiple pregnancies).

Differential Diagnosis

- It should be differentiated from: 1. Abdominal wall lipoma 2. Hematoma 3. Soft tissue sarcoma.

Treatment

- Through a lengthy transverse incision, herniotomy & later, closure of the defect layer-by- layer using non-absorbable interrupted sutures. But, ideally a mesh is required to cover the defect properly. - Laparoscopic dual mesh placement is also useful.

COMPLICATIONS OF HERNIA SURGERY

- Twenty years ago when tissue repair was popular, recurrence was the most worrisome complication. Nowadays, since recurrence rate has been reduced with the standard usage of mesh, mesh-related complications have become more common than recurrence. - Inguinodynia (pain in the inguinal region), mesh extrusion, mesh infection, mesh erosion are now the worrisome problems. - Complications of hernia surgery can be classified as early, delayed, late, or life- threatening. - According to technique, complications can be classified into those related to open surgery or laparoscopic (TEP/TAPP).

Complications of Open Hernia Surgery Complications of TEP/TAPP

- Infection - SC emphysema - Seroma / hematoma formation - Pneumothorax - Groin pain – osteitis pubis - Hyper-carbia - Inguinodynia (neural) - Vascular - Ischemic orchitis (thrombosis of pampiniform - Neural venous plexus (0.5%). - Visceral - Injury of vas deferens – injury of viscera - Infection - Hydrocele formation - Ileus - Recurrence - Conversion - Dysejaculaion: Painful, burning sensation just - Recurrence before, during or after ejaculation due to cremaster dysfunction or vas stricture (0.25%).

CHAPTER 9 Inguino-Scrotal Swellings Other than Hernias

CAUSES

- Inguino-scrotal swellings almost necessarily arise in the constituents of the spermatic cord or in a persistent funicular process. - The following must be considered: 1. Hernia (reducible, irreducible, strangulated) (Refer back) 2. Hydrocele (congenital hydrocele, infantile hydrocele, hydrocele of the cord, hydrocele of the hernial sac). 3. Varicocele. 4. Other spermatic cord lesions (causing thickening of the cord): a) Traumatic (hematocele of cord). b) Inflammatory. c) Malignant thickening of the cord. 5. Testicular causes.

HYDROCELE

Types of Hydrocele that Present with Inguino-scrotal Swelling

Congenital Hydrocele - It is a persistence of the processus funicularis throughout its length, and differs from a congenital hernia in that its communication with the abdomen is shut off, or so nearly shut off that bowel or omentum cannot enter it (Figure 1). - The swelling fills the tunica vaginalis and obscures the testis, and continues as a tubular prolongation up the inguinal canal. It is elastic or fluctuant, and trans-illuminates easily. - It is differentiated from a hernia by its translucency, and absence of both impulse on cough and reducibility. - Though irreducible (digital pressure on the hydrocele does not usually empty it), many congenital hydroceles have a small communication with the abdomen indicated by its reduction in size during the hours of sleep. - The appearance of bilateral congenital hydrocele in a baby is often an index of peritoneal mischief, such as tuberculous peritonitis.

Infantile Hydrocele - It occurs when a completely patent processus funicularis remains unobserved in infancy and becomes distended during adult life. The tunica and processus vaginalis are distended to the inguinal ring, but there is no connection with the peritoneal cavity - It has the same physical characters mentioned above, but it does not vary in size.

Encysted Hydrocele of the Cord - It occurs if the processus funicularis is shut off from both, the tunica vaginalis and peritoneum, leaving a patent portion in between, which becomes distended with fluid (Figure 1). - It may give an inguinal, scrotal or inguino- scrotal swelling depending on the patent part of the funicular process. - There is a smooth oval swelling near the spermatic cord, which is liable to be mistaken for an inguinal hernia. The swelling is characteristically cystic and translucent. Figure 1. Hydrocele - Traction test is positive. The swelling moves downwards and becomes less mobile if the testis is pulled gently downwards

Hydrocele of the Hernial Sac - There is collection of fluid in the hernial sac, the opening of which at the internal ring being closed by a plug of omentum. - There is a history of hernia, and the testis is felt separate.

Pathogenesis

A hydrocele can be produced in four ways: 1. By connection with a hernia of the peritoneal cavity in the congenital variety, which presents as hydrocele of the cord 2. By excessive production of fluid within the sac, e.g. Secondary hydrocele 3. Through defective absorption of fluid 4. By interference with lymphatic drainage of scrotal structures (elephantiasis)

Complications

Among complications of hydrocele are the following: 1. Rupture usually occurs as a result of trauma, but may be spontaneous. On rare occasions cure results after the fluid has been absorbed. 2. Transformation into a hematocele occurs if there is spontaneous bleeding into the sac or as a result of trauma. Acute hemorrhage into the tunica vaginalis sometimes results from testicular trauma and it may be difficult without exploration to decide whether the testis has been ruptured. If the hematocele is not drained, a clotted hematocele usually results.

3. The sac may calcify. Clotted hydrocele may result from a slow spontaneous ooze of blood into the tunica vaginalis. It is usually painless and by the time the patient seeks help, it may be difficult to be sure that the swelling is not due to a testicular tumor. Indeed, a tumor may present as a hematocele. 4. Infection, which may lead to ―pyocele‖. 5. Severe infection can, occasionally, be introduced by aspiration. Simple aspiration, however, often may be used as a temporary measure in those cases where surgery is contraindicated or must be postponed. 6. Post-herniorrhaphy hydrocele is a relatively rare complication of inguinal hernia repair. It may be due to interruption to the lymphatics draining the scrotal contents. 7. Atrophy of testis in long standing cases.

Diagnosis

A primary hydrocele is described as having the following characteristics: - Transillumination: positive - Fluctuation: positive - Impulse on coughing: negative (positive in congenital hydrocele) - Reducibility: absent - Testis cannot be palpated separately, except in funicular hydrocele, and encysted hydrocele.

Treatment

- Most hydroceles appearing in the first year of life seldom require treatment as they resolve without treatment. - Hydroceles that persist after the first year or occur later in life require treatment only in selected cases, such as patients who are symptomatic with pain or a pressure sensation, or when the scrotal skin integrity is compromised from chronic irritation; the treatment of choice is surgery and the operation is conducted via an open access technique aiming to excise the hydrocele sac. Anesthesia is required for the operation and general anesthesia is of choice in children, while spinal anesthesia is usually sufficient in adults. Local infiltration anesthesia is not satisfactory because it cannot abolish abdominal pain due to traction on the spermatic cord. In long-standing cases, hydrocele fluid may be opalescent with cholesterol and may contain crystals of tyrosine. - After aspiration of a primary hydrocele, fluid re-accumulates over the following months and periodic aspiration or operation is needed. For younger patients, operation is usually preferred. - Sclerotherapy is an alternative; after aspiration, 6% aqueous phenol (10-20 ml) together with 1% Lidocaine for analgesia can be injected, and this often inhibits reaccumulation. These alternative treatments are generally regarded as unsatisfactory treatment because of the high incidence of recurrences and the frequent necessity for repetition of the procedure.

VARICOOCELE

Surgical Anatomy (Veins of the Testis)

1. Pampiniform Plexus: Intercommunicating veins accompanying the testicular artery in the spermatic cord. They form 2-3 testicular veins in the inguinal canal and then one testicular vein at the level of the deep inguinal ring (DIR). 2. Cremasteric Veins: Anastomose freely with testicular veins to provide an alternative venous return. They leave the cord in the inguinal canal, piercing the posterior wall to end in the inferior epigastric vein. 3. Veins of the Vas: a small vein that passes the DIR, then passes medially into the pelvis to end in the inferior vesical vein and finally into the internal iliac vein.

Definition of Varicocele

Varicocele is pathological alteration in the venous drainage of the testis; defined as a state of varicosity (dilatation, elongation and tortuosity) of the testicular veins (Figure 2). Varicocele may be primary or secondary.

Figure 2: Varicocele: engorgement of the pampiniform plexus

Pathophysiology - Often the greatest concern with respect to varicocele is its effect on male fertility. The relationship between varicocele and infertility is unclear. Some men with the condition are fertile, some have sperm that are normal in shape and move normally but are compromised in function, and some have sperm with abnormal shapes or that do not move well. Theories as to how varicocele affects sperm function include damage via excess heat caused by the blood pooling and oxidative stress on sperms. - Tobacco smoking and mutations in the gene expressing glutathione S-transferase both put men at risk for infertility; these factors may also exacerbate the risk that varicocele will affect fertility.

Signs and Symptoms - Varicocele occurs in about 15% to 20% of all men. Its incidence increases with age. - It is an inguino-scrotal swelling (chiefly scrotal).

- It is nearly always on the left side, the patient being usually a young adult with dragging pain or heaviness in the scrotum, particularly after prolonged standing (Figure 2). - The distended veins of the pampiniform plexus resemble a ―bag of worms‖. With a lax scrotum, the blue color of veins can be seen. - A varicocele is said to have an impulse on coughing, but the sensation is that of a fluid thrill or a small tap on the examining finger rather than an impulse. Figure 2. Left varicocele - Squeezing empties the swelling, which refills immediately. Recumbency with the scrotum raised also causes it to disappear.

Clinical Classification (Dubin)

Group Description

Group 1 (large) - Easily visible at a distance. - Palpation reveals a bulk of dilated veins >2 cm in diameter on Valsalva‘s maneuver. Group 2 (moderate) o Less easily seen. o Palpation reveals a bulk of dilated veins 1-2 cm in diameter. Group 3 (small) - Cannot be seen by inspection. - Bulk of veins < 1 cm by palpation (but +ve reflux with Valsalva‘s).

Diagnosis Following discovery of the sign of swelling comprising a mass, varicocele can be confirmed with scrotal ultrasound, which will show dilation of the vessels of the pampiniform plexus to be greater than 2 mm.

Complications of Varicocele

1. Atrophy of the testis due to chronic congestion (pressure and thermal effect). 2. Oligospermia (sterility, if bilateral) due to the same effects. 3. 2ry hydrocele, due to chronic congestion of the testis. 4. Repeated attacks of thrombosis due to blood stagnation in the veins. 5. Redundancy of the scrotum may interfere with the patient‘s normal activities, and also makes the testis more liable to torsion and trauma, and may cause neurosis.

Differences between 1ry & 2ry Varicocele

Criteria Primary Varicocele Secondary Varicocele

Incidence More common Less common

Etiology - Genetic factor (familial - Abdominal mass or tendency). retroperitoneal fibrosis. - Wall weakness - Hypernephroma invading the - Venous hypertension renal vein, or causing (congestion). compression from outside. Age Young adults. Older age (40-50 years). Side Left side (96%), right side (2%), According to cause and site of bilateral (2%) obstruction.

Onset Gradual. Rapid and sudden. Clinical 1. Dragging pain. 1. Painless. Picture 2. Impulse and thrill on cough. 2. No impulse and no thrill. 3. Bowing test +ve. 3. Bowing test -ve. 4. Softer and smaller testicle. 4. Larger and firmer testicle. 5. No evidence of 1ry cause. 5. Evidence of 1ry cause.

Treatment - The two most common surgical approaches are retro-peritoneal (abdominal using laparoscopic surgery), infra-inguinal/sub-inguinal (below the groin), and inguinal (groin using percutaneous embolization). - Possible complications of this procedure include hematoma (bleeding into tissues), hydrocele (accumulation of fluid around the affected testicle), infection, or injury to the scrotal tissue or structures. In addition, injury to the artery that supplies the testicle may occur, resulting in a loss of a testicle.

Prognosis

- Whether having varicocele surgery or embolization improves male fertility is controversial, as good clinical data is lacking. There is tentative evidence that varicocelectomy may improve fertility in those with obvious findings and abnormal sperm. - Evidence for sclerotherapy is unclear as of 2015.

Important Clinical Notes

Why does 1ry varicocele occur more on the left side than the right ?? 1. Higher incidence of left testicular vein abnormal valves (absent, malformed or incompetent). 2. The left testicular vein ends in the renal vein at a right angle, which impedes the flow. 3. Blockage of the valve at the left testicular vein by the SMA or left colon. 4. Earlier descent of left testis  lower level in the scrotum  longer & more dependent veins. 5. Nutcracker mechanism: compression of left renal vein between the abdominal aorta and superior mesenteric artery (SMA). 6. Compression of left renal vein by the ligament of Trietz or arched testicular artery. 7. The left adrenal vein enters the left renal vein opposite to the stoma of the left testicular vein causing turbulence of blood and impedance of flow.

Why is spermatogenesis impaired in cases of varicocele ?? 1. Disturbed scrotal thermoregulatory system  impaired testicular function due to  intra-scrotal temperature. 2. Mechanical pressure of the dilated pampiniform plexus may  testicular dysfunction. 3. Stasis of blood  hypoxia and tissue destruction especially of the germinal epithelium of the testis. 4. Impaired epididymal environment which may be produced by varicocele  disturbed motility of the sperms. 5. Retrograde passage of toxic materials (mainly cortisol) from the left renal or left adrenal vein to the pampiniform plexus. 6. Hormonal (chemical) factors: Alteration in testicular function of infertile men with varicocele > fertile men with varicocele: - Catecholamines (found more in the left testicular vein and cause spermatic dysfunction). - Prostaglandins (PGDs E & F) (higher levels in the left internal spermatic vein  inhibited spermatogenesis due to reduced blood flow through the testis and acceleration of contractility of the accessory sex organs, which  reduced maturity of sperms). - Serrotonin  dys-spermatogenesis due to its vaso-constrictive effect on testicular blood supply.

How can you diagnosis subclinical cases ? 1. Doppler echography: With the patient recumbent, the probe is placed over the spermatic cord. With Valsalva‘s maneuver, venous pressure increases  reversal of blood flow in the testis (i.e. incompetent valves). 2. Scrotal thermography: Scrotal temperature reflects that of underlying structures. With venous stagnation, temperature is elevated. 3. Retrograde spermatic venography (via a catheter in the left renal vein): It shows reflux. It is a definitive test but invasive & therefore not used for screening and has been replaced by Doppler and thermography.

OTHER SPERMATIC CORD LESIONS

Trauma (Hematocele of the Cord)

- It presents as an ill-defined opaque swelling which is tender and irreducible. - It occurs soon after trauma such as a kick in the groin.

Inflammation

- Inflammatory thickening of the cord may be an extension of inflammation of the epididymis and testis. - Tuberculous funiculitis o There is generalized fullness of the cord. o More typically the ―beaded‖ vas may be felt. - Filarial funiculitis o It is usually acute with high fever swelling and redness of the skin. o The cord becomes thickened, tender and matted. o There is always a well-pronounced hydrocele. o The prostate and seminal vesicles are normal. o The patient usually comes from an endemic area. o Blood shows leukocytosis and eosinophilia and may show microfilaria. o Sometimes a residual mass is felt in the cord after the acute attack resolves. o It resembles a bilharzial mass, but differentiation can be made by the following:

Filarial Mass Bilharzial Mass

- Cord is matted - Cord not matted. - Mass diffusely affect the cord. - Mass in the lower 2 inches of the cord. - Normal prostate. - Enlarged, firm, irregular prostate. - Patient comes from an endemic - History of bilharziasis. area. - Ova in urine. - No bilharzial ova in urine.

Lymph Varix (Lymphangiectasis, Diffuse Hydrocele of the Cord)

- There is dilatation and tortuosity of the lymphatic vessels of the cord, caused by obstruction due to filaria. - It gives an impulse (thrill-like) on coughing and reduces spontaneously on lying down. - It may feel soft, cystic or doughy. - It is diagnosed by the history of periodic attacks of fever with development of swelling and tenderness of the cord, eosinophilia and living microfilaria in blood.

Neoplastic

1. Diffuse lipoma of the cord - It is rather rare. - The cord feels soft and lobulated. - The swelling is irreducible and has no impulse on cough. 2. Malignant extension from the testis - It may occur along its lymphatics. - The inguinal condition is, however, over-shadowed and explained by the scrotal one (i.e. the testicular tumor). 88

HEPATO-BILIARY PANCREATIC SURGERY

CHAPTER 1 Surgical Jaundice

INTRODUCTION / DEFINITIONS

- Jaundice (derived from French word ‗jaune‘ for yellow or icterus, which is the Latin word for jaundice) is a yellowish staining of the skin, sclera and mucous membranes by deposition of bilirubin in these tissues. - Jaundice indicates excessive levels of conjugated or unconjugated bilirubin in blood and is clinically apparent when bilirubin level exceeds 2 mg/dl. - It is most apparent in natural sunlight. - Causes of jaundice can be classified into pre-hepatic, hepatic or post-hepatic. Post-hepatic jaundice is also termed obstructive or surgical jaundice as this type is amenable to surgical treatment.

SURGICAL ANATOMY OF THE HEPATO-BILIARY SYSTEM

- The biliary system can be broadly divided into two components, the intra-hepatic and the extra-hepatic tracts. - The secretory units of the liver (hepatocytes and biliary epithelial cells), the bile canaliculi, bile ductules and the intrahepatic bile ducts make up the intra-hepatic component of the biliary tree. - The extra-hepatic bile ducts (right and left), the common hepatic duct (CHD), the cystic duct, the gallbladder (GB), and the common bile duct (CBD) constitute the extra-hepatic component of the biliary tree. - The cystic and CHDs join to form the CBD which is approximately 8-0 cm in length and 0.4-0.8 cm in diameter. The CBD can be divided into three anatomical segments: supra-duodenal, retro-duodenal, and intra-pancreatic (Figure 1). It then enters the medial wall of the duodenum, courses tangentially through the submucosal layer for 1- 2 cm, and terminates in the major papilla in the second portion of the duodenum (Figure 2). The distal portion of the duct is encircled by smooth muscle that forms the sphincter of Oddi. The CBD may enter the duodenum directly (25%) or join the pancreatic duct (75%) to form a common channel, termed the ampulla of Vater.

Figure 1. Anatomy of the common bile duct (CBD)

Figure 2. Anatomy of the biliary tree. The CBD enters the medial wall of the duodenum, courses tangentially through the submucosal layer for 1-2 cm, and terminates in the major papilla in the second portion of the duodenum

PHYSIOLOGY/BIOCHEMISTRY OF BILIRUBINE PRODUCTION AND TRANSPORT

- Bile is produced in the liver and contains bile salts, water, cholesterol, electrolytes, and bilirubin, which is a breakdown product of hemoglobin. - The formation of bilirubin from heme is essential for mammalian life, because it provides the body with the main means of elimination of heme. Eighty percent of the circulating bilirubin is derived from heme of hemoglobin from senescent red blood cells (RBCs) destroyed in the reticuloendothelium of the bone marrow, spleen, and liver. Ten to twenty percent of the bilirubin comes from other sources such as myoglobin, cytochromes, and other heme-containing proteins processed in the liver. - Initially, heme is oxidized to the green pigment biliverdin, which is then reduced to bilirubin, which is virtually insoluble in aqueous solutions. In blood it is reversibly, but tightly bound to plasma albumin. - Hepatic uptake of bilirubin occurs with the dissociation of the albumin-bilirubin complex with subsequent translocation of bilirubin into the hepatocyte. - In the hepatocytes, conjugation of bilirubin involves its esterification with glucuronic acid to form, first, a monoglucuronide, then a diglucuronide. The principal enzyme involved is uridine diphosphate (UDP)-glucuronyl transferase. Conjugation renders bilirubin water-soluble and is essential for its elimination from the body in bile and urine. - Conjugated bilirubin is excreted in bile through the biliary and cystic ducts to enter the gallbladder, where it is stored; or it passes through the ampulla of Vater to enter the duodenum. Inside the intestines, some bilirubin is excreted in the stool as stercobilin (responsible for the dark color of stools), while the rest is metabolized by the gut flora into urobilinogens and then reabsorbed. The majority of the urobilinogens are filtered from the blood by the kidney and excreted in the urine. A small percentage of the urobilinogens are reabsorbed in the intestines and re-excreted into the bile through the entero-hepatic circulation

ETIOLOGY

Causes of surgical jaundice may be congenital or acquired as shown in Table 1. Table 1: List of causes of surgical jaundice CONGENITAL CAUSES

1. Biliary atresia. 2. Cystic fibrosis Disease 3. Choledochal cysts

ACQUIRED CAUSES

1. Gallstone disease (Calcular a) Choledocholithiasis: obstructive jaundice): - Primary: Formed inside the CBD - Secondary: Migrating from the GB b) Mirizzi Syndrome 2. Tumors (Malignant obstructive a) Primary tumors jaundice): - The gallbladder - Bile ducts (cholangiocarcinoma) - Head of pancreas - Ampulla of Vater - Peri-ampullary carcinoma - Metastatic tumors b) Metastases deposited in the lymph nodes (LNs) at the porta-hepatis. Primaries of these metastatic deposits may be located in the lower esophagus, stomach, breast, or even the liver itself. 3. Trauma a) Iatrogenic e.g. during cholecystectomy, liver resections, etc. b) Others e.g. stab wound, gunshot, etc 4. Strictures a) Benign b) Malignant c) Primary sclerosing cholangitis

PATHOPHYSIOLOGY OF OBSTRUCTIVE JAUNDICE

- In obstructive jaundice, the pathophysiological effects reflect the absence of bile constituents in the intestines and their spillage into the systemic circulation. - Stools become pale because less bilirubin reaches the intestine. Absence of bile salts can produce malabsorption, leading to steatorrhea and deficiencies of fat- soluble vitamins (particularly A, K, and D); vitamin K deficiency can reduce prothrombin levels and cause coagulopathy.

- Bilirubin retention produces mixed hyper-bilirubinemia (jaundice). Some conjugated bilirubin reaches the kidney and darkens the urine. High levels of circulating bile salts are associated with, but may not cause, pruritus.

CLINICAL FEATURES

- A good history, physical examination and diagnostic tests are the requisites for the evaluation of the jaundiced patient. - Jaundice, dark urine (liquorice colored), pale stools and generalized pruritus are the hallmark of obstructive jaundice. - The main aim of history-taking and physical examination is the differentiation between calcular (benign) and malignant obstructive jaundice (Table 2). - History of fever, biliary colic and intermittent jaundice may be suggestive of calcular obstructive jaundice. - Weight loss, abdominal mass, pain radiating to the back and progressively deepening jaundice may be suggestive of malignant obstructive jaundice. - Deep jaundice (with a greenish hue) that appears to fluctuate in intensity may be due to a peri-ampullary cancer.

Table 2: Differences between calcular and malignant obstructive jaundice

Features Calcular Malignant

1. History Long (months or years) Short (days or weeks)

2. Symptoms - Dyspepsia Fatty food intolerance Usually absent - Biliary colic Present Absent - Anorexia Absent Present - Weight loss Absent Present 3. Onset of jaundice After a heavy meal and an Accidental discovery i.e. attack of typical biliary colic jaundice usually told by (Painful Jaundice) others ( Painless Jaundice) 4. Course of jaundice Intermittent Progressive 5. Cholangitis Present Absent 6. Previous attacks ± Never

Cholangitis

- Definition: It means infection of the biliary system. - Clinical presentation: It can present as either: a) Charcot‘s triad: right upper quadrant pain, jaundice, and fever with rigors. b) Reynolds pentad: right upper quadrant pain, jaundice, and fever with rigors, hypotension and an altered mental status.

Courvoisier's Law

Courvoisier's law In a jaundiced patient, if gallbladder is palpable, it is unlikely to be due to gallstones Explanation of the law Malignant obstruction is usually a complete obstruction, which leads to chronically elevated intra-ductal pressures. This contrasts with obstructions caused by gallstones; stones usually cause only partial obstructions (related to a ―ball- valve‖ action of the stone) leading to less consistent intra-ductal pressure elevations and less gallbladder dilatation. Exceptions of the law 1. A malignant obstruction above the cystic duct will not cause GB dilatation. Therefore, absence of a palpable GB in a jaundiced patient does exclude a malignant cause 2. Rarely, a stone may get impacted at the cystic duct while another obstructs the CBD leading to a palpable mucocele of the GB and obstructive jaundice. Nevertheless, this case should never underscore the fact that for patients presenting with jaundice and an enlarged palpable GB the possibility of a neoplastic process involving the head of pancreas or the ampulla of Vater should be strongly considered.

INVESTIGATIONS

Biochemistry / Hematology

- Elevated serum bilirubin level with a preponderance of the conjugated fraction is the rule. - The serum gamma glutamyl trans-peptidase (GGT) level is also raised in cholestasis. - The alkaline phosphatase may be elevated up to ten times normal. - Elevated white cell count (WBC) may be present in cholangitis. - Tumor markers like CA 19-9, CEA and CA-125 are usually elevated in pancreatic cancers, cholangiocarcinoma and peri-ampullary cancers, but they are non-specific and may be elevated in other benign diseases of the hepato- biliary tree.

Imaging

Goals of Imaging 1. To confirm the presence of an extra-hepatic obstruction (i.e. to verify that the jaundice is indeed post-hepatic rather than hepatic), 2. To determine the level of the obstruction, 3. To identify the specific cause of the obstruction, and 4. To provide complementary information relating to the underlying diagnosis (e.g. staging information in cases of malignancy

Ultrasonography (US) It is the most important initial tool of investigation as it can provide sure evidence of biliary tree obstruction; namely intra-hepatic bile duct dilatation. In addition it can provide data regarding: - The size of the extra-hepatic biliary tree - The level of the obstruction, - The cause and additional information related to the disease (e.g. GB stones, hepatic metastases, hepatic parenchymal change).

Computed Tomography (CT) Scan of the Abdomen It is very useful for staging of pancreatic and biliary malignancies. It provides excellent visualization of the tumor and its relation to surrounding structures, as well as visualization of the liver and lymphadenopathies.

Endoscopic Ultrasound (EUS) - With regard to the biliary system, EUS is useful for the detection and staging of ampullary tumors, evaluation of benign and malignant bile-duct strictures and finally in evaluating relationships to vascular structures. - EUS enables the aspiration of cysts and biopsy of solid lesions.

Direct Visualization of the Biliary Tree 1. Endoscopic retrograde cholangio-pancreatography (ERCP) 2. Percutaneous trans-hepatic cholangiography (PTC): PTC provides direct visualization of the level of obstruction. Both ERCP and PTC are invasive and may be associated with complications such as cholangitis, biliary leakage, pancreatitis, and bleeding. 3. Magnetic resonance cholangio-pancreatography (MRCP) It is a non-invasive technique for visualization of the biliary and pancreatic ductal system.

TREATMENT OF OBSTRUCTIVE JAUNDICE

Treatment of obstructive jaundice is treatment of the cause

Surgically Correctable Jaundice

The term surgically correctable jaundice refers to a broader spectrum of diseases where surgery can relieve jaundice.

1. Pre-hepatic (hemolytic) jaundice: Hereditary Spherocytosis: - Hereditary spherocytosis is a disorder of the RBC membrane that leads to sequestration and destruction of the spherocytic RBC in the spleen resulting in hemolytic anemia. - Splenectomy is curative and serves as the sole mode of therapy. 2. Hepatic jaundice - End-stage liver disease requiring liver transplantation 3. Post-hepatic (obstructive) jaundice

CHAPTER 2

Surgical Diseases of the Gall Bladder

CHOLELITHIASIS

The spectrum of manifestation of gallstones is very wide. Figure 1 illustrates the most common of these manifestations.

Figure 1: Spectrum of gallstone manifestations and complications

Asymptomatic Gallstones

Clinical Presentation Asymptomatic gallstones are usually discovered on routine imaging studies or incidentally at laparotomy for unrelated problems.

Management - There is no role for prophylactic cholecystectomy in most patients with asymptomatic gallstones. - Prophylactic cholecystectomy may be warranted in patients with asymptomatic gallstones who have other risk factors for gallbladder cancer as outlined later.

- Children with gallstones have a relative indication for cholecystectomy due to the general difficulty of declaring and interpreting symptoms in this population. - Management of gallstones discovered at laparotomy remains controversial because the literature is conflicting with regard to the incidence of biliary symptoms after surgery in patients in whom the gallbladder is not removed.

Symptomatic Gallstones

Clinical Presentation - Biliary colic is the main symptom and is initiated by impaction of a gallstone in the outlet of the gall bladder (GB). An attack has characteristic periodicity, location, and timing. The pain comes in waves lasting 30 minutes to several hours and is typically situated in the epigastrium or right upper quadrant, occasionally with concomitant back or left upper quadrant symptoms. The pain is commonly severe after a meal with such intensity as to wake-up the patient from sleep. - Other symptoms include nausea and vomiting.

Diagnosis - Physical signs include mild right upper quadrant tenderness, although there may be few abdominal findings during an attack. If jaundice is present, another cause should be sought. - Ultrasound (US) diagnosis is based on the presence of echogenic structures having posterior acoustic shadows (stones). There is usually little or no associated GB wall thickening or other evidence of cholecystitis. The bile ducts must be assessed for evidence of dilatation or choledocho-lithiasis (bile duct stones).

Treatment - Laparoscopic cholecystectomy (LC) is the appropriate treatment of patients with symptomatic gallstones, as described below.

Acute Calculous Cholecystitis

It is initiated by obstruction of the cystic duct by an impacted gallstone. Persistence of stone impaction leads to inflammation of the gallbladder.

Diagnosis - Diagnosis can be made by a combination of local and systemic signs of inflammation, correlated with imaging findings. - Local inflammatory signs include right upper quadrant pain and tenderness as well as Murphy sign, which is inspiratory arrest during deep palpation of the right upper quadrant. - Systemic signs include fever, leukocytosis, and an elevated C-reactive protein (CRP) level. Mild jaundice may be present, but severe jaundice is rare and suggests the presence of common bile duct (CBD) stones, cholangitis, or obstruction of the CBD caused by external compression from Mirizzi syndrome.

- Complications, such as gangrene, perforation, or cholangitis, are suggested by moderate leukocytosis (>20,000 cells/µL). - Liver function tests (LFTs), including serum bilirubin, alkaline phosphatase, alanine transaminase (ALT), aspartate transaminase (AST), and serum amylase, may also be abnormal. - Ultrasonography (US) may reveal GB wall thickening, peri-cholecystic fluid, and a sonographic Murphy sign (tenderness over the GB when compressed by the US probe). - Computed tomographic (CT) scanning is now frequently performed to evaluate the patient with acute abdominal pain. It can demonstrate gallstones, although it is less sensitive for these than US. Other signs of acute cholecystitis on CT scan include GB wall thickening, peri-cholecystic fluid, edema, and emphysematous changes. - Radionuclide cholescintigraphy can be useful as an adjunct in the diagnosis of acute cholecystitis. Scintigraphic scanning with (HIDA) enables visualization of the biliary system. The radionuclide is concentrated and secreted by the liver, allowing visualization of the bile ducts and the GB normally within 30 minutes. Since the test depends on hepatic excretion of bile, it may not be useful in jaundiced patients. Non- filling of the GB after 4 hours is diagnostic of acute cholecystitis. Although its sensitivity and specificity are higher than US, its expense and total study duration limit it from being the first imaging choice.

Management - Initial steps for patients with acute cholecystitis include hospitalization, intravenous (IV) fluid resuscitation, and parenteral antibiotics. - In Mild/ responsive cases for medical treatment 1. Early approach: within first 72 hours of the attack onset 2. Delayed approach: after 6 weeks of treatment - In Complicated cases/ or failed medical treatment: 1. Emergency cholecystectomy 2. Percutaneous Cholecystostomy Several prospective, randomized trials have compared early versus delayed (6 weeks) LC for acute cholecystitis. Recent meta-analyses of the existing literature showed no significant differences in early versus delayed procedures with regard to mortality, conversion rate, bile duct injury, and peri-operative complications.

Choledocholithiasis

Choledocholithiasis is generally due to gallstones that originate in the GB and pass through the cystic duct into the CBD. Stones rarely originate in the hepatic or common ducts in Western countries, but intra-hepatic stones are more common in Asia.

Diagnosis - The most common manifestation of uncomplicated choledocholithiasis is jaundice, with bilirubin levels typically between 3-10 mg/dL. - Cholangitis is often caused by choledocholithiasis. 111

- Biliary colic is common. - Ultrasonography usually demonstrates GB stones and bile duct dilatation. Because of obscuring gas in the duodenum, ductal stones are visible in only about 50% of cases. - Diagnosis may be confirmed by magnetic resonance cholangio-pancreatography (MRCP), endoscopic retrograde cholangio-pancreatography (ERCP) or percutaneous trans-hepatic cholangiography (PTC), which can opacify the biliary tree and demonstrate the intra-ductal stones. - Occasionally, diagnosis of choledocho-lithiasis is confirmed by intra-operative cholangiography (IOC) at the time of cholecystectomy.

Management Management depend on the clinical situation as follows: - Patients with low risk of choledocho-lithiasis have no evidence of LFT derangements, jaundice, cholangitis, pancreatitis, or imaging evidence of CBD stones. In these patients, standard management consists of LC. - Patients with high risk of choledocho-lithiasis have US evidence of CBD stone, bilirubin levels >4 mg/dL, clinical ascending cholangitis, or dilated CBD on US with a bilirubin >1.8 mg/dL. In these patients, ERCP is recommended prior to interval LC. ERCP with sphincterotomy carries a <1% risk of mortality and a 5-10% risk of morbidity, principally acute pancreatitis. - In cases with ectatic CBD (>1.5cm in diameter); biliary enteric bypass is needed.

Biliary Pancreatitis

- Biliary Pancreatitis is caused by blockage of pancreatic secretions by passage of a gallstone into the common biliary pancreatic channel. The greatest risk is carried by small (<2 mm) stones. - Once the acute episode of pancreatitis has resolved, the GB should be removed during the same admission to avoid recurrent pancreatitis. - A longer delay may be justified in patients who have had severe pancreatitis and in whom local inflammation or systemic illness contraindicates surgery.

Gallstone Ileus

- Gallstone ileus is a small bowel obstruction caused by a gallstone, an uncommon complication resulting from a gallstone eroding through the GB into the adjacent bowel (usually duodenum). The stone migrates until it lodges in the narrowest portion of the small bowel, just proximal to the ileo-cecal valve. - Patients present with symptoms of bowel obstruction and ascending cholangitis from the cholecysto-enteric fistula. - Treatment is exploratory laparotomy and removal of the obstructing gallstone by milking it back to an enterotomy made in healthy intestine. The entire bowel should be searched diligently for other stones, and cholecystectomy with closure of the fistula should be performed if the patient is stable and the inflammation is not too severe.

111

Laparoscopic Cholecystectomy (LC)

Indications - Laparoscopic cholecystectomy is the treatment of choice for all cases of symptomatic gallstone disease. - In case of asymptomatic gallstones, LC is indicated in the following conditions: 1. Calcified GB 2. Gallstone > 3cm in diameter 3. Sickle cell disease 4. Diabetic patients (relative indication) 5. Young patients (relative indication) - The complication rate of LC is low and is available for 95% of patients, with excellent recovery and return-to-work times.

Contraindications - Concomitant diseases that prevent use of a general anesthetic. - Patient's refusal of open cholecystectomy should urgent conversion be required.

Technique - Because mis-identification of the cystic duct is the commonest cause of biliary injury, the surgeon must use a technique to provide conclusive identification of the cystic duct and artery in Calot‘s triangle and hepato-cystic triangle is displayed in Figure 2.

Figure 2: Hepatocystic triangle and triangle of Calot. Hepatocystic triangle (blue): Upper boundary of hepatocystic triangle is the inferior border of liver. Laterally, the cystic duct and neck of the gallbladder, medially, the common hepatic duct. Triangle of Calot (yellow): Upper boundary is the cystic artery. Lateral the cystic duct. Medial the CBD

112

- In the critical view of safety technique, the triangle of Calot is dissected free of fat, fibrous, and areolar tissue. Importantly, the lower end of the GB must be dissected off the liver bed. A complete dissection demonstrates only two structures (i.e. the cystic duct and cystic artery) entering the GB, constituting the critical view of safety. To assist with anatomical definition, IOC may be used, especially whenever the critical view is not achieved.

Complications - LC appears to be associated with a higher incidence (2.5/1,000) of major bile duct injury than open cholecystectomy. In addition, there are also risks of injury of other structures, including the hepatic artery and the bowel. - Spilled and retained gallstones can be the source of infrequent, but serious long-term complications such as abscess and fistula formation. - Factors associated with an increased rate of conversion to an open procedure include emergent cholecystectomy, male sex, age >60 years, obesity, severe GB inflammation, choledocholithiasis, and prior upper abdominal surgery.

Open Cholecystectomy

- Open cholecystectomy is performed in patients (1) who have contraindications to LC, (2) who require conversion from LC because of inability to complete the laparoscopic procedure, or (3) who are undergoing laparotomy for another operation (e.g. pancreatico-duodenectomy). - Conversion to an open operation in the face of a difficult laparoscopic procedure should never be viewed as a surgical failure or complication, but rather as a way to avoid potential injury to the patient. - A partial cholecystectomy is advocated when the ductal and vascular structures in the triangle of Calot cannot be safely identified in the setting of severe acute inflammation. In this situation, the fundus is opened and stones are extracted. The anterior wall of the GB is excised, leaving the posterior wall with the liver bed. The mucosa is then fulgurated, and a drain is left near the infundibulum.

113

Summary

Diagnosis of the different manifestations of gallstones is summarized in Table 1.

Table 1: Diagnosis of the different manifestations of gallstones

BENIGN STRICTURES AND BILE DUCT INJURIES

Etiology

- Benign strictures occur in association with a number of conditions, including (1) chronic pancreatitis, (2) choledocholithiasis, (3) primary sclerosing cholangitis (PSC), (4) prior 114

hepatic transplantation, (5) trauma, or (4) iatrogenic injury after instrumentation or surgery. LC is the leading cause of iatrogenic bile duct injuries and subsequent benign strictures. - Biliary malignancy may masquerade as a benign stricture. Attempts to differentiate between the two should be made prior to surgery because the patient may not be a candidate for a curative resection if advanced cancer is found.

Bismuth Classification

Bismuth classification for biliary strictures is based on transections or occlusions at various levels of the CBD as shown in Figure 3.

Figure 3: Bismuth classification of biliary strictures (Types I-V)

Clinical Presentation

- Clinical Presentation depends on the type of injury. Approximately 25% of major bile duct injuries are recognized at the time of the initial procedure. Intra-operative signs of a major ductal injury include unexpected bile leakage, abnormal IOC, and delayed recognition of the anatomy after transection of important structures. - If an injury is not recognized intra-operatively, the patient usually presents with symptoms within 1 week and within 3-4 weeks after the initial procedure. - Patients with a bile leak often present with right upper quadrant pain, fever, and sepsis secondary to biloma and may have bile drainage from a surgical incision. - Patients with occlusion of the CBD without a bile leak present with jaundice. Occasionally, a delayed presentation of months or years is seen, especially in case of ischemic biliary stricture.

Diagnosis

- Ultrasound (US) abdomen is usually the initial imaging, which can detect collections and /or biliary dilatation. - Axial imaging with CT scan or magnetic resonance imaging (MRI) is useful for detecting abdominal bile collections that require percutaneous drainage.

115

- MRCP with angiography is now often the initial imaging test of choice because of its ability to define the biliary anatomy and any associated vascular injuries. - On-going bile leaks can also be diagnosed by HIDA scan. - ERCP is for diagnostic and therapeutic purposes, such as biliary stent deployment. - In the case of occlusion of the CBD, PTC can demonstrate the biliary anatomy distal to the occlusion and be used for decompression of the biliary tree.

Management

Management depends on the clinical presentation. - If the injury is identified at the time of the initial procedure, the surgeon should proceed directly to open exploration and repair, only if trained with complex techniques in hepato-biliary surgery, or to control life-threatening hemorrhage. Otherwise, the patient should be resuscitated, a drain should be placed in the right upper quadrant, and the patient immediately referred to a hepato-biliary specialist. - Some simpler injuries can be successfully managed with ERCP and sphincterotomy and stenting. Occlusive lesions, usually with clip occlusion, require decompression of the proximal system via PTC. - Ideally, in delayed diagnosis, temporization for at least 8 weeks can allow the acute inflammation to resolve. Control of sepsis, percutaneous drainage, and adequate nutrition should be optimized before definitive repair. In addition, if there is a concern about a concomitant vascular injury, definitive repair should be delayed to identify areas of ductal ischemia more easily, which should not be incorporated in the repair. - Operative repair is best achieved by means of a Roux-en-Y hepatico-jejunostomy after debridement of the bile duct to viable tissue. All bile ducts must be accounted for, and an adequate blood supply must be apparent for each. A tension-free mucosa-to-mucosa anastomosis constructed with fine absorbable suture is desired. Excellent long-term outcomes have been described with anastomotic stricture is the most common, yet infrequent, complication.

BENIGN STRICTURES AND BILE DUCT INJURIES

Clinical Presentation

- Acute cholangitis is a potentially life-threatening bacterial infection of the biliary tree typically associated with obstruction of the ductal system. - Although acute cholangitis is often associated with choledocho-lithiasis, other causes include benign and malignant strictures of the bile ducts or at biliary-enteric anastomoses, parasites, and indwelling tubes or stents, but almost always this occurs with incomplete biliary decompression (e.g. stent occlusion). - ERCP without concomitant stenting in the presence of a stricture may lead to cholangitis above the stricture. Therefore, patients should routinely be pre-treated with antibiotics in case a stent cannot be placed.

116

Diagnosis

- Patients present with a spectrum of disease severity, ranging from subclinical illness to acute toxic cholangitis. - Fever is present in >90% of patients. Charcot triad (fever, jaundice, and right upper quadrant pain) is present in only 50-70% of patients, and Reynold‘s pentad (Charcot triad with hemodynamic instability and mental status changes) in <10% of patients, mostly in the elderly and those with a septic course. - Laboratory data may demonstrate leukocytosis and abnormalities in LFTs. - Ultrasonography or CT scan can reveal gallstones and biliary dilatation, but definitive diagnosis is made by ERCP or PTC. These studies are both diagnostic and therapeutic because they demonstrate the level of obstruction and allow culture of bile, removal of stones or indwelling foreign bodies, and placement of drainage catheters if necessary.

Treatment

- Initial management of cholangitis includes IV antibiotics appropriate for the coverage of the most common Gram-negative aerobic and anaerobic organisms. - In patients with acute toxic cholangitis or in patients who fail to respond to antibiotic therapy, emergent decompression of the biliary tree via ERCP or PTC is required. - Cholangitis in patients with indwelling tubes or stents generally requires stent removal and replacement. - Definitive operative therapy for benign or malignant biliary tract strictures should be deferred until a later date.

GALL BLADDER CANCER

Pathology

- Histologically, nearly all gallbladder cancers are adenocarcinomas, and concomitant cholecystitis is frequently present. - Tumors spread primarily by direct extension into liver segments IV and V but also via lymphatics along the cystic duct to the CBD.

Presentation

- Gall bladder cancer is the most common malignancy of the biliary tract. It is more aggressive than cholangio-carcinoma and has a poor prognosis with median survival of 5-8 months. - Because of its generally advanced stage at presentation, only a small percentage of patients with a pre-operative diagnosis of GB cancer are resectable for potential cure. - Polyps 1.5 cm or greater in diameter have a 46-70% prevalence of cancer, whereas in those smaller than 1 cm, the risk of malignancy is <5%. - Malignant polyps also tend to be sessile in nature and echogenic on ultrasound.

117

- Prophylactic cholecystectomy should be considered for polyps >1 cm in size or meeting morphologic criteria. - Other risk factors include porcelain gallbladder, PSC, and anomalous junction of the pancreato-biliary duct.

Diagnosis

- Approximately one-third of these tumors are diagnosed incidentally during cholecystectomy, found in 0.3% to 1% of all cholecystectomy specimens. - Symptoms of stage I and II GB cancer are often directly caused by gallstones rather than the cancer, whereas stage III and IV cancers present with weight loss and symptoms typical of CBD obstruction. - Suggestive ultrasound findings include thickening or irregularity of the GB, a polypoid mass, or diffuse wall calcification indicative of porcelain GB.

Treatment

- Mucosal disease confined to the GB wall (Tis and T1a tumors) is often identified after routine LC. Because the overall 5-year survival rate is as high as 80%, cholecystectomy alone with negative resection margins, including the cystic duct margin, is adequate therapy. - Patients with a pre-operative suspicion of GB cancer should undergo open cholecystectomy because port site recurrences and late peritoneal metastases (associated with bile spillage) have been reported even with in situ disease. - T2 tumors have invaded the muscularis and may be treated by radical cholecystectomy that includes the GB, the GB bed of the liver, and the hepato-duodenal ligament, para- duodenal, peri-pancreatic, hepatic artery, and celiac lymph nodes (LNs). Presence of LN metastases or extension of disease beyond the GB wall into local organs (T3Ñ T4 disease) requires more radical resection. Depending on the extent of local invasion, extirpation may range from wedge resection of the liver adjacent to the GB bed to resection of 75% of the liver. - Improvement in survival has been demonstrated after radical resection. - Because of the aggressive nature of this malignancy, adjuvant chemo-radiation is often recommended, but little proof of efficacy is available. Most GB cancers have invaded adjacent organs, extended into the porta hepatis, or distantly metastasized before clinical diagnosis. Extensive liver involvement or dis-contiguous metastases preclude surgical resection. Jaundice may be palliated by percutaneous or endoscopically placed biliary stents. Duodenal obstruction can be surgically bypassed if present.

118

CHAPTER 3 Acute Pancreatitis

DEFINITION AND EPIDEMIOLOGY

- Acute pancreatitis describes an acute inflammatory process of the pancreas that can range from mild interstitial pancreatitis to severe pancreatitis with pancreatic necrosis and concomitant multi-organ failure. Acute pancreatitis is typically rapid in onset and most commonly encountered in its mild form. - The increased frequency of acute pancreatitis may be due to rising incidence of obesity, a risk factor for the development of gallstones and, by extension, gallstone pancreatitis. - Acute pancreatitis confers a heavy financial burden on the health care system and significant physiological stress on the patient. The average length of hospital stay for a patient with acute pancreatitis is approximately 5-6 days. In addition, acute pancreatitis may be accompanied by life-threatening complications as well as significant morbidity and mortality.

ETIOLOGY

- The two most common causes of acute pancreatitis are: 1. Cholelithiasis / choledocholithiasis 2. Alcohol consumption (deﬁnitions vary as does duration with consumption between 50-80 g or 4–7 drinks/day) with frequency estimates of 40% and 30%, respectively. - Other etiologies are hyper-triglyceridemia (>1000 mg/dL), medications, trauma, infections, iatrogenic (surgical or post-ERCP), genes, anatomy (pancreatic divisum, and sphincter of Oddi dysfunction, which remain controversial), as well as, autoimmunity.

Gallstones and Biliary Pancreatitis

- Biliary pancreatitis, synonymous with gallstone pancreatitis, is a form of acute pancreatitis caused by the passage of gallstones through the cystic duct and into the distal common bile duct (CBD) where they can obstruct the biliary and pancreatic ducts. Pancreatic ductal obstruction is felt to be the inciting event in gallstone pancreatitis. - Incidence is highest in patients with small gallstones, or microlithiasis, as these stones are more likely to escape the gallbladder (GB) and transit the cystic duct to reach the CBD. Large stones are more likely to be retained in the GB. Gallstone pancreatitis typically presents and tended to have a more benign clinical course with infrequent ICU admissions.

119

- Obesity is a risk factor for the development of gallstone pancreatitis and may increase the risk of development of severe pancreatitis, including pancreatic necrosis. Obesity also increases the risk of developing local complications such as pancreatic fluid collections. However, obesity is not associated with increased mortality in acute pancreatitis. - Other risk factors associated with the development of gallstone acute pancreatitis include pregnancy, elevated alanine aminotransferase (ALT), advancing age, weight gain, female gender, and rapid weight loss. Unsaturated fats, coffee, and moderate alcohol consumption appear to reduce the risk of developing gallstones and thus may reduce the risk for gallstone acute pancreatitis.

Alcoholic Pancreatitis

- Alcohol is a well-known precipitant of acute pancreatitis, although the incidence of acute pancreatitis in heavy alcohol consumers is not more than 2-3% per year, suggesting that there are as yet undetermined environmental or genetic factors that influence the development of acute pancreatitis in this population. - Currently, 17.6 million Americans have an alcohol use disorder, and some data suggest that the incidence of alcoholic acute pancreatitis is on the rise. Furthermore, alcoholic acute pancreatitis has the highest associated risk of overall mortality, with the odds of death increased 90% as compared with biliary pancreatitis, possibly due to poor baseline nutrition.

Iatrogenic Pancreatitis

- Iatrogenic pancreatitis most commonly occurs following endoscopic retrograde cholangio-pancreatography (ERCP). Post-ERCP pancreatitis has an incidence of approximately 3.5%. - Young age, biliary sphincter balloon dilatation in intact papilla, pancreatic duct contrast injection, normal bilirubin, precut sphincterotomy or pancreatic sphincterotomy, and suspected sphincter of Oddi dysfunction are risk factors for post-ERCP pancreatitis. - Pancreatitis may also occur following abdominal surgery, cardiac surgery, liver biopsy, and abdominal procedures performed by interventional radiologists. It can also be caused by retained intra-abdominal foreign bodies as well as iatrogenic hypercalcemia due to total parental nutrition, among other causes.

Drug-induced Pancreatitis

- Medications thought to induce acute pancreatitis have been classified on the level of evidence to support the association. - Class I medications are defined as those where recurrence of acute pancreatitis was confirmed upon re-challenging. Class I is further subdivided into 1a (other causes of pancreatitis ruled out) and 1b (alternative etiologies not ruled out). - Class II medications do not meet strict criteria for class 1 but exhibit a consistent latency period in a preponderance of reported cases. 111

- Class III and IV medications refer to those in which two or one published case report of medication- induced pancreatitis has been reported, respectively. - Medications implicated in the development of drug-induced pancreatitis are summarized in Table 1.

Table 1: Drugs (medications) implicated in development of pancreatitis

Class Drug(s) - Antimicrobials - Tetracycline, sulfonamide, pentamidine, didanosine, - Anti-convulsants metronidazole - Diuretics - Valproic acid - Immunosuppressants - Furosemide, thiazides - Non-steroidal anti-inflammatory - Azathioprine, 6-mercaptopurine drugs - Sulindac, salicylates - Anti-proliferative drugs - Tamoxifen, L-asparaginase - Others - Estrogen

Hyper-triglyceridemia and Hyper-calcemia

- Elevated triglyceride serum levels, typically exceeding 500 mg/dL, can be seen in various conditions, including poorly controlled type-2 diabetes mellitus (DM), obesity, alcoholism, third trimester pregnancy, renal disease, hypothyroidism, and familial hypertriglyceridemia. - Patients should have fasting triglyceride levels checked after their pancreatitis has resolved before diagnosing hyper-triglyceridemia, as serum triglycerides can be artificially elevated during an episode of acute pancreatitis. - Hypercalcemia is also a recognized etiology of acute pancreatitis. Hypercalcemia can be associated with a malignancy (often in the setting of bony metastases or multiple myeloma), total parenteral nutrition, sarcoidosis, vitamin D toxicity, and infusions of peri-operative high-dose calcium during cardio-pulmonary bypass. If muscular/myopathic, urological, or nervous system symptoms co-exist with acute pancreatitis, patients should be evaluated for hyperparathyroidism (HPT).

Autoimmune Pancreatitis

- It is a predominantly lymphocytic inflammatory process that results in eventual organ fibrosis and dysfunction. - While many diagnostic criteria have been proposed, the modified Japan Pancreas Society Criteria require a combination of typical imaging (CT, MRCP, or ERCP) and either serology (IgG4 or IgG totals, etc.) or pancreatico-biliary / extra-intestinal findings (sialadenitis, nephritis, or IgG4 pneumonitis) for diagnosis.

111

Infectious Causes

- Infectious causes of acute pancreatitis are rare and have mostly been described in case reports. - Parasitic: the most common are Toxoplasma, Cryptosporidium, and Ascaris. - Viral: examples include mumps, Coxsackie virus, hepatitis B, cytomegalovirus (CMV), and varicella-zoster virus. - Bacterial: examples include Mycoplasma, Legionella, Leptospira, and Salmonella. - Fungal: Aspergillus is the only fungus that has been shown to cause acute pancreatitis.

Inherited Forms

- Mutations, up-regulation, and genetic variants in several genes have been implicated in acute pancreatitis, namely, the trypsinogen gene (PRSS1) and trypsin inhibitor (SPINK1), cystic ﬁbrosis trans-membrane regulator (CFTR) variants, and endothelial ion/water channel CLDN2 risk allele.

Other Causes

- Neoplasms: Rarely, tumors such as ampullary cancer or intra-ductal papillary mucinous neoplasm of the pancreas can cause acute pancreatitis. - Congenital malformations, including pancreas divisum, annular pancreas, and anomalous pancreatico-biliary union, among others, have also been implicated.

Idiopathic Acute Pancreatitis

- Between 10% and 30% of cases of acute pancreatitis may be idiopathic in nature. - It may be explained by undetected micro-lithiasis, unrecognized drug-induced pancreatitis, or sphincter of Oddi dysfunction.

PATHOPHYSIOLOGY

- The inciting events in acute pancreatitis begin in pancreatic acinar cells after a primary injury promotes pancreatic enzyme activation (primarily trypsin, although other proteases such as elastase and chymotrypsin may be involved), with subsequent enzymatic ―spilling‖ (Figure 1) - The enzymes diffuse into the interstitial and endothelial spaces and begin auto-digestion of the gland. Tissue breakdown products potentiate vascular injury, with local recruitment of cytokine and Arachidonic acid metabolite-secreting leukocytes. These agents produce edema and oxidative stress. - The increase in vascular permeability promotes thrombosis and hemorrhage and can lead to pancreatic ischemia and necrosis. Increased vascular permeability can also lead to bacterial translocation into the pancreatic bed and result in infected pancreatic necrosis, a life-threatening complication of acute pancreatitis. - In severe cases, systemic inflammatory response syndrome (SIRS), renal failure, shock, myocardial stress, fever, or acute respiratory distress syndrome (ARDS) may develop. 112

Figure 1: Pathophysiology of acute pancreatitis

- Alcoholic acute pancreatitis may have a slightly different pathogenesis. Alcohol potently stimulates the release of secretin and cholecystokinin, which are the major contributors to pancreatic secretion. Also, the rising ethanol concentration in acinar cells causes an increase in cytosolic calcium, which is required for vesicular zymogen activation. This relationship between cytosolic calcium and zymogen activation may also help to explain the association between hypercalcemia and acute pancreatitis.

DIAGNOSIS

Clinical Presentation

- The classic presentation of acute pancreatitis includes mild to severe epigastric abdominal pain (often radiating to the back) as well as nausea and vomiting. The pain is typically constant in nature and is not aggravated by coughing, movement, or respiration. Pain tends to be more severe in a supine position and may lessen if the patient leans forward in a sitting position. - Patients may be pale, distressed, have distention, jaundice, tachycardia and fever. - Turner‘s sign (flank bruising) or Cullen‘s sign (bruising surrounding the umbilicus) may be present in severe cases. - Some patients may have a more florid presentation that includes hypotension or shock due to intra-vascular volume depletion and third-spacing of fluids.

113

- Other clinical findings that can be present in acute pancreatitis include dehydration and decreased urine output. - Findings that may be seen in more severe presentations include hypotension despite volume replacement and a corresponding rise in hematocrit secondary to hemo- concentration, metabolic acidosis, ARDS/respiratory failure, renal failure, and fluctuation in serum calcium levels.

Diagnostic Criteria

- The Revised Atlanta Criteria of 2012 (updated from 1992) requires two of three conditions be met to diagnose acute pancreatitis: 1. Abdominal pain consistent with acute pancreatitis (i.e. epigastric abdominal pain with possible radiation to the back). 2. Lipase or amylase ≥ 3 times the upper limit of normal, and/or 3. Characteristic imaging features of acute pancreatitis by CT, MRI, or US.

Biochemical Diagnostic Parameters

- Elevation of serum amylase and lipase levels to >3 times the upper limit of normal in conjunction with the appropriate clinical history are the mainstays in the diagnosis of acute pancreatitis. - In general, amylase and lipase levels do not correlate with either the severity of the attack or with overall prognosis. In addition, serum amylase and lipase levels neither assist in generating an overall prognosis nor in predicting complications of acute pancreatitis. - A fall in enzymes accompanied by clinical improvement is often an adequate indication that the pancreatitis is resolving in most patients. Persistent elevation of serum amylase and lipase levels may suggest pancreatic ductal disruption and/or necrosis.

Imaging

- Plain X-ray The presence of calcifications may suggest chronic pancreatitis. Signs that may be seen on radiograph in acute pancreatitis include a ―sentinel loop,‖ which represents a dilated segment of small intestine or colon displaying ileus, and ―colon cut-off sign,‖ which represents a functional spasm in the descending colon resulting in a termination of air in the distal colon near the splenic flexure. - Ultrasonography (US) A hyper-echoic, diffusely enlarged pancreas is often seen on trans-abdominal US in acute pancreatitis. Ultrasonography is also a useful and economic choice for evaluating patients with suspected gallstone pancreatitis. This study can identify stones in the GB, evidence of acute cholecystitis (GB wall thickening or peri-cholecystic fluid), and CBD dilatation (often suggestive of an obstructing CBD stone) and in some cases can directly visualize choledocho-lithiasis. Bowel gas may obscure the pancreas on US imaging. Ultra- sonography does not assist in diagnosing the extent of pancreatic necrosis or inflammation. 114

- CT scan Specific Imaging of the pancreas is recommended only in patients whom the diagnosis is unclear, for those who fail to improve within the ﬁrst 48–72 hours, or to assess for complications. CT scans with intravenous (IV) contrast are helpful in patients with known or suspected moderate to severe pancreatitis. With this study, the entire pancreas can be well visualized and complications of pancreatitis such as fluid collections, pseudocysts (Figure 2A), and/or areas of necrosis (Figure 2B) can be rapidly identified. There is an excellent correlation between contrast-enhanced CT results and the development of early and late necrosis. The degree of necrosis is also an excellent prognostic factor. However, small areas of necrosis can be missed via contrast CT.

Figure 2. A: Computed tomography (CT) scan obtained from a patient with acute pancreatitis showing multiple well- demarcated pseudo-cysts.

A Figure 2B: Computed tomography (CT) scan showing severe necrotizing pancreatitis (arrows).

- Magnetic resonance imaging (MRI) Abdominal MRI is another commonly utilized imaging modality in patients with acute pancreatitis. Studies performed with MRI use gadolinium for contrast, which carries a lower risk of side effects or renal injury than contrast used with CT scans. MRI is also highly effective at identifying fluid collections and pancreatic necrosis. It has a greater sensitivity for detecting mild acute pancreatitis as compared with CT scan. MRI may be preferred over CT scan if biliary pancreatitis is suspected as magnetic resonance cholangio-pancreatography (MRCP) can be performed. - Endoscopic ultrasound (EUS) Endoscopic ultrasound can be used to evaluate the CBD for the presence of stones that may require removal via ERCP. If stones are seen via EUS, ERCP can typically be performed at the same time

115

PROGNOSTIC FACTORS

- Several scoring systems to assess the severity of acute pancreatitis and prognosis of patients (e.g. Ranson‘s criteria, Glasgow and Acute Physiology and Chronic Health Evaluation II [APACHE-II]) have been developed. - In 1976, Ranson reported the use of a series of 11 objective findings that correlate with severity in patients with acute pancreatitis (Table 2).

Table 2: Ranson‘s Criteria for Scoring of Acute Pancreatitis

Criteria for Acute Pancreatitis not due to Gallstones

At Admission During the Initial 48 hours - Age >55 y - Hematocrit fall > 10 points - WBCs >16,000/mm3 - BUN elevation >5mg/dL - Blood glucose >200 mg/DL - Serum calcium <8mg/dL

- Serum LDH >350 IU/L - Arterial PO2 <60 mmHg - Serum AST >250 IU/dL - Basic deficit >4 mEq/L - Estimated fluid sequestration >6L

Criteria for Acute Gallstone Pancreatitis

At Admission During the Initial 48 hours - Age >70 y - Hematocrit fall >10 points - WBCs >18,000/mm3 - BUN elevation >2 mg/dL - Blood glucose >220 mg/DL - Serum calcium <8 mg/dL - Serum LDH >400 IU/L - Basic deficit >5 mEq/L - Serum AST >250 IU/dL - Estimated fluid sequestration >4L

- These scoring systems may be helpful early in the clinical course of acute pancreatitis, although their usefulness is diminished as the disease progresses. - Poor prognostic factors: These include: 1. Renal failure 2. Respiratory failure 3. Multi-organ system failure (MSOF) 4. Fluid collections 5. Necrosis 6. Increased ICU length of stay 7. Shock. - Other prognostic factors include CT severity index (CTSI) score described by Balthazar et al (Table 3).

116

Table 3: CT Severity Index (CTSI) Score

TREATMENT

- Most patients with mild acute pancreatitis recover with supportive measures. Patients should have nothing by mouth status for at least 24-48 hours. In the absence of cardiopulmonary complications, vigorous hydration with IV fluids should be administered until adequate urine output (UOP) is achieved and maintained. Pain management, typically with narcotics, should be implemented as well. Some data suggest the superiority of a patient-controlled analgesia pump with the agent Meperidine instead of morphine as it might increase sphincter of Oddi pressure. If the underlying cause of the episode of acute pancreatitis is amenable to correction (e.g. choledocho-lithiasis), therapeutic interventions such as ERCP with biliary sphincterotomy and duct clearance and/or cholecystectomy may be indicated. - Other important etiologies that may be correctable are alcohol use/abuse, hypercalcemia, hyper-triglyceridemia, and drug-induced pancreatitis. - There is little to be gained from daily monitoring of serum amylase and lipase levels. Patients can be gradually returned to oral intake as abdominal pain recedes and hunger returns. Over the course of mild acute pancreatitis, most laboratory abnormalities should show improvement and resolution without further intervention within 3-7 days. - Patients with more severe acute pancreatitis, manifested as the development of peri- pancreatic fluid collection, pancreatic pseudo-cysts, pancreatic necrosis, and/or the development of respiratory, renal, or circulatory compromise, require more aggressive management, and ICU admission is often warranted. - In general, a multi-disciplinary approach with both medical and surgical teams is generally beneficial. Renal failure may warrant hemodialysis, and patients with respiratory failure may require mechanical ventilation.

117

- As with mild pancreatitis, nothing by mouth status is recommended in severe acute pancreatitis for at least the first 48 hours after diagnosis. If pancreatic necrosis is seen, enteral feeding is often performed to reduce the risk of bacterial translocation from the gut to the necrotic pancreatic bed, improve intestinal wall integrity, and promote gut motility. - Parenteral nutrition was once recommended in patients with severe pancreatitis, but it has been associated with increased length of stay, costs, and complication and mortality rates as well as increased systemic and local infections as compared with enteral nutrition. Aggressive pain management and intravenous fluid replacement are recommended. Vital signs and urine output should be monitored every few hours for the first 24 to 48 hours. - The use of prophylactic antibiotics in patients with pancreatic necrosis (in an attempt to avoid infection) remains controversial. Some studies have demonstrated benefit in this regard, while others have not shown an advantage. Imipenem, Meropenem, and Fluoro- quinolones are commonly used in this setting as these agents have a high degree of pancreatic penetrance. Although antibiotic prophylaxis cannot be universally recommended in the setting of acute pancreatitis, 4 situations in which administering antibiotics may be appropriate are: systemic inflammatory response syndrome or sepsis, MSOF, proven extra-pancreatic or pancreatic infections, or an increase in C-reactive protein (CRP) with evidence of pancreatic or extra-pancreatic infection. - Patients who develop pancreatic necrosis must be closely monitored for the development of infected pancreatic necrosis. Infected pancreatic necrosis is most commonly treated with surgical debridement, although endoscopic debridement is possible, and percutaneous drainage of pus can be a consideration as well. The timing of surgical intervention varies; surgical therapy may be required urgently if patients are septic. Delaying surgery, if possible, may allow necrotic pancreatic tissue to be well demarcated at the time of surgery. Infected pancreatic necrosis, pancreatic abscesses, and infected pseudocysts are the most common indicators for surgery in the acute phase of the illness, with more minimally invasive techniques favored if possible, although open abdominal procedures may be required. - Different types of peri-pancreatic fluid collections resulting from an attack of acute pancreatitis are illustrated in Table 4. Most acute fluid collections and/or pseudo-cysts do not require interventions unless they become infected or cause significant extrinsic compression of other organs. In this case drainage is indicated and it is initially attempted via endoscopic/ultrasound-guided maneuvers. Failure to improve after these measures may warrant surgical drainage.

118

Table 4: Different types of peri-pancreatic fluid collections resulting from an attack of acute pancreatitis

119

- General scheme for treatment of acute pancreatitis is outlined in Figure 3.

Figure 3: General scheme for treatment of acute pancreatitis

121

CHAPTER 4 Pancreatic Tumors

CLASSIFICATION OF PANCREATIC TUMORS

According to Cell of Origin

Pancreatic tumors are classified according to the cell of origin into exocrine tumors and endocrine tumors (Table 1). - Exocrine tumors: originating from cells in the exocrine pancreas (ductal and acinar) - Endocrine tumors: originating from endocrinal / hormone-producing cells i.e. A cells, B cells, D cells and D1 cells - Benign tumors of the pancreas are extremely rare

Table 1: Types (Classification) of Pancreatic Neoplasms

Pancreatic Neoplasm Frequency

1. Pancreatic exocrine neoplasms 90% - Invasive ductal adenocarcinoma 85% - Intra-ductal papillary mucinous neoplasms (IPMN) 3-5% - Serous cystic neoplasms 1-2% - Mucinous cystic neoplasms 1-2% - Solid pseudo-papillary neoplasm 1-2% 2. Pancreatic endocrine neoplasm (PEN) 3-4% 3. Miscellaneous epithelial neoplasms 1-2% - Teratoma - Lymphoepithelial cyst

121

PANCREATIC EXOCRINE NEOPLASMS

Pancreatic Ductal Adenocarcinoma (PDAC)

Incidence - It is the most common type of pancreatic tumors (85-90%)

Risk Factors 1. Cigarette smoking 2. Heavy alcohol consumption 3. Heavy consumption of red or processed meat 4. Chronic pancreatitis 5. Obesity 6. Diabetes Mellites (DM) - New onset NIDDM in elderly patients: Older patients >50 years of age with new- onset DM have about an 8-fold elevated risk of having pancreatic cancer compared with the general population - Long-term DM (2-8 years duration)

Pre-malignant Pancreatic Lesions 1. Mucinous cystic neoplasms (MCN) 2. Intra-ductal papillary mucinous neoplasms (IPMN)

Genetic Predisposition - Familial syndromes associated with increased risk of PDAC - Approximately, 10% of PDAC occur in conjunction with a defined genetic syndrome; these include the following: a) Peutz-Jeghers syndrome. b) Lynch syndrome. c) Familial pancreatitis. d) Familial pancreatic cancer. e) Melanoma-pancreatic cancer syndrome. f) Hereditary breast-ovarian cancer syndrome.

The Peri-ampullary Region - Peri-ampullary region is ―the region located within 2 cm around the ampulla of Vater. - Peri-ampullary carcinoma is a term to define carcinomas arising from the head of the pancreas, the ampulla of Vater, the distal common bile duct (CBD) and the duodenum adjacent to the ampulla (Table 2). - Although, the mode of presentation and treatment options for peri-ampullary tumors are similar, their prognosis are quite different with that for adenocarcinoma of the pancreas being much worse than for the other tumors. - Most patients with peri-ampullary cancers will present with biliary obstruction due to the location of the tumor. Often, this obstruction will lead to symptoms of obstructive jaundice i.e. dark color urine, clay color stools, and pruritis.

122

Table 2: Types of Peri-ampullary Carcinoma

Type of Carcinoma Frequency

1. Adenocarcinoma of head of pancreas 50 % 2. Tumor from the ampulla of Vater 30 % 3. Distal common bile duct carcinoma 10 % 4. Duodenal carcinoma adjacent to ampulla 10 %

Clinical Presentation - Early pancreatic cancer symptoms are generally non-specific, which contributes to its delayed diagnosis (diagnosis is often delayed until disease is at an advanced stage) - Only 15- 20% of patients present with resectable disease - Most lesions are non-resectable at presentation; the disease is locally advanced in about 25-30% of patients and metastatic in about 50- 60% of patients - Clinical presentation depends on tumor location and size (Table 3). - Location: 1. Head: 65% 2. Body and tail: 30% 3. Diffuse pancreatic involvement: 5% - Early pancreatic cancer may present with: 1. Non-specific GIT symptoms such as nausea and vomiting 2. Mild epigastric pain radiating to the middle of the back 3. Anorexia and unexplained weight loss. - Enlarging tumors in the head of the pancreas usually present with obstructive jaundice, dark urine, clay stools and pruritis. - Physical Examination 1. Jaundice 2. Epigastric fullness 3. Courvoisier gallbladder (palpable GB) 4. Scratch marks from pruritus 5. Migratory thrombophlebitis (Trousseau sign) 6. Late cases: o Epigastric mass o Ascites o Troisier sign (palpable enlarged left supraclavicular lymph node, Virchow node)

Table 3: Differences in presentation between pancreatic head versus body and tail lesions

Clinical Presentation Pancreatic Head Pancreatic Body and Tail 1) Jaundice Yes No 2) Size at discovery Usually smaller Usually larger 3) Metastasis at discovery Less frequent More frequent

123

Laboratory Investigations - Routine lab tests - Dedicated lab tests 1. Bilirubin (total and direct) 2. Markers of cholestasis: alkaline phosphatase (AP), Gamma glutamyl transferase (GGT) 3. Tumor markers a) Carboxylated Antigen 19-9 (CA19-9): - It is not tumor-specific i.e. is commonly elevated in pancreatic and hepato- biliary diseases and in many other malignancies. - It is high in cases of biliary obstruction regardless of etiology and does not necessarily indicate cancer in jaundiced patients. Pre-operative measurement of CA19-9 is therefore best performed after biliary decompression is complete and bilirubin is normal. - Serial measurement of CA19-9 is one of the validated follow-up tools b) Carcino-Embryonic Antigen (CEA) c) CA 125

Imaging Studies: Assessment of Jaundice in Pancreatic Head Lesions 1. Abdominal Ultrasonography (US): It provides sure evidence of biliary tree obstruction namely intra-hepatic bile duct dilatation. 2. Endoscopic retrograde cholangio-pancreatography (ERCP) - Diagnostic: double duct sign or distal CBD stricture - Biopsy: Punch biopsy from peri-ampullary lesions 3. Magnetic resonance cholangio-pancreatography (MRCP)

Imaging Studies: Assessment of the Lesion 1. Multi-detector computed tomography (MDCT): pancreatic protocol - It is the gold standard for diagnosis and staging. - It allows evaluation of resectability through assessing the relation of the tumor to nearby arterial structures and venous structures; namely, the Portal vein (PV), the superior mesenteric vein (SMV), the superior mesenteric artery (SMA) and the celiac artery (CA). - It allows staging by detecting the presence or absence of liver metastases and/or lymph nodal spread outside the field of resection.

2. Magnetic Resonance Imaging (MRI): - It is most commonly used as a problem-solving tool. When contrast enhanced CT cannot be obtained (severe allergy to iodinated intravenous contrast material) - Disadvantages: Higher cost- lack of widespread availability compared to CT 3. Endoscopic Ultrasound (EUS) - EUS is not recommended as a routine staging study. - EUS may be used as a complementary to CT staging - Disadvantage: Highly operator-dependent and high cost.

124

4. Role of Biopsy - For operable pancreatic masses, biopsy proof of malignancy is not required before surgical resection and a non-diagnostic biopsy should not delay surgical resection when the clinical suspicion of pancreatic cancer is high - Pancreatic biopsy should be reserved for patients with locally unresectable or metastatic disease prior to chemotherapy. - Biopsy of a pancreatic lesion should be performed endoscopically with either ERCP or EUS-FNA. - Percutaneous biopsy of pancreatic mass is generally inadvisable owing to potential for tumor seeding along the needle track. Percutaneous US- or CT-guided pancreatic biopsy should be reserved for evaluation of suspected metastatic lesions, typically liver metastases and not for the lesion itself. 5. Positron Emission Tomography (PET) Scan - It is sensitive in detecting extra-pancreatic metastatic disease of questionable morphology. 6. Diagnostic Staging Laparoscopy: - It is used in some institutions to rule out peritoneal metastases not detected on imaging, particularly those that may occur in body and tail of pancreas.

At the end of assessment, lesions should be classified into either: resectable, border-line resectable, or unresectable (Table 4)

Table 4: Criteria Resectability and Irresectability of Pancreatic Cancer

Resectable Borderline Resectable Unresectable

No distant metastases No distant metastases Distant metastases No arterial contact Less than 180-degree SMA Greater than 180-degree SMA encasement. encasement No venous contact Reconstructable Non-reconstructable SMV/portal vein contact SMV/portal vein contact

Treatment 1. Resectable Tumors A. For tumors in the head of the pancreas: Surgical resection with the intent of cure in the form of pancreatico-duodenectomy (Whipple procedure) in which the followings are resected: - pancreatic head and neck - distal 40% of the stomach - whole duodenum - proximal 10% of the jejunum - lower part of the common bile duct (CBD) along with the gallbladder (GB)

125

Then the continuity of the gastrointestinal tract (GIT) is restored via - Pancreatico-jejunostomy - Hepatico-jejunostomy - Gastro-jejunostomy B. For tumors in the body and tail of the pancreas: - Surgical resection in the form of distal pancreatectomy and splenectomy 2. Border Line Resectable Tumors - Neoadjuvant chemotherapy is given pre-operatively to potentially permit cases with borderline resectability to achieve a partial response with therapy in the hope of improving chance for a complete, margin-free (R0) resection. - This is followed by reassessment by MDCT, if it becomes resectable, trial resection is attempted. This decision is made under the direction of multidisciplinary surgeon and oncologist consultations 3. Unresectable Tumors: Palliative measures for a) Biliary obstruction: - Endoscopic stenting - Percutaneous trans-hepatic biliary drainage - Surgical bilio-enteric bypass i.e. hepatico- or cholecysto-jejunostomy b) Gastric outlet obstruction: - Surgical gastro-jejunostomy - Endoscopic stenting c) Severe tumor-associated pain: - Narcotics administration - Celiac plexus neurolysis

PANCREATIC ENDOCRINE NEOPLASMS

The common pancreatic neuro-endocrine tumors (pNETs) are listed in Table 5, which summarizes the hormone causing the syndrome, signs and symptoms as well as the frequency of the tumor or syndrome. Table 6 summarizes the malignant potential of pancreatic endocrine tumors.

126

Table 5: Summary of Common Pancreatic Neuro-Endocrine Tumors (pNETs)

Name of Tumor Hormone Causing Signs or Symptoms % of All (Syndrome) the Syndrome functional pNETs

Insulinoma Insulin Hypoglycemia (weakness, 35-40 % sweating, tremors, palpitation, confusion, visual changes, etc)

Gastrinoma Gastrin Abdominal pain, 16-30 % (Zollinger-Ellison) refractory peptic ulcer disease, secretory diarrhea

Glucagonoma Glucagon Dermatitis (migratory <10 % necrolytic erythema, DM, diarrhea, DVT (4D- syndrome) VIPoma (Verner- VIP Profuse watery diarrhea, <10 % Morrison) severe dehydration, hypokalemia, achlorhydria (WDHA syndrome) Somatostatinoma Somatostatin DM, cholelithiasis, <5 % steatorrhea, anemia, steatorrhea

pNETs: Pancreatic Neuro-Endocrine Tumors: DM: diabetes mellitus, VIP: vasoactive intestinal peptide, DVT: deep vein thrombosis

Table 6: malignant potential of pancreatic neuro-endocrine tumors (pNETs)

Type of Pancreatic Neuro-Endocrine Tumors Malignant Potential

1. Functioning (hormone-producing) - Insulinoma (B cells) 10 % - Gastrinoma 60 % - Glucagonoma (A cells) 80 % - Somatostatinoma (D cells) 90 % - VIPoma 80 % - PPoma (PP cells) 80 %

2. Non-functioning Tumors 92 %

127

CHAPTER 5 Liver Cysts and Abscesses

OVERVIEW

- The precise prevalence and incidence of liver cysts are not known because most do not cause symptoms; however, liver cysts have been estimated to occur in 5% of the population. No more than 10-15% of these patients have symptoms that bring the cyst to clinical attention. - Cystic lesions of the liver include the following: 1. Simple cysts 2. Multiple cysts arising in the setting of polycystic liver disease (PCLD) 3. Parasitic or hydatid (Echinococcal) cysts 4. Cystic tumors 5. Abscesses 6. Ductal cysts (choledochal cysts and Caroli‘s disease) are differentiated from hepatic cysts by involvement of the bile ducts. - The evaluation of a patient with a simple liver cyst involves carefully recording the patient history and performing a physical examination plus an imaging study to define the anatomy of the cyst. - The imaging in patients with hepatic cysts usually includes ultrasonography (US), which is readily available, non-invasive, and highly sensitive and/or computed tomography (CT) scan, which is also highly sensitive and is easier for most clinicians to interpret, particularly for treatment planning. - Other tests are generally not necessary in the evaluation of hepatic cysts. Percutaneous aspiration should be avoided because the laboratory and cytological evaluation of the simple cyst fluid is non-diagnostic, and a small risk exists of inducing anaphylaxis from leakage from the hydatid cyst or of causing abscess formation in a previously sterile cyst. - Histological assessment of the excised cyst wall should be routinely undertaken to identify the presence of an unsuspected neoplasm, such as cystadenoma.

128

SIMPLE LIVER CYSTS

- Etiology: The cause of simple liver cysts is not known, but they are believed to be congenital in origin. - Pathology: The cysts are lined by biliary-type epithelium. Typically, the fluid within the cyst has an electrolyte composition that mimics plasma. Bile, amylase, and white blood cells (WBCs) are absent. The cyst fluid is continually secreted by the epithelial lining of the cyst. For this reason, needle aspiration of simple cysts is not curative, and recurrence is the norm. - Clinical presentation: Simple cysts generally cause no symptoms, but may produce dull right upper-quadrant pain if large in size. Patients with symptomatic simple liver cysts may also report abdominal bloating and early satiety. Occasionally, a cyst is large enough to produce a palpable abdominal mass. Jaundice caused by bile duct obstruction is rare, as are cyst rupture and acute torsion of a mobile cyst. - Investigations: Liver function test (LFT) results, such as transaminases or alkaline phosphatase, may be mildly abnormal, but bilirubin, prothrombin time (PT), and activated partial thromboplastin time (aPTT) are usually within normal range. Simple cysts have a typical radiographical appearance. They are thin-walled and uniform with a homogenous low-density interior on CT scan. - Management: Treatment of simple cysts of the liver is indicated only in symptomatic patients. Asymptomatic patients do not require therapy, because the risk of developing complications related to the lesion is lower than the risk associated with treatment. When the cysts become large and cause symptoms, such as pain, treatment is warranted. Surgical treatment of simple liver cysts involves "deroofing" the cyst by excising the portion of the wall that extends to the surface of the liver. Excision of this portion of the cyst wall at the liver surface produces a saucer-type appearance in the remaining cyst so that any fluid secreted from the remaining epithelium leaks into the peritoneal cavity where it can be absorbed. The cyst wall should be sent to pathology to confirm the diagnosis and exclude cystadenoma or cystadenocarcinoma. In simple cysts, histology of the cyst wall generally reveals a layer of simple cuboidal epithelium. Historically, treatment of symptomatic hepatic cysts required laparotomy, but today, cyst deroofing can be successfully performed laparoscopically and is currently considered the standard of care.

POLYCYSTIC LIVER DISEASE (PCLD)

- Etiology: Adult PCLD (AD-PCLD) is congenital and is usually associated with autosomal dominant polycystic kidney disease (AD-PKD). In patients with PKD, the kidney cysts usually precede the liver cysts. PKD often results in renal failure, whereas liver cysts only rarely are associated with hepatic fibrosis and liver failure. - Clinical Presentation: Polycystic liver disease (PCLD) rarely arises in childhood. These cysts are observed at the time of puberty and increase in adulthood. Women are more commonly affected, and an increase in cyst size and number is correlated with estrogen

129

level. In PCLD, hepatomegaly may be prominent, and patients occasionally progress to hepatic fibrosis, portal hypertension, and liver failure. Complications (e.g. rupture, hemorrhage, and infection) are rare. However, patients do present with abdominal pain as the cysts enlarge. - Investigations: In the setting of PCLD, greater abnormalities in LFT results are found, but liver failure is rare. Renal function test results, including blood urea nitrogen (BUN) and creatinine levels are often abnormal and should be performed on initial evaluation. Diagnosis of PCLD is confirmed by means of US or CT scan with multiple liver cysts identified. - Management: Treatment of PCLD is indicated only in symptomatic patients. Only those patients with clearly disabling pain should be considered for surgery. The surgical goal is to decompress as much of the cystic liver as possible. This can be accomplished by a combination of deroofing and fenestration or, in selected patients, by resection of the involved portion of the liver. Recurrence of symptoms with either procedure is high as new cysts replace those that have been resected. Small numbers of patients have been treated with liver transplantation.

NEOPLASTIC CYSTS

- Etiology: Liver tumors with central necrosis visualized on imaging studies are often misdiagnosed as liver cysts. True intra-hepatic neoplastic cysts are rare. The cause of cyst-adenomas and cyst-adenocarcinomas is unknown. - Pathology: These cystic tumors are lined with biliary-type cuboidal or columnar cells and are surrounded by ovarian-like stroma. Cystadenoma is a pre-malignant lesion with neoplastic transformation to cyst-adenocarcinoma confirmed by tubulo-papillary architecture and invasion of the basement membrane. - Clinical Presentation: Cystadenoma most often occurs in middle-aged women. However, cyst-adenocarcinoma equally affects both, men and women. Most patients are asymptomatic or have vague abdominal complaints of bloating, nausea, and fullness. These patients, like all those with hepatic cysts, eventually present with abdominal pain. Rarely, they present with evidence of biliary obstruction. - Investigations: With cystic tumors, as with simple cysts, LFT results are normal. There may be mild abnormalities in some patients. Carbohydrate antigen (CA) 19-9 levels are elevated in some patients. Cystadenoma and cyst-adenocarcinoma usually appear multi- loculated with internal septations, heterogeneous density, and thicker, irregular hypervascular cyst wall. Unlike many tumors, cystadenoma and cystadenocarcinoma are rarely associated with calcifications.

HYDATID CYSTS

- Etiology: Hydatid cysts are caused by infestation with the parasite Echinococcus granulosus. This parasite is found worldwide, but it is particularly common in areas of sheep and cattle farming.

131

- Pathogenesis / Pathology: The adult tapeworm lives in the digestive tract of carnivores, such as dogs or wolves. Eggs are released into the stool and are inadvertently ingested by the intermediate hosts, such as sheep, cattle, or humans. The egg larvae invade the bowel wall and mesenteric vessels of the intermediate host, allowing circulation to the liver. In the liver, the larvae grow and become encysted. The hydatid cyst has 3 layers: (1) adventitia, an inseparable fibrous tissue due to reaction of the liver to the parasite (2), laminated membrane (ectocyst), formed of the parasite itself; it is whitish, elastic, which can be peeled-off readily from the adventitia, and (3) germinal epithelium (endocyst), the only living part, lining the cyst and produces daughter cysts. When carnivores ingest the liver of the intermediate host, the scolices of the daughter cysts are released in the small intestines and grow into adult worms, thus completing the life cycle of the worm. - Clinical Presentation: Patients with hydatid cysts, like those with simple cysts, are most often asymptomatic, but pain may develop as the cyst grows. Larger lesions typically cause pain and are more likely to develop complications than simple cysts. At presentation, patients generally have a palpable mass in the right upper quadrant. - Complications: Cyst rupture is the most serious complication of hydatid cysts. Cysts may rupture into the biliary tree, through the diaphragm into the chest, or freely into the peritoneal cavity. Rupture into the biliary tree may result in jaundice or cholangitis. Free rupture into the peritoneal cavity may cause anaphylactic shock. As with simple cysts, patients with hydatid cysts may develop secondary infection and subsequent hepatic abscesses. - Investigations: Eosinophilia is noted in approximately 40% of patients, and Echinococcal antibody titers are positive in nearly 80% of patients. Casoni‗s test (intradermal test—70% sensitive—historical interest. Hydatid cysts can be identified by the presence of daughter cysts within a thick-walled main cavity. Ultrasound may reveal rosettes of daughter cysts, double-contoured membrane of the cyst, and calcification of cyst wall (in adventitia). CT scan abdomen is more accurate in identifying cyst characteristics — cart-wheel-like—multi-vesicular rosette-like, calcification, floating membrane, and hydatid sand. In patients who are jaundiced with hydatid disease, MRCP or ERCP should be performed to determine whether the cyst has ruptured into the bile duct. - Management: Patients with hydatid cysts should be treated to prevent complications related to cyst growth and rupture. 1. Medical therapy with anti-hydatid agents (Albendazole and Mebendazole) is relatively ineffective. These drugs are used as adjuvant treatment, but they do not replace surgical or percutaneous therapy in symptomatic patients. However, they may have a role in small accidentally discovered hydatid cysts. In surgically treated patients, the use of anti-hydatid agents is generally given peri-operatively; continuation is limited to those who have spillage of cyst fluid at the time of operation or to those with cyst rupture. Anti-hydatid agents are used in conjunction with percutaneous treatment. Medical therapy should be started 4 days before percutaneous treatment and continued either for 1 month (Albendazole) or for 3 months (Mebendazole).

131

2. PAIR (puncture, aspiration, injection, and re-aspiration) is a percutaneous treatment technique for hydatid disease. In this minimally- invasive method, a needle is introduced into the cyst under US guidance. Cyst fluid is aspirated and analysed. Hypertonic saline is then injected and re-aspirated. Its use in the treatment of hydatid cysts has been somewhat controversial. However, as this technique safety and efficacy have been reported in the literature, it has been increasingly accepted as a treatment option for hydatid disease although its use is still limited. It is contraindicated in non- cooperative patients and in Inaccessible or risky location of the liver cyst and cyst communicating with the biliary tree. 3. Most liver hydatid cysts are managed surgically. Treatment of hydatid cysts is associated with two technical problems; first, risk of anaphylaxis from spillage of cyst fluid containing eggs and larvae into the peritoneal cavity and, secondly, recurrence caused by residual eggs in incompletely removed germinal membranes. To prevent these problems, the cyst contents are aspirated and replaced with a hypertonic saline solution to kill residual daughter cysts in the germinal membrane. The abdomen is opened, and the peritoneal cavity is packed with mops [black or colored mops are used to identify white scolices clearly so as to pick up all and prevent any spillage]. Fluid from the cyst is aspirated and scolicidal agents [Cetrimide, Chlorohexidine,

alcohol, hypertonic saline (15%-20%), 10% Povidone iodine or H2O2] are injected into the cyst cavity (formalin should not be used). The cyst is then opened at most superficial part, contents aspirated. The goal of the procedure is to excise the lamellated and germinal membrane, leaving the fibrous component of the cyst wall in situ (cystectomy). The residual cavity is usually packed by a pedicled omentum Laparoscopic cystectomy is becoming more popular. Contraindications are deeply situated cyst, densely adherent cyst, and inaccessible cysts. Main problem with laparoscopic cystectomy is spillage and difficulty in preventing it. Attempts to excise the entire cyst wall without opening the cyst (peri-cystectomy) or to perform formal hepatectomy for hydatid cysts have largely been abandoned because of increased surgical morbidity. However, Liver resection is occasionally done in peripherally situated cyst or if diagnosis of cystic tumour could not be excluded.

AMEBIC LIVER ABSCESS

- Incidence: Nowadays, amebic liver abscess (also known as tropical abscess) is generally less common than pyogenic abscess. - Etiology: Entameba histolytica is the causative agent. It is contracted by ingestion of food or water contaminated by the cyst stage of the parasite. Amebiasis generally only involves the intestine but can invade the mesenteric venules resulting in liver abscesses. Its only host is the human. - Clinical Presentation: The patient is toxic with fever, chills and rigors, loss of appetite, reduced weight, and jaundice. The abdomen shows localized guarding and rigidity, as well as a mass in right upper abdomen (tender, soft liver). Patients may have a history of diarrhea and dysentery.

132

- Sequelae and complications: An amebic abscess can rupture into the peritoneum, pleural cavity, pericardial cavity, bronchus (causing a broncho-pleural fistula leading to coughing out of anchovy sauce pus), bare-area of liver (causing retro-peritoneal abscess), the intestines, or into the skin (amebiasis cutis). - Investigations: Total WBC count may be increased. Liver function tests may show altered bilirubin and albumin. Serum alkaline phosphatase, AST and ALT levels are elevated. Serological tests (ELISA) are reliable in non-endemic areas. US abdomen shows altered echogenicity (an-echogenic, hypo-echogenic), size, location, number of abscess, nature of the liver. CT scan shows raised diaphragm; abscess cavity (low density area)—its size, location, number; presence of effusion; changes in the lung. Sigmoidoscopy/colonoscopy is used to identify the active ulcers. Scrapings of the ulcer show trophozoites. - Management: Treatment is mainly medical using metronidazole. Aspiration with injection of amebicidal agent is indicated in case of large abscess done under US guidance. Aspirated fluid is sent for culture and sensitivity, cytology and for study of trophozoites.

PYOGENIC LIVER ABSCESS

- Etiology 1. Ascending cholangitis: commonest route. 2. Portal vein sepsis: appendicitis, diverticulitis, inflammatory bowel disease (IBD), etc. 3. Distant infections (through hepatic artery) 4. Super added infections: amoebic liver abscess, hydatid cyst. 5. Cryptogenic liver abscess — No identified primary infection. 6. Trauma; becoming a common cause. 7. Direct extension: from suppurative cholecystitis, subphrenic abscess, perforation, peri-nephric abscess. 8. Umbilical vein sepsis. - Clinical Presentation: It is of increasing incidence especially in elderly and diabetic patients or immuno-compromised individuals. It may complicate cholangitis, appendicitis, or diverticulitis. Clinical diagnosis is suggested when anorexia, fever, malaise, upper right abdominal discomfort are present. - Investigations: Micro-organisms isolated are most often bowel flora. E. coli (commonest), Klebsiella, Proteus. Enterococci, streptococci viridians in polymicrobial infection. Leukocytosis is generally present. Abdominal US and CT scan are diagnostic. US-guided aspiration of pus for culture and sensitivity is usually needed. Blood culture may be positive. - Management: Patients with liver abscesses are immediately started on antibiotics according to culture and sensitivity. If abscesses are small, patients may respond to medications alone. More likely, these patients will require the addition of percutaneous drainage. Percutaneous drainage and antibiotics are usually adequate treatment. If abscesses persist despite attempted percutaneous drainage, surgical drainage is

133 indicated. Other surgical indications include large cysts at risk of rupture and abscesses not anatomically amenable to percutaneous treatment. Treating the primary causes should not be forgotten. In general, abscesses are adequately managed by means of antibiotics and percutaneous drainage.

134

CHAPTER 6 Liver Tumors

Classification (Table 1)

Table 1: Benign and malignant tumors of the liver

Benign

- True neoplasms - Liver cell adenoma (LCA) - Non-neoplastic lesions - Hemangioma - Focal nodular hyperplasia Malignant - Primary hepatocytes - Hepatocellular carcinoma (HCC) (hepatoma) - Bile ducts - Cholangio-carcinoma - Mixed - Cholangio-hepatoma - Secondary - From GIT, pancreas or breast cancer

BENIGN TUMORS OF THE LIVER

Hemangioma

- Incidence: It is the commonest BT of the liver. It affects all ages with a mean age in adults 50 years AND occurs in females > males (5:1). It affects both lobes equally. - Etiology is not well-understood, but may be related to estrogen receptors (accelerated growth with high estrogen states; puberty, pregnancy and oral contraceptive pills-OCPs). - Pathology 1. Grossly: Well-delineated, flat, red-blue lesions that may coalesce partially on sectioning ± fibrosis, calcification and thrombosis. It may attain a large size of 10 cm or more (giant hemangioma) (Figure 1). 2. Microscopically: It is of the cavernous type showing cavernous vascular spaces lined by flattened epithelium underlying fibrous septae of various widths. - Clinically 1. It is usually < 5 cm, harmless and found incidentally during US or CT scan for unrelated reason. It may remain stable in size or increase minimally over time. 2. Pain related to uncomplicated hemangioma is probably due to an associated disorder. 3. Large hemangiomas can be asymptomatic or manifest with pain or an abdominal mass. 4. Large lesions in the left lobe may cause pressure effects.

135

- Complications 1. Alteration of internal structure, such as inflammation (low-grade fever, weight loss, abdominal pain, ↑ ESR, anemia, thrombocytosis abd ↑ fibrinogen level). 2. Alteration in coagulation, which → systemic disorders (Kasabach-Merit syndrome: intra-vascular coagulation, clotting and fibrinolysis within the hemangioma, which may progress to → 2ry ↑ systemic fibrinolysis and thrombocytopenia → death (20-30%). 3. Hemorrhage → hemo-peritoneum (internal hemorrhage). It is rare and may result from spontaneous rupture or after anti-coagulation therapy. - Diagnosis 1. US shows a focal lesion, a classically echogenic mass of uniform density, < 3 cm in diameter, with acoustic enhancement and sharp margins. 2. CT scan (Figure 2): Criteria of Dx are (1) low attenuation on non-contrast CT, (2) peripheral enhancement of the lesion followed by central enhancement on contrast CT and (3) contrast enhancement of the lesion on delayed scans. 3. Dynamic MRI: Hemangioma is a hypo-intense lesion on T1-weighted sequences and strongly hyper-intense on T2-weighted sequences with a "light bulb pattern". 4. Biopsy is contra-indicated if hemangioma is suspected.

Figure 1. Gross appearance of hemangioma Figure 2. CT scan showing hemangioma (arrow)

- Treatment 1. Asymptomatic hemangiomas that are diagnosed at laparotomy should not be resected as the natural history is towards involution and calcification. Just patient assurance is required as it has no tendency to turn to malignancy. 2. Surgical resection is indicated in presence of (1) severe symptoms, (2) complications and (3) to exclude malignancy. It includes enucleation or hepatic resection (laparoscopic) depending on the size and anatomical location on the lesion.

Focal Nodular Hyperplasia (FNH)

- It is the 2nd most common BT of the liver that usually affects women between 30-50 years. - This is a harmless lesion of the liver whose main importance is the difficult differentiation from other focal lesions. - Etiology: Hyperplastic reaction resulting from arterial malformation (↑ arterial flow → hyper-perfusion of local liver parenchyma → 2ry hepatocellular hyperplasia) (a regenerative procedure). Contraceptive pills stimulate the development & growth of FNH.

136

- Pathology 1. Grossly, FNH is a nodular firm mass, which is slightly paler than the normal liver and has prominent surface vasculature. The cut-section shows a stellate scar with radiating fibrous septa that divide the lesion into lobules (Figure 3). 2. Histologically, it resembles cirrhosis with regenerating nodules. The central scar and fibrous septa are apparent Figure 3. Focal nodular hyperplasia - Clinical presentation 1. Asymptomatic. 2. Abdominal pain or discomfort caused by pressure of large FNH or severe pain due to torsion of pedunculated lesions. - Complications: 1. Rupture 2. Bleeding (rare). No risk of malignancy. - Diagnosis / Investigations 1. US: It is hyper- or iso-echoic on US examination. The central scar is slightly hyper- echoic (difficult to visualize). Color Doppler shows a central feeding artery with a stellate or spoke-wheel pattern corresponding to the artery running from the central scar to fibrous septa. 2. CT scan: It also shows a hypo- or iso-echoic mass. The central scar is seen in 30% of cases only. FNH enhances at the arterial phase, but the central scar is hypodense. The lesion returns to become iso-dense in the portal phase. 3. MRI: A hypo- or iso-intense lesion is seen on T1-weighted images and iso- or slightly hyper-intense on T2- weighted images. - Treatment 1. Asymptomatic lesions require no treatment regardless of their number or size. Patient assurance is needed and contraceptive pills should be discontinued. 2. Surgical resection is indicated in symptomatic patients or in doubtful cases i.e. cases that are suspicious of malignancy are explored and subjected to frozen section examination, which is diagnostic.

Liver Cell Adenoma (LCA)

- Definition This lesion is a rare true benign neoplasm of hepatocytes, usually solitary (but may be multiple) and sometimes pedunculated. - Etiology 1. It occurs almost only in women. It is strongly associated with OCPs and androgen steroid therapy. Cessation of pill-intake can result in tumor regression. 2. It can occur spontaneously or associated with underlying metabolic disease including Type I glycogen storage disease, iron overload related to β-Thalassemia and DM.

137

- Pathology

1. Grossly, it is soft, well-circumscribed and light yellow in color (Figure 4). It is multiple in 1/3 of cases. It is sometimes encapsulated with fleshy appearance ± areas of hemorrhage and necrosis. 2. Histologically, it is formed of sheets of regular hepatocytes with thin-walled vessels, yet no portal triads. - Clinical features / complications 1. Small LCA is asymptomatic and is Figure 4. Well-circumscribed adenoma within discovered incidentally on US or at the liver (arrow). laparotomy. 2. Sometimes, a large LCA causes right upper quadrant pain or discomfort. 3. Complications are rare and include malignant transformation (10%) and spontaneous rupture causing internal hemorrhage (20-40%), more in women, during pregnancy. - Investigations 1. US: Well-delineated heterogenous liver mass. Hyper-echogenicity is due the presence of fat and glycogen. 2. CT scan: Hyper-vascular & heterogenous on the arterial phase and becomes iso- or hypo-dense on the portal phase due to arterio-venous shunting. Distinctive features from FNH are the smooth surface, present of hemorrhage and necrosis & the tumor capsule. 3. MRI: It is the best tool because of its higher sensitivity for fat and hemorrhage. Adenomas appear iso- or hyper-intense (due to fat content) on T1-weighted images and mildly hyper-intense on T2-weighted images. Heterogeneity of signal intensity has been considered one of the most constant features of hepatocellular adenoma. - Treatment 1. Liver resection is indicated if doubt exists as to the possibility of malignancy, and for large (> 4 cm) symptomatic tumors. 2. In other cases (small lesions < 3 cm), the tumor is regularly checked for any change in size (it may regress), contraceptive pill-intake is discontinued and pregnancy avoided.

MALIGNANT TUMORS OF THE LIVER

A. PRIMARY LIVER CANCER

Pathological Types

- Hepatocellular carcinoma (HCC) - Hepatoblastoma (in infancy) - Mixed HCC and cholangiocarcinoma. - Intra-hepatic cholangiocarcinoma.

138

Epidemiology

- In contrast to BTs, 1ry liver malignancies are, by far, commoner in men. - Hepatocellular carcinoma (HCC) is the main 1ry liver malignancy and is prevalent in the Far East and in equatorial African nations. The world‘s highest incidence is in Mozambique. - Cholangio-carcinoma arising from the intrahepatic bile ducts is much less common than HCC and it also has its highest incidence in the Far East.

Etiology

Hepato-cellular Carcinoma (HCC) - The following conditions are associated with the development of HCC: 1. Infection with hepatitis B virus (HBV), or hepatitis C virus (HCV). 2. Liver cirrhosis of any cause. The highest incidence of HCC is with post-hepatitic, followed by alcoholic, cirrhosis (90% of HCC occurs in patients who are carriers of hepatitis virus or who have cirrhosis). Prolonged infection with HBV or HCV → integration of the viral DNA into the host genome, thus starting the malignant changes. 3. Aflatoxin ingestion: This substance is formed by the fungus Aspergillus flavus, which grows on grains that are stored under moist warm conditions.

Cholangiocarcinoma - Development is related to infestation with Clonorchis sinensis (liver fluke) that causes Asciatic cholangitis.

Pathology

Hepato-cellular Carcinoma (HCC) - Gross appearance: The tumor is yellow in color & commonly arises in a cirrhotic liver. It assumes different forms: 1. Massive form, i.e. forming a localized mass (Figure 5). The fibro-lamellar variant of HCC is a variety of the massive type that affects non-cirrhotic young females & hence has a better prognosis. 2. Nodular form, i.e. multiple nodules scattered over the liver. 3. Diffuse form, characterized by diffuse Figure 5. Hepatocellular carcinoma (HCC) infiltration throughout the liver. - Microscopically: HCC is formed of malignant hepatocytes with little stroma, but high vascularity from the HA. Cells possess acidophilic cytoplasm and large nuclei. In well- differentiated cases, cells are similar to normal liver cells, but in anaplastic cases the similarity is less striking. The fibro-lamellar type is an exception as it contains numerous fibrous septa that make it resemble FNH & the cells are deeply eosinophilic.

139

- Complications 1. Spread by the lymphatic and venous routes producing porta hepatis nodal enlargement, peritoneal nodules and less commonly lung deposits. 2. Spontaneous rupture → subcapsular hematoma or massive intra-peritoneal hemorrhage.

Intra-hepatic Cholangio-carcinoma - The tumor is an adenocarcinoma of the lining epithelium of the intra-hepatic bile ducts. - It spreads widely inside and outside the liver, and by the time the tumor is discovered, the disease is usually so advanced to cure.

Hepato-cholangio-carcinoma - This is a mixed type that behaves like HCC.

Hepatoblastoma - This is a form of HCC that occurs in children. It is termed hepatoblastoma because of the similarity to fetal liver cells.

Other 1ry Malignant Tumors of the Liver - These include: Angiosarcoma, epitheloid hemangio-endothelioma, undifferentiated embryonal sarcoma, and leiomyosarcoma

Clinical Features

Different types of presentation depend on the race and Country + tumor type and size. 1. Deterioration of health of a known cirrhotic is a common presentation. 2. Small lesions may be accidentally discovered by an US done for another purpose. 3. Where HCC is a common, an early detection program is recommended by annual US and AFP estimation (↑ AFP or appearance of a focal lesion on US, warrants further investigations for early detection of the tumor). 4. HCC in black Africans grows rapidly producing pain, jaundice, fever and a tender mass, which is commonly mistaken for an amebic liver abscesses. 5. Late cases present by pain, jaundice, ascites, hepatomegaly, anorexia, loss of weight and probably massive intra-peritoneal hemorrhage.

Investigations

Laboratory Tests 1. Serum AP ↑. The rest of LFTs may show the pattern of cirrhosis if present. 2. Serum AFP is a tumor marker that is present in the serum of the fetus. The normal adult level is 0-10 ng/mL. Its serum level ↑ highly in HCC and testicular teratoma. A level >200 ng/mL is suggestive and >2000 ng/mL is diagnostic of HCC. It may also ↑ in other benign liver diseases, but to a lesser extent. Serum AFP returns to normal level after a successful tumor resection. Re-elevation in the follow-up period signifies a recurrence.

141

Imaging 1. US Abdomen: It can detect the focal hepatic lesion, P) patency and presence of ascites. 2. Tri-phasic CT scan (Figure 6) and/or MRI can detect HCC (number, site, size vascular invasion) and extent of tumor spread in the liver. It can also detect portal vein (PV) invasion and ascites. 3. Chest X-ray, CT scan and PET scan to search for chest metastases.

Biopsy - The value of pre-operative guided-biopsy is controversial because of the possible risk of hemorrhage from the highly vascular tumor.

Figure 6. Triphasic CT scan showing enhancement of the lesion (HCC) in the arterial phase & wash-out of contrast in the venous portal phase.

Treatment

Treatment of 1ry MTs of the liver is either curative or palliative:

Curative Treatment Options Palliative Treatment Options

1. Hepatic resection: For patients with Child 1. TACE (Trans-Arterial Chemo- A where resection is done with adequate Embolization) (the best palliative option) free margin & adequate residual liver 2. Local radiotherapy volume. 3. Targeted chemotherapy (Sorafinib) 2. Liver transplantation (OLTx, LDLT) for 4. Systemic chemotherapy patients within Milan Criteria, regardless 5. Ligation of hepatic artery of the Child class. 6. Percutaneous ethanol injection 3. Local ablation for solitary tumors, < 3 cm.

141

Operable Cases The ideal treatment for a tumor localized to one liver lobe is to resect that lobe. The usual operation for such situation is either right hemi-hepatectomy (resection of segments 5- 8) or left hemi-hepatectomy (resection of segments 2, 3 and 4) (Figure 7). The presence of cirrhosis in most cases poses some problems due to: 1. Bleeding tendency. 2. Poor function of the remaining 1/2 of liver 3. ↓ capacity of liver cell regeneration. For these reasons, hepatic resection is Figure 7. Segments of the liver. Hepatic resection is reserved for patients with fair liver functions. named according to the segments resected.

Inoperable Cases The following palliative options are available 1. Trans-Arterial Chemo-Embolisation (TACE) combines the benefits of inducing tumor ischemia and those of selective chemotherapy. The chemotherapy agent is loaded on a particulate material like gelfoam and is injected by angiographic techniques into the HA. The artery is blocked → tumor ischemia and the drug is selectively delivered to its target. 2. Systemic chemotherapy. 3. Intra-arterial selective chemotherapy via a catheter inserted in the HA. 4. Ligation of HA: As the blood supply of the tumor is exclusively through the HA, its ligation produces some regression. 5. Percutaneous 90% ethanol injection (under US guidance) → tumor necrosis.

Management According to Staging The most accepted staging of HCC that links clinical data and therapeutic options is: The Barcelona Clinic Liver Cancer (BCLC) (Figure 8)

Figure 8: The Barcelona Clinic Liver Cancer (BCLC) staging of HCC

142

- Stage 0: Patients with very early HCC are optimal candidates for resection. - Stage A: Patients with early HCC are candidates for radical therapies (resection and ablation, liver transplantation; or percutaneous treatments). - Stage B: Patients with intermediate HCC may benefit from chemo-embolization. - Stage C: Patients with advanced HCC may receive new agents in the setting of a RCT. - Stage D: Patients with end-stage disease will receive symptomatic treatment.

B. LIVER METASTASES

Incidence

- Liver Metastases are 20 times more common than 1ry malignancies.

Sources

Metastases Can Reach the Liver Through: 1. Portal circulation (commonest rout): From carcinoma of colon, rectum, pancreas or stomach. 2. Hepatic artery: Carcinomas of the lung, breast, kidney, uterus or ovaries are common examples. 3. Lymphatics. 4. Direct spread from tumors of the gall bladder, stomach or colon.

Metastatic Hepatic Focal Lesions 1. Colo-rectal hepatic metastases. 2. Neuro-endocrine hepatic metastases. 3. Non-colorectal, non-neuroendocrine hepatic metastases. Pathology

- Metastases may occur in the same time of discovery of the 1ry tumor (synchronous) or months or years after resection of the 1ry (metachronous). - Liver metastatic nodules are usually multiple, white, and umbilicated because of central necrosis (Figure 9). - They are usually adenocarcinomas. - Over 90% of patients have tumor deposits in

other organs. Figure 9. Liver secondaries (metastases)

Clinical Features

- In addition to the manifestations of the 1ry MT, the patient suffers from weight loss, fatigue and anorexia, right upper abdominal pain, jaundice, ascites and/or palpable hepatomegaly.

143

Investigations

- Laboratory tests usually reveal anemia + ↑ serum alkaline phosphatase and bilirubin. - Imaging by US, CT scan (Figure 10), or MRI shows the number and sites of metastases, which affects the line of treatment.

Figure 10. CT scan showing multiple metastases in the liver

Treatment

- The presence of liver metastases indicates an advanced inoperable tumor. - The treatment in such cases, usually by chemotherapy, is considered palliative. - A few selected cases of metastatic colorectal cancer, if localized to one segment, can be treated by liver resection aiming at cure. Chemotherapy 1. Systemic chemotherapy is administered in the presence of other organ involvement. 2. Selective intra-arterial chemotherapy via a catheter surgically inserted in the HA, is indicated when the liver is the only organ with metastases. This technique reduces the systemic side effects of anti-cancer agents & allows delivery of higher doses to the liver. Liver Resection - The prerequisites include: 1. A completely resectable colorectal cancer with no apparent residual tumor. 2. No extra-hepatic metastases are found after a thorough search. 3. Liver metastases that are resectable with a safety margin: - A solitary metastatic nodule → wedge resection. - Multiple metastases confined to one surgical lobe → right or left hemi-hepatectomy. - Liver resection is done in the same session of the colorectal operation, or later if the liver deposits appear in the course of the patient‘s follow-up. Cure rate = 25%.

Prognosis

- In patients with isolated hepatic metastases, the extent of liver disease is the prime determinant of survival, and when left untreated, survival is measured in months. - Surgery is safe and potentially curative in treatment of colo-rectal metastases to the liver. - The current 5-year survival after a margin-negative hepatic resection is 40%, while the 10- year survival approaches 20% only.

144

CHAPTER 7

Organ Transplantation and Liver Transplantation

TERMS AND TYPES OF TRANSPLANTATION

Organ transplantation - It is a surgical procedure in which a failing organ in a recipient is replaced by a functioning one from a donor with matching tissue and blood compatibility.

Donor - A donor is the healthy individual who accepts to donate his tissue to the diseased patient after receiving proper consent without any coercion.

Recipient - A recipient is the patient who has a failing organ function that endangers his life and is willing to receive the new functioning organ from the donor.

Auto-transplantation - It is the type of transplantation where tissues (auto-grafts) are transferred within the same person e.g. skin grafts and vein grafts.

Allo-transplantation - It is the transplant of an organ or tissue (allograft) between two genetically non-identical members belonging to the same species. Most human tissue and organ transplants are allografts (from one human to another). Such genetic differences between the organ donor and the recipient makes it easy for the recipient's immune system to identify the transplanted organ as a foreign body and starts to act against it to attempt destroying it, which is known as transplant rejection.

Isograft - It is the transplantation of organs or tissue between two genetically identical individuals (such as identical twins). Iso-grafts differ from allografts in that they do not trigger the recipient‘s immune response since it is transplanted from a genetically identical person.

145

Xenograft and Xeno-transplantation - It is the transplantation of organs or tissue between members of two different species e.g. porcine heart valve transplant.

Orthotopic transplantation - It entails placement of the graft in its normal anatomical site.

Heterotopic transplantation - It entails placement of a graft in a site different from where it is normally located.

Split transplantation - It is the type of transplantation in which an allograft procured from a deceased donor can be split to benefit 2 recipients, such as liver allograft, which can be split into right lobe and left lobe or right tri-segment lobe and left lateral lobe to fit into an adult recipient and a pediatric recipient, respectively.

Domino transplantation - It is the type of transplantation where a healthy donor donates his organ to a diseased recipient after taking out his failing organ. This failing organ in return can be used in another recipient who is in a grave situation and who can benefit from such failing organ for some time until a healthy donor is available to offer him his organ. This type is suitable only in liver grafts with amyloidosis, or lungs with cystic fibrosis.

BASIC IMMUNOLOGY OF TRANSPLANTATION

- Donor allografts trigger the recipient‘s immune response owing to antigenic differences between them, which promote antibody formation and consequently lead to graft rejection. Accordingly, prior to transplantation, histocompatibility matching between donor antigens and recipient antigens is required to minimize rejection. - The most 2 important antigen groups that should be tested for compatibility between donor and recipient are: 1. ABO blood group antigens 2. Human leukocyte antigens (HLA) - Major organs liable for transplantation: 1. Heart 2. Lung 3. Liver 4. Kidney 5. Pancreas 6. Small bowel - Tissues liable for transplantation: 1. Skin 2. Cornea 3. Pancreatic islets 4. Bone marrow 5. Tendon 6. Cornea 7. Heart valve 8. Bone 9. Veins

146

Types of Rejection

Hyper-acute Rejection - This type of rejection occurs immediately within few hours following transplantation. - It usually occurs due to the presence of preformed antibodies in recipient‘s blood against HLA antigens expressed in the donor‘s allograft. - It also occurs with organ transplantation from an ABO-incompatible donor.

Acute Rejection - It usually occurs during the first few days up to 6 months after transplantation. - It is usually cell-mediated immune reaction (by T-cell lymphocytes), but may also involve humoral immune reaction (antibody-mediated), or both.

Chronic Rejection - It usually occurs more than 6 months following transplantation. - It may be immunological or non-immunological. - It is the most common cause of allograft failure.

ORGAN PROCUREMENT

Organ procurement can be performed from: 1. Living donors who can donate organs as one kidney, right or left lobe of the liver, or a lobe of the lung. 2. Deceased donors who can donate a whole organ and may be classified into: a) Donation after donor brainstem death (DBD). b) Donation after donor circulatory death (DCD).

INDICATIONS OF LIVER TRANSPLANTATION

Liver transplantation is indicated for patients suffering from irreversible decompensated end-stage liver disease (ESLD) due to any of the following factors:

Acute Liver Failure 1. Acute viral hepatitis (HAV, HBV, HEV). 2. Drug- or toxin-induced hepatotoxicity (e.g. Acetaminophen hepatic toxicity). 3. Autoimmune hepatitis. 4. Wilson‘s disease.

Chronic Liver Failure 1. Chronic viral hepatitis (HCV, HBV) 2. Alcoholic liver disease. 3. Non-alcoholic fatty liver disease and steato-hepatitis 4. Autoimmune hepatitis 5. Cryptogenic liver cirrhosis

147

Malignant Diseases of the Liver 1. Hepatocellular carcinoma (HCC), within Milan criteria (single lesion within 5 cm or up to 3 lesions, none of them exceeding 3 cm in diameter). 2. Carcinoid tumor 3. Cholangio-carcinoma 4. Hemangio-endothelioma

Metabolic Liver Diseases 1. Wilson‘s disease 2. Hereditary hemochromatosis 3. Alpha-1 antitrypsin deficiency 4. Glycogen storage disease

Vascular Diseases 1. Budd-Chiari syndrome 2. Veno-occlusive diseases of the liver

Cholestatic Liver Diseases 1. Primary biliary cholangitis (PBC) 2. Primary sclerosing cholangitis (PSC) 3. Biliary atresia 4. Alagille syndrome (a genetic disorder that affects primarily the liver and heart)

Others 1. Adult polycystic liver disease 2. Liver trauma 3. Caroli disease 4. Amyloidosis

LIVING DONORS

Investigations for Living Donor Assessment

Laboratory Tests 1. Complete blood count (CBC) 2. Liver function tests (LFTs) - Total and direct bilirubin - Serum albumin and prothrombin - Liver enzymes: AST, ALT - Cholestatic enzymes: ALP, GGT 3. Coagulation profile: INR, PT, PTT 4. Renal function tests: BUN, serum creatinine 5. Hepatitis profile for HBV, HCV, HAV, HEV, HDV 6. Chronic infections as CMV, EBV 7. Urinalysis for drug abuse

148

Cardiac Assessment 1. Electrocardiography (ECG) 2. Echo-cardiography 3. Cardiac catheterization.

Imaging Studies 1. Chest X-ray 2. Abdomino-pelvic ultrasound (US) 3. Duplex US of the portal vein and hepatic artery 4. Triphasic abdominal CT with volumetry and/or MRI 5. CT angiography

Ideal Liver Transplant Living Donor

Criteria of an ideal liver transplant living donor: 1. Age: 8-45 years 2. BMI< 30 3. No previous upper abdominal surgery 4. No history of any chronic diseases as hypertension or diabetes mellitus. 5. No evidence of drug abuse or smoking 6. No evidence of HCV, HBV, or bilharziasis 7. No coercion to donation 8. Related to the recipient 9. Absence of any financial benefit

CONTRAINDICATIONS OF LIVER TRANSPLANTATION

Absolute Contraindications

1. Severe cardiopulmonary disease. 2. Extra-hepatic malignancy (oncological criteria for cure not met). 3. Active alcohol/substance abuse. 4. Acute alcoholic hepatitis. 5. Active infection/uncontrolled sepsis. 6. Lack of psychosocial support or inability to comply with medical treatment. 7. Brain death.

Relative Contraindications

1. Advanced age. 2. Acquired immune deficiency syndrome (AIDS). 3. Cholangio-carcinoma. 4. HCC exceeding the transplantable criteria. 5. Diffuse portal vein thrombosis.

149

IMMUNO-SUPPRESSION AFTER LIVER TRANSPLANTATION

Steroid-based triple therapy is followed in the form of: 1. Corticosteroids. 2. Anti-proliferative drugs such as mycophenolate. 3. Calcineurin inhibitors such as cyclosporine A or tacrolimus.

COMPLICATIONS OF LIVER TRANSPLANTATION

Complications of liver transplantation include the following: 1. Biliary complications: stricture – leak - stone formation 2. Vascular complications: hepatic artery thrombosis or stenosis - Portal vein thrombosis - Hepatic vein or inferior vena cava (IVC) occlusion or thrombosis. 3. Parenchymal complications: Recurrent disease (as HCV, HCC) - acute or chronic liver allograft rejection 4. Opportunistic infections 5. Immunosuppressive drugs-induced complications

151

CHAPTER 8

Abdominal Trauma

TOPOGRAPHIC ANATOMY

Definitions (Figure 1) - Thoraco-abdominal area: Transverse nipple line to costal margin - Anterior abdomen: Costal margin to groin crease to anterior axillary lines bilaterally - Flank area: Anterior axillary line to posterior axillary line, costal margin to iliac crests - Back: Medial to posterior axillary lines, tip of scapula to iliac crests - Torso: All the above

Figure 1. Topographic anatomy of the abdomen

The Abdomen is More Than Just the Abdomen - Abdominal trauma may involve the Figure 2. Parts of following (Figure 2): the abdomen that may be involved in 1. Intra-peritoneal cavity case of abdominal 2. Retro-peritoneal cavity and trauma pelvis 3. Thoraco-abdominal injuries 4. Cardiac box injuries (heart and great vessels ) 5. Diaphragm and bladder (innocent by-standers)

151

CLASSIFICATION

According to Etiology 1. Penetrating Abdominal Trauma (PAT) - Stab wounds (75%) - Gunshot wounds (high-velocity, low-velocity) (25% ) 2. Blunt Abdominal Trauma (BAT) - High-energy transfer (Motor Vehicle Accident – MVA) (75%) - Low-energy transfer (fall, fight) (25%) 3. Mixed Pattern: Explosions

According to Pathogenesis or Mechanism of Injury (MOI)

1. Blunt Abdominal Trauma (BAT) - Examples: Road traffic accident (RTA), run-over, falls, blows, etc. - Multiple organ damage is quite often. - Most commonly injured organs: Small intestine > colon > liver - Pressure effect → burst injury, - Crushing effect → crush injury - Acceleration and deceleration effect → shear injury.

2. Penetrating Abdominal Trauma (PAT) Stab Wounds - Examples: Knives, ice picks, pens, coat hangers, broken bottles, etc. - Multiple organ injuries are less often than with BAT. - Most commonly injured organs: Liver, small bowel &spleen. - Direct effect. Gunshot Wounds - Examples: Bullets and pellets - Often multiple organ injuries, bowel perforations - Most commonly injured organs: Small bowel, colon & liver - Direct effect - Kinetic energy (cavitation).

According to Grading

AAST Concept (American Association for the Surgery of Trauma) - Grading of abdominal injuries is done according to the AAST concept according to certain CT-based criteria, mainly relying on the following: 1. Presence of hematoma 2. Presence of laceration 3. Presence of vascular injury 4. Effect on the collecting system. - Accordingly, solid abdominal organs (spleen, liver, kidneys and pancreas) can be classified (graded) as follows: 152

Grading of the Splenic Injury (Figure 3)

Grade Description

Grade I Hematoma: Subcapsular, non-expanding hematoma (< 10% surface area) Laceration: Capsular tear, non-bleeding, parenchyma involved <1cm deep Grade II Hematoma: Subcapsular, nonexpanding hematoma (10-50% surface area); nonexpanding, intra-parenchymal hematoma (<2cm in diameter) Laceration Bleeding capsular tear or parenchymal laceration 1-3 cm deep without trabecular vessel involvement Grade III Hematoma Subcapsular hematoma (> 50% surface area or expanding); ruptured subcapsular hematoma with active bleeding; or intra-parenchymal hematoma (> 2 cm in diameter or expanding) Laceration Parenchymal laceration > 3 cm deep or involving trabecular vessels Grade IV Hematoma Ruptured intra-parenchymal hematoma with active bleeding Laceration Involving segmental or hilar vessels producing major (> 25% splenic volume) Devascularization Grade V Laceration Completely shattered or avulsed spleen Vascular Hilar vascular injury that devascularizes the entire spleen

Figure 3. Grading of splenic injury

Easy way to remember Grade 1 is < 1 cm Grade 2 is about 2 cm Grade 3 is > 3 cm Grade 4 is > 10 cm Grade 5 corresponds to total devascularizaIon or maceration

153

Grading of the Liver Injury (Figure 4)

Easy way to remember Grade 1 is < 1 cm Grade 2 is about 2 cm Grade 3 is > 3 cm Grade 4 is > 10 cm, or unilobar maceration Grade 5 is bilobar maceraIon, venous injury Grade 6 is avulsion Figure 4. Grading of hepatic injury

Grading of the Renal Injury (Figure 5)

Grade Extent of Renal Injury

1 Contusion: Microscopic or gross hematuria, no injury with any imaging Hematoma: Subcapsular with no parenchymal laceration 2 Non-expanding peri-renal hematoma or cortical laceration < 1cm deep with no urinary extravasation. 3 Parenchymal laceration extending > 1cm into the cortex with no urinary extravasation. 4 Parenchymal laceration extending through the cortico-medullary junction & into the collecting system. 5 Multiple major lacerations resulting in shattered kidney or avulsion of the renal hilum that devascularizes the kidney. Figure 5. Grading of renal injury

154

Easy way to remember • Grade 1 is contusion or subcapsular hematoma only • Grade 2 is laceration < 1 cm without injury to collecting system • Grade 3 is laceration > 1 cm without injury to collecting system • Grade 4 is injury to collecting system or large laceration • Grade 5 is shattered or devascularized kidney

Grading of the Pancreatic Injury (Figure 6)

Figure 6. Grading of pancreatic injury

155

CLINICAL PRESENTATION

Physical Examination

Signs of Intra-Peritoneal Injury - Hypovolemia - Peritonism. - Distention (pneumo-peritoneum, gastric dilation, or ileus) - SC emphysema - Entrance & exit wounds to determine path of injury. - Gastrointestinal hemorrhage: Mouth or rectal - Ecchymosis: 1. Umbilicus (Cullen's sign) (Figure 7) 2. Flanks (Gray Turner’s sign) (Figure 8): Retro-peritoneal hemorrhage 3. Back: Retroperitoneal hemorrhage 4. Abdominal contusions – Seat belt sign (Figure 9),

Figure 7. Cullen's sign: ecchymosis around the Figure 8. Grey-Turner's sign: ecchymosis around umbilicus the umbilicus

Be cautious ! Clinical assessment is mandatory but is not enough because of. - Silent presentation Blunt trauma can be evolving. - Limitations because SEAT-BELT SIGN of the degree of level of consciousness, distracting injury and spinal cord injury. Figure 9. Abdominal contusion: Characteristic Seat-belt sign

156

INVESTIGATIONS

Laboratory Investigations

- CBC (complete blood count): ↑ WBC (leucocytosis) - ↓ HCV (hematocrit value) - ↑ CRP (C-reactive protein). - LFTs (liver function tests) - ↑ lipase (pancreatic enzyme).

Imaging Studies

Plain X-Ray A plain radiogram may show the following: - Fractures: nearby visceral damage - Air: Subcutaneous - Free intra-peritoneal air (Figure 10) - Retro-peritoneal air - Foreign bodies & missiles (Figure 11)

Figure 10. Plain X-ray abdomen showing air under Figure 11. Plain X-ray abdomen showing diaphragm (free intra-peritoneal) foreign body (missile)

FAST (Focused Assessment with Sonography for Trauma) - To diagnose free intra-peritoneal blood after blunt trauma, 4 areas should be examined: 1. Peri-hepatic and hepato-renal space (Morrison’s pouch) (Figure 12) 2. Peri-splenic (Figure 13) 3. Pelvis (Douglas pouch/recto-vesical pouch) (Figure 14) 4. Pericardium (sub-xiphoid) - Sensitivity 60-95% for detecting 100 mL - 500 mL of fluid - Extended FAST (E-FAST): Add thoracic windows to look for pneumothorax (Figure 15) - Advantages of FAST 1. Portable 2. Fast (<5 minutes), 3. No radiation or contrast material injected 4. Less expensive when compared to CT scan

157

- Disadvantages of FAST 1. Operator-dependent 2. Limited by obesity, substantial bowel gas and subcutaneous (SC) air. 3. Not good for assessment of the retro-peritoneum, or detection of diaphragmatic defects. 4. Not suitable for solid parenchymal damage 5. Cannot distinguish blood from other fluid collections.

Figure 12. FAST of the hepato-renal space (Morrison pouch) for injuries of liver and kidney

Figure 13. FAST of the peri-splenic region for assessment of injuries of the spleen

Figure 14. FAST of the recto-vesical pouch (Douglas pouch)

158

Figure 15. FAST of the pericardium (sub-xiphoid area) CT scan - Advantages 1. Accurate for solid organ lesions and intra-peritoneal hemorrhage (Figure 16 A-D) 2. Can guide non-operative management of solid organ damage - Disadvantages 1. Expensive. 2. Radiation & contrast exposure. 3. Less sensitive for injury of the pancreas, diaphragm & mesentery.

Figure 16. CT scan of the abdomen showing solid organ & intra-peritoneal lesions (injuries)

159

Angiography - To embolize bleeding vessels or solid visceral hemorrhage from blunt abdominal trauma (BAT) in an unstable patient - Rarely, for diagnosing intra-peritoneal & retro-peritoneal hemorrhage after penetrating abdominal trauma (PAT)

Diagnostic Peritoneal Lavage (DPL) - Procedure (under sterile conditions) 1. Attempt to aspirate free peritoneal blood: >10 mL is +ve for intra-peritoneal injury 2. Insert lavage catheter. 3. Lavage peritoneal cavity with saline - Positive test 1. In blunt trauma, or stab wound to anterior, flank, or back: RBC count > 100,000/mm3 2. In lower chest stab wounds or GSW: RBC count > 5,000-10,000/mm3 - DPL has been largely replaced by FAST and CT scan - Limited indications 1. In blunt trauma, used to triage the patient who is hemodynamically unstable and has multiple injuries with an equivocal FAST examination 2. In stab wounds, for immediate diagnosis of hemo-peritoneum, determination of intra- peritoneal organ injury & detection of isolated diaphragm injury

Summary of Assessment According to Region

Abdomen Intra-peritoneal cavity Retro-peritoneal cavity & pelvis - Imaging: FAST - CT scan - Pelvic X-ray - DPL - CT scan - Exploratory laparoscopy - Exploratory laparotomy - Exploratory laparotomy Thorax (Thoraco-Abdominal Injuries) Heart & Great Vessels (Cardiac Box Injuries) - Chest X-Ray - Cardiac FAST - Chest X-Ray Diaphragm and Bladder (innocent bystanders) - Diagnostic laparoscopy - CT cystogram

TREATMENT

Management of BAT (Algorithm) (Figure 17) Non-operative Management - Indication Criteria - Reliable FAST - Good quality CT scans - Experienced radiologist - Intensive care (ICU) setting available - Hemodynamically stable victim - Minor-Grade Injury - Absence of active hemorrhage - Absence of peritoneal signs

161

Operative Management - Indication Criteria - Lack of reliable FAST or good quality CT scans, or experienced radiologist - Lack of Intensive care (ICU) setting - Hemodynamically unstable victim - High or increasing packed RBCs transfusion requirement. - High-Grade Injury - Active hemorrhage - Peritonism

Figure 17. Algorithm for Management of BAT

Management of PAT (Algorithm) (Figure 18)

Local Wound Exploration

Aims - To determine the depth of penetration in case of stab wounds. - If peritoneum is violated, more diagnostic investigations should be ordered Procedure - Prepare the wound site - Extend wound the wound under local anesthesia - Carefully examine the wound – Avoid blind probing (to avoid injury of organs) Indication - Anterior abdominal stab wounds, less clear for other areas

161

Laparoscopy

Indication - Most useful to evaluate penetrating wounds to thoraco-abdominal region in stable patient especially for injury of the diaphragm Advantages - Accurate: Sensitivity 87.5%, specificity 100% - Organs can be repaired via the laparoscope e.g. diaphragm, solid viscera, stomach, small bowel. Disadvantages - Poor sensitivity for hollow visceral injury & the retro-peritoneum - Complications may occur from trocar misplacement. - In case of diaphragmatic injury, pneumothorax may occur during insufflation

Figure 18: Algorithm for Management of PAT

162

GASTRO-INTESTINAL SURGERY

163

CHAPTER 1 Gastro-Esophageal Reflux Disease (GERD)

Introduction

- Reflux esophagitis is inflammation of the lower esophageal mucosa 2ry to reflux of gastric and/or duodenal contents up into the esophagus. - Gastro-esophageal reflux may be asymptomatic and is regarded as physiological. Short periods of reflux, especially after eating can also be regarded as normal. - Significant reflux, leading to symptoms (including nocturnal episodes), is termed gastro- esophageal reflux disease (GERD), which is now a common cause of dyspepsia in the ‗developed‘ world and can lead to esophagitis and its complications.

Epidemiology

- GERD affects up to 60% of the population at some time in their lives, although up to 40% of these patients will have no evidence of esophagitis at endoscopy. - It is rare in certain parts of the world (e.g. the Orient) but is increasing rapidly in the developed world and represents a major drain on health care resources. Not only is the cost of drug therapy and surgery for GERD increasing dramatically, but GERD also causes significant economic consequences in terms of work absenteeism.

Factors Involved in the Normal Prevention of Reflux

1. Lower esophageal sphincter (LES): (a) Intrinsic LES pressure, (b) Total length of LES and (c) intra-abdominal length of LES 2. Angle of His (‗flap valve‘ mechanism) 3. Crural fibers of the diaphragm (‗pinchcock‘ action) 4. Mucosal rosette (prominent mucosal folds at the gastro-esophageal junction - GEJ) 5. Swallowed saliva (lubrication and neutralization) 6. Ante-grade esophageal peristalsis 7. Normal gastric motility/emptying.

Pathophysiology

- There is an underlying anatomical or physiological defect leading to failure of the normal anti-reflux mechanism. The major cause of significant GERD is inappropriate transient LES relaxations (TLESRs).

164

- Substances that have been implicated include smoking, alcohol, coffee, chocolate and excess dietary fat intake. The role of Helicobacter pylori (H. pylori) is uncertain.

Causes of GERD (Table 1)

Table 1: Primary and secondary causes of gastro-esophageal reflux disease (GERD)

Primary Causes Secondary Causes

1. Decreased LES pressure 1. Poor esophageal motility 2. Decreased total LES length (<2–3 cm) 2. Excess gastric acid production 3. Decreased intra-abdominal LES length (<1–2 cm) 3. Impaired gastric emptying 4. Loss of angle of His 4. Excess duodeno-gastric reflux 5. Sliding hiatus hernia 5. Decreased saliva production 6. Diaphragmatic crural defect 7. Loss of mucosal rosette (e.g. due to inflammation)

Clinical Features of GERD - Typical symptoms include heartburn, retrosternal or epigastric pain, regurgitation and water brash, dysphagia and odynophagia (painful swallowing). - Atypical symptoms include back pain, cardiac-type chest pain, chronic wheeze or asthma (especially at night), sore throat, hoarse voice, halitosis or dental decay.

Investigations

Endoscopic Examination - It is the mainstay of investigation for GERD, allowing direct visualization of the lining of the esophagus and stomach. - Esophageal abnormalities that can be diagnosed at endoscopy include hiatus hernia (HH), esophagitis, Barrett‘s esophagus, strictures and tumors. - The severity of reflux esophagitis can also be graded at endoscopy, and biopsies can be taken from the mucosa to facilitate histological examination of the tissues. - Other diagnostic techniques employed at endoscopy include brush cytology and washings, vital staining and fluorescence detection to ↑ recognition of minor mucosal abnormalities.

Radiological Contrast Studies - Barium swallow may show structural abnormalities (e.g. strictures, tumors and diverticula). It also yields information regarding esophageal function and the competence of the LES. - Video fluoroscopy allows dynamic visualization of esophageal function and can demonstrate reflux of contrast up into the esophagus. - Although radiological contrast studies have been largely superseded by endoscopy, barium swallow and video fluoroscopy still have a role, especially where esophageal function needs to be demonstrated and in patients who refuse endoscopy.

165

Esophageal pH Measurement - Intra-esophageal pH measurement is performed as an ambulatory test over a 24-hour period. This involves the passage of a fine pH probe into the lower esophagus, which is connected to a small recording device worn by the patient. - This makes it possible to measure the duration, frequency and severity of reflux attacks, and it is now the ‗gold standard‘ means of quantifying the degree of acid reflux.

Esophageal Manometry - Using this technique, it is possible to determine the length and position of the LES and to measure the LES pressure. - A short or weak LES is often a major cause of reflux esophagitis. A "LES" pressure of < 4 mmHg is abnormal and may be an indication for surgery. It is also possible to measure the resting and maximum contracting pressures at various levels within the esophagus and to study the propagation of peristaltic waves down the esophagus.

Classification of Reflux Esophagitis (Endoscopic Examination)

Savary-Miller Classification This was devised in 1967 by Savary and Miller, who graded the degree of esophagitis into grades 1 to 4, based on the extent of the inflammation and/or erosions, together with the presence or absence of complications, such as scarring, strictures or columnar metaplasia as shown in Table 2.

Table 2: Savary-Miller classification (grading) of reflux esophagitis Grade Description Grade 1 Erythema or diffusely red mucosa; edema causing accentuated fluid loss Grade 2 Isolated round or linear erosions extending from the gastro-esophageal junction in relation to the folds Grade 3 Confluent erosions extending around the entire circumference or superficial ulceration, but without stenosis Grade 4 Complicated: erosions (as described above) plus deep ulceration, stricture or columnar-lined esophagus

Los Angeles Classification The Los Angeles Classification represents a further attempt to subdivide uncomplicated reflux esophagitis into 4 grades based on both the length and width of the lesions as shown in Table 3.

Table 3: Los Angeles Classification (grading) of reflux esophagitis Grade Description Grade A One (or more) mucosal break(s) no longer than 5 mm that does not extend between the tops of 2 mucosal folds Grade B One (or more) mucosal break(s) longer than 5 mm that does not extend between the tops of 2 mucosal folds Grade C One (or more) mucosal break(s) that is continuous between the tops of 2 or more mucosal folds, but which involves < 75% of the esophageal circumference Grade D One (or more) mucosal break(s), which involves at least 75% of the esophageal circumference.

166

Complications

The 2 most common complications are benign stricture and Barrett‘s esophagus.

1. Benign (Peptic) Strictures - These occur in inadequately treated patients. - They present with dysphagia and endoscopy is essential to exclude malignancy. - Treatment is by endoscopic dilatation followed by either long-term proton-pump inhibitors (PPI) or anti-reflux surgery to prevent recurrence.

2. Barrett’s Esophagus - It is the replacement of normal squamous epithelium by metaplastic columnar epithelium due to long-standing reflux. Its importance lies in its malignant potential. - In order to diagnose Barrett‘s esophagus, intestinal metaplasia must be identified histologically. - Treatment is a combination of acid suppression and life-long endoscopic surveillance. Anti-reflux surgery may have a role and endoscopic ablation techniques have produced encouraging early results.

Hiatal (Hiatus) Hernia (HH)

Definition - It is the "protrusion (or herniation) of the upper part of the stomach into the chest cavity through the esophageal hiatus because of a tear or weakness in the diaphragm".

Classification - Four types of esophageal hiatal hernia are identified (Table 4) (Figure 1)

Type of HH Description

Type I HH (Sliding) It is the cephalad displacement of the gastro-esophageal junction (GEJ) (85%) through the hiatus into the mediastinum. It is usually small, asymptomatic and reducible. Commonly associated with GERD.

Type II HH (Rolling) It is superior migration of the fundus of the stomach alongside the GEJ and (10%) = para- esophagus into the mediastinum with GEJ in normal intra-abdominal esophageal location. May present by abdominal and chest pain, hiccough, early satiety, regurgitation, post-prandial bloating, arrhythmia, dysphagia & dyspnoea. About 40% of patients present with acute features of perforation/gangrene/ bleeding. Type III HH It is combination of type I and type II Type IV HH HH containing other abdominal viscera e.g. transverse colon and omentum

167

Figure 1. Hiatus hernia: sliding (Type 1) & para-esophageal (rolling) (Type 2)

Management

General Measures - Avoidance of certain foods and drinks (alcohol, coffee, chocolate and fatty or spicy foods). - Diet (weight loss). - Cessation of smoking (because smoking has a potent effect at lowering LES pressure) - Other lifestyle changes include avoidance of tight abdominal garments, alteration of eating habits (change in timing, type and volume of meals) + elevation of the head of the bed.

Medical Therapy - Antacids act by neutralizing the acid pH within the stomach & lower esophagus, but their benefits are often short-lived.

- H2-receptor antagonists (e.g. Cimetidine, Ranitidine, Famotidine, Nizatidine) have been largely superseded by PPIs, which have a more powerful acid-suppressant effect. - Proton-pump inhibitors (PPIs) (e.g. Omeprazole, Lansoprazole, Pantoprazole, Rabeprazole) are powerful acid suppressants and now form the mainstay of R/ for GERD. • They block the proton pump within the mucosal cells of the stomach lining, thus inhibiting the active transport of hydrogen ions across the membrane and thereby almost completely abolishing gastric acid production. Their clinical effect better than

H2-receptor antagonists, leading to better acid suppression and more effective symptom control and healing of ulcerative esophagitis in most patients. A full-dose therapy should be started until the esophagitis has healed, followed by long-term maintenance therapy using a reduced (usually half-strength) dose. • Recently, a more powerful ‗second generation‘ PPI has also been introduced. Esomeprazole is the s-isomer of Omeprazole & has even greater & more long-lasting anti-secretory activity. At present, it is largely used in more severe disease (grades III and IV) or as 2nd-line treatment where ‗first generation‘ PPIs failed to control symptoms.

168

- Mucosal protection agents e.g. Sucralfate help to protect against other (non-acidic) agents in the refluxate. However, sucralfate has an unpleasant taste and may produce constipation. - Prokinetic agents e.g. Metoclopramide, Domperidone and Erythromycin, improve gastric emptying and clearance of contents from lower esophagus, but are of little benefit in the treatment of reflux esophagitis - Eradication of H. pylori infection: There is little evidence that this is of any benefit in the treatment of reflux esophagitis & is not currently recommended as standard treatment.

Surgical Treatment

Advantages - Anti-reflux surgery is the most effective long-term treatment for GERD as it prevents the reflux of all gastric contents. - Surgery also avoids the problem of poor patient compliance with long-term medication. Side-effects of surgery: - Dysphagia (usually short-lived), inability to belch, the so-called ‗gas bloat‘ syndrome and excessive flatus. Indications 1. Failure of medical treatment (persistent symptoms or complications despite full-dose PPIs). 2. Complications e.g. ulceration, stricturing, Barrett‘s esophagus and respiratory problems, such as wheezing or coughing. 3. Age: Many patients, especially the young, prefer surgery to prolonged lifelong medication. Pre-operative assessment 1. Endoscopic evaluation to assess the degree of esophagitis, note the size of HH if present and, more importantly, to exclude other UGI pathology that may mimic symptoms of GERD 2. Esophageal manometry to assess position and function of LES, contractility of the esophageal body musculature and propagation of peristalsis. It is therefore important to exclude achalasia and other esophageal motility disorders. In some centers, the type of anti-reflux surgery is tailored according to the manometric assessment. Types of anti-reflux surgery Many types of anti-reflux surgery have been described and most lead to excellent results. Trans-abdominal and trans-thoracic routes have been widely adopted, and both can be performed through either open or minimal access (laparoscopic/thoracoscopic) techniques, (Figure 2). 1. Nissen (360 o) fundoplication: It is the ‗gold standard‘ trans-abdominal operation, in which the fundus of the stomach is wrapped completely around the lower esophagus, following a posterior hiatal repair. The Nissen procedure is very effective at controlling reflux, but it is easy to make the wrap too tight and thus produce dysphagia and gas bloat

169

. 2. Partial fundoplication: In an attempt to ↓ dysphagia, various partial fundoplications have been described. The rationale behind these procedures is to create a sufficient wrap to prevent reflux but not so tight. The major modifications in use are the Lind (270o), anterior partial (e.g. Watson), posterior partial (e.g. Toupet) and sub-total fundoplications. Although the use of a partial fundoplication may ↓ the risk of dysphagia, concerns have been expressed over the long- term durability of these procedures. 3. Other less common trans-abdominal approaches: They include the Hill posterior gastropexy, and Collis–gastroplasty. The latter is particularly useful in patients in whom the gastro-esophageal junction (GEJ) cannot be reduced below the diaphragm. 4. Trans-thoracic approach: The most common is the Belsey Mark IV procedure. Here the lower esophagus is mobilized up to the level of the aortic arch and is then sutured to the gastric fundus and to the diaphragm, to ↓ any hiatus hernia & wrap the fundus over the front of the esophagus.

Figure. 2. Different types of anti-reflux surgery

Results (outcome) of surgery - Anti-reflux surgery produces effective symptom control in 85-90% of patients. - The major side-effects include persistent heartburn (5-8%), dysphagia (3-8%), gas bloat and inability to belch and/or vomit. - Other less common complications include esophageal perforation, pneumothorax, chest infection, splenic damage and deep vein thrombosis (DVT).

171

CHAPTER 2 Complications of Peptic Ulcer Disease

HEMORRHAGE

Peptic ulceration is responsible for about 44% of all upper gastro-intestinal (GI) hemorrhage. One-third of patients with peptic ulcer (PU) hemorrhage have no previous history or diagnosis of PU disease. Hemorrhage is the leading cause of death due to PU disease with 5-10%. Bleeding usually occurs from posteriorly located duodenal ulcers, within 2 cm from the pylorus and typically erodes the gastroduodenal artery.

Clinical Assessment

- Presentation is by hematemesis, melena or rarely, in severe bleeding, bleeding per rectum. - Hypovolemic shock, in severe cases. - Abdominal examination is normal

Management

- Assessment and resuscitation 1. Shocked patients should receive prompt volume replacement and anti-shock measures. 2. Red blood cell (RBC) transfusion should be considered after loss of 30% of circulating blood volume 3. Correction of coagulopathy - Insertion of a nasogastric (NG) tube and lavage. - Although spontaneous cessation of bleeding occurs in 70% of patients, endoscopy is still indicated as the first-line investigation in order to identify and often treat the bleeding point. It should be performed within 24 hours of the initial presentation, especially in patients with hypotension, age older than 60 years and multiple co-morbidities. - Laboratory findings: anemia - Imaging: non-specific

171

Major Endoscopic Stigmata of Recent Hemorrhage (Table 1)

Table 1: Endoscopic stigmata of recent hemorrhage

Stigmata Risk of Rebleeding 1. Pulsatile or oozing hemorrhage >85% 2. Fresh clot on ulcer 85% 3. Adherent clot on ulcer 50% 4. Visible vessel in base of ulcer 40%

Endoscopic Control of Hemorrhage In the presence of major signs of recent hemorrhage, endoscopic therapy significantly reduces the risk of re-bleeding. Methods include the following: - Injections 1. Adrenaline: typically, a 0.5-1 ml of diluted adrenaline (1:100000) into each of the four quadrants of the ulcer base. 2. Ethanol 3. Variceal sclerosants (sclerosing agents) - Heater probe - Clips - Bipolar electro-coagulation - Nd-YAG laser

Pharmacological Therapy In conjunction with endoscopic therapy, pharmacological therapy is started: - Acid suppression by proton-pump inhibitors (PPIs) to maintain intra-gastric pH > 6 to stabilize clots and prevent rebleeding. Omeprazole 80 mg bolus injection is administered followed by 8 mg/hour intravenous (IV) infusion for 72 hours. - Tranexamic acid - Eradication H-pylori infection

Indications of Emergency Surgical Treatment of a Bleeding PU - Continuous bleeding e.g. spurting vessel at endoscopy that does not stop with adrenaline - Rebleeding in hospital - Transfusion requirements of 4-6 units in 24 hours

Principles of Emergency Surgical Treatment of a Bleeding PU Bleeding duodenal ulcer - Exploratory midline incision - Duodenotomy - Stop bleeding by under-running the bleeding vessel with a suture. - Ligation of the gastroduodenal artery - Definitive treatment by truncal vagotomy and pyloroplasty if the patient‘s condition permits. Bleeding gastric ulcer - Antrectomy and gastro-jejunostomy to prevent re-bleeding and exclude malignancy.

172

PERFORATION

Perforation of PU is usually related to non-steroidal anti-inflammatory drug (NSAID) intake. It usually occurs in the anterior aspect of duodenal ulcers. It is more common in males than females, and more common in duodenal ulcers than gastric ulcers.

Clinical Features (Peritonitis and Sepsis)

- Sudden severe epigastric pain (acute abdomen) then becomes diffuse abdominal pain. - Starts as chemical peritonitis. After 12 hours, bacterial peritonitis develops. - Sepsis: fever, tachycardia - Abdominal examination: tenderness, rebound tenderness, board-like rigidity, silent abdomen. - 50% of patients have no history of peptic ulcer disease. - The perforation may become sealed by omentum and intestinal loops and the condition improves. These patients can be managed conservatively. - Fluid may track along the right paracolic gutter to the right iliac fossa causing pain in the right iliac fossa

Investigations

- Laboratory findings: leukocytosis - Plain X-ray abdomen standing: free air under right copula of diaphragm (Figure 1). - Ultrasound (US): large amount of free intra-peritoneal fluid collection. - Computed tomography (CT) scan: pneumo-peritoneum. - Endoscopy and barium meal are contraindicated.

Figure 1: Plain X-ray abdomen, erect position, showing air under right copula of diaphragm (arrow)

173

Pre-operative Preparation

- Nothing per mouth - Vigorous fluid resuscitation - Monitoring urine output - Correction of electrolyte imbalance - Nasogastric (NG) tube - Broad-spectrum Antibiotics - Analgesia e.g. opioids - Central venous pressure (CVP) measurement - Proton pump inhibitors (PPIs)

Surgical Treatment of Perorated Duodenal Ulcer

- Laparotomy: Midline exploratory incision - Peritoneal lavage/toilets to remove all toxic fluid and food debris. - Omental (Graham) patch to reinforce the approximated edges of the ulcer (Figure 2) - The operation can be performed laparoscopically.

Figure 2: Omental patch for treatment of perforated duodenal ulcer

Surgical Treatment of Perforated Gastric Ulcer

Options include the following: - Simple closure - Wedge resection - Biopsy - Antrectomy and gastro-jejunostomy

174

GASTRIC OUTLET OBSTRUCTION (PYLORIC STENOSIS)

In a chronic process, duodenal ulcer can cause stricturing and narrowing of the first part of the duodenum, leading to gastric outlet obstruction, but an obstructing tumor is a much more common cause nowadays.

Clinical Presentation

- Long history of peptic ulcer disease - Projectile non-bilious vomiting of undigested bad odor food. - Epigastric pain - Weight loss and dehydration - Electrolyte disturbances: 1. Hypochloremic metabolic alkalosis 2. Hypokalemia 3. Hypocalcemia and tetany. - Inspection: Visible peristalsis - Palpation: 1. Palpable distended stomach 2. Succession splash

Investigations

- Laboratory findings: anemia, hypo-albuminemia, metabolic alkalosis, hypokalemia, elevated urea and creatinine levels. - Imaging 1. Barium enema reveals a markedly distended stomach that may reach the pelvis with a characteristic soap-dish appearance (Figure 3). 2. CT scan with IV contrast - Endoscopy and biopsy: usually the scope cannot pass to the duodenum.

Figure 3: Barium meal showing markedly dilated stomach (soap-dish appearance)

175

Management

- Exclusion of malignancy by CT scan, endoscopy and biopsy from the stricture. - In mild cases, inflammation and partial obstruction may resolve with gastric lavage, acid suppression therapy and antispasmodics - In severe cases, a hard fibrotic stricture develops causing a dilated atonic stomach.

Pre-operative Preparation - Correction of fluid and electrolyte abnormalities by normal saline and potassium. - Nasogastric lavage - Proton pump inhibitors

Surgical Treatment - Truncal vagotomy and a drainage procedure either gastro-jejunostomy (Figure 4) or pyloroplasty. - Endoscopic balloon dilatation, but it has inferior results than surgery.

Figure 4: Simple loop gastro-jejunostomy (the stomach is joined to the jejunum)

OTHER COMPLICATIONS

1. Hour-glass deformity of the stomach 2. Recurrence of ulceration

176

CHAPTER 3 Gastric Neoplasms

BENIGN GASTRIC TUMORS & POLYPS

The widespread use of endoscopy has increased the frequency of detection of gastric wall lesions in symptomatic & asymptomatic patients.

Benign Gastric Tumors

- 40% mucosa-based - 40% muscularis-based

Gastric Polyps

Types 1. Hyperplastic (regenerative) polyps - These are the commonest (80%) and occur in association with gastritis and peptic ulcer (inflammatory origin). 2. Adenomatous polyps - True gastric adenomas are rare (only 5% of gastric polyps). - The majority lie in the antral lesion - May be sessile, villous, pedunculated, or lobulated. - May be single or multiple - The progression of a gastric polyp into carcinoma is very rare (contrary to what occurs in the colon). 2. Inflammatory fibroid polyps: rare found in the gastric antrum. 3. Myoepithelial hamartomas (Polyps of Peutz-Jeghers syndrome) 4. Heterotopic pancreatic tissue.

Treatment - Endoscopic excision of gastric polyps provides a minimally invasive approach to diagnosis and treatment. Polyps < 2 cm are easily snared. - Larger polyps or sessile polyps are best removed operatively to obtain a clear margin & complete removal - Malignant polyps  treat as carcinoma

177

GASTRIC CANCER

Types of Gastric Cancer include adenocarcinoma (94%), lymphoma (4%) & GIST (Gastrointestinal Stromal Tumors) (1%). The typical patient with gastric cancer is a male (male-to-female ratio, 2:1) & 40-70 y (The disease is rare before the age of 40 y).

Adenocarcinoma of the Stomach

Etiology (Risk Factors) 1. Diet (major role): Geographic areas with high consumption of salty & smoked foods (containing polycyclic hydrocarbons, nitrosamines). Hypothesis: ↑ dietary nitrates  bacterial proliferation in an achlorhydria stomach ↑ N-nitrosamine production  stomach cancer. NB. Raw vegetables, vitamin C & antioxidants are protective 2. Cigarette smoking: It lowers vitamin levels. 3. Male gender, black race, low socioeconomic class (this applies to USA population) 4. Occupational hazards: miners, metal workers exposed to dusts of asbestos and wood. 5. Blood group A (relative risk = 1.2 times). 6. H-pylori infection (gram –ve microaerophilic bacterium in gastric pits)  ↑ incidence. 7. Gastric polyps: Villous adenomatous polyp (2%) has a malignant potential. 8. Pernicious anemia (10% risk of gastric carcinoma). 9. Chronic GU: It carries a small risk 10. Gastrectomy (gastric dumping syndrome) 0-5 times the risk (15 or more years). 11. Chronic atrophic gastritis: It is the most researched risk factor in gastric carcinoma due to: - The incidence of atrophic gastritis in patients with pernicious anemia is 3-6 folds ↑. - Several risk factors mentioned above are thought to act by causing damage to the gastric mucosa with subsequent development of atrophic gastritis. - There is now increasing evidence for the progression of gastric atrophy to intestinal metaplasia, dysplasia & carcinoma in-situ.

Site of Stomach Cancer - Antrum/lower 3rd: most predominant (40%) - In the last decades, proximal cancers (cardia a GE junction) have risen from 10% to 30% of gastric cancers. This is because distal tumors are affected by altering the risk factors. There is also ↑ in tumors in the cardia region associated with gastro-esophageal reflux. - Multicentric in 20% of cases

Spread of Gastric Carcinoma Direct extension - To the omentum, transverse colon and mesocolon and left lobe of liver. - Extension of antral tumors to the duodenum. - Extension of upper third tumors to the esophagus. Lymphatic spread - To LNs along the greater & lesser curvature, then to the celiac LNs, to the splenic hilum & root of mesentery, to retro-peritoneal & hepato-duodenal LNs & then to para-aortic nodes. Blood spread: Mainly to the liver and peritoneal cavity.

178

Pathology

Gross appearance 1. Polypoid 2. Fungating 3. Ulcerative 4. Scirrhous

Histologic types (Lauren, Finnish or DIO classification)

Diffuse (D) Intestinal (I) Mixed (O) Young age Old age No history of chronic gastritis History of chronic gastritis have a mixed Arise from normal gastric mucosa & not Surrounded with intestinal metaplasia morphology associated with intestinal metaplasia or between both other precancerous conditions (D & I) No gland formation Gastric cancer cells resemble intestinal cells with gland formation Infiltrates the gastric wall without Form localized expanding or forming discrete masses. ulcerating lesions Less differentiated (signet ring cells) Tendency to be well differentiated.

Spreads by lymphatics & transmural Spreads hematogenously Genetic predisposition No genetic predisposition Linitis plastic, signet ring carcinoma Fungating or polypoid masses Association with blood group A No association Penetrates the gastric wall at an early Tends to be localized with less stage tendency to disseminate Worse prognosis Better prognosis

Clinical Presentation 1. Vague postprandial fullness, indigestion & malaise. 2. Recent dyspepsia in a patient >50 years and receiving treatment as benign ulcer for 6-12 months without investigations. 3. Anorexia and loss of weigh denote advanced disease. 4. Epigastric mass (25% of cases): It usually denotes incurability but not irresistibility. 5. Anemia (Fe deficiency type due to chronic blood loss) 6. Complications a. Vomiting (gastric outlet obstruction by a pyloric tumor) b. Dysphagia (due to a tumor in the cardia) c. Acute presentation: hematemesis and melena (in advanced cases) d. Presentation with metastases: - To the neck (Virchow node). - To the pelvis (Blumer shelf). - To the ovaries (Krunkenberg tumors). - To the peritoneum (ascites).

179

Investigations

Laboratory - Anemia (in 40% of cases). - CEA (↑ in 65% of cases). - Liver function tests (LFTs). - Electrolyte panel. Imaging studies - Barium meal: false negative (20%) especially in ulcerating tumors. - Chest X-ray: for detection of extra-gastric lesions. - Liver US & abdominal CT scan: for determining the extent of the tumor, para-aortic LNs involvement & hepatic secondaries. - EUS: (endoscopic US) to assess depth of the tumor (T) & detect enlarged perigastric & celiac lymph nodes (LNs) Gastroscopy and Biopsy - Multiple mucosal biopsies; 6 in number, should be taken Laparoscopy - To assess the stage and the curability of the disease.

AJCC Classification "Staging of Gastric Carcinoma"

Tumor (T) Nodes ( N) Metastases (M)

T1 Limited to mucosa and No No metastases to LNs. M0 No metastases. submucosa. N1 Metastases to peri-gastric LNs M1 Distant T2 Extends to the serosa. within 3 cm of the 1ry tumor. metastases T3 Penetrates the serosa. N2 To LNs > 3cms from the 1ry tumor T4 Invades the adjacent (left. gastric, splenic, celiac LNs). organs. N3 LNs in porta hepatic, behind pancreatic head, root of mesentery and around middle colic vessels.

Treatment

Surgical treatment: is the only curable treatment Objective Removal of the tumor + uninvolved stomach margin (minimum 6 cm) and duodenum + regional LNs (corresponding to the location of the tumor) + adjacent organs (if involved).

- Antral tumor: Distal gastrectomy+ omentectomy + 3-4 cm duodenum + subpyloric LNs + reconstruction with GJ or gastro-duodenostomy. - Body tumor and extensive tumors: Total gastrectomy + Splenectomy (debatable) + reconstruction (pouch or Roux en Y esophago-jejunostomy). - NB: Routine splenectomy does not improve survival. Thus, splenectomy should not be performed unless there are adhesions with the tumor to or infiltration of the spleen. - Cardia tumor: Esophago-gastrectomy (via 2 incisions)

181

Japanese classification of gastric resections for cancer based on radicality (R)

R0 Gastrectomy with incomplete removal of N1 nodes. R1 Gastrectomy with complete removal of N1 nodes (along the lesser & greater curvatures). R2 Gastrectomy with removal of N2 nodes (LNs along the main arteries: left gastric, common hepatic, splenic, celiac). R3 Gastrectomy with removal of N3 (LNs in the porta hepatic, behind the head of pancreas, root of mesentery, around the middle colic vs.). R4 Gastrectomy with removal of N4 nodes

Palliative resection - If life expectancy is > 2 ms, palliative gastrectomy is better to be done than palliative GJ. - For inoperable cases of cardiac tumors: Intubation with a Celestin tube is done to overcome the dysphagia. Adjuvant chemotherapy - Not of much value (response is only 20%) - Regimens used: 5 FU or Adriamycin or Mitomycim or a combination (FAM)

Non-epithelial Gastric Tumors

Gastric Lymphomas All are non-Hodgkin type (B cell mucosa associated with MALT lymphoma) Clinical presentation - Bulky tumor: palpable mass (50%) when discovered versus 25% in adenocarcinoma). - Symptoms are mild in relation to tumor size, but my present with bleeding & perforation. Diagnosis - Barium meal + CT scan (for staging) - Gastroscopy with biopsy & brush cytology. Treatment - Low-grade lymphoma  Cyclophosphamide. - High-grade lymphoma  surgical resection (this gives the best results + total abdominal radiation + intra-operative staging (Needle biopsy both hepatic lobes. Celiac & para-aortic LN biopsy, splenectomy if directly invaded) + post-operative chemotherapy (patients with high risks of recurrence). The 5-y survival is 50%.

GIST (Gastrointestinal Stromal Tumors) - Definition: These are mesenchymal neoplasms derived from interstitial cells of Cajal (pacemaker cells controlling peristalsis). - Pathology: GISTs are large tumors with high mitotic rate and tend to behave malignantly. Stomach is the commonest site. - Treatment: GIST is the only type that has a real systemic medical R/ because of the expression of KIT (tyrosine kinase receptor). Clinical trials have shown partial response rates in patients treated with Imatinib for advanced, recurrent or metastatic GISTs. It has been used in the pre-operative R/ to ↓ tumor size before surgical resection

Gastric Carcinoids ………. Extremely RARE

181

CHAPTER 4 Small Intestinal Tumors

The small bowel is the least common site for primary cancer. The small bowel contains > 90% of the mucosal surface area of the gastro-intestinal (GI) tract, but only constitute 1.1- 2.4% of all GI primary malignancies. The incidence of small intestinal tumors decreases from duodenum to ileum.

RISK FACTORS OF DEVELOPING SMALL INTESTINAL CANCER

1. Ingestion of smoked or cured foods 2. Consumption of red meat 3. Celiac sprue 4. Crohn‘s disease 5. Peutz-Jeghers syndrome 6. Hereditary non-polyposis colo-rectal cancer 7. Familial adenomatous polyposis (FAP) 8. Acquired immunodeficiency syndrome (AIDS)

CLASSIFICATION OF TUMORS OF THE SMALL INTESTINE

A. BENIGN TUMORS OF THE SMALL INTESTINE

Benign neoplasms of the small bowel account for 30-50% of small bowel tumors. They are most frequently encountered in the duodenum as incidental findings during endoscopic examinations.

Adenoma

Adenomas are the most common neoplasms of the small intestine, and are mostly encountered in the duodenum. Adenomas have different types that include: 1. Tubular adenoma: low malignant potential, pedunculated and presents with bleeding. Endoscopic polypectomy is the treatment of choice. 2. Villous adenoma: sessile lesion with high malignant potential, mostly encountered in the peri-ampullary area. Treatment is by endoscopic polypectomy for benign lesions, or surgical local excision. 3. Tubo-villous adenoma: similar to villous adenoma 4. Bruner‘s gland hyperplasia: hyperplasia of the gland of the first part of the duodenum is a benign condition, treated by endoscopic polypectomy

182

Hamartoma

- Small intestinal hamartomas are usually a part of Peutz-Jeghers syndrome, characterized by familial intestinal hamartomatous polyposis (multiple polypi in the jejunum and ileum 25% gastric, 50% colonic), melanosis of oral mucous membrane, lips as well as palmar and plantar surfaces, intermittent intussusception, and bleeding. - Rare malignant potential - Increased incidence of ovarian, breast, endometrial, and pancreatic tumors. - Treated by surgical resection or endoscopic polypectomy

Hemangioma

- Present by bleeding, occult or overt. - Diagnosed and treated by selective angiography and surgical resection

B. PRIMARY MALIGNANT TUMORS OF THE SMALL INTESTINE

Primary malignant small intestinal neoplasms are summarized in Table 1, which also shows their incidence and predominant site.

Table 1: Primary malignant small intestinal neoplasms

Tumor Incidence Predominant Site 1. Adeno-carcinoma 40-50% Duodenum 2. Carcinoid tumor 20-40% Ileum 3. Lymphoma 15-20% Ileum 4. Gastro-intestina stromal up to 15% No regional variation tumor (GIST)

C. PRIMARY MALIGNANT TUMORS OF THE SMALL INTESTINE

- The small bowel is frequently affected by metastases either from direct extension (local invasion by cancers), intra-peritoneal carcinomatosis, or hematogenous spread. - Common primary malignancies that can give small intestinal metastasis include malignant melanoma (commonest), ovarian, bladder, breast or lung cancer

CLINICAL PRESENTATION OF SMALL INTESTINAL TUMORS

- The prevalence of small bowel tumors identified at autopsy is 0.2-0.3%, which is significantly higher the rate of operations for small bowel tumors. - This suggests that the majority of small bowel tumors are asymptomatic. - The most common signs and symptoms of small intestinal neoplasms include the following: 1. Abdominal pain (67%) 2. GIT bleeding (occult, melena or manifest bleeding) (41%). 3. Weight loss (38%).

183

4. Nausea and vomiting (33%). 5. Bowel obstruction (13%). 6. Intussusception (in adults, usually benign) 7. Palpable mass (7-18%) (GIST) 8. Perforation (25%) (lymphomas) 9. Painless jaundice (peri-ampullary carcinoma)

DIAGNOSIS OF SMALL INTESTINAL TUMORS

Laboratory Tests

- Laboratory tests are non-specific, with the exception of elevated serum 5-hydroxyindole acetic acid levels in patients with the carcinoid syndrome. - Elevated CEA levels are associated with small intestinal adeno-carcinomas, but only in the presence of liver metastases.

Imaging Studies

Plain Abdominal Radiographs - Plain X-rays of the abdomen may show an associated small bowel obstruction, or rarely a calcified abdominal mass

Contrast (Barium) Radiography of the Small Intestine - Contrast radiography of the small intestine may demonstrate benign and malignant lesions. - Enteroclysis is reported to have a sensitivity of over 90% in the detection of small bowel tumors and is the test of choice, particularly for tumors located in the distal small bowel. - Upper GI with small bowel follow-through examinations have reported sensitivity of only 30%-44%.

Computed Tomography (CT) Scan - Magnetic Resonance Imaging (MRI - CT scan has a low sensitivity for detecting mucosal or intra-mural lesions, but can demonstrate large tumors and is useful in the staging of intestinal malignancies. Sensitivity depends on tumor location 100% in the duodenum, 17% in the jejunum and 70% in the ileum. - CT enteroclysis (helical CT with enteroclysis)

Bleeding scan and angiography - Tumors associated with significant bleeding can be localized with angiography or radioisotope-tagged RBC scans.

Endoscopic Studies - Biopsy

- Upper GI endoscopy: Tumors located in the duodenum can be visualized and biopsied on upper GI endoscopy.

184

- Endoscopic ultrasonography: It can offer additional information such as the layers of the intestinal wall involved by the lesion. - Colonoscopy: It can occasionally visualize the distal ileum successfully. - Intra-operative enteroscopy: It can be used to directly visualize small intestinal tumors beyond the reach of standard endoscopic techniques. - Capsule endoscopy and double-balloon endoscopy have been recently used to evaluate the small bowel.

MALIGNANT SMALL BOWEL TUMORS

Adenocarcinoma

Incidence - Location: 40% in the duodenum – Peri-ampullary lesions. - Age: Mean age is 60 years - Gender: Male predominance (2:1)

Conditions which Increase the Relative Risk of Adenocarcinoma 1. Familial Adenomatous Polyposis (FAP) Coli and Gardner‘s syndrome. 2. Villous adenomas. 3. Hereditary non-polyposis colorectal cancer (Lynch II syndrome). 4. Inflammatory bowel disease (BD) (Crohn‘s disease and ulcerative colitis). 5. Peutz-Jeghers syndrome.

FAP and adenocarcinoma - Patients with FAP have a nearly 100% cumulative life-time risk of developing duodenal adenomas that have the potential to undergo malignant transformation. - The risk of duodenal cancer in these patients is over 100-fold that in the general population. Duodenal cancer is the leading cause of cancer-related death among patients with FAP who have undergone colectomy.

Crohn‘s disease and adenocarcinoma - Patients with Crohn‘s disease have a 12 fold increased risk of small bowel cancer than normal population. - Adenocarcinomas developing in Crohn‘s disease are most found in the ileum, and symptoms often mimic those of Crohn‘s disease. - Risk factors of developing adenocarcinoma in a patient with Crohn‘s disease include (1) long duration of disease, (2) male gender, (3) fistulas, (4) surgically excluded loops of small bowel, (5) strictures, and (6) immuno-suppressive drugs.

Management - How are Adenocarcinomas Treated? - Resection-anastomosis: For adenocarcinomas of the small bowel, a wide resection of the segment harbouring the tumor (5-cm safety margin distally and proximally) with wide excision of corresponding mesentery is done to achieve regional lymphadenectomy. Continuity of the small bowel is then restored by anastomosis. - Right hemicolectomy: This is done exclusively for adenocarcinomas of the distal ileum. 185

- Pancreatico-duodenectomy: It is performed in case of duodenal tumors. - Palliative Surgery: In the presence of locally advanced or metastatic disease, palliative intestinal resection or bypass (gastro-jejunostomy and choledocho-jejunostomy) is done. - Endoscopic stents: Endoscopic wall stent placement for duodenal obstruction and bile stent placement for obstructive jaundice - Chemical splanchnicectomy: Alcohol neurolysis (chemical splanchnicectomy) may be used for pain control - Chemotherapy has no proven efficacy in the adjuvant or palliative treatment of small intestinal adenocarcinomas.

Prognosis - Localised disease: 80% long-term survival. - Advanced disease: 10%-15% five-year survival

Carcinoid Tumor

- Carcinoid tumors arise from Kulchitsky cells in the crypts of Lieberkuhn (ECL cells) – Neuroendocrine cells - (APUD system) - They secrete several endocrine and vasoactive peptides e.g. Serotonin, Substance P, Dopamine, Histamine, Prostaglandins (E and F). - 85% of carcinoid tumors occur in the appendix, ileum and rectum, in order of frequency, and 30-40% have multiple synchronous lesions in the jejunum - The primary tumor is usually mostly asymptomatic, discovered incidentally - They may metastasize to the liver causing carcinoid syndrome; reddish blue cyanosis, flushing attacks, diarrhea, asthmatic attacks, pulmonary and tricuspid stenosis, right- sided heart failure. - Diagnosis is achieved by the demonstration of increased level of 5-HIAA in urine. - Non-carcinoid synchronous tumors are found in up to 25% of patients (typically tumors of the breast, lung, stomach, or colon). - Approximately, 25-50% of patients with carcinoid tumor-derived liver metastases will develop manifestations of the carcinoid syndrome - Treatment 1. For small bowel: surgical resection 2. For the appendix: appendicectomy (appendectomy) 3. For metastatic disease: enucleation or liver resection 4. Somatostatin analogue: Octreotide.

Small Intestinal GIST

- The terms leiomyoma and leiomyo-sarcoma have been replaced by a more general term, gastro-intestinal stromal tumor (GIST). - GISTs arise from the interstitial cell of Cajal, an intestinal pace-maker cell (theory). - GISTs may have myogenic and/or neural features, or they may lack differentiation. - They can present with benign or malignant characteristics. - Tumor markers for GIST are CD 117 and CD 34

186

Management of Small Intestinal GIST - Surgical resection of the entire tumor, along with local extensions and localized peritoneal or liver metastases, is the treatment of choice. - If diagnosis is known pre-operatively, segmental intestinal resection can be performed and wide lymphadenectomy can be avoided as GISTs are rarely associated with lymph node metastasis - GISTs are resistant to radiotherapy and chemotherapy, their role in adjuvant treatment is not defined. - The specific treatment of GIST is Tyrosine Kinase inhibitor (Gleevec) Imatinib, which is used with those with advanced or metastatic disease.

Important Markers of Prognosis - Tumor size. - Mitotic activity - Spread of the disease outside the bowel wall. - Poor histological grade. - Short time from symptoms to diagnosis.

Small Intestinal Lymphoma

- Lymphoma may involve the small intestine as primary neoplasms without systemic nodal disease or as secondary tumors as a part of disseminated lymphoma. - Primary GI lymphomas are the most common of extra-nodal lymphomas; most commonly located in the ileum (contains the highest concentration of lymphoid tissue). - Although partial small bowel obstruction is the most common mode of presentation, 10% of patients with small intestinal lymphoma present with bowel perforation. - Most are Non-Hodgkin‘s B-cell lymphomas with intermediate or high grade features.

What defines primary lymphoma of the gut ? 1. Absence of peripheral lymphadenopathy 2. Absence of mediastinal lymphadenopathy 3. Normal white blood cell (WBC) count 4. Absence of bone marrow lymphoma 5. At laparotomy, the disease is restricted to the bowel and proximal lymph nodes with no involvement of the liver or the spleen

Conditions with impaired mucosal barrier increase the risk of small bowel lymphoma? 1. Mal-absorptive or inflammatory conditions e.g. celiac sprue, Crohn‘s disease. 2. Helicobacter pylori infection → small bowel lymphoma 3. Immunosuppressed patients due to: - Pharmacological e.g. post-transplant immunosuppressants e.g. cyclosporine - HIV infection (AIDS): GI lymphomas (B-cell non-Hodgkin's lymphoma) and Kaposi‘s sarcoma can appear in any organ. - Diseases that cause immunological dysfunction e.g. rheumatoid arthritis - Congenital e.g. agammaglobulinemia and congenital immunodeficiency syndrome

187

Types of primary intestinal lymphoma They are Non-Hodgkin‘s lymphoma (NHL), mainly of the B-cell type: 1. Mature (peripheral) B-cell - Extra-nodal Marginal Zone B-cell lymphoma → (MALT Type). - Diffuse large B-cell→ High-grade and MALT type. - Mantle cell. - Burkitt‘s lymphoma 2. Mature T cell - Enteropathy type T-cell (EATCL)

Management of Small Intestinal Lymphomas - Localized small intestinal lymphoma should be treated with segmental resection of the involved intestine and adjacent mesentery. - If the small intestine is diffusely affected by lymphoma, chemotherapy rather than surgical resection should be the primary therapy. - Surgical resection for stage I or II lymphoma, debulking for large complicating tumors in stage III or IV - All patients receive chemotherapy - The role of radiation therapy is unclear

188

CHAPTER 5 Bariatric Surgery

MORBID OBESITY – CONCEPTS AND DEFINITIONS

Obesity has become one of the most widespread health problems in the world. Prevalence of obesity in USA increased from 12% to 21% between 1991 and 2001 = 15 million people. Obesity is the 2nd most common cause of death from a modifiable behavioural risk factor

Who is Considered Obese ?

Body Mass Index (BMI) Concept Weight measurement alone is not ideal for determination if the patient is obese or not; that is why the Body Mass Index (BMI) concept is used. - BMI is defined by the ratio of an individual‘s height to weight [BMI = body weight (Kg) / height (m2)]. - Normal: The normal BMI ranges from 20-25. - Morbid obesity: An individual is considered morbidly obese if he or she has a BMI of 40 or more, or 35 or more and experiencing obesity-related health conditions, such as diabetes mellitus (DM) and hypertension (HTN). - Super-obesity: Persons with BMI above 50 are considered Super Obese. Super-obesity should be dealt with as a special entity because of the following: 1. They suffer much more from obesity-related health problems. 2. They impose much higher risk to surgery and anesthesia. 3. They need special beds, chairs, trolleys, surgical tables and surgical instruments with extra-lengths. 4. They are better to be dealt with at dedicated institutions.

Apples versus Pears Concept Not only the absolute value of BMI is important, but also the distribution of fat. Having an apple-shaped body means that you carry a higher proportion of fat around your abdomen, whereas a pear-shape indicates you carry most of fat around your legs and hips (Figure 1). - For example, if you have a bit of a beer belly or a spare tyre around you, you are more of an apple shape! If you are wider around the hips you are a pear-shape. In general, being more pear-shaped means you are at lower risk of chronic disease than being an apple- shape and this mainly has to do with your visceral and subcutaneous (SC) fat storage.

189

- Visceral fat is the fat that sits deep in our abdomen and surrounds our organs, while SC fat is stored under our skins and is the fat that you can feel in the skin folds. - The visceral fat is the most dangerous and it increases our risks of type-2 DM, cardiovascular disease, hypertension, inflammation and other diseases.

Figure 1. Apples versus pears body types

1. COMMON OBESITY-RELATED CONDITIONS

Obesity is associated with the following conditions:

1. DM-Type 2: People who are obese become resistant to insulin, which regulates blood glucose levels leading to Type 2 diabetes. 2. HTN/heart disease: The obese person usually gets HTN, which results in stroke and damage to the heart and kidneys. 3. Osteoarthritis of weight-bearing joints: Additional weight placed on joints, especially knees and hips can cause rapid wear and tear, along with pain and inflammation. Similarly, the strain on bones and muscles in the back leads to disk problems, pain and reduced mobility. 4. Sleep apnea/respiratory problems: Fat deposits in the tongue and neck can block the air passages, especially in patients who sleep on their backs. This interferes with sleep leading to daytime drowsiness and headache. 5. Gasto-esophageal reflux disease (GERD) (hiatal hernia and heart-burn): Excess weight weakens and overloads the gastro-esophageal junction and thus allows stomach acid to escape into the esophagus (reflux) leading to heart-burn and acid indigestion. About 10- 15% of patients with even mild heart-burn develop Barrett's esophagus (a pre-malignant change in the lining membrane and a cause of esophageal cancer). 6. Depression: People who are obese must deal with constant, depressing emotional challenges. 7. Infertility: Obesity affects male and female hormones, disrupting normal cycles and function leading to difficulty or inability to conceive.

191

8. Urinary stress incontinence: A large, heavy abdomen relaxes the pelvic muscles, compounding the effects of childbirth. This weakens the urinary bladder sphincter, allowing leakage when coughing, sneezing, or laughing.

TREATMENT OF MORBID OBESITY

There are several arms for the management of the obesity problem: 1. Non-invasive: Exercise and medical therapy. 2. Invasive, non- surgical: Intra-gastric balloon insertion 3. Surgical: Bariatric surgery

Non-invasive Treatment

- Diet – low in calories, fat and carbohydrates - Exercise – 40 minutes, 5 times per week - Behavior Modification – eat 3 sensible meals per day, avoid snacks - Drugs/Prescription medications 1. Appetite suppressants 2. Anti-depressants 3. Drugs that reduce fat absorption Disadvantages of non-invasive therapy 1. Most patients (95-97%) regain most or all of the weight that was lost within 2-5 years following diet or drug treatment 2. The average amount of weight loss is relatively small (5-20 Kg) 3. Drug therapy may be associated with severe complications 4. Very difficult for most people to maintain these programs in the long-term 5. ―Yo-Yo‖ effect of many different programs leads to significant weight fluctuations

Intra-gastric Balloon Insertion

- This is balloon manufactured from non-irritant material to be placed in the stomach by the aid of upper gastrointestinal (UGI) endoscopy. After its placement in the stomach, it is inflated with methylene blue solution to a volume of 500-700 cc (Figure 2). - It decreases the functioning volume of the stomach in order to restrict food-intake. - It aims at a decrease of 25-30 kg of the patient‘s body weight - It has the advantage of being a simple and short procedure. - Its main disadvantage is that it must be removed after six months. Thus, most patients regain weight after its extraction. - It is very useful as an adjuvant procedure before operating on super-obese patients in order to obtain some weight reduction, which helps in improving their.

191

Figure 2. Intra-gastric balloon insertion for treatment of morbid obesity

Bariatric Surgical Procedures

Definition - The name bariatric comes from the Greek words baros – meaning weight and iatreia – meaning treatment - Bariatric surgery describes a group of operations performed primarily to reduce body weight (treat morbid obesity). This is typically achieved through inducing anatomical alteration to the gastro-intestinal tract (GIT). These operations aim at decreasing food reservoir, speeding food passage through the GIT and/or shortening the journey through the bowel.

Classification Bariatric operations are classified according to the mechanism of action into: 1. Restrictive Operations This group of operations works mainly by limiting food reservoir, which limits food- intake. Rapid food passage occurs in some of these operations. This, when present, acts as a 2ry mechanism. 2. Mal-absorptive Operations Here, the main mechanism of action is to induce malabsorption through short-circuiting of the food passage via ―bypassing‖ a variable length of the small intestine. All these operations have also a restrictive action added to it. Again, food passage is faster.

Indications 1. BMI >40 2. BMI >35 + one or more co-morbidity such as: - Osteoarthritis - Disc prolapse - DM type-2, dyslipidemia - Hypertension - Obstructive sleep apnea

192

Prerequisites for Having a Bariatric Operation 1. Age: 18-65 years (patients out of this range are only operated upon in special conditions) 2. Psychological stability and integrity. 3. Normal eating behavior 4. Normal endocrine function 5. Ability and willingness to attend for follow-up 6. Fitness for surgery and anesthesia 7. Failure of other measures of weight loss (diet and exercise)

Operative Procedures

Laparoscopic Sleeve Gastrectomy - This is a commonly performed restrictive operation - A narrow vertical pouch is designed along the lesser curvature of the stomach connecting the esophagus to the pylorus. The resulting 150 cc stomach pouch resembles a ―sleeve‖ hence the name ―sleeve gastrectomy‖ (Figure 3) - Advantages: laparoscopic sleeve gastrectomy does not involve malabsorption, abnormal tracts, blind tracts, or the placement of a foreign body Figure 3: Sleeve gastrectomy

Laparoscopic Gastric Bypass - These are a group of mal-absorptive operations in which the stomach is divided into 2 parts: a smaller proximal part that is anastomosed to the small intestine 1.5-3 meters distal to the duodeno-jejunal junction; and a larger distal part that is left attached to the duodenum through the pylorus (Figure 4) - This ―excluded‖ distal part of the stomach does not receive food. It only secretes acid and enzymes that flow distally

through the duodenum to meet the food at Figure 4. Laparoscopic Gastric Bypass the site of the gastro jejunostomy

193

Post-operative Care

Post-operative instructions - After surgery, patients should remain on a high-protein and low-fat diet and supplement their diet with multi-vitamins, iron and calcium, usually on a twice-daily basis. - Ursodiol may be given to minimize the risk of developing gallstones during the period of acute weight loss. - Patients must modify their eating habits by avoiding chewy meats and other foods that may inhibit normal emptying of their stomach pouch. Post-operative tests - Nutritional and metabolic blood tests must be performed on a frequent basis; at 6 months and 12 months after surgery, and then annually thereafter. Post-operative body contouring - Massive weight loss is associated with negative consequences for the body, such as flabby skin, abdominal skin overhang and pendulous breasts. - The excess skin does not regain the tightness it had before the weight gain. Redundant rolls of tissue may also be associated with intertrigo and significant hygiene problems. - Surgical correction of these body deformities can significantly enhance physical and physiological changes. Examples include abdominoplasty, buttock lift, thigh lift, mastopexy, etc. - The usual time lapse between gastric bypass and plastic surgery procedures is 12-18 months.

Complications of Bariatric Surgery 1. Heavy patients are prone to certain hazards due to their weight during transport, positioning on the operating table and during endotracheal intubation. 2. Delayed recovery from anesthesia with possible need for post-operative mechanical ventilation. 3. Increased risk of deep vein thrombosis (DVT). 4. Post-operative hemorrhage from the stapled cut-edge of the stomach. 5. Leakage of gastric or intestinal contents through a defective staple line or sutured anastomosis. 6. Intra-operative injury to the esophagus or spleen.

194

CHAPTER 6 Acute Abdomen and Peritonitis

DEFINITION OF ACUTE ABDOMEN

- Acute abdomen: Any serious acute intra-abdominal condition attended by sudden, spontaneous severe pain in the abdomen that is less than 24 hours in duration. - Acute surgical abdomen: Pain arising in a previously apparently healthy person which lasts more than six hours.

ETIOLOGY OF ACUTE ABDOMEN

Surgical Causes of Acute Abdomen (Figure 1) Gastro-intestinal Tract (GIT) Disorders - Non-specific abdominal pain (43%) - Appendicitis (24%) - Small and large bowel obstruction (5%) - Perforated peptic ulcer (3%) - Bowel perforation - Diverticulitis (2%) - Gastritis and gastroenteritis

Liver, Spleen and Biliary Tract Disorders - Acute cholecystitis (9%) - Acute cholangitis - Hepatic abscess - Ruptured hepatic tumour - Spontaneous rupture of spleen - Splenic infarct

Pancreatic Disorders - Acute pancreatitis (2%)

Urinary Tract Disorders - Renal colic (4%) - Renal infarct

195

Gynecological Disorders - Ruptured ectopic pregnancy - Twisted ovarian tumour - Ruptured ovarian follicle cyst - Pelvic inflammatory disease (PID)

Vascular Disorders - Ruptured aneurysm - Mesenteric vascular occlusion

Peritoneal Disorders - Peritonitis - Intra-abdominal abscess

Post-operative Causes - Bleeding - Anastomotic leak - Bowel obstruction - Abscess - Abdominal compartment syndrome

Figure 1. Common surgical causes of acute abdomen

196

Medical Causes of Acute Abdomen

Endocrine and Metabolic Disorders - Uremia - Diabetic crisis - Addisonian crisis - Acute intermittent porphyria - Hereditary Mediterranean fever

Hematological Disorders - Sickle cell crisis

Toxins and Drugs - Lead poisoning - Food poisoning

Infections and Inflammatory Disorders - Henoch-Schonlein purpura - Systemic lupus erythematosis (SLE) - Polyarthritis

Tropical Diseases - Typhoid - Malaria - Tuberculous (TB) peritonitis - Amebiasis - Worm infestations

Cardiac / Pulmonary Disorders - Pleurisy - Pneumonia - Cardiac disease (e.g. myocardial infarction - MI)

Nervous System Disorders - Disease of spine affecting nerve roots

Common Acute Abdominal Conditions in Infants and Children

- Acute appendicitis - Non-specific mesenteric adenitis - Intussusception - Intestinal volvulus - Meckel‘s diverticulitis

197

CLINICAL DIAGNOSIS

History-Taking / Symptoms

Abdominal pain - Characterizing the pain is the key: onset, duration, location, character, etc. 1. Visceral pain → dull, poorly localized (distension, inflammation or ischemia) 2. Parietal pain → sharper, better localized 3. Kidney / ureter → flank pain - Onset 1. Sudden (hemorrhage, perforation or torsion) 2. Gradual (inflammation) - Progression of pain (Figure 2)

Figure 2: Progression of pain in case of perforation, obstruction (colic) and inflammation

- Referral of pain The different sites of referred pain from various pathologic disorders are illustrated in Figure 3

Figure 3: Sites of referred pain from different pathologic disorders

198

GI symptoms 1. Nausea, vomiting (bilious or bloody) 2. Constipation is the rule in most acute abdomen (due to ileus or obstruction) 3. Diarrhea (?? bloody) 4. Both, nausea and diarrhea? Medical? 5. Change in symptoms with eating?

Constitutional symptoms 1. Fever 2. Chills 3. Jaundice (passage of clay stools and dark urine denotes obstructive jaundice)

History-Taking - Non-steroidal anti-inflammatory drug (NSAID) use (e.g. for duodenal ulcer) - Drinking alcohol (pancreatitis) - Prior surgeries (adhesions…) - Hernia (obstruction, strangulation) - Urine output (dehydration)

Common Causes of Pain According to Site

The following is an illustrated summary of the different causes of pain according to the site (location) of pain, whether generalized or localized (Figures 4-12).

- Perforation - Mesenteric ischemia - Abdominal Aortic Aneurysm (AAA) - Acute pancreatitis - DM

Figure 4. Common causes of diffuse (generalized) abdominal pain

199

Figure 5: Causes of central abdominal pain Figure 6: Causes of epigastric pain - Early appendicitis - Duodenal ulcer (DU) - Small bowl obstruction - Gastric ulcer (GU) - Acute pancreatitis - Esophagitis - Ruptured AAA - Acute pancreatitis - Mesenteric thrombosis - AAA - Acute gastritis

Figure 7: Causes of right upper quadrant pain Figure 8: Causes of left upper quadrant pain - Acute cholecystitis - GU - DU - Pneumonia - Acute pancreatitis - Acute pancreatitis - Retro-cecal appendicitis - Splenic rupture - Splenic abscess - Right pneumonia - Subphrenic abscess - Acute peri-nephritis - Subphrenic abscess

211

Figure 9: Causes of right iliac fossa pain Figure 10: Causes of right iliac fossa pain - Acute appendicitis - Diverticulitis – IBS - Constipation - Mesenteric adenitis - Meckel‘s diverticulum - PID - Perforated - DU - Diverticulitis - IBS - Rectal Cancer - PID - Ectopic pregnancy - Ureteric colic - Ureteric colic - Ectopic pregnancy

Figure 11: Causes of supra-pubic pain Figure 12: Causes of loin pain - Acute urinary retention - Muscle strain - UTI - Cystitis - UTIs - PID - Renal stones - Ectopic pregnancy - Pyelonephritis - Diverticulitis

211

Clinical Examination

- General examination - Vital signs - Abdominal examination - Examination of hernial orifices - PR examination - Scrotal examination.

INVESTIGATIONS

Laboratory Test

1. Complete blood count (CBC): Leukocytosis 2. Electrolytes: Vomiting causes acidosis and dehydration 3. Amylase: Pancreatitis 4. Liver function tests (LFTs): Jaundice – hepatitis 5. Urine analysis: UTI – stones - hematuria

Imaging Studies

1. Plain X-ray (supine and erect): Obstruction – free air – stones 2. Ultrasound: Cholecystitis – gynecological pathology – collection 3. CT scan: Diagnostic 4. Angiography: Ischemia - bleeding

TREATMENT

Primary Care

- Monitoring vital signs and pain assessment - Foley‘s urinary catheter - Nothing orally (NPO) until surgical intervention is ruled out - Nasogastric (NG) tube with low continuous suction - IV fluids or nutritional support - Analgesia for pain

Decision to Operate (Indications of Surgery)

1. Peritonitis: Tenderness, rebound tenderness, involuntary guarding 2. Severe / unrelieved pain 3. ―Vitally unstable‖ (hemorrhage or sepsis): Tachycardia, hypotensive, white count 4. Intestinal ischemia, including strangulation 5. Pneumo-peritoneum 6. Complete or ―high grade‖ obstruction

212

Individual Causes of Acute Abdomen

Acute Appendicitis (Figure 13) - Any age - Shifting pain - Pain before vomiting - Clinical picture depends on the site of appendix - High WBC count with shift to the left - Ultrasound to exclude other pathology especially in females - Treatment: Appendectomy

Figure 13: Acute inflammation of the appendix (acute appendicitis)

Perforated Peptic Ulcer - Sudden onset - Commonly in the duodenum (DU > GU with a ratio of 6:1) (Figure 14) - Ulcer may be acute or chronic - Board like rigidity - Plain X-ray (KUB) shows air under diaphragm (pneumo-peritoneum) (Figure 15) - History of a precipitating factor

Figure 14: Perforated DU (arrow) Figure 15: Air under diaphragm (arrow)

213

Acute Cholecystitis - Diffuse or colicky right hypochondrial pain - Overlying tenderness and rigidity - Difficult to feel the gall bladder (GB) - Ultrasound (US): cholecystitis – gall stones (Figure 16)

Figure 16: Cholecystitis and gallstones seen on ultrasound (arrows)

Acute Pancreatitis - Severe epigastric pain - Pain is referred to back - Profuse vomiting - The patient may be shocked - Serum amylase within 12 hours (elevated) - Serum lipase (elevated) is more diagnostic - US and CT scans are diagnostic

Acute Intestinal Obstruction - Common causes: Hernia - adhesions – intussusception – cancer – volvulus - Symptoms according to site and cause of obstruction - In general the higher up the gut, the more severe the symptoms - Pain very severe referred to epigastrium, umbilical or hypogastrium - Clinically- distension, features of shock - Plain X-ray: Multiple fluid levels

(step-ladder appearance) (Figure 17) Figure 17: Plain X-ray showing multiple fluid levels

214

Obstruction Proximal Small Intestine - Vomiting very early, frequent and violent, green and bilious - Distension is not an early feature

Obstruction Distal Small Intestine - Pain is less severe than proximal small bowel obstruction - Vomiting and distension delayed

Large Bowel Obstruction - Distension is an early feature except in intussusception - Pain less acute, shock and vomiting late. - Can be due to strangulation of bowel where tenderness on applying pressure is positive. - Obstruction can be due to volvulus, colon cancer, impacted fecal matter, etc.

Simple Bowel Obstruction - Colicky abdominal pain - Vomiting - Abdominal distension - Absolute constipation - Multiple air-fluid levels on plain X-ray

Strangulated Bowel Obstruction - Severe pain not relieved by NG tube - Localised tenderness and guarding - Fever and tachycardia - Leucocytosis

Acute Diverticulitis - Rare before 40 years - Sigmoid commonly affected (Figure 18) - Diagnosis relies on clinical presentation - Gastrograffin enema may be helpful - CT scan is diagnostic (Figure 19)

Figure 18: Diverticulitis Figure 19: CT showing diverticulitis (arrows)

215

Vascular Acute Abdomen - Acute mesenteric ischemia: Usually acute occlusion of the superior mesenteric artery (SMA) by a thrombus or an embolus (Figure 20) - Chronic mesenteric ischemia: The patient is typically a smoker, vasculopathy with severe atherosclerotic vessel disease - Ischemic colitis - Any inflammation, obstructive, or ischemic process can progress to perforation - Ruptured abdominal aortic aneurysm (AAA)

Figure 20: Acute mesenteric ischemia (inset)

Mesenteric Ischemia - Patient is over 50 years, with valvular or atherosclerotic heart disease - Arrhythmia or history of myocardial infarction (MI). - Bleeding per rectum may be the early manifestation - CT scan is diagnostic

216

CHAPTER 7 Intestinal Obstruction

Definition Intestinal Obstruction means that the luminal contents cannot pass through the gut tube.

CLASSIFICATION / ETIOLOGY

Intestinal obstruction has several types and can be classified in several ways:

1. Acute, sub-acute & chronic or acute-on-top of chronic: - Acute obstruction = Small bowel obstruction (SBO) - Chronic obstruction = Large bowel obstruction (LBO) - Acute-on-top of chronic= obstruction start as large-bowel and progress as small-bowel due to yielding of ileo-cecal valve

2. Dynamic & adynamic (according to peristalsis) - Dynamic (mechanical) obstruction means that luminal contents cannot pass through the gut because the lumen is blocked (Dynamic = audible, loud & noisy peristalsis). Causes of dynamic intestinal obstruction: Remember that in dynamic obstruction there is peristalsis working against an obstructing agent trying to overcome it (this peristalsis is always heard by auscultation & could be seen on inspecting the abdomen as a wave-like movement). This obstructing agent could be in the wall, in the lumen or outside the wall a. Intra-luminal (in the lumen): e.g. impacted feces, bezoars, gallstones, meconium b. Intra-mural (in the wall): e.g. malignant (Figure 1) or benign stricture such as Crohn's disease (Figure 2), radiation stricture, post-ischemic stricture) c. Extra-mural (outside the wall) (Figure 3): e.g. bands/adhesions, hernias (Figure 4), volvulus (Figure 5), intussusception (Figure 6), or carcinomatosis - Adynamic (functional) obstruction means that luminal contents cannot pass through the gut tube because of disturbances in gut motility that prevent coordinated peristalsis from one region of the gut to the next i.e. absent mechanical element. Causes of adynamic intestinal obstruction: a. Absent peristalsis e.g. paralytic ileus b. Non-propulsive e.g. mesenteric vascular occlusion [MVO], or pseudo-obstruction)

217

Special Considerations - Intra-abdominal adhesions related to prior abdominal surgery are the most common etiologic factor of small-bowel obstruction. - In contrast to colonic obstruction, SBO is uncommonly caused by neoplasm. Cancer- related SBO is more commonly caused by extrinsic compression or invasion by advanced malignancies arising in organs other than the small bowel.

Figure 1. Obstructing colonic cancer with a classical apple-core appearance on barium enema

Figure 2. Crohn's disease of small bowel Figure 3. Commonest causes of extra-luminal obstruction

Figure 4. Strangulated inguinal hernia (pre- operatively, it is diagnosed by the 4 local cardinal signs of strangulation i.e. the hernia becomes tense, tender, irreducible & with no impulse on cough)

Figure 5. Sigmoid volvulus with classical appearance on X-ray & intra-operatively

218

Figure 6. GIST of the jejunum causing obstruction by jejuno-ileal Intussusception

- Superior mesenteric artery (SMA) syndrome; a rare etiology of obstruction caused by compression of the 3rd part of duodenum by the SMA as it crosses over it. - Closed loop obstruction: The bowel is obstructed at both, proximal and distal points e.g. volvulus and tight carcinomatous stricture of the colon with a competent ileo-cecal valve (Figure 7).

Figure 7. Closed loop obstruction: tight carcinoma of the sigmoid colon with a competent ileo-cecal valve causing perforation of the hepatic flexure and transverse colon. The patient underwent total colectomy (as shown) with ileo- rectal anastomosis

PATHOPHYSIOLOGY

- Simply as blockage occurs, gas and air distend the bowel proximal (closest) to the blockage causing distension of the proximal segment leading to distension, pain and vomiting. - Collapse of the distal segment will occur as a result of defecation and/or diagnostic enema leading to constipation that would be absolute to stools & flatus. As the process continues, gastric, bilious (bile from the liver) and pancreatic secretions begin to form a pool. Water, electrolytes & proteins accumulate in the area. - Distension is produced by gas and fluids 1. Gas: Swallowed + fermented + diffused from the blood). 2. Fluids: Saliva (1-1.5L), gastric (1.5-2.5L), bile 1L, pancreatic 1 ½ L, small bowel (3 L). - With ongoing gas and fluid accumulation, the bowel distends and intra-luminal and intra- mural pressure ↑. If the intra-mural pressure becomes high enough, microvascular perfusion to the intestine is impaired → intestinal ischemia. This condition is termed strangulating bowel obstruction. Strangulation of a bowel segment may cause necrosis (death of the tissue), perforation and loss of fluid & blood

219

- Since intestinal contents cannot go downstream from the stomach, nausea and vomiting occur in most patients. If the obstruction has persisted for too long or the bowel has been significantly damaged, bowel sounds decrease, eventually becoming silent. Presentations 1. Acute obstruction 2. Chronic obstruction 3. Acute-on-chronic obstruction 4. Subacute obstruction (incomplete obstruction) Obstruction may be: 1. Simple: intact blood supply 2. Strangulating/strangulated: direct interference to blood flow Strangulation may be due to: 1. External compression; hernial orifices/ adhesions/ bands 2. Interruption of mesenteric flow: Volvulus or intussusception 3. ↑ intra-luminal pressure: closed-loop obstruction 4. 1ry obstruction of intestinal circulation: mesenteric infarction What is Ileus? It is a functional obstruction of the bowel due to lack of propulsive peristalsis of the bowel stops the movement of bowel contents downward. Causes of ileus include: 1. Anesthesia, 2. Interruption of nerve supply to the bowel, 3. Intestinal ischemia. 4. Abdominal wound infections, intra-abdominal sepsis 5. Electrolyte imbalance (loss of potassium → lack of intestinal peristalsis) 6. Metabolic diseases.

CLINICAL PICTURE & DIAGNSOIS

Dynamic Obstruction

1. Pain: Colicky abdominal pain. 2. Nausea and vomiting 3. Abdominal distension 4. Absolute constipation: Passage of flatus and/or stool up to 6 hours after presentation may occur and does not exclude intestinal obstruction 5. Bowel sounds: are classically high-pitched and active, coming on in rushes coincident with cramp pain These features vary according to 1. Location of the obstruction: distension is most pronounced if the site of obstruction is in the distal ileum, may be absent if the site of obstruction is in the proximal jejunum. 2. Duration of the obstruction 3. Underlying pathology 4. Presence or absence of intestinal ischemia: strangulated obstruction with ischemia → tachycardia, localized abdominal tenderness, fever, marked leukocytosis, & acidosis.

211

Other manifestations of bowel obstruction 1. Dehydration & hypokalemia. 2. Abdominal tenderness 3. Pyrexia & mild leukocytosis

Diagnosis

Tools of Diagnosi 1. Clinical examination 2. Double enema test 3. Plain X-ray abdomen (supine abdominal films) 4. CT scan abdomen to diagnose obstruction and reveal its etiology. 5. Abdominal US 6. Upper and lower GI series (Gastrografin enema and Gastrografin follow-through). Gastrografin is used as the contrast material because it is water-soluble (Barium studies are not recommended).

The Strategy of Diagnosis The aim of work-up for diagnosis is to: 1. Distinguish mechanical obstruction from ileus 2. Determine etiology of obstruction 3. Discriminate partial from complete obstruction 4. Discriminate simple from strangulating obstruction

Features of Supine Abdominal Films (Figure 8) - Small bowel obstruction: triad of (1) dilated small bowel loops > 3 cm in diameter, (2) air-fluid levels that are generally central a lie transversally in a step-ladder fashion across the abdomen & (3) no air in the colon. - Jejunum: characterized by its valvulae conniventes, which completely pass across the width of the bowel and are regularly spaced giving a concertina effect. - Ileum: featureless - Cecum: rounded gas shadow in the right iliac fossa - Large bowel: haustral folds which, unlike valvulae conniventes, are spaced irregularly and the indentations

are not placed opposite one another. They do not Figure 8. Plain X-ray abdomen usually traverse the complete width of the bowel showing Multiple fluid levels in SBO

TREATMENT OF ACUTE INTESTINAL OBSTRUCITON

Management Strategy

1. Gastric decompression using a nasogastric (NG) tube to reduce nausea, distension and the risk of vomiting and aspiration

211

2. Fluid & electrolyte resuscitation 3. Urinary catheter insertion to calculate the urine output (UOP) 4. Blood transfusion and/or blood products (FFP), if needed 5. Antibiotics as bacterial translocation may occur in the sitting of bowel obstruction 6. Relief of obstruction, usually surgical.

Surgical Treatment

When to operate ? - Not too early and not too late - Indications of early surgical intervention 1. Obstructed or strangulated external hernia 2. Internal intestinal strangulation 3. Acute obstruction Aim of surgery - Why operate ? - The goal is to operate before the onset of irreversible ischemia How to operate ? What to do ? - Operative assessment is directed to 1. The site of obstruction 2. The nature of the obstruction 3. The viability of the gut: non-viable bowel is resected. Criteria suggesting viability are normal color, peristalsis & marginal arterial pulsations. - Exploration 1. Incision: Midline/Right paramedian 2. Palpate the cecum: Distended cecum = LBO, while collapsed cecum = SBO - Procedure – dealing with the cause 1. Adhesions → Division (lysis) 2. Bands → Release (cut) 3. Hernias → Reduction & repair 4. Tumors → Resection or bypass or diverting stoma (ileostomy – colostomy)

Specific Causes of Obstruction

Large Bowel Obstruction (LBO)

1. Obstructing lesion in the cecum or ascending colon: - Removable (resectable) → emergency right hemicolectomy. - Irremovable (irresectable) → proximal stoma (ileostomy) or ileo-transverse bypass. 2. Obstructing lesion in the left colon → left hemicolectomy 3. Obstructing recto-sigmoid lesions - Resection anastomosis ± on-table colonic-lavage, ± a covering stoma - Total colectomy with ileo-rectal anastomosis, in patients with multiple colonic carcinomas or closed-loop obstruction with devitalized or perforated cecum. - Stenting followed later by resection anastomosis

212

- Colostomy and mucous fistula: The proximal colon is brought to the surface as a colostomy and the distal bowel as mucous fistula, to facilitate subsequent extra- peritoneal closure - Hartmann`s procedure: End-colostomy closure of the rectal stump. A 2nd-stage colorectal anastomosis can be planned when the patient is fit. - Colostomy: Later on, resection anastomosis is performed and finally closure of stoma in patients unfit for surgery at time of presentation 4. Rectal cancer - Anterior resection (resection of the rectum through an abdominal approach) o High anterior resection: Resection of distal sigmoid colon and upper rectum o Low anterior resection: For lesions in the upper & mid rectum o Extended low anterior resection: For lesions in the distal rectum - Abdomino-perineal resection (APR) o Excision of the rectum, anal canal & anus with a permanent colostomy

Obstruction by Adhesions and Bands

Etiology of adhesive intestinal obstruction 1. Operative injury 2. Bacterial peritonitis 3. Radiotherapy 4. Ischemic injury 5. Chemical injury 6. FB reaction Problems of adhesive intestinal obstruction 1. Small intestinal obstruction (SBO) 2. 2ry female infertility 3. Ectopic gestation 4. Chronic abdominal and pelvic pain 5. Hazardous re-operation Factors that limit adhesion formation 1. Good surgical techniques 2. Minimize contact with gauze. 3. Cover anastomosis and raw peritoneal surfaces 4. Seprafilm: Hyaluronic acid and Carboxy-methylcellulose Bands 1. Congenital: e.g. obliterated vitello-intestinal duct. 2. Acquired: A string band following previous bacterial peritonitis. Treatment Figure 9. Small bowel obstruction 2ry - NG tube decompression & IV rehydration. to intra-peritoneal fibrous band - Laparotomy, if needed, to divide causative adhesions. adhesion - R/ of recurrent intestinal obstruction due to adhesions: 1. Repeat adhesiolysis alone (open / laparoscopic) 213

2. Adhesiolysis with seprafilm 3. Noble`s intestinal plication (Figure 10) 4. Child-Phillips trans-mesenteric plication (Figure 11). 5. Baker intestinal intubation (Figure 12). Their relative efficacy remains unclear

Figure 10. Noble's plication Figure 11. Child-Philips plication Figure 12. Baker's intubation

Internal Hernia

Definition - Portion of the small intestine becomes entrapped in one of the retroperitoneal fossae or into a congenital mesenteric defect. Potential sites of internal herniation - The foramen of Winslow. - A hole in the mesentery. - A hole in the transverse mesocolon. - Defects in the broad ligament. - Congenital or acquired diaphragmatic hernia. - Duodenal retro-peritoneal fossae, left para-duodenal & right duodeno-jejunal. - Cecal/appendiceal retro-peritoneal fossae - superior, inferior & retro-cecal. - Inter-sigmoid fossa. Treatment of internal herniation - The standard R/ is to release the constricting agent by division - This should not be undertaken in cases of herniation involving the foramen of Winslow, mesenteric defects & the para-duodenal / duodeno-jejunal fossae, as major blood vessels run in the edge of the constriction ring. The distended loop in such circumstances must first be decompressed with minimal contamination & then reduced.

Enteric Stricture Causing SBO - Inflammatory stricture 2ry to TB or Crohn`s disease - Malignant stricture: lymphoma is common, whilst carcinoma and sarcoma are rare. - Presentation is usually subacute or chronic - Standard surgical R/: Resection & anastomosis. In Crohn`s disease stricturoplasty may be considered in the presence of short multiple strictures without active sepsis.

214

Bolus Obstruction - Food - Tricho-bezoars & phyto-bezoars - Stercoliths - Worms: Ascaris lumbricoides - Gallstones: in the elderly, 2ry to erosion of a large gallstone through the GB into the duodenum. Classically, there is impaction about 60 cm proximal to the ileo-cecal valve (Figure 13).

Figure 13. Impaction of a gallstone in the ileum (60 cm proximal to the ileo-cecal valve).

Gastrointestinal Obstruction in Infancy & Childhood 1. Infantile hypertrophic pyloric stenosis 2. Malrotation & volvulus neonatorum 3. Anorectal malformations 4. Intussusception 5. Intestinal atresia 6. Meconium ileus 7. Hirschsprung`s disease (Aganglionic megacolon)

CHRONIC INTESTINAL OBSTRUCITON

Organic

- Intra-mural: fecal impaction - Mural: colorectal cancer, diverticulitis, strictures (Crohn`s disease, ischemia), anastomotic stenosis. - Extra-mural: adhesion (small bowel only), metastatic deposits, endometriosis

Functional

- Hirschprung`s disease, idiopathic megacolon, pseudo-obstruction

215

ADYNAMIC OBSTRUCITON

Ileus and pseudo-obstruction designate clinical syndromes caused by impaired intestinal motility and are characterized by symptoms and signs of intestinal obstruction in the absence of a lesion-causing mechanical obstruction.

Paralytic ileus

Varieties (Causes) 1. Post-operative 2. Infection: Intra-abdominal sepsis, abscess, peritonitis 3. Reflex ileus: following fractures of the spine or ribs, retro-peritoneal hemorrhage. 4. Metabolic: uremia, hypokalemia, hypo-thyroidism 5. Medications: anticholinergics, opiates, calcium channel blockers

Clinical Features - Distension - Effortless vomiting - Absence of bowel sounds - No passage of flatus - Pain is not a feature - X-ray: gas-filled intestinal loops with multiple fluid levels - CT scan is the test of choice

Management of Paralytic Ileus - Nasogastric suction - Fluid and electrolyte balance - Removal of the 1ry cause - No place for routine use of peristaltic stimulants. - If paralytic ileus is prolonged; laparotomy is performed to exclude a hidden cause and facilitate bowel decompression

Pseudo-obstruction

- Definition: Obstruction, usually of the colon, in the absence of a mechanical cause or acute intra-abdominal disease. It is associated with a variety of syndromes where there is an underlying neuropathy &/or myopathy. - Small intestinal pseudo-obstruction 1. Primary (i.e. sporadic or familial visceral myopathy, neuropathy). 2. Secondary (e.g. scleroderma, muscular dystrophy, spinal cord injury, viral infection) - Colonic pseudo-obstruction 1. Acute form: Ogilvie syndrome 2. Chronic form

216

Mesenteric Ischemia

Causes of acute mesenteric ischemia - Arterial or venous thrombosis – Embolism – Vasculitis - Surgical trauma to vessels (e.g. aortic reconstruction) Gut ischemia: important anatomical consideration - Marginal artery of Drummond - Marginal artery of Dwight - Griffiths‘ point - The arc of Riolan Acute Mesenteric Ischemia Clinical features and diagnosis - Sudden onset of severe abdominal pain in a patient with atrial fibrillation or atherosclerosis. Abdominal pain is classically out of proportion to the physical findings - Persistent vomiting and defecation occur early with subsequent passage of altered blood. - Hypo-volemic shock rapidly ensues. - Mild abdominal tenderness initially. Rigidity is a late feature. - 40% of patients receive no treatment or an ―open & close‖ laparotomy - Laboratory tests: Leucocytosis, acidosis, hyper-amylasemia - Imaging: Plain X-ray, angiography, CT scan. Treatment - Full resuscitation - Massive resection of affected bowel (Figure 14) unless flow is restored within 6 hours - Second look laparotomy - In early cases: embolectomy or revascularisation of the SMA by aorto-SMA bypass. - Anti-coagulations early in the post-operative period. - IV alimentation after extensive enterectomy. - Small bowel transplantation in selected cases. Prognosis - Mortality rate = 60-85% !!

Figure 14. Extensive arterial mesenteric ischemia causing gangrene of a large segment of bowel (resected during operation)

Chronic Mesenteric Ischemia (Intestinal Angina) - Cause: insufficient blood flow to the bowel during periods of the increased metabolic demand associated with digestion. - Symptoms: Recurrent postprandial pain, bloating, flatulence, constipation, or diarrhea, associated with weight loss and steatorrhea (50%). - Patients are usually in their 50s or 60s and have other evidence of generalized atherosclerosis.

217

CHAPTER 8 Upper Gastrointestinal (UGI) Bleeding

Definition

Upper gastrointestinal (UGI) bleeding means bleeding from the gastro-intestinal tract (GIT) proximal to the ligament of Treitz. Bleeding site is no more distal to the duodenum.

CAUSES OF UGI BLEEDING

Causes of UGI result from the esophagus, stomach or duodenum (Table 1).

Table 1: Etiology of UGI bleeding

Esophageal Causes Gastric Causes Duodenal Causes

- Esophageal varices - Gastric peptic ulcer - Duodenal peptic ulcer - Hiatal hernia - Stress ulcer Duodenal diverticulum - Reflux esophagitis - Erosive gastritis - Duodenitis - Esophageal carcinoma - Portal hypertensive gastropathy - Carcinoma of duodenum - Mallory Weiss tear - Gastric varices - Lymphoma - Dieulafoy lesion - Hemobilia - Mentenier's syndrome - Crohn's disease - Gastric tumors (carcinoma, - Duodenal diverticulum lymphoma, GIST) - Rupture of retro-gastric aortic aneurysm (aorto-enetric fistula). GIST: Gastro-intestinal stromal tumor

Most common causes in Egypt (in order of frequency) 1. Esophageal varices (75%) 2. Erosive gastritis 3. Duodenal ulcer (DU) 4. Cancer stomach 5. Gastric ulcer (GU)

218

CLINCIAL PRESENTATION

1. Hematemsis - Fresh blood (significant bleeding) - Small amount of altered blood (coffee grounds) = mild bleeding that already settled. 2. Melena - Liquid, jet black or black with reddish tinge pungent characteristic smell, quite unlike smell of feces. - DD from Iron therapy (sticky feces with dark grey rather than black) - Massive bleeding may cause fresh rectal bleeding or large fresh clots per rectum. 3. Both hematemesis &melena

The severity of the attack can be evaluated by the presence & extent of hypovolemia. 1. Mild: no significant hypovolemia. 2. Moderate: hypovolemia that responds to volume replacement (crystalloids & blood) & thereafter the patient is stable. 3. Severe: active continued major bleeding rendering resuscitation with blood transfusion difficult, or recurrent major re-bleeding after successful resuscitation. Presentation according to onset 1. Sudden massive hematemesis → shock 2. Slow (usually melena) → chronic anemia

TREATMENT OF ACUTE UGI BLEEDING

Resuscitation

- All patients should be kept nothing by mouth at least until endoscopy has been performed. - Fluid replacement & blood transfusion o Send blood for grouping & save, start slow IV. o Blood transfusion if Hgb is <10 g/dl with evidence of acute blood loss. o Goal of a hematocrit value (HCV) > 30%. o Rapid transfusion in hemodynamically stable patient should be avoided (Heart failure and re-bleeding). o Warm the blood to 25oC by warmer. o Fresh frozen plasma (FFP) every 6 units of stored blood (15 ml/kg) or every 4 U packed RBCs. o Citrate toxicity is avoided by 10 ml of 10% Ca gluconate after the first 6 units then every 2 subsequent units. Hypokalemia is carefully monitored.

Surgical Treatment

Indications of Surgery 1. Patients with severe hemorrhage that resuscitation with volume replacement cannot keep up with the losses.

219

2. Elderly patients older than 60 y if > 4 units of blood are necessary during the initial resuscitation and patiett who has one recurrence of bleeding after initial successful control

Lines of Surgery 1. The use of a definitive ulcer-curing operation is mandatory in patients with hemorrhage, but is optional in patients with perforation. 2. Pyloromyotomy under run the vessel. 3. PG or TV + GJ (truncal vagotomy and gastro-jejunostomy) 4. Recurrent bleeding in both is 13% 5. Mortality of PG 8% is double that of TV + GJ.

Treatment of Bleeding Peptic Ulcer Disease

Bleeding occur in 20% of patients with DU. It may occur without history of dyspepsia.

Diagnosis - Typical history - Epigastric tenderness - Endoscopy: not only diagnosis 1. Site: posterior. 2. Risk signs: a. Visible vessel (blue or red) ………………… 50% risk of re-bleeding. b. Spurter (arterial) …………………………… 50% risk of re-bleeding c. Overlying adherent clot …………………….. 10% risk of re-bleeding d. Red or black spot in the base of the ulcer ….. 10% risk of re-bleeding

Treatment Approximately, 80% will stop without intervention, so any technique should be subjected to controlled trial.

A. Medical treatment: H2 blockers, proton pump inhibitors, somatostatin, prostaglandins. B. Endoscopic techniques for hemostasis: 1. Injection methods - Adrenaline (1:10,000): Tissue pressure, active vasospasm, activation of platelets - Sclerosants as STD, polidocanol act by damaging the endothelium. - Absolute alcohol: rapid dehydration … tissue fixation. 2. Laser photocoagulation: Argon, Nd YAG. 3. Diathermy, bipolar 4. Heater probe 5. Topical agent: Collagen, clotting factors, cyanoacrylate glue, fibrin sealant, thrombin. 6. Mechanical methods: Clips, staples, sutures. 7. Microwave and argon plasma coagulator: (under study).

221

NB. The endoscopic techniques for visible vessels and active bleeding are:  Thermal  Electrocoagulation  Photocoagulation  Injection therapy C. Insertion of NG tube, lavage with ice cold saline to induce gastric mucosal constriction.

D. Pharmacological control of bleeding: H2-blockers, sucralfate, proton-pump inhibitors, vasopressin (or analogues) and somatostatin. No drug has appreciable effect on ulcer E. Vascular embolization. F. Surgical treatment - With the expansion and increased efficacy of endoscopic techniques, fewer patients are treated surgically nowadays. - Indications for surgery (in about 25%) 1. Massive bleeding requiring >10 units. 2. Rebleeding after cessation (Death rate >30%) so need Early surgery 3. Visible bleeding vessel on endoscopy (50% rebleeding). - Relative indications: giant DU or GU, visible vessel, shortage of blood and previous bleeding. G. With stopping of bleeding, after 12-24 h the patient can eat to ↑ the morale, stimulate muscle contraction and ↓ acidity Order hematocrit and occult blood in stools (twice daily). Rebleeding PU - Incidence: 5% - Most patients re-bleed within 2 days from the time of first episode - Re-bleeding carries tenfold increase in mortality (MR = 25%). - Risk signs carry a 40% risk of re-bleeding compared to only 4% in absence of risk signs. - If risk signs are present → active treatment, if re-bleeding → surgery. Prognosis - MR of emergency operation is 8-10% versus 1-2% in elective or semi-elective surgery

Treatment Options for Erosive Gastritis

1. Near total gastrotomy + Roux-en-Y anastomosis. 2. Ligation of all blood vessels to the stomach. 3. Vagotomy and pyloroplasty. 4. Conserve until transfusion requirements are 12 units or more.

Treatment of Esophageal Varices

General Plan - Treatment of shock and guard against liver failure and encephalopathy - Stop bleeding by chemical, mechanical, physical or surgical methods.

Pharmacological Treatment - Vasopressin: IV bolus: 20 units / 100 ml G 5% / 20 min or Infusion 0.4 units / min. It has 3 side effects; myocardial infarction, gut ischemia &local gangrene - Glypressin: It has lower side effects. Bolus dose, longer action

221

- Somatostatin: Synthetic analogue (Octereotide), half-life 1-2 hours, greater potency, little side effects. Dose = mg/8 h for 48 hours on 250 ml of glucose 5%

Surgical Treatment Indications for surgery - Non stoppage of bleeding after 2 sessions of sclerotherapy - Bleeding gastric varices (5-50% of cases) Operative lines - Emergency decongestion: Mortality = 40%. Portal vein thrombosis occurs in 30% of cases - Emergency porto-caval shunt is obsolete - The preferred technique is esophageal transection with stapling.

222

CHAPTER

9 Differential Diagnosis of Abdominal Masses

ABDOMINAL EXAMINATION

Exposure

- From the nipple to the knee, - If embarrassing, cover the lower abdomen with a sheet.

Inspection

Abdominal Contour - Normal contour: The abdomen is flat from the xiphoid to the pubis and the umbilicus is at the center of the abdomen. - Generalized distension (Figure 1): Due to fat, fetus, feces, flatus, fluid, full-sized tumors - Localized bulge (Figure 2): Due to mass, organomegaly, hernia.

Figure 1: Generalized abdominal distention Figure 2: Localized distention (arrow)

Palpation

Superficial Palpation - To gain the patient‘s confidence - To detect local temperature, tenderness, parietal mass, or hypersthesia

223

Deep Palpation - To feel the following: 1. Abdominal organs: Liver, spleen, kidneys 2. Abdominal aorta 3. Any masses - If masses are felt, note the following: 1. 6S: Site, size, shape, surface, skin overlying, special character: 2. Consistency 3. Tenderness 4. Pulsations 5. Mobility with respiration or with hand.

Parietal versus Intra-Abdominal Mass

The rising-up test, with the aid of imaging, can differentiate between parietal and intra-abdominal masses.

Parietal Swellings - Extends over the costal margin - Moves anterior and posterior with respiration - More prominent on rising-up test

Intra-abdominal Swellings - Disappears beneath costal margin - Moves up and down with respiration - Less prominent on rising-up test

1. COMMON PARIETAL SWELLINGS

Skin

- Sebaceous cyst - Papilloma - Melanoma - Squamous cell carcinoma (SCC)

Subcutaneous Tissue

- Lipoma: Subcutaneous (SC), inter-muscular - Neurofibroma - Hemangioma - Lymphangioma

Muscles

- Rectus sheath hematoma - Desmoid tumor

224

ABDOMINAL QUADRANTS / REGIONS

Nine Regions

The abdomen is divided by 2 vertical planes and 2 transverse planes into 9 regions: - The 2 vertical planes are mid-clavicular or pass from the mid-inguinal point upwards, on each side. - The 2 transverse planes: 1. The upper is roughly midway between the xiphoid and umbilicus, or touching the lower costal margin (subcostal line), or at the trans-pyloric plane of Addison‖, which passes at the lower edge of L1. 2. The lower plane is roughly midway between the umbilicus and symphysis pubis, or between the highest points of the iliac crests (inter-costal line) or between the tubercles of the iliac crest (inter- tubercular line).

Accordingly, the 9 regions are as follows (Figure 3): 1. Right hypochondrium. 2. Epigastrium 3. Left hypochondrium. 4. Right lumbar region. 5. Umbilical region. 6. Left lumbar region. 7. Right iliac fossa (RIF). 8. Hypogastrium (supra-umbilical region) 9. Left iliac fossa (LIF). Figure 3. The nine regions of the abdomen

Four Quadrants

The abdomen can also be divided into 4 quadrants by a midline vertical plane passing from the xiphoid to the symphysis pubis and a transverse plane passing horizontally through the umbilicus.

Accordingly the 4 quadrants of the abdomen are: 1. Upper right quadrant (URQ) 2. Upper left quadrant (ULQ) 3. Lower right quadrant (RLQ) 4. Lower left quadrant (LLQ)

Figure 4 demonstrates the 9 regions of the abdomen (a) and the 4 quadrants of the abdomen (b)

225

Figure 4: The 9 abdomino-pelvic regions (a) and 4 abdomino-pelvic quadrants (b)

Figure 5 deomonstrates the specific organs occupying the different 9 regions of the abdomen, which aids the diagnosis of a ‗mass# occupying a certain region

Figure 5. The different organs occupying the 9 regions of the abdomen

226

CLINICAL APPROACH

In a patient presenting with an abdominal mass, the following clinical features, should be carefully assessed: - Pain: Site, nature, aggravating or relieving factors, duration of pain, referred pain. - Vomiting: Type, content, hematemesis, relation to food, frequency. - Jaundice: It is an important factor in relation to liver, gall bladder or pancreatic masses. - Bowel habits: Constipation, diarrhea, bloody diarrhea, furious diarrhea, tenesmus. - Decreased appetite and weight. - Inspection of the mass: Anatomical location, margin, movement with respiration. - Palpation of the mass: Site, extent, surface, tenderness, consistency, movement with respiration, mobility, borders, plane of the swelling (by leg-raising test), presence of other masses. - Often the mass needs to be examined for change of position—in sitting, in standing, in side position, after a brisk walk, in knee-elbow position for retro-peritoneal mass and for puddle sign (but difficult to keep patient in this position). - Percussion is an important aspect of examination in case of an abdominal mass. Percussion over the mass is important to predict the anatomical location of the mass. 1. If the mass has a dull note, then it is in the anterior abdominal wall, or in front of the bowel intra-abdominally like liver, spleen, gall bladder (GB). 2. If the mass is with an impaired resonant note, then the mass is arising from the bowel like stomach, colon, or small bowel. 3. If the mass is resonant on percussion, then it is probably in the retro-peritoneal region. 4. Other than this, liver dullness, free fluid in the abdomen should be elicited during percussion. - Per-rectal (PR) examination: It is done to look for any secondaries in recto-vesical pouch, primary tumor or relation of lower abdomen masses (pelvic masses). - Per-vaginal (PV) examination: It is done to assess pelvic masses.

MASS IN THE RIGHT HYPOCHONDRIUM

A. Palpable Liver Mass in the Right Hypochondrium

Characteristics

- It is horizontally placed. - It usually moves with respiration. - The upper border is not felt. - It is dull on percussion (this dullness continues over liver dullness above). - Fingers cannot be insinuated under the right costal margin.

227

Causes of an Enlarged Liver

1. Soft, Smooth, Non-Tender Liver - Hydro-hepatosis: It is due to obstruction of the common bile duct (CBD) causing dilatation of the intra-hepatic biliary radicles. - Congestive cardiac failure. - Hydatid cyst of the liver: The mass is well-localized in the liver with a typical hydatid thrill. Three fingers are placed over the mass widely; when the central finger is tapped, fluid movement is elicited in lateral two fingers (Three finger test).

2. Soft, Smooth, Tender Liver Amoebic liver abscess - It is due to entameba histolytica infestation - Common in males (20:1), fever, pain, intercostal tenderness, tender liver - It is more common in alcoholics and cirrhotics - Single abscess is common (70%), most common in the right postero-superior lobe (80%) - The liver often gets adherent to the anterior abdominal wall and will not move with respiration. Inter-costal tenderness and right-sided pleural effusion are common. - Chocolate-colored Anchovy sauce pus is classical - Secondary infection can occur (30%) and is life-threatening due to septicemia - It can be acute or chronic; both mimics hepatoma - The most common site of rupture is into the lungs. - The most dangerous rupture is into pericardium (left lobe abscess) - Liver failure can develop in cirrhotic patients - Mimics cholecystitis, subphrenic abscess, hepatoma - Total count, LFT, prothrombin time, US abdomen are relevant investigations - Chest X-ray may show left-sided sympathetic pleural effusion - CT scan to differentiate it from hepatoma - Treatment: Drugs like metronidazole, injection dehydroemetine, chloroquine tablets, diloxanate furoate; US-guided aspiration after controlling prothrombin time using vitamin K or fresh frozen plasma (FFP). If it recurs, percutaneous guided drainage using a pigtail catheter, or open laparotomy and drainage with placement of Malecot‘s catheter may be used.

3. Hard, Smooth Liver (Hepatic Tumors) - Hepatoma (HCC) - Solitary secondary in liver.

Hepatoma / hepato-cellular carcinoma (HCC) - Common etiologies are afla toxins, hepatitis B and C virus infection, alcoholic cirrhosis, hemochromatosis, smoking, hepatic adenoma, clonorchis sinensis, polyvinyl chloride - Uni-centric tumor and right lobe involvement is more common

228

- Fibro-lamellar variant is common in the left lobe, not related to hepatitis or cirrhosis and without alpha-feto protein (AFP) level raise. There are increased serum vitamin

B12 binding capacity and neurotensin levels. - It can be multifocal/indeterminate/spreading/expanding— Okuda classification - Presents as a large smooth hard liver mass — later jaundice, fever, pain and tenderness, ascites and bruit over the mass. Occasionally there can be multiple nodules when it is multi-centric. - A rapidly growing tumor can be also soft. - Hepatoma often can also be tender due to tumor necrosis or stretching of the liver capsule. - A vascular bruit may be heard over the liver during auscultation. - Spreads to lymphatics, blood and direct spread - Mimics amebic liver abscess, secondaries, hydatid cyst, polycystic liver disease - LFT, CT scan, raised AFP, liver biopsy (only needed) are the investigations - Hemi-hepatectomy in early operable growth is the treatment - Hepatic artery ligation / intra-arterial chemotherapy /chemoembolization / percutaneous ethanol or acetic acid injection / radiofrequency ablation/chemotherapy using adriamycin, carboplatin, gemcitabine — are palliative procedures

4. Hard, Multi-Nodular Liver - Multiple secondaries in liver Here, hard nodules show umbilication due to central necrosis.

B. Palpable Gall bladder (GB) in the Right Hypochondrium

Characteristics

- It is smooth and soft (except in carcinoma of the GB). - It is mobile horizontally (side-to-side). - It moves with respiration. - It is located right of the right rectus muscle, below the right costal margin or below the lower margin of the palpable liver. - It is dull on percussion.

Causes of a Palpable Gall Bladder

1. Soft, non-tender gall bladder - Mucocele of the gall bladder. - Enlarged gall bladder in obstructive jaundice due to carcinoma head of the pancreas or peri ampullary carcinoma or growth in the CBD. 2. Hard gall bladder: Carcinoma gall bladder 3. Tender gall bladder: Empyema GB

229

C. Other Masses in the Right Hypochondrium

1. Peri-cholecystic inflammatory mass: It is tender, smooth, firm or soft, non-mobile, intra- abdominal mass often with guarding. 2. Kidney mass arising from upper pole: due to renal cell carcinoma or hydronephrosis.

MASS IN THE EPIGASTRIUM

Palpable Left Lobe of the Liver

- It is in the epigastric region. - Its upper border cannot be felt. - It moves with respiration. - It extends towards the left hypochondrium. - It is dull on percussion.

Features of Stomach Mass

- It is located in the epigastric region. - It is an intra-abdominal mass that moves with respiration. - It is resonant or impaired resonant on percussion. - The mass may be better felt on standing or on walking. It is often mobile, unless it gets adherent posteriorly. Its presentation depends on the gastric region from which it arises: 1. Pylorus: all margins of the mass are well felt. It is mobile, with features of gastric outlet obstruction. 2. Body: the mass is horizontally placed without any features of obstruction. 3. Upper part of the stomach near the gastro-esophageal junction: it causes dysphagia. 4. Fundus: the mass is in the upper part of the epigastric region towards left side. - Gastro-intestinal stromal tumor (GIST) of stomach is smooth and firm. - Stomach carcinoma is nodular and hard (Figure 6). It is the most common cause of stomach mass

Figure 6: Gastric carcinoma present with a hard, nodular mass

231

- Management of gastric carcinoma 1. Early growth in the pylorus: lower radical gastrectomy with removal of the tumor, proximal 5 cm clearance, nodal clearance, greater and lesser omentum, distal pancreas and spleen (now not regularly removed; it is removed to clear splenic lymph nodes and Billroth II anastomosis or Roux-en-Y anastomosis is done. Post-operatively adjuvant chemotherapy should be given (5-fluorouracil, mitomycin, epirubicin, cisplatin). 2. Growth in body, proximal growth, diffuse carcinoma and generalized linitis plastica are the indications of total radical gastrectomy with esophago-jejunal anastomosis 3. Neoadjuvant chemotherapy in advanced gastric cancer prior to gastrectomy 4. Instillation of mitomycin C impregnated charcoal intra-peritoneally to control lymphatic disease (Japan) 5. Palliative procedures like palliative partial gastrectomy, anterior gastro-jejunostomy, Devine‘s exclusion procedure, luminal stenting in proximal inoperable growths, chemotherapy are used in inoperable cases 6. In early carcinoma, proper lymph nodal clearance is important

Abdominal Masses of Pancreatic Origin (Figure 7)

A. Pancreatic Cystic Lesions (inflammatory cystic or cystic neoplasm) B. WOPN (walled-off pancreatic necrosis), C. Pseudo-cyst of the Pancreas - Mass in the epigastric region, smooth, soft, and may be tender if it gets infected. - It does not move with respiration. - It is not mobile. - It has got transmitted pulsation. - It is confirmed by placing the patient in knee-elbow position. - The lower border is well felt, but the upper border is not clear. - It is resonant on percussion. - Baid test: As the stomach is pushed in front, a Ryle‘s tube when passed can be felt per abdomen on palpation.

Figure 7: Different pancreatic tumors arising from different parts of the pancreas.

231

- Investigations for pseudo-cyst of pancreas 1. Ultrasound (US): a commonly done procedure 2. CT scan: ideal choice 3. LFT, serum amylase, prothrombin time 4. ERCP to find out communications 5. Barium meal (not done nowadays): shows widened vertebro-gastric angle - Indications of intervention 1. Cyst size bigger than 6 cm 2. Mature thick wall cyst 3. Infected cyst - Complications 1. Rupture — 3% 2. Infection — 20% 3. Bleeding — torrential 7% 4. Cholangitis - Interventions 1. Roux-en-Y cysto-jejunostomy is ideal 2. Cysto-gastrostomy (Jurasz procedure) commonly done cysto-duodenostomy 3. Cysto-gastrostomy with external drainage if infected (Smith operation) 4. Endoscopic stenting 5. Laparoscopic cysto-gastrostomy (popular and safer than open cysto-gastrostomy) 6. Guided aspiration helps, but high recurrence rate is high (70%) D. Pancreatic Cystic Neoplasms (Figure 8) - Mass is smooth, firm, does not move with respiration, and non-mobile - Resonant on percussion. - The patient complaints of back pain.

Figure 8: Pancreatic cystic neoplasms present with a smooth, firm mass.

Serous cyst-adenomas (SCAs) - Have thick, fibrous walls and contain clear fluid. - Almost all SCAs are benign.

232

Intra-ductal Papillary Mucinous Neoplasms (IPMNs) - Start in the ducts that connect the pancreas to the intestine. - They are the most common type of pre-cancerous cyst. - They produce large amounts of proteins that form mucus (mucin) in the cyst lining and fluid. - It is difficult to predict if and when an IPMN will become cancerous, although research indicates that those that involve the main pancreatic duct are at a higher risk. Mucinous Cystic Neoplasms (MCNs) - These cysts are pre-cancerous growths that can start in the body and tail of the pancreas. - They almost always develop in women rather than men. - Large ones that contain tiny walls that divide the cyst into compartments, called septations, may be more likely to become cancerous. E. Pancreatic Cancer (advanced cancer head pancreas, cancer body pancreas) - It is the fourth leading cause of cancer deaths, being responsible for 8% of all cancer- related deaths. - Approximately, 75% of all pancreatic carcinomas occur within the head or neck of the pancreas, 15-20% occurs in the body of the pancreas, and 5-10% occurs in the tail. - Palpable GB (i.e. Courvoisier sign) - Advanced intra-abdominal disease: Presence of ascites, a palpable abdominal mass, hepatomegaly from liver metastases, or splenomegaly from portal vein obstruction - Advanced disease: Para-umbilical subcutaneous (SC) metastases (or Sister Mary Joseph nodule) - Possible presence of palpable metastatic mass in the rectal pouch (Blumer shelf) - Possible presence of palpable metastatic cervical lymph nodes (LNs): Nodes may be palpable behind the medial end of the left clavicle (Virchow node) and other areas in the cervical region

Colonic Mass

- It is due to carcinoma of transverse colon. - It is mobile, horizontally placed, nodular, hard mass (Figure 9), which does not move with respiration. - The cecum will be dilated and palpable. - It is resonant or impaired resonant on percussion. - Patient has bowel symptoms, loss of appetite and decreased weight.

233

Figure 9. A colonic mass is hard, nodular and horizontally placed.

Para-aortic Lymph Node Mass

- Mass in the epigastric region, which is deeply placed, non-mobile, not moving with respiration. - It is vertically placed, above the level of the umbilicus and resonant on percussion. - Causes of enlargement are secondaries, lymphomas or tuberculosis (TB).

Aortic Aneurysm

- It is smooth, soft, pulsatile (expansile pulsation, which is confirmed by placing the patient in knee-elbow position). - It is vertically placed above the level of the umbilicus, non-mobile, not moving with respiration and resonant on percussion.

MASS IN THE LEFT HYPOCHONDRIUM

Enlarged Spleen

Characteristics - Spleen has to enlarge three times to be palpable clinically. - It enlarges from left costal margin towards the right iliac fossa. - It moves with respiration, mobile, obliquely placed, smooth, soft or firm, with a notch on the anterior edge, which is directed downwards and inwards. - Fingers cannot be insinuated over the upper border. - ―Hook sign‖ is positive, i.e. one cannot insinuate the fingers under the left costal margin. - It is dull on percussion.

234

Figure 10: Splenomegaly (enlarged spleen) presents with a mass in the left hypochondrium.

Causes of splenomegaly - Infectious 1. Protozoa: Bilharziasis, malaria 2. Viral: Cytomegalovirus (CMV), Ebstein-Barr virus (EBV) 3. Bacterial: Septicemia, TB, typhoid - Cellular infiltration: Amyloidosis, sarcoidosis - Collagen disease: Felty‘s disease - Space-occupying lesions: Hydatid cyst - Cellular proliferative disorders: Leukemias, lymphoma, spherocytosis, hemolytic anemia, myelo-fibrosis, sarcoidosis - Infarction: Emboli, splenic artery or vein thrombosis - Trauma: Splenic sub-capsular hematoma

Left-Sided Colonic Mass

- It is mobile, nodular, and resonant. - It does not move with respiration. - It is commonly due to carcinoma colon.

Left Renal Mass from Upper Pole of any Cause

- It has got features of a renal mass.

Left-Sided Adrenal Mass

- It does not move with respiration. - It is not mobile. - It is a deeply placed mass. - Often, it crosses the midline. - It is resonant on percussion. - It mimics a kidney mass.

Mass Arising from the Tail of the Pancreas

- Clinical features are same as other pancreatic masses. - Causes are pseudo-cyst in tail of the pancreas and cyst-adenomas.

235

MASS IN THE LUMBAR REGION

Palpable Kidney Mass

Characteristics - There is fullness in the loin, which is better observed in the sitting position. - The mass moves with respiration. - It is vertically placed. - It is bimanually palpable. - It is ballotable. - The renal angle is dull on percussion (normally it is resonant due to colon). - There is a band of resonance in front due to reflected colon. - It does not cross the midline.

Causes of Enlarged Kidney 1. Hydronephrosis - It is smooth, soft, and lobulated. - It is a non-tender mass - It is non-mobile. 2. Pyonephrosis - History of throbbing pain in the loin, pyuria and fever with chills. - It is smooth, soft and tender kidney mass - It is non-mobile. 3. Polycystic kidney - History of loin pain and hematuria. - Hypertension, anemia and features of renal failure. - Usually bilateral, but one side can present earlier than the other. - Lobulated smooth surface. 4. Renal cell carcinoma - History of a mass in the loin, hematuria, fever and dull pain. - The mass is nodular and hard. - It does not cross the midline. - Initially the mass is mobile; eventually it infiltrates gets fixed and becomes non- mobile.

Mass from the Ascending Colon on Right Side or Descending Colon on Left Side

- History of altered bowel habits with decreased appetite and weight. - The mass is nodular, hard, does not move with respiration and is not ballotable. - It is resonant or there is impaired resonance on percussion. - Renal angle is resonant. - Proximal dilated bowel may be palpable.

236

Adrenal Mass

- The mass is nodular and hard. - It does not move with respiration. - It is not mobile and often crosses the midline. - It is felt on deep palpation. - It is resonant in front. - It is not ballotable.

Retro-peritoneal Tumors and Cysts

- Retro-peritoneal tumors are not mobile and do not fall forward in knee-elbow position. They are deeply placed mass which are usually smooth and hard. They may be retro- peritoneal sarcomas or teratomas or lymph node (LN) mass. - Retro-peritoneal cysts are smooth, soft, with same features as retro-peritoneal tumors

N.B. Cystic Lesions in the Abdomen

- Mucocele/empyema of the GB - Pseudo-cyst of the pancreas - Hydatid cyst of the liver - Congenital non-parasitic cyst of the liver - Hydronephrosis - Mesenteric cyst - Ovarian cyst - Omental cyst - Aneurysm - Retro-peritoneal cyst - Cyst-adeno-carcinoma of the ovary - Loculated ascites

MASS IN THE UMBILICAL REGION

Common Causes

- Mesenteric cyst - Omental cyst - Ovarian cyst (pedunculated) - Small bowel tumors - Extension of masses from other region - Transverse colon mass - Mass in the body of pancreas - Mesentery mass - LN mass: secondaries (primary from GIT, testis, ovary, melanoma), lymphoma, TB - Retro-peritoneal tumor

237

Mesenteric Cyst

- Tillaux Triad 1. Soft intra-abdominal umbilical mass. 2. Mobile in the direction perpendicular to the attachment of the mesentery. 3. Resonant mass. - May precipitate intestinal obstruction, volvulus.

Omental Cyst

- It is smooth, soft and non-tender. - It moves with respiration. - It is mobile in all directions. - It is dull on percussion.

Small Bowel Swellings

1. Small bowel lymphomas. 2. Small bowel carcinomas. 3. Intussusception. - The Mass in umbilical, usually towards left and above the umbilicus. - Occasionally, towards the right side. - Mass is intra-abdominal, sausage-shaped, with concavity towards umbilicus, well- defined, smooth, firm and mobile. - Mass does not move with respiration. - Mass contracts under palpating fingers. - Often, the mass disappears and reappears. - It is resonant or there is impaired resonance on percussion. - ―Red currant jelly‖ stool with features of intestinal obstruction may be present.

MASS IN THE RIGHT ILIAC FOSSA (RIF)

Common Causes

- Appendicular mass or abscess - Carcinoma cecum - Ileo-cecal tuberculosis (TB) - Ameboma - Psoas abscess - Lymph node mass either mesenteric or external Iliac LNs - Bony swellings - Ectopic kidney - Undescended testis (Abdominal) - Actinomycosis - Crohn‘s disease - Iliac artery aneurysm - Gynecological: ovarian cyst, tubo-ovarian mass, uterine mass (pedunculated fibroid)

238

Appendicular Mass

- It is smooth, firm, tender mass in the right iliac fossa. - It is not mobile and does not move with respiration. - It is resonant on percussion. - It is a well-localized mass with distinct borders

Appendicular Abscess

- It is smooth, soft, tender and dull mass in the right iliac fossa - It has indistinct borders.

Carcinoma of the Cecum

- It is a nodular, hard mass in the RIF, and does not move with respiration. - It is mobile but mobility may be restricted once it gets adherent to psoas muscle. - Mass is resonant or there is impaired resonance on percussion. - Often features of intestinal obstruction may be present.

Ileo-cecal Tuberculosis (TB)

- Mass in the right iliac fossa, which is smooth, hard, resonant and non-tender. - It does not move with respiration and has a restricted mobility. - The cecum may be pulled-the up to the lumbar region due to fibrosis.

Ameboma

- History of dysentery with pain in the RIF. - Smooth, hard, well-defined mass in the RIF, non-mobile. - It may or may not be tender.

Psoas Abscess

- It is a localized, smooth, soft, non-mobile mass in the RIF. - Psoas spasm (flexion of the hip joint) is typical. - Spine may show tenderness and para-spinal spasm (spinal movements are restricted)

5. MASS IN THE LEFT ILIAC FOSSA (LIF)

Causes

1. Carcinoma sigmoid or descending colon 2. Bony masses 3. Ovarian/uterine masses 4. Psoas abscess 5. Ectopic kidney 6. Lymph node mass 7. Undescended testis

239

MASS IN THE HYPOGASTRIUM

Bladder Mass

- It is in the midline. - It is dull on percussion. - The lower border is not felt. - It can be mobile in a horizontal direction. - The mass reduces in size after emptying the bladder. - It can be felt on per-rectal examination. - It is either carcinoma bladder (common) or leiomyoma or sarcoma bladder.

Uterine Mass

- It is a midline mass, which is smooth, hard. - The lower border, which extends into the pelvis, is not felt. - It is felt on per-vaginal (PV) examination.

Ovarian Mass

- It is a pelvic soft tissue mass. - As in all lower abdomen masses PR and/or PV examination is a must.

INVESTIGATIONS OF AN ABDOMINAL MASS

Laboratory Test - Hematocrit value (HCV) - Liver function tests (LFTs) - Renal function tests - Stool/urine analysis.

Imaging Studies - US abdomen. - Barium studies: Barium meal - Barium enema - Barium meal - Follow through. - CT scan - MRI - MRCP - Endo-sonography.

Endoscopic/Laparoscopic Procedures - Gastroscopy - Colonoscopy - ERCP. - Diagnostic laparoscopy.

Tapping - Ascitic tap

241

Biopsy - US-guided biopsy - CT-guided biopsy.

Renal Investigations - Laboratory: Serum urea and creatinine - Imaging: IVU - RGP - isotope renogram - Endoscopy: Cystoscopy.

Exploration - Exploratory laparotomy.

IMPORTANT CLINICAL NOTES

- A hard mass in the abdomen is commonly malignant - A firm mass may be tuberculous, lymphoma or many benign conditions - A soft mass is hydronephrosis, pseudo-cyst, mesenteric cyst, omental cyst, or loculated ascites - In TB abdomen, the mass may be doughy in consistency due to thickened parietal peritoneum or omentum - It is difficult to elicit fluctuation in the abdominal mass as mass cannot be fixed properly - Plane of the swelling should be checked by leg raising/head-raising test / Valsalva manoeuver / knee-elbow test - Bimanual palpation, ballotability, renal angle inspection, palpation and percussion should be done in case of a renal mass - Intrinsic mobility should be checked. Different mobilities / movements are—gallbladder shows side to side; stomach lateral; ovarian mass—all over; mesenteric cyst—right angle to line of mesentery; transverse colon mass—vertical; small bowel mass all over; appendicular mass/pancreatic mass/ nodal mass (para-aortic)/retroperitoneal mass do not show any mobility; cystadenocarcinoma of pancreas may show false mobility (tree top mobility) - Percussion is very important method of examination to find out the anatomical plane of the mass. Mass in front of the bowel like liver/spleen/GB/parietal mass is dull on percussion. Mass from the bowel is resonant on percussion e.g. stomach, small bowel and colon. Retro-peritoneal masses like cyst, sarcoma, nodal mass, aneurysm, pancreatic mass are resonant on percussion - Succussion splash and auscultation-percussion tests are done for gastric outlet obstruction in pyloric stenosis - Digital examination of rectum (PR) and left supraclavicular examination for nodes is a must. PR is done to see Blumer shelf secondaries in the recto-vesical pouch. Per-vaginal examination should be done in pelvic masses in females - The urinary bladder should be emptied while examining a pelvic mass - Bimanual examination is done often under general anesthesia in case of a pelvic mass

241

- Auscultation for bruit depends on condition and location of the mass (over epigastrium, over liver) - Other relevant systemic examination is a must in examination of mass abdomen — respiratory system, skeletal and neurological systems - Masses that may appear and disappear 1. Pseudocyst of pancreas (communicating) 2. Hydronephrosis (intermittent) 3. Choledochal cyst 4. Intussusception

242

COLO-RECTAL SURGERY

243

CHAPTER 1

Functional Colorectal Disorders

CONSTIPATION

Physiology of Defecation

- The stimulus to defecation is distention of the rectum. - Relaxation of the internal anal sphincter (IAS) - Contraction of the external sphincter (EAS) - Sampling response - Squatting position - The angulation between the rectum and the anal canal is straightened out (Figure 1). - Valsalva maneuver coordinated with pelvic floor relaxation. - Inhibition of the EAS, which permits passage of the fecal bolus.

Figure 1. Normal bowel evacuation at rest (A) and during straining (B): pelvic floor and anal function

What is the Normal Defecation Frequency?

- It ranges from 2-3 time/week

244

Definition of Constipation

- Constipation is the infrequent passage of stool. - It is generally defined as ≤ 3 bowel movements per week, which may be associated with straining to defecate, the passage of hard stools, tenesmus, or the need for self-digitation to evacuate stool.

Epidemiology

- Prevalence: 14% of the general population experiences chronic constipation. - Gender: ♀ > ♂ (3:1)

Classification

According to Course

- Acute or chronic - Chronic Functional Constipation Subtypes 1. Slow-transit constipation 2. Pelvic floor dys-synergia = paradoxical contraction or inadequate relaxation of the pelvic Floor muscles during attempted defecation. (is any disturbance of muscular coordination, resulting in uncoordinated movements) obstructed defecation (dys-synergia or anismus) 3. Inflammatory bowel disease (IBS) with constipation

According to Etiology

- Causes and differential diagnosis of constipation are summarized in Table 1. 1. Primary constipation (functional constipation) o It means constipation in the absence of an identifiable medical disorder. o It is most commonly due to poor diet and insufficient exercise. 2. Secondary constipation o It means constipation due to a medical disorder or medication

245

Table 1: Causes and differential diagnosis of constipation

Type of Constipation Causes Examples

Primary Constipation / Normal transit constipation Functional constipation Slow transit constipation Pelvic floor dysfunction - Pelvic floor dys-synergia

Gastrointestinal Causes - Colorectal carcinoma - Diverticulosis / diverticulitis - Anal cancer - Stricture (colonic – rectal) - Thrombosed hemorrhoids - Incomplete bowel obstruction - Complete bowel obstruction - Volvulus - Ileus - Obstructed hernia - Irritable bowel syndrome (IBS) Neurological Causes - Autonomic neuropathy (DM) - Stroke - Hirschsprung disease - Parkinson disease - Chaga‘s disease (megacolon) - Botulism - Multiple sclerosis - Peripheral neuropathy (DM, vitamin B12 deficiency) - Spinal cord injury Secondary Constipation - Pelvic dys-synergia Endocrine Causes - Hypothyroidism - DM - Electrolyte imbalance (hypokalemia, hypercalcemia) - Hyperparathyroidism (HPT) Connective Tissue Disorders - Scleroderma - SLE - Dermatomyositis - Amyloidosis Drug-Induced - Opioid analgesics - Iron supplements - Antacids - Anti-cholinergics - Anti-depressants (tricyclic) - Calcium channel blockers - Bile acid resins - NSAIDs - Beta-blockers - Calcium supplements - Anti-psychotics - Anti-parkinsonism - Anti-convulsants - 5-HT3-receptor antagonists

246

Pathophysiology

- Both primary and secondary constipation can cause changes in stool consistency and defecation habits. External factors such as lack of exercise or inadequate fluid and fiber intake (primary constipation)/internal factors such as changes within the colon or rectum (secondary constipation) → slow passage of stool → prolonged absorption of water by the bowel → dry, hard stool → painful defecation → sensation of incomplete and irregular bowel emptying → constipation. - Mechanism of altered bowel motility 1. Effective peristalsis of the bowel is controlled by intrinsic (e.g. myenteric plexus) and extrinsic (e.g., sympathetic, and parasympathetic) innervation. 2. Any alteration in bowel innervation may lead to ineffective peristalsis. - Drugs (e.g. calcium channel blockers, opiates, antispasmodics, antidepressants) → altered autonomic outflow and bowel muscle contraction. - Endocrine pathology (e.g. hypothyroidism) → down-regulated bowel motility - Neurological pathology (e.g. spinal injury, enteric neuropathy) → disease or trauma of bowel innervation - Ineffective peristalsis → difficult passage of stool regardless of stool consistency → sensation of incomplete and irregular bowel emptying.

Causes of Obstructed Defecation (Mechanical Causes)

Recto-anal Intussusception (Internal or Occult Rectal Prolapse / Procidentia)

- Definition Circumferential, full thickness, enfolding of the mid-rectum during straining on defecography (Figure 2). This enfolding extends into the anal canal, without reaching the anus. - Classification 1. First-degree when the protrusion is invisible. 2. Second-degree when it is visible on straining, 3. Third-degree when visible externally - Etiology 1. Diastasis of the levator 2. Deep Douglas pouch 3. Redundant recto-sigmoid 4. Loss of rectal horizontal position (due to lose attachment to the sacrum) Figure 2. Defecography shows Recto- anal intussusception (arrows)

247

Rectocele

- Definition: Herniation of the rectal wall through a defect in the recto-vaginal septum in the direction of the vagina (Figure 3). - Classification 1. First-degree: 2-4 cm 2. Second-degree: 4-6 cm 3. Third-degree: > 6 cm - Etiology 1. Vaginal childbirth, 2. Chronic ↑ in abdominal pressure 3. Congenital or inherited weakness in the pelvic support system Figure 3. Rectocele (herniation of - Treatment: Most surgeons advocate operative repair the rectal wall) when a symptomatic rectocele is large (> 3 cm) or if it fails to empty sufficiently on defecography.

Enterocele

- Definition: Prolapse of the small bowel into the recto-genital space (Figure 4). - Classification (Pathological) 1. Congenital (unusual deep Douglas pouch) 2. Pulsion-mediated (caused by chronic ↑ of abdominal pressure) 3. Traction (associated with a loss of support of the pelvic floor) - Etiology 1. Primary: Multiparity, advanced age, general lack of elasticity, obesity & ↑ intra- abdominal pressure. 2. Secondary: After gynecological surgical Figure 4. Enterocele (prolapsed small procedures, especially hysterectomy. bowel)

Diagnosis

Patient History - Diagnostic criteria for functional constipation (Rome diagnostic criteria for functional constipation) (Table 2) must include two or more of the following: 1. Straining during > ¼ (25%) of defecations 2. Lumpy or hard stools (Bristol Stool Form Scale 1-2) > ¼ (25%) of defecations 3. Sensation of incomplete evacuation > ¼ (25%) of defecations 4. Sensation of anorectal obstruction/blockage > ¼ (25%) of defecations

248

5. Manual maneuvers to facilitate > ¼ (25%) of defecations (e.g. digital evacuation, support of the pelvic floor) 6. Fewer than three SBM per week 7. Loose stools are rarely present without the use of laxatives. 8. Insufficient criteria for irritable bowel syndrome

- Criteria fulfilled for the last 3 months with symptom onset at least 6 months prior to diagnosis

Table 2. Rome diagnostic criteria for functional constipation in adults

Symptoms / Diagnosis Rome I Rome II Rome III Rome IV

>25% of >25% of >25% of >25% of Straining to evacuate defecations defecations defecations defecations

>25% of >25% of >25% of >25% of Lumpy or hard stools defecations defecations defecations defecations

Sensation of >25% of >25% of >25% of >25% of incomplete evacuation defecations defecations defecations defecations

Sensation of anorectal >25% of >25% of >25% of - obstruction/blockage defecations defecations defecations

Manual maneuvers to >25% of >25% of >25% of - facilitate defecations defecations defecations defecations

Less than three Yes Yes Yes Yes evacuations/week

Number of criteria for ≥2 ≥2 ≥2 ≥2 diagnosis

12 weeks/12 Chronological factor 3 months 3-6 months 3-6 months months

249

Physical Examination - Inspect the anorectal area for possible fissures or hemorrhoids - Check the anal wink reflex: An absent anal wink reflex suggests a pathology (e.g., sacral nerve injury). - Digital examination of the rectum - Check for rectal carcinoma. - Test the sphincter tone to evaluate for pelvic floor dysfunction.

Investigations - Laboratory investigations: exclude hypokalemia, hypothyroidism, diabetes mellitus - Imaging 1. Abdominal X-ray 2. Colon transit time o Procedure: The patient ingests a number of radiopaque markers (plastic rings containing radiopaque material) (Figure 5) in a meal and abdominal radiographs are obtained to monitor the clearance of the rings from the colon. o Normal colonic transit time is 20-56

hours, and most adults will clear all Figure 5: Colon transit time markers the markers in 4-5 days. o Pre-procedural evaluation: Laxatives (and possibly GI-active medications) should be temporarily dis-continued, otherwise, no special preparation is needed. o Technique: 20 or 24 markers are ingested in a single dose, and then serial daily abdominal radiographs are obtained (Figure 6)

Figure 6. Day one after oral intake of the capsule markers.

251

- Colonoscopy: to exclude mechanical obstruction (e.g. tumor, stenosis). - Anorectal manometery and electromyography [EMG] (lack of anorectal inhibitory reflex for Hirschsprung disease, high rectal volume tolerability for rectal inertia and internal sphincter achalasia due to lack of nitrinergic fibers) - Cineodefecography (occult prolapse, sigmoidocele, perineal descent &nd paradoxical puborectalis contraction) - Suction capsule rectal biopsy (for absence or presence of ganglia, and plexus of nerves and possible histochemical staining procedures)

Treatment

Laxatives (Table 3)

Table 3: Laxative agents, mechanism of action and adverse effects

Class Agents Mechanism of Action Adverse Effects

Osmotic - Polyethylene - Increase of osmotic - Diarrhea Laxatives glycol (PEG) pressure draws water into the - Dehydration (very effective intestinal lumen → stimulation - Hypernatremia and well- of intestinal motility. - Hypermagnesemia tolerated, best - Lactulose is degraded by - Severe flatulence initial treatment) intestinal microbiota into lactic (lactulose and sorbitol) - Glycerin acid and acetic acid: - Osmotic laxative misuse is - Magnesium - Induces nitrogen (NH4+) frequently seen in patients hydroxide excretion. with bulemia nervosa - Magnesium - Used in the treatment of hepatic - Overuse causes metabolic citrate encephalopathy acidosis. - Lactulose - Sorbitol Stimulant / - Senna - Stimulation of nitric oxide- - For short-term use only Secretory - Bisacodyl mediated epithelial cell - Diarrhea Laxatives secretion of electrolytes into the - Hypokalemia colonic lumen - Bisacodyl overuse can - Myenteric cause metabolic acidosis neuronal depolarization → colo - Senna may cause n contractions melanosis coli.

Emollient - Docusate - Emulsification (i.e. integration - Diarrhea stool of water and fat) of stool - Metabolic alkalosis softener → softening of stool → easier - Bloating passage through the intestinal - Cramping tract Bulk- - Methylcellulose - Bulk-forming - Bloating forming - Psyllium husks laxatives are indigestible, not - Worsening constipation or Laxatives - Polycarbophil systemically absorbed. ileus if the patient does not - Soluble fibers increase water take enough water with absorption in the doses intestinal lumen → stretching of the bowel wall → stimulation of peristalsis

251

- Biofeedback Techniques for Treatment of Anismus Most studies report good short-term efficacy with improved psychological quality of life, whereas long-term follow-up studies showed a fading effect over time. They include: 1. Sensory training 2. Electro-myographic feedback 3. Manometric feedback. - Surgery (Not for psychological patients) 1. Surgical treatment of rectocele: Trans-vaginal, trans-anal, trans-perineal, and abdominal repair 2. Surgical treatment of rectal intussusception: perineal procedures (Delorme's procedure) 3. Surgical treatment of rectal prolapse (intra-rectal intussusception): Abdominal procedures (open technique) – laparoscopic procedures. 4. Refractory mechanical or functional outlet problems (fecal diversion) 5. Refractory slow transit (subtotal colectomy + ileo-rectal anastomosis)

INCONTINENCE

Definition

- Incontinence is defined as ―recurrent uncontrolled passage of fecal material for at least one month in an individual with a developmental age of at least 4 years‖. - It occurs in 1.4-18% of population.

Factors Necessary for Maintaining Fecal Continence (Figure 7)

- Highly compliant rectal wall. - Normal anorectal angel. - Formed fecal materials. - Normal anal canal and sphincter function. - Anal cushions.

Figure 7: Factors necessary for maintenance of fecal continence (+ anal and rectal sensation)

252

Causes of Incontinence

Any change or inadequacy of the previous factors will lead to a degree of incontinence.

Inadequate Rectal Compliance - Absent rectal reservoir (ileo-anal anastomosis, low ant. resection) - Rectal ischemia - Rectal neoplasms - Altered stool consistency - Inflammatory bowel disease (IBD) - Infectious diarrhea - Laxative abuse - Radiation enteritis - Short bowel syndrome (SBS) - Malabsorption syndrome

Inadequate Rectal Sensation - Dementia, - Cerebrovascular accident (CVA) - Multiple sclerosis (MS) - Brain or spinal cord injury/neoplasm - Sensory neuropathy - Tabes dorsalis - Overflow incontinence - Fecal impaction – leading cause of incontinence in institutionalized elderly patients - Diabetes Mellitus (DM)

Anatomical Sphincter Defect (Internal or External Sphincter) - Traumatic - Obstetric injury – prolonged difficult labor with forceps application, episiotomy complications, third or fourth-degree lacerations - Anorectal surgery – anal fistula surgery (the most common operative procedure that results in fecal incontinence), hemorrhoidectomy.

Pelvic Floor Denervation - Degenerative neurogenic factors are a common cause of non-surgically related incontinence. - Primary (idiopathic neurogenic incontinence) - Pudendal neuropathy: Denervation of puborectalis muscle and external sphincter muscles - Secondary - Injuries to spinal cord, cauda equina - Congenital abnormalities: spina bifida – myelo-meningocele

Rectal Prolapse - Approximately, 60-70% of patients with rectal prolapse suffer from incontinence. 253

Types of Incontinence

- Gas / soft or liquid stool /solid stool - Passive or with awareness - Mild (soiling); moderate; severe

Scoring

Clinical scoring was adopted by Wexner (Cleveland clinic) for detection of the condition severity (Table 4).

Table 4 Cleveland Clinic Scoring System (Wexner`s scoring)

Never Rarely Sometimes Usually, Always Solids 0 1 2 3 4 Liquids 0 1 2 3 4 Flatus 0 1 2 3 4 Use of Pad 0 1 2 3 4 Lifestyle 0 1 2 3 4 Alteration

Diagnostic Evaluation

- Clinical examination especially perineal and neurological examinations. - Laboratory investigations - Procto-sigmoidoscopy: Rigid or flexible procto-sigmoidoscopy can be performed in the office setting and identify characteristic mucosal inflammatory changes e.g. IBD. - Endoanal ultrasonography (US) (Figures 8 and 9): It is a non-invasive tool that is used with increasing frequency to examine anal sphincter integrity. It is a relatively comfortable procedure for the patient and is capable of accurately identifying sphincter defects.

Figure 8: Normal endoanal US Figure 9: Anterior defect IAS and EAS (arrow)

254

- Magnetic resonance imaging (MRI) and dynamic MRI (Figures 10 and 11): - It is valuable in detection of anal sphincter s defects. MRI should be considered in potential candidates for anal sphincters repair.

Figure 10: Normal MRI and sphincters Figure 11: Defect of IAS and EAS

- Ano-rectal Manometry: It is an objective method of studying the physiology of the ano-rectal sphincter mechanism. The principle is based on measurement of the resistance in terms of pressure that the sphincters offer to a constant flow of water. - Electromyography (EMG): Anal EMG is the recording of myo-electrical activity from the striated sphincter at rest, during voluntary & reflex contraction, and simulated defecation. - Pudendal Nerve Terminal Motor Latency (PNTML): It is a simple method of assessing pudendal nerve function. This test indicates the integrity of the distal motor innervation of the pelvic floor musculature. It is important in the evaluation of neurogenic fecal incontinence. - Defecography and video defecography (Figure 12): Barium, thickened to the consistency of stool, is introduced into the rectum. o Evacuation is monitored with fluoroscopy. o Assessment of the anorectal angle at rest and during defecation. o The following may be detected: 1. Excessive perineal descent 2. Failure of the puborectalis muscle to relax 3. Rectocele 4. Internal intussusception.

255

Figure 12: Video defecography

Treatment Options

The choice of treatment needs to be individualized according to the patient, etiology and severity of incontinence.

Non-invasive Treatment 1. Sphincter exercises: These can help improve the bowel control, especially if the main problem is urgency and there is no major defect in the muscles. 2. Bowel habit regulation: Some people find that the bowel responds well to a regular habit. 3. Skin (peri-anal) care and coping with soreness or irritation with careful personal hygiene. 4. Medications: Constipating agents, such as loperamide, codeine phosphate and co- phenotrope (diphenoxylate and atropine), are valuable agents in the treatment of fecal incontinence 5. Biofeedback: It is a behavioral technique that captures the ―bio‖ logical information about an individual. This information is "feed" back to the individual using auditory or visual feedback. The individual uses this information to gain sensitivity, and with practice, control over bodily functions that were dysfunctional or over-functioning and previously thought to be uncontrollable.

Minimally-invasive Procedures 1. Evacuation enema: Another alternative for conservative treatment of anal incontinence is the daily use of colonic irrigation. Usually, patients are instructed to fill their rectum with 500-1000 ml of water while they are seated on a toilet. However, colonic irrigation can potentially cause severe adverse effects and must be carefully administered. 2. Antegrade continent enema (Malone procedure): For patients with rectal evacuation disorders, regular antegrade irrigation of the colon is employed to achieve evacuation. 3. Anal encircling procedure (Thiersch Procedure): It was originally advocated for treatment of rectal prolapse, then for the treatment of anal incontinence. Complications of the procedure include infection, stricture, fecal impaction, persistent incontinence and pain. 4. Anal plugs: Anal plug is another non-surgical option for patients who want to avoid surgery.

256

5. Radiofrequency Energy (SECCA procedure): The addition of temperature-control technology in the last decade has allowed radiofrequency to be utilized for more specific therapeutic applications. 6. Tissue augmentation: Injections directly fill the asymmetric anal canal defect using a bulking agent to augment the internal anal sphincter (IAS) muscle. Bulking agents include Polytetrafluoroethylene paste (PTP implants), Teflon, Polytex, Autologous fat, Micro-carbon coated beads, Collagen & silicone injection (Figure 13). These are injected trans-sphincteric ally to the IAS. This has been recently shown to ↑ anal canal resting pressure in healthy volunteers. This novel agent may have a future role in the treatment of passive fecal incontinence associated with a weak IAS.

Figure 13: Internal anal sphincter (IAS) defect treated by injectable agents (silicone topical phenylephrine gel)

Invasive (Surgical) Treatment

A. Sphincter Repair Sphincter repair is considered in patients with an isolated sphincter defect because the remaining muscle usually maintains the ability to contract. There are 3 approaches to sphincter repair: namely, apposition, placation, and overlapping. 1. Direct apposition - It involves mobilization of the external anal sphincter (EAS) and suture of the muscle ends in an end-to-end fashion. 2. Post-anal repair - It was devised by Parks for the treatment of idiopathic and neurogenic incontinence. - The puborectalis limbs are approximated and the EAS is plicated. 3. Overlapping repair (Figure 14) - It is preferable to a simple apposition of the sphincter muscles. - Patients best suited are those in whom incontinence is 2ry to an anterior sphincter defect. - Obstetric and iatrogenic causes are among the commonest surgically correctable faecal incontinence.

257

Figure 14: Overlapping repair

4. Total pelvic floor repair - It is the combination of anterior levator plasty and IAS plication with post-anal repair. - This operation was proposed by Keighley for patients who had a suboptimal outcome after post-anal repair. 5. Anterior levator plasty and EAS plication - It is preferred by some surgeons > post-anal repair, which has a poor functional outcome, especially on long-term results. - It is used mainly in post-obstetric fecal incontinence). 6. Puborectalis plication - Physical finding of lax puborectalis musculature, obtuseness of the ano-rectal angle and bulging of the anterior rectal wall into the proctoscope suggest incontinence.

B. Muscle Wrap The most widely used for the creation of a new anal sphincter are transposed skeletal muscles. Muscles that may be suited for muscle wrapping are the following: 1. Sartorius muscle - It has segmental vascularisation, which restricts the arch of rotation if the muscle is transposed, the sartorius muscle seems to be less-well suited. 2. Gluteus maximus - The choice of this muscle to recreate an anal sphincter is logical because it is a powerful muscle that often is used as an accessory muscle to continence. 3. Gracilis muscle - First used for sphincter-wrapping in 1952 by Pickerel in the treatment of children with faecal incontinence due to a neurogenic cause. - It has been applied on a wide scale with mixed results. Although active contraction of the muscle is forceful enough to hold up stool, still all-day continence is seldom achieved. This is also because the gracilis, like all skeletal muscles, is unable to have a sustained contraction.

258

C. Artificial Sphincter Replacement (Figure15) - Artificial sphincters have been used to treat urinary incontinence since 1973. - In 1987, Christiansen and Lorenzen applied a device (called the American Medical System) to a patient with fecal incontinence. The implantation procedure involves the surgical insertion of a cuff in a tunnel around the anal canal, tubing between the cuff and an abdominal incision, a balloon behind the rectus abdominis, and a pump in the labia majora or scrotum.

Figure 15: Artificial anal sphincter device in place

D. Neuromodulation 1. Dynamic graciloplasty (Figure16) - Gracilis muscle is wrapped around the anal verge and its nerve supply is connected to a neuro-stimulator via electrodes (Figure 17) and the neurostimulator placed subcutaneously. This neuro-stimulator maintains muscle contraction and continence. This overcomes the problem of skeletal muscle fatigue. - The average patient accepted for dynamic graciloplasty has a severe incontinence for many years, has had maximal conservative treatment, biofeedback training and one or more previous operations for restoration of continence.

Figure 16: Dynamic graciloplasty Figure 17: Neuro-stimulator

259

2. Sacral Nerve Stimulation (SNS) (Figure 17) - Nowadays, stimulation of the sacral nerve roots is successfully used to control urinary incontinence, but only recently has experience gained with this technique been transferred to the field of ano-rectal disturbances, above all fecal incontinence. - The concept is to recruit residual function of the ano-rectal continence organ by electrical stimulation of its peripheral nerve supply.

Figure 17: sacral nerve stimulation.

E. Diverting Stoma - Although fecal diversion may be considered a failure of fecal incontinence treatment, it is an effective, safe, and appropriate operation for certain patients with severe incontinence.

261

CHAPTER 2

Diverticular Disease and Polyps

DIVERTICULAR DISEASE

Types

- Diverticula can occur in a wide number of positions in the gut, from the esophagus to the recto-sigmoid. - There are two varieties: 1. Congenital: All 3 coats of the bowel are present in the wall of the diverticulum, e.g. Meckel‘s. 2. Acquired: The wall of the diverticulum lacks a proper muscular coat. Most alimentary diverticula are thought to be acquired.

A. DIVERTICULA OF THE SMALL INTESTINE

Most of these diverticula arise from the mesenteric side of the bowel, probably as the result of mucosal herniation through the point of entry of blood vessels.

Duodenal Diverticula

There are two types: 1. Primary. Figure 1. Duodenal Mostly occurring in older patients on the diverticula nd rd inner wall of the 2 and 3 parts (Figure on the inner 1). These diverticula are found wall of the nd incidentally on barium meal and are 2 part of duodenum. usually asymptomatic. They can cause problems locating the ampulla during ERCP. 2. Secondary. Diverticula of the duodenal cap result from long-standing duodenal ulceration.

261

Jejunal Diverticula

- These are usually multiple and of variable size (Figure 2). Clinically, they may (1) be symptomless, (2) cause abdominal pain, (3) produce a malabsorption syndrome or (4) present as an acute abdomen with acute inflammation and occasionally perforation. - They are more common in patients with connective tissue disorders. In patients with major malabsorption problems, they give rise to anemia, steatorrhea, hypo-

proteinemia or vitamin B12 deficiency. - Resection of the affected segment with end- to-end anastomosis (R-EEA) can be an

effective treatment. Figure 2. Multiple jejunal diverticula

Meckel’s Diverticulum

- Meckel's diverticulum represents the patent intestinal end of the vitello-intestinal tract (VIT) (Figure 3)

Incidence and Location - It is present in 2% of the population. - It is situated on the anti-mesenteric border of the small intestine, commonly 60 cm (2 feet) from the ileo-cecal valve, and is usually 3–5 cm (2 inches) long. Many variations occur - "2% – 2 feet – 2 inches" is a useful aide-mémoire (Figure 4).

Figure 3. Vitello-intestinal tract (blue arrow) Figure 4. Meckel's diverticulum: The rule of "two"

262

Pathology - A Meckel‘s diverticulum possesses all 3 coats of the intestinal wall & has its own blood supply (Figure 5). It is ∴ vulnerable to infection & obstruction in the same way as the appendix. Indeed, when a normal appendix is found at surgery for suspected appendicitis, a Meckel‘s diverticulum should be sought by inspection of an appropriate length of terminal ileum. - In 20% of cases, the mucosa contains heterotopic epithelium, namely; 1. Gastric

2. Colonic Figure 5. Meckel's diverticulum has all 3. Sometimes pancreatic tissue. 3 coats of the intestinal wall

Clinical Presentation - In order of frequency, these symptoms are as follows: 1. Severe hemorrhage (Figure 6) caused by peptic ulceration. Painless bleeding occurs per rectum and is maroon in color. An operation is sometimes required for serious progressive gastrointestinal (GI) bleeding. When no lesion in the stomach or duodenum can be found, the terminal 150 cm of ileum should be carefully inspected. 2. Meckel‘s diverticulitis (Figure 7) may be difficult to distinguish from the symptoms of acute appendicitis. When a diverticulum perforates, the symptoms may simulate those of a perforated duodenal ulcer (DU). At operation, an inflamed diverticulum should be sought as soon as it has been demonstrated that the appendix and Fallopian tubes are not at fault.

Figure 6. Hemorrhage from Meckel's Figure 7. Inflammation of Meckel's diverticulum diverticulum

263

3. Chronic peptic ulceration (PU) • As the diverticulum is part of the mid-gut, the pain, although related to meals, is felt around the umbilicus. • It is caused by the presence of ectopic gastric epithelium in the wall of the Meckel's diverticulum (Figure 8).  The base of the diverticulum should be palpated intra-operatively to rule out any thickening that may represent ectopic tissue.

Figure 8. Ectopic gastric tissue in a Meckel's diverticulum (white arrow)

4. Intussusception (Figure 9): In most cases, the apex of the intussusception is the swollen, inflamed (Figure 10), with heterotopic epithelium at the mouth of the diverticulum.

Figure 9. Intussusception of Meckel's Figure 10. Swollen & inflamed apex of the diverticulum intussusception

264

5. Intestinal obstruction. The presence of a band between the apex of the diverticulum & the umbilicus (Figure 11) may cause obstruction either by the band itself or by a volvulus around it. If neglected, it may cause gangrene of the bowel. (Figure 12).

Figure 11. a band between the apex of Figure 12. If neglected, it may cause the diverticulum & the umbilicus gangrene of the bowel.

- ‗Silent‘ Meckel‘s diverticulum An aphorism attributed to Dr. Charles Mayo is: ‗a Meckel‘s diverticulum is frequently suspected, often sought & seldom found‘. A Meckel‘s diverticulum usually remains symptomless throughout life and is found only at necropsy. When a silent Meckel‘s diverticulum is encountered in the course of an abdominal operation, it can be left provided it is wide mouthed and the wall of the diverticulum does not feel thickened (Figure 13). Where there is doubt and it can be removed without appreciable additional risk, it should be resected. - Exceptionally, a Meckel‘s diverticulum is found in an inguinal or a femoral hernia sac – Littre‘s hernia (Figure 14).

Figure 13. "Silent Meckel's Figure 14. Meckel's diverticulum in an diverticulum" could be left alone inguinal hernia (Littre's hernia - rare)

265

Investigations (Imaging) - Meckel‘s diverticulum can be very difficult to demonstrate by contrast radiology; small bowel enema would be the most accurate investigation (Figure 15). - Technetium-99m (99mTc) scanning may be useful in identifying Meckel‘s diverticulum as a source of GI bleeding (Figure 16).

Figure 15. Small bowel enema showing Figure 16. Technetium-99m (99mTc) Meckel's diverticulum (white arrow) scanning showing bleeding from Meckel's diverticulum (black arrow) Treatment - Meckel‘s diverticulectomy o A Meckel‘s diverticulum that is broad-based should not be amputated at its base & invaginated in the same way as a vermiform appendix, because of the risk of stricture. Furthermore, this does not remove heterotopic epithelium when it is present. o Alternatively, a linear stapler device may be used (Figure 17). - Where there is induration of the base of the diverticulum extending into the adjacent ileum, it is advisable to resect a short segment of ileum containing the diverticulum, restoring continuity with an end-to-end anastomosis (Figure 18).

Figure 17. Meckel's Figure 18. Resection of adjacent diverticulectomy using stapler segment of small bowel

266

B. DIVERTICULAR DISEASE OF THE COLON

Prevalence (by Age)

< 50 years ………………. 5-10 % > 50 years. ……………... 30 % > 70 years ………………. 50 % > 85 years ………………. 65 %

Pathogenesis

- The start is disordered colonic motility, which leads to segmentations of the colon into "bladders" (Figure 19) separated by contraction rings with pressures reaching 90 mmHg inside these bladders. Segmentation causes high pressure and the pulsion force responsible for diverticulosis (Figure 20) i.e. herniation of colonic mucosa through the circular muscle at the points where the blood vessels penetrate the colonic wall (Figure 21).

Figure 19. disordered colonic motility → segmentations of the colon

Figure 20. Segmentation of the colon causes high pressure and the pulsion force responsible for diverticulosis

Figure 21. Herniation of colonic mucosa through the circular muscle at the points where blood vessels penetrate the colonic wall

267

Clinical Presentation (Table 1)

- Approximately, 90% of cases are asymptomatic - It is important to distinguish between diverticulosis, which may be asymptomatic, and clinical diverticular disease in which the diverticula cause symptoms. - Diverticulitis is the result of inflammation of one or more diverticula, usually with some peri-colitis. It is not a precancerous condition, but cancer may coexist - Pain is due to diverticulosis or due to inflammation (diverticulitis). Pain due to diverticulosis o Colicky, of variable severity and duration (may be for days) o Felt in left iliac fossa (LIF) and hypogastrium o Precipitated by food & stress, and relieved by flatus o Stools are small hard pieces (sheep droppings) Pain due to diverticulitis o Simulates left-sided appendicitis. Well localized + rigidity, tenderness and rebound tenderness, with a possible palpable inflammatory phlegmon

Table 1: Clinical Picture of Sigmoid Diverticular Disease

Elective Cases (Mild) Emergency Cases

- Distension, - Persistent lower abdominal pain, usually in the LIF ± - Flatulence peritonitis, could be caused by diverticulitis. Fever, - Sensation of heaviness in malaise & leucocytosis can differentiate diverticulitis the lower abdomen that may from painful diverticulosis. be indistinguishable from - The patient may pass loose stools or may be those of irritable bowel constipated; syndrome (IBS). - Tender left lower abdomen, but occasionally also in the RIF if the sigmoid loop lies across the midline. - The sigmoid colon is often palpable, tender and thickened. - PR exam may, but not usually, reveal a tender mass. - Any urinary symptoms may denote a vesico-colic fistula, which → pneumaturia (flatus in urine) & even fecaluria (stools in urine).

Clinical Picture of Complications 1. Perforation - Localized → left-sided peritonitis, and if walled off, a fluctuant tender mass may be palpable (peri-colic abscess) - Free → generalized peritonitis, with rapid general condition deterioration 2. Diverticulitis

268

3. Obstruction - Large bowel obstruction (LBO) is due to repeated inflammation with dense fibrous tissue constriction - Small bowel obstruction (SBO) may be due to adhesions at the site of large bowel inflammation 4. Fistula (5%) - Abscess will burst into a nearby organ causing fistulae (small bowel, uterus, vagina, abdominal wall or perineum, and urinary bladder) - Colo-vesical fistula is heralded by urgency and dysuria, and its manifestations are pneumaturia & fecaluria 5. Hemorrhage (15%) - The two common causes of massive lower GIT bleeding (that may require blood transfusion) in elderly patients are diverticulosis and vascular ectatic lesions.

Natural History of Diverticulosis (Figure 22)

Figure 22. Natural history of diverticulosis. It may be asymptomatic (70%) or present with profuse bleeding (5-15%) or inflammation (diverticulitis often with peri-colitis) that may be complicated with abscess formation, perforation, obstruction or fistula formation.

Hinchey Classification of Diverticulitis (Table 2) (Figure 23)

Table 2: Hinchey classification (staging) of diverticulitis

Stage Severity Pain Systemic Investigation Management I Peri-colic abscess LIF Possibly Delayed Bowel rest, IV or phlegmon no change barium antibiotic, DVT enema, prophylaxis & fluids endoscopy II Pelvic or intra- Severe, Mild toxic CT scan Percutaneous drainage abdominal abscess fullness - LIF III Non-feculent Peritonitis Toxic CT scan Resuscitation + peritonitis operation IV Feculent peritonitis Peritonitis Severe Proceed to Resuscitation + toxicity, operation immediate operation shock

269

Figure 23. Classification of diverticulitis (Hinchey Classification System).

Diagnosis / Investigations

1. In the acute phase, computerized tomography (CT) scan (Figures 24 & 25) is particularly useful (high specificity) by identifying the following: - Bowel wall thickening, - Abscess formation (Figure 26) - Extra-luminal disease or other disease. - In addition, CT-guided percutaneous drainage may be done and postpone further operative procedures.

Figure 24. Multi-slice CT scan showing diverticula

271

Figure 25. Multi-slice CT scan with Figure 26. CT scan with showing diverticular virtual colonography showing diverticula abscess with fluid level (yellow arrow).

2. In elective cases: - Barium enema (Figures 27-34) and endoscopy (sigmoidoscopy and colonoscopy) are the main methods of diagnosis. Barium enemas are usually reserved for patients who have recovered from an attack of acute diverticulitis for fear of causing perforation or peritonitis. Barium enema is done to exclude a carcinoma and to assess the extent of the disease. - Water-soluble contrast enemas (Figure 35) are helpful in sorting out patients with large bowel obstruction. In the acute situation, it is good at detecting intra-luminal changes & leakage (Figure 36). 3. In presence of inflammation or localized perforation: - CT scan is the investigation of choice. 4. In presence of obstruction and free perforation: - Plain X-ray abdomen is diagnostic; also US is informative as regards fluid collections. 5. In presence of fistula: - Barium enema is the best method + colonic endoscopy (Figures 37 and 38). - In case of colo-vesical fistula, cystoscopy and biopsy should be added. 6. In the presence of hemorrhage: - Angiography and colonoscopy are of value.

271

Figure 27. Barium enema showing sigmoid Figure 28. Barium enema showing a large diverticular disease (saw-teeth appearance) white filling defect in the sigmoid colon caused by a arrow). peri-colic abscess (white arrow).

Figure 29. Barium enema showing diverticular Figure 30. Barium enema showing area of disease involving the whole colon. spasm with a zigzag appearance in the sigmoid colon (white arrow)

272

Figure 31. Barium enema showing 2 areas of Figure 32. Barium enema showing a colo- narrowing (white arrows), one in the sigmoid vesical fistula denoted by the black arrow. colon & the other in the mid-descending colon, each representing a stricture 2ry to diverticulitis.

Figure 33. Barium enema showing colo-enteric Figure 34. Barium enema showing a walled-off fistula denoted by the black arrows. peri-colic abscess with a colo-vaginal fistula.

273

Figure 35. Water-soluble contrast enema Figure 35. Water-soluble contrast enema showing multiple diverticula of the sigmoid showing leakage of contrast due to diverticular colon (white arrows). disease.

Figure 35. Colonoscopy showing the "mouth" of Figure 36. Colonoscopy showing the "mouth" of multiple diverticula. multiple diverticula with inflammation.

274

Differentiation of Diverticulitis from Carcinoma of the Colon (Table 3)

Table 3: Differences between diverticulitis and carcinoma of the colon

Criteria Diverticulitis Carcinoma - History Long Short - Pain More common 25% painless - Mass 25% have tenderness over the Sausage shaped, felt in left iliac inflammatory mass fossa, sometimes the above loaded colon is the only thing palpable - Bleeding 17% often perfuse, periodic 65% usually small amounts persistently present - Radiograph Diffuse change, with the Localized, shouldering, characteristic characteristic saw teeth and apple core appearance multiple diverticula appearance - Sigmoidoscopy Inflammatory change over an area, No inflammatory signs, however, and/or with mouths of diverticulae seen diverticula can be also present. colonoscopy scattered Carcinoma is biopsied

Treatment of Diverticular Disease

1. Patients with Diverticular Pain - Bulk laxative + antispasmodics - Sigmoid colectomy if conservative treatment fails

2. Patients with Uncomplicated Diverticulitis - Conservative treatment (bowel rest, IV fluids, bed rest, antibiotics for 4-5 days, pain control with Meperidine [better than Morphine]. After 3 weeks, endoscopy is performed to rule out malignancy, then 4-6 weeks later, elective resection with primary anastomosis is performed (if appropriate). - Indications of elective surgery a. ≥ 2 acute attacks, successfully treated medically b. One attack requiring hospitalization in patient < 40 Figure 37. Primary resection and end-to-end anastomosis years old. c. One complicated attack d. One attack in an immuno-compromised patient e. Inability to rule out colonic carcinoma - Operative procedure o The ideal operation carried out as an interval procedure after careful preparation of the gut is a one-stage resection. o This involves removal of the affected segment and restoration of continuity by end-to- en anastomosis (Figure 37).

275

3. Treatment of Complicated Diverticular Disease

Diverticulitis with perforation - Stage 1 (contained peri-colic abscess) → resection + end-to-end anastomosis (EEA) ± protecting colostomy - Stage 2 (walled-off pelvic abscess due to leaking peri-colic abscess) → Hartmann procedure (Figure 38) - Stage 3 (generalized purulent peritonitis due to perforated pelvic abscess) → (1) Hartmann procedure, or (2) 1ry resection and anastomosis after on-table lavage in selected cases (Figure 39), or (3) 1ry resection and anastomosis after on-table lavage with covering ileostomy - Stage 4 (generalized fecal peritonitis due to perforated diverticulum) → exploration and emergency Hartmann procedure

Figure 38. Hartmann procedure (end-colostomy in the left iliac fossa & closure of the distal rectal stump).

Figure 39. Resection and end-to-end anastomosis after on-table colonic lavage.

276

Obstruction - If the obstruction is due to small bowel adhesions → adhesolysis and resection of the affected segment with EEA. - In colonic obstruction in the presence of inflammatory edema and adhesions, or the bowel is loaded with feces → it is better to perform Hartmann procedure and avoid EEA. A primary anastomosis with a covering proximal stoma can also be used.

Fistulation - Most patients are best treated by elective sigmoid resection with 1ry anastomosis and repair of the fistulous opening in the affected organ. - In the case of a colo-vesical fistula, it is usually possible to ‗pinch off‘ the affected bowel from the bladder, close it & then resect the sigmoid (elective resection, 1ry re-anastomosis + insertion of Foley catheter for 7-10 days). - In very difficult cases, a staged procedure with a preliminary defunctioning stoma may be necessary.

Hemorrhage - Fortunately the hemorrhage, although severe, is rare to cause shock; only profound anemia requiring transfusion is common. It usually responds to conservative treatment, occasionally it requires resection. Emergent / elective resection, 1ry re-anastomosis - Endoscopy can locate the area of hemorrhage (rectum, sigmoid or above the sigmoid) in a good % of cases. If the source cannot be located, then subtotal colectomy and ileostomy is the safest option

Diverticular disease with carcinoma - Diverticular disease and carcinoma coexist in 12% of cases. - Exploration may be necessary but, even then, differentiation may be difficult until histological investigations are available.

Laparoscopic Surgery - In selected cases, laparoscopic surgery has been used for sigmoid resection. - This has the benefit of decreased hospital stay and costs. - However, there is little high-quality

research in the field to advocate its true Figure 40. Diverticula seen during merits. laparoscopy

277

POLYPS OF THE COLON AND RECTUM

Definition A polyp is a swelling arising from colonic mucosa on a single pedicle producing a visible protrusion above the surface of the surrounding normal large bowel mucosa (Figure 41)

Classification (Tables 4 and 5) Polyps can be classified according to different variables as follows: - Congenital - Acquired - Neoplastic - Non-Neoplastic - Benign - Malignant

- Symptomatic - Asymptomatic - Sessile - Pedunculated Figure 41. A polyp is merely a descriptive - Single - Multiple term of any lesion elevated above the mucosal surface of the large bowel. - A Disease - Syndrome

A. BENIGN COLO-RECTAL POLYPS

Table 4: Histological classification of benign colo-rectal polyps

Polyps Single Multiple Inflammatory Benign lymphoid polyp Pseudopolypi may be multiple in IBD. diverticular disease, Bilharziasis Hamartomatous - Juvenile polyp (70%) - Juvenile polyposis (30%) - Peutz-Jeher polyp - Peutz-Jeher syndrome - Cronkhite-Canada syndrome - Cowden disease Hyperplastic or Metaplastic polyp Often multiple metaplastic Neoplastic Adenoma (tubular, tubulo- Familial adenomatous polyposis (FAP) (epithelial) villous or villous) Neoplastic Leiomyoma – Lipoma - Often single (submucosal) Neuro-fibroma - Hemangioma

Table 5: Classification according to their malignant potential

Polyps with NO Malignant Potential Polyps with NO Malignant Potential Non-neoplastic Neoplastic (Adenoma) - Inflammatory - Tubular (0-25% villous tissue) - Hamartomatous - Tubulo-villous (25%-75% villous tissue) - Hyperplastic - Villous (75%-100% villous tissue). - Lymphoid

278

Inflammatory Polyps (Pseudo-polyps)

- These lesions develop as by-products of the ulcers that penetrate into the submucosa, leaving islands of adjacent regenerative mucosa (Figure 42). - They are associated with ulcerative colitis (UC), Crohn's disease, chronic dysentery, ischemic colitis and schistosomiasis. They can be associated with bilharzial colonic mass. - They are multiple, concentrated in the sigmoid area, different in sizes and stalk length, bleed on touch, can be easily avulsed leaving a bleeding ulcer, and are associated with excessive mucus and blood-tinged mucus. Figure 42. Inflammatory colonic polypi - Diagnosis rests on histopathology.

Hamartomatous Polyps

Hamartomatous polyposis syndromes include (1) juvenile polyposis syndrome, (2) Peutz-Jeher syndrome and (3) Cronkhite-Canada syndrome

Juvenile Polyposis Syndrome - Juvenile polyps occur in infants and children <10 y, usually single (70%) and often multiple, pedunculated (Figure 43), usually within the reach of the finger. They affect males > females, with familial tendency, present by bleeding/rectum and treated by excision through the endoscope. - Juvenile polyposis means the presence of 10 juvenile polyps in the GIT. It is inherited as autosomal dominant (AD) disease due to mutation of SMAD4 gene on chromosome 18. Thus, they are "developmental malformations" affecting the glands and lamina propria, occurring commonly in the

rectum of children <5 years. In adults, they are called "retention polyps". The risk of Figure 43. Juvenile colonic polypi colon cancer is 50%. There is also ↑ risk of (pedunculated) gastric, duodenal and pancreatic cancers

279

Peutz-Jeher Syndrome - It is autosomal dominant, due to mutations of the STK11 gene on chromosome 19. There is a very strong familial tendency - Characterized by peri-oral and buccal mucosa pigmentations (Figure 44) and hamartomatous polyps throughout the GIT (Figure 45) mainly in the small bowel (78%), colo-rectal (70%) and stomach (40%). - Both, gastro-intestinal (GI) and non-GI cancers are common. The GI cancers include colon, pancreas, stomach, small bowel and esophagus. Non-GIT cancers include breasts, ovaries, uterus and lungs.

Figure 44. Peri-oral melanosis Figure 45. Melanosis & small polyp

Cronkhite-Canada Syndrome - Gastrointestinal (GI) hamartomatous polyposis causes diarrhea, weight loss and abdominal pain - Extra-GI manifestations include alopecia, cutaneous hyper-pigmentation and onycho- dystrophy - Treatment: Nutritional support, corticosteroids, acid suppression and antibiotics - The 5-year mortality rate (MR) may reach 55% with most deaths resulting from GI bleeding, sepsis and congestive heart failure (CHF).

Metaplastic (Hyperplastic) Polyps Figure 46. Hyper-plastic - They constitute the majority of non-neoplastic (meta-plastic) polyp (black polyps, with prevalence rates of 20-34% at arrow) autopsy and screening colonoscopic studies. - Predominantly located in distal colon & rectum. - Plaque-like protrusions, small (<0.5cm), with smooth glistening surface (Figure 46). - After biopsy, they only require observation.

Lymphoid Polyps

- They present with mucosal nodularity (Figure 47) representing lymphoid hyperplasia (Figure 48)

281

Figure 47. Lymphoid polyps Figure 48. Lymphoid hyperplasia

Neoplastic / Epithelial (Adenomatous) Polyps (three main types)

1. Adenomas Facts and Figures - ↑ with age (50% by 70 years as compared to 33% of population by 50 years ) - 70 % of all colo-rectal polyps are adenomatous - 70 % are located in the left colon - 70 % are solitary (30% synchronous) - 70 % are small (<1 cm in size) - 7 % have severe dysplasia, 3-5% have invasive cancer

Adenoma-Carcinoma Sequence Regardless of etiology, most colo-rectal cancers (CRC) arise from adenomas as shown in Figure 49.

Figure 49. Adenoma–carcinoma sequence. CRC: colo-rectal cancer

281

Factors determining the risk of malignant transformation (Table 6)

Table 6: High- and low-risk factors of malignant transformation of adenomatous polyps

High Risk Low Risk

- Large size (> 1.5 cm) - Small size (<1 cm) - Sessile or flat - Pedunculated - Severe dysplasia - Mild dysplasia - Villous architecture (>25% villous - Tubular architecture component) - Single polyp - Polyposis syndrome (multiple polyps - >3 of any size)

2. Tubular or Tubulo-villous adenoma - Vary in size from few mm to 7 cm. - As they grow they attain a darker color and look more vascular and become pedunculated

3. Villous Adenoma - Sessile, large 3-7 cm and expand in a carpet like fashion around the lumen and vertically. - The surface is coarsely granular, there is always very much mucus and it bleeds on touch. - It usually affects the rectum. - They carry a high risk of colorectal cancer (CRC) - It should be managed as carcinoma in-situ.

Differences between Tubular and Villous Polypi (Table 7)

Table 7: Characteristic differences between tubular and villous polypi

Tubular Villous >85% <15% Males > females Females > males Often multiple Single, spread in a carpet-like fashion around the lumen of the bowel 70% are pedunculated 90% are sessile Varies in size from 1-7 cm Same MP: consists of closely packed gland MP: covered with a single layer of colonic tubules with varying grades of mucosa, which may show changes of differentiation carcinomatous degeneration About 5% turn malignant About 30% turn malignant

282

Familial Adenomatous Polyposis (FAP) (and its variants)

- They present 1% of CRC. Figure 50. Familial - Hundreds of different sized polypi (Figure adenomatous polyposis (FAP) 50). coli. (>100 polyps) - Familial inheritance as an autosomal dominant gene with ≈ 100% penetrance. - Patients develop adenomas by the mean age of 15 y (puberty), and develop CRC by the age of 40 y. - It is a disease of abnormal tumor initiation, caused by mutations of APC gene (tumor suppressor gene) on chromosome 5q21 that encodes for a protein, which functions in cell adhesion and signal transduction. Mutations results in truncated protein & affects cell growth (Figure 51)

Figure 51. Mechanism of carcinogenesis in FAP

- Screening of FAP 1. Genetic screening of family members for APC mutations (Figure 52) 2. Annual flexible sigmoidoscopy starting at 10-12 y until the age of 40 years, then every 3-5 years. If polyposis is present, colectomy should be considered 3. Upper GIT Endoscopy every 1-3 y is also recommended to evaluate for upper GI adenomas - Diagnosis of FAP 1. Endoscopy / biopsy Figure 52. Protein truncation test showing mutation (arrow) 2. Genetic tests: Protein truncation test (will show mutation) - DNA sequencing

283

- Extra-colonic manifestations of FAP 1. Congenital hypertrophy of retinal pigmented epithelium (CHRPE) (Figure 53) 2. Gardner‘s syndrome: Desmoid tumors (Figure 54), osteomas (Figures 55 and 56) and multiple epidermoid (sebaceous) cysts and connective tissue tumors. 3. Turcot syndrome: CNS malignancies including medulloblastoma and glioblastoma 4. Duodenal, hepato-biliary-pancreatic and thyroid tumors

Figure 53. CHRPE in FAP (arrow) Figure 54. CT showing desmoid tumors in FAP

Figure 55. Plain X-Ray showing skull Figure 56. Plain X-Ray showing mandibular osteoma in FAP osteoma in FAP

Attenuated FAP - It is a variant of FAP - Number: < 100 adenomas - Age: Late age of onset: Adenomas at 44 years and CRC at 56 years - Location: Proximal distribution of adenomas - Surveillance: Colonoscopy is used for surveillance - Treatment: The infrequent involvement of the rectum supports the role of total colectomy & ileo-rectal anastomosis (IRA).

Hereditary Non-Polyposis Colo-rectal Cancer (HNPCC) - It represents 2-5% of all CRC - Autosomal dominant with 70-80% penetrance - It takes only 3-5 ys to develop CRC from adenomas

284

- Molecular genetics of HNPCC (accelerated progression) (Figure 11): Mutations of MMR genes → replication errors during DNA synthesis (micro-satellite instability) → acceleration of genetic mutations. HNPCC patients develop adenomas at the same rate as the general population. Once these adenomas develop, however, defective DNA repair ensues & mismatches accumulate. Thus, it takes only 3-5 y to develop CRC from adenomas. - HNPCC: Lynch Syndromes 1. Lynch syndrome I - Early onset of CRC (40-45 y). - Occurs predominantly proximal to the splenic flexure (60-70%). - Increased frequency of synchronous and metachronous lesions (33%) 2. Lynch syndrome II - Same features of Lynch I syndrome + extra-colonic malignancies such as endometrial (most common, gastric, small bowel, hepato-biliary, ovarian, ureteral and renal tumors. - Screening for NHPCC 1. Colonoscopy every 2 years starting at ages 20-25 or years younger than the earliest diagnosis of CRC, whichever is earlier until 40y, and then annually 2. Flexible sigmoidoscopy is not acceptable, due to the proximal location of tumors 3. Trans-vaginal US and endometrial aspiration annually starting at 25-35 years - Diagnosis of NHPCC Due to lack of phenotypic markers like polyps, diagnosis is based on family history of CRC only Amsterdam Criteria 1 1. One member less than 50 years of age 2. Two involved generations 3. Three family members affected, one of whom is a first-degree relative of the other two Amsterdam Criteria 2 Same as Amsterdam 1, but includes all HNPCC-related tumors

Clinical Features and Diagnosis of Polyps

- Most polyps are asymptomatic; many of them can be diagnosed by occult blood in stools. - Bleeding per rectum is the most common symptom (ranges from mild to severe) - Bouts of colonic colic due to intussusception. - Rectal manifestations (protrusion, tenesmus and excessive mucus discharge) (if in excessive amounts it may lead to hypokalemia and metabolic acidosis) - Diagnosis is by either barium enema and double contrast or endoscopy (better for biopsy). There is a definite malignant potential to colorectal polyps (adenomatous). - Predicting severity by endoscopy: 1. Mild disease: < 5 rectal adenomas 2. Moderate: 3-19 adenomas 3. Severe: 20 or more adenomas.

285

Treatment of Polyps

Factors affecting Treatment of Colorectal Figure 57. Polyps Endoscopic snaring of a 1. Location: Colon or rectum small polyp 2. Number: Single or multiple 3. Morphology: Pedunculated or sessile 4. Histology: Benign or malignant.

Excision (Polypectomy) - Pedunculated polyps Diathermy snaring or coagulation using the flexible sigmoidoscope or

colonoscope with CO2 insufflations, and insulated snares may safely remove polyps (Figure 57). - Sessile polyps If possible  colonoscopic polypectomy o Larger polyps may require  piecemeal removal o 5-8 polyps  snaring polypectomy o > 8 polyps  removal of affected segment (segmental colectomy) If endoscopic removable is not possible  operative removal o Colon  colectomy o Rectum  staged with EUS or MRI a. Benign / early malignant (T1No)  Trans-anal local excision or TEMS (may need further radical surgery) b. Other malignant polyps  radical excision (APR /anterior resection) - Complications of Polypectomy 1. Risk of immediate bleeding & also delayed bleeding depending on vasculature of the stalk 2. Colonic perforation (uncommon, right colon > left due to thinner bowel wall). 3. Polyps may be missed due to incomplete examination, blind spots or poor preparation

Radical Surgery - Factors determining the need for radical surgery (unfavorable histology): 1. Cancer within 2 mm of cut edge of stalk. 2. Poorly differentiated cancer. 3. Lympho-vascular invasion. 4. Unfavorable shape - Adenomatous polyps containing cancer present a dilemma for the endoscopist. Should the patient undergo colectomy or not? Polyps with "unfavorable" histology (mentioned above) are an indication for bowel resection while those with "favorable histology" can be spared.

286

B. MALIGNANT COLO-RECTAL POLYPS

Definition

- A malignant polyp is one that contains invasive cancer. Malignant cells that have invaded through the mucularis mucosa into the submucosa (Figure 58)

Figure 58. Malignant polyp with malignant cells reaching the submucosa.

Conventional Treatment of Malignant Polyps

- If an experienced colonoscopist is not available, open colotomy provides a way of surgical polypectomy (better to use a frozen section to proceed for segmental colectomy in case malignancy is shown) - Villous papilloma (broad base, lower position and tendency to turn malignant or to harbor malignancy, 25% of which is often missed except with total excision biopsy) a. For the usual low lesion and that of moderate size → total excision; trans-anally or trans-sphincterically (York Mason). b. For high or extensive lesions → low anterior resection (low-stapled colo-anal anastomosis) - Familial Adenomatous polyposis (FAP) Timing of surgery In FAP, timing of the operation depends on the clinical presentation as shown in Table 8

Table 8: clinical presentation and timing of surgery for treatment of FAP

Clinical Presentation Timing of Surgery - Asymptomatic patient with modest May wait for a few years, as long as number of small adenomas colonoscopic surveillance is performed yearly - Symptomatic patient with large number Early surgery of adenomas - Suspicious of CRC Very early or urgent surgery

287

Surgical options - Total procto-colectomy + ileal Figure 59. pouch-anal anastomosis (TPC- Total procto- IPAA): colectomy It is usually suitable for most (TPC) + patients with FAP (Figure 59). ileal pouch - Total colectomy with ileo-rectal anal anastomosis anastomosis (TC-IRA). (IPAA) - Other surgical options include: o TC-IRA for attenuated FAP (AFAP). o Total procto-colectomy + terminal ileostomy (Figure 60): Indicated for low rectal cancers, poor sphincters and desmoid tumors Role of Medical treatment in FAP ? - Sulindac (NSAID) and Celecoxib (COX-2 inhibitor) shown to control and decrease the number of colorectal adenomas in FAP - It is not a definitive treatment, but a temporizing treatment (e.g. when surgery needs to be delayed) - It may control pouch and rectal polyposis after initial prophylactic surgery

Figure 60. Total procto-colectomy (TPC) + terminal ileostomy

- Surgical Treatment of HNPCC 1. Total colectomy with ileo-rectal anastomosis (TC-IRA) 2. Restorative proctocolectomy with ileal pouch-anal anastomosis (TPC-IPAA) 3. Segmental colectomy not recommended because of high rate of metachronous CRC 4. TAHBSO (trans-abdominal hysterectomy-bilateral salpingo-oophorectomy) for endometrial cancer

288

CHAPTER 3

Inflammatory Bowel Disease (IBD)

ULCERATIVE COLITIS (UC)

Definition

- Ulcerative colitis (UC) is a type of I nflammatory bowel disease (IBD) that results in inflammation and ulcers of the colon and rectum.

Pathology

- The disease starts in rectum (90%) and spreads continuously proximally to involve: 1. The rectum only (25%) 2. The left side only (25%) 3. The colon sub totally (15%) 4. The colon totally (25%) with back-wash ileitis in 10-20% of cases - It is predominantly a mucosal disease with the characteristic crypt abscesses and specific goblet cell mucus depletion. Chronic inflammatory cell infiltration and proliferative granulation tissue (GT) and edematous mucosa causing Pseudo-polypi are present in chronic cases. - Endoscopic picture (gross appearance) 1. Loss of normal vascular pattern 2. Red, edematous mucosa with friable granular changes 3. Contact bleeding on sigmoidoscopic or colonoscopic passage 4. In chronic cases, pseudo-polypi (these are edematous mucosa between denuded undermined ulcerations), confluent ulcerations

Clinical Features

- Patients generally fall into following categories: 1. Severe acute colitis 2. Intermittent relapsing colitis 3. Chronic persistent colitis 4. Asymptomatic disease - Approximately, 85% of patients follow a chronic relapsing course, 10% may follow chronic continuous course and 5% may suffer from single attack

289

Severity of the Attack - Assessment of disease severity 1. Mild = < 4 stools/day. Systemically well 2. Moderate = > 4 stools/day. Systemically well 3. Severe = > 6 stools/day. Systemically unwell (, fever, anemia, hypo-albuminemia) 4. Toxic colitis (Cleveland criteria) - Severe colitis + at least two of the following 1. Heart rate >100/ m 2. Temperature > 38.5 3. Leukocytosis >10.500 4. Hypo-albuminemia <3g/dl

Cardinal Features are "Intermittent Attacks of" 1. Rectal bleeding 2. Diarrhea 3. Mucous discharge 4. Abdominal pains 5. Weight loss - There is complete absence of symptoms between attacks in 60% of cases. - Relapses occur with variable frequency (5% of patients suffer single attack) - Symptoms correlate well with the severity of the disease (bleeding and diarrhea) a. Mild: < or 4 motions/day b. Moderate: > 4 motions /day without systemic signs of illness c. Severe: > 6 motions with systemic signs (fever >37.5ₒC, tachycardia >90, low albumin <3g or loss of weight >3Kg).

Differential Diagnosis of Bloody Diarrhea

- Amebic colitis - UC or other forms of IBD - Spurious diarrhea of cancer rectum - Rarely, bilharzial colitis

Investigations

Laboratory - CBC (complete blood count), ESR (erythrocyte sedimentation rate), CRP (C-reactive protein) - Liver function test (LFTs), mainly serum albumin - Electrolytes Endoscopy - Colonoscopy with rectal biopsy (or sigmoidoscopy, as rectal involvement is present in > 95%, however, the extent of the disease should be determined by barium enema) Radiology (barium enema is contraindicated in toxic mega colon) - Plain X-ray for toxic mega colon (transverse colon >5.5 cm) and for perforations - Barium enema and double-contrast (double wall sign, loss of hausterations, mucosal irregularities). CT scan is not as useful in UC as in Crohn‘s disease (CD)

291

Medical Treatment

5-Aminosalicylic Acid - Used in mild / moderate UC & CD. - 5-ASA block production of prostaglandins & leukotrienes - Sulfasalazine was first agent described - Topical preparation may be used in those with left-sided colonic disease - Maintenance therapy of proven benefit in those with UC

Corticosteroids - Often used in those in whom 5-ASA therapy is inadequate - Also used in those presenting with acute severe disease - Can be given orally, topically or parenterally - Use should be limited to acute exacerbations of the disease - Of no proven value as maintenance therapy in either UC or CD - Use must be balanced against side effects

Immunosuppressive and Immune Modulators Agents - Often used in those in whom steroids cannot be tapered or discontinued - Agents used include: o Azathioprine: Effective in both UC & CD. o Methotrexate: Effective in CD o Cyclosporine o Infleximab: Anti-TNF-α therapy

Antibiotics as metronidazole

Surgical Treatment

The ideal Operation for UC would: 1. Remove the diseased bowel. 2. Return the patient to health. 3. Lower the risk of developing cancer. 4. Obviate the need for permanent ileostomy. 5. Preserve the anus for defecation. 6. Maintain continence. 7. Have few complications. 8. Be done in one stage.

For Toxic Megacolon

Anti-diarrheal medications, narcotics, hypokalemia, colonoscopy or barium enemata are prohibited

Indications of surgery 1. Failure of medical treatment (a trial of 3 or 5 days) 2. The presence or occurrence of complications like mega colon, hemorrhage or perforation 291

Procedure - Subtotal colectomy (STC) with ileostomy and Hartmann pouch The colon is divided at the distal sigmoid rather than the rectum and suture the stapled distal stump above the fascia and below the skin. In case it blows-up, a mucous fistula is created. The mobilization of distended friable colon is always associated with occasional perforation which, increase the morbidity and mortality of the operation To maintain remission - No role for steroids systemically - Retention enemata (Budesonide enemacort) are used - 5-ASA is the cornerstone of drugs (Olsalazine Dipentum is the best) - Immunosuppressive drugs (Azathioprine or 6-Mercaptopurine) are steroid-sparing drugs and are occasionally used in UC contrary to CD where it is usually used. - Cyclosporine may be used in severe cases that fail to respond to conventional treatment.

For Non-acute Cases

Indications of surgery 1. Severe disease not responding to medical treatment (intractability). 2. Chronic disease with anemia, frequent stools, urgency and tenesmus 3. Steroid-dependent disease (with failure of steroid sparing drugs) 4. Risk of neoplastic changes during review colonoscopy (severe dysplasia or villous adenomatous changes) Operation Options - The four current surgical options for treating ulcerative colitis are: 1. Total (pan)-procto-colectomy & ileostomy (Brooke ileostomy) (TPC, ileostomy) 2. Total procto-colectomy & continent ileostomy (Barnett or Kock pouch) (TPC, KP), 3. Total procto-colectomy + ileal pouch + ileo-anal anastomosis (TPC, IPAA). 4. Total abdominal colectomy with ileo-rectal anastomosis (TAC, IRA), - A covering ileostomy is a must in: 1. Patients receiving steroids at the time of surgery. 2. Patients who are nutritionally compromised. 3. When the vascularity or tension of the pouch is suspected. - Anal transition zone (ATZ) is usually spared if stapled anastomosis is used. It gives better functional results (sampling is better). Hand-sewn anastomosis after mucosectomy is done if there is dysplasia, or in cases of FAP (familial adenomatous polyposis) to avoid cancer.

Total Procto-colectomy with Ileostomy (TPC, ileostomy)

- Indications: TPC with ileostomy remains the benchmark procedure for treatment of UC. - Advantages 1. The diseased colon is removed 2. UC medications can be stopped 3. Patients are able to return to normal health and activities 4. The risk of cancer is obviated 5. The procedure is done in one stage.

292

- Disadvantages 1. The need for a permanent ileostomy and stoma appliance with its potential complications (skin irritation, intestinal obstruction, stoma retraction, or para-stomal herniation). 2. Lack of control over stool evacuation 3. The complications associated with pelvic dissection 4. The potential for a perineal wound.

Total Procto-colectomy with Continent (Kock) Pouch (TPC, KP)

- Indications: Prior to 1983 "TPC, KP" was a popular alternative to TPC with Brooke ileostomy. Though several centers continue to promote its advantages as a treatment for UC, the popularity of KP has waned. Currently KP is limited to those patients who are: 1. Not candidates for an IPAA 2. As a modification for patients who currently have a Brooke ileostomy. 3. As an alternative to Brooke ileostomy for those patients requiring ileal pouch excision - Advantages 1. All of the diseased bowel is removed 2. Patients are off of medications 3. Patients return to health and normal activities 4. The risk of cancer is obviated 5. Patients are continent and do not have to wear a stoma appliance. - Disadvantages 1. It is a 2- or 3-stage operation 2. The risk of pouchitis (inflammation of the pouch) 3. Complications of pelvic dissection 4. Patients must catheterize the pouch to evacuate the contents 5. A 50% reoperation rate is reported because of nipple valve slippage (continence is maintained by the creation of a nipple valve fashioned by intussuscepting a portion of the ileum into the pouch. Several techniques have been employed to prevent nipple valve slippage including the use of fascia, mesh and intestines in the Barnett modification. Nipple valve slippage results in either incontinence of the pouch or the more urgent problem an inability to catheterize and empty the pouch. Other valve specific complications include bleeding, ischemia, and valve necrosis).

Total Procto-colectomy with Ileal Pouch Anal Anastomosis (TPC, IPAA)

- Advantages 1. All diseased bowel is removed 2. Patients may stop UC medications 3. Patients return to health and normal activity 4. The cancer risk is obviated 5. Stool is passed through the anus 6. Continence is preserved.

293

- Disadvantages 1. It requires 2 or 3 stages 2. Complications associated with pelvic dissection 3. Patients have a mean stool frequency of 6/day one of which is nocturnal 4. Incontinence rates vary from 5-20% 5. Many patients must take anti-diarrheal preparation 6. Pouchitis. - Results: Patients with an IPAA report the highest levels of satisfaction compared to patients with either a Brooke or continent ileostomy. However, complications such as stool frequency, urgency & incontinence can detract from the quality of life. - Pouch Design Stool frequency is affected by stool volume, pouch compliance, pouch capacity, & the patient's response to distention. As shown in Figure 1, Pouches may be constructed from either 2 limbs ("J" pouch), 3 limbs ("S" pouch), or 4 limbs ("W" pouch). Though J- pouches are the easiest to construct, they have the lowest capacitance & compliance. S- pouches have a longer reach into the pelvis than J-pouches, but the efferent limb must be < 2 cm in length to prevent outlet obstruction. Anal transition zone (ATZ) is usually spared if stapled anastomosis is used (Figure 2). W-pouches have a greater capacitance and compliance than either J- or S-pouches, but this does not translate into a functional advantage.

Figure 1. Ileo-anal anastomosis with pouch. A substitute rectum is made from joined folds of ileum to form an expanded pouch of small intestine. The pouch is then joined directly to the anus at the level of the dentate line, all other rectal mucosa having been removed. Three ways of forming a pouch are illustrated: (a) a simple reversed ‘J‘; (b) an ‗S‘ pouch; (c) a ‗W‘ pouch.

294

Figure 2. Stapled ‗J‘ pouch with stapler creating a pouch–anus anastomosis.

- Diverting Ileostomy Diverting loop ileostomy has been an integral part of TPC, IPAA. It protects the anastomosis and reduces pelvic sepsis. However, closure of an ileostomy is not without complications + added cost of a 2nd surgery and hospitalization. TPC, IPAA can be performed without an ileostomy, particularly in patients in good nutritional condition and not on steroids or 6-MP. - Pouchitis and Other complications Pouchitis is the most common complication and patients present with increased stool frequency, urgency, incontinence, cramping abdominal pain and a flu-like generalized malaise. On pouchoscopy the mucosa is inflamed and may be ulcerated. The fast response to Metronidazole supports the role of anaerobic bacteria overgrowth as a cause or significant factor in the etiology. Fortunately few patients' symptoms are severe enough to require pouch excision. Other conditions to be considered include: 1. Idiopathic pouchitis 2. Anastomotic stricture with bacterial overgrowth 3. Pouch anal abscess or fistula 4. Ischemia 5. Cytomegalovirus (CMV) infection 6. Recurrent UC in retained rectal mucosa 7. Crohn's disease (CD) 8. Irritable bowel syndrome (IBS).

Total Abdominal Colectomy with Ileal Rectal Anastomosis (TAC, IRA),

This procedure removes the abdominal colon, but leaves the rectum intact. - Indications: It was an alternative to TPC and ileostomy for younger patients in an effort to delay an ileostomy until a more acceptable time. Now, it is used in those patients who: 1. Have rectal sparing, or are not suitable candidates of IPAA 2. Have a picture suggestive of indeterminate colitis.

295

- Advantages 1. Improvement in health 2. No pelvic dissection 3. It is done in one stage 4. Patients have about 3 stools/day 5. Continence is preserved 6. Patients do not have a stoma. - Disadvantages 1. All diseased bowel is not removed 2. Risk of cancer is reduced, but not eliminated (after 25 years, 13% of patients with IRA for UC developed cancer in the rectal remnant).

Complications of UC

1. Toxic Mega-Colon or Acute Dilatation (10%) - Usually occurs in the first attack of total colitis - Predisposed to buy drugs inhibiting motility and hypokalemia, barium enema and colonoscopy. - The condition is heralded by general condition deterioration and diagnosed by plain X ray - Because of high risk of perforation, treatment is by surgery after 5 days trial of medical treatment.

2. Colonic Perforation (2%) - Can occur ± dilatation due to use of barium enema or colonoscopy with aggression

3. Massive Hemorrhage (3%)

- Responds to conservative treatment and transfusions

4. Colonic Stricture (10%) - Benign strictures occur commonly in the rectum and transverse colon (usually with total colitis) - It is an absolute indication of colonoscopy and biopsy if seen be enema

5. Colonic Dysplasia and Carcinoma (5%) - The risk depends on (1) extent of disease, (2) duration (>10 years) and (3) onset during childhood - The majority lies in recto-sigmoid and transverse colon - Flat plaques of dysplasia (high grade) are treated as carcinomata

6. Peri-anal Suppurative Diseases (15%) - Apart from hemorrhoids (frequently present) suppuration and fissure are more common in Crohn‘s disease (CD)

296

CROHN’S DISEASE (CD)

Definition

Crohn's disease is a type of inflammatory bowel disease (IBD) that may affect any segment of the gastrointestinal tract (GIT) from the mouth to the anus.

Pathology

- Common sites 1. Ileocolic ……………………….. 40% 2. Colon ………………………….. 25% 3. Small intestine ………………… 20% 4. Ano-rectal (alone) …………..… 5% - It is a chronic trans-mural inflammatory disorder affecting all GIT with segmental distribution and skip lesions - The pathology is characterized by trans-mural infiltration by chronic inflammatory cells, edema, non-caseating epithelioid cell granulomas and angitis. The affected segment is extremely thickened and has a woody feeling (hose-pipe lesions). Mesenteric lymph nodes (LNs) are enlarged and mesenteric fat wraps the gut - Anal lesions (anal CD) are common and are manifested as dusky edematous skin tags, indolent ulcers and deep fissures, recurrent perianal suppuration, fistulae or sinuses) - There are 2 forms of CD (present clinically): perforating & non-perforating. - Endoscopic picture 1. Earliest lesion is pallor of mucosa + discrete aphthoid (aphthous-like) ulcers 2. Later cobble stoning, mucosal thickening and linear ulcerations 3. Deep fissures may penetrate to adjacent structures causing abscesses and fistulae - Comparison of pathological features between UC and CD (Table 1)

Table 1: Comparison of pathological features between UC and CD

Ulcerative Colitis (UC) Crohn's Disease (CD) Lesions continuous - superficial Lesions patchy – penetrating Rectum always involved Rectum normal in 50% Terminal ileum involved in 10% Terminal ileum involved in 30% Granulated ulcerated mucosa Discretely ulcerated mucosa No fissuring Cobblestone appearance with fissuring Normal serosa Serositis: common Muscular shortening of colon Fibrous shortening Fibrous strictures: rare Strictures: common Fistulae: rare Entero-cutaneous or intestinal fistulae in 10% Anal lesions in <20% Anal lesions in 75%. Anal fistulae and chronic fissures. Malignant change well recognized Possible malignant change

297

Degrees of CD

Mild-to-Moderate - Ambulatory patient - Tolerating oral feeding - No manifestations of 1. Toxicity (high fever, rigors, prostration) 2. Abdominal tenderness and painful mass 3. Obstruction 4. >10% weight loss

Moderate-to-Severe - Failed medical treatment of mild (5-ASA, antibiotics "Quinolones or Metronidazole" and Budesonide) - Patients with more prominent symptoms of: 1. Fever (mild fever) 2. Abdominal pains and tenderness without a mass 3. Intermittent nausea and vomiting without frank obstruction 4. Weight loss and/or significant anemia

Severe to Fulminate (should be hospitalized) - Symptoms despite steroid or Infleximab therapy - Patient presenting with 1. High fever, rigors or prostration 2. Persistent vomiting with evidence of obstruction 3. Evidence of abdominal mass or rebound tenderness 4. Significant weight loss or cachexia

CD in Remission (whether induced medically or surgically) - Patients asymptomatic or without inflammatory sequelae or evidence of residual disease - Patients how need steroids for well-being are not in remission

Clinical Picture

- Systemic features in CD are > in UC. The presentation of CD can vary widely according to the age of patient, location (50% ileo-cecal and 25% present with colitis) and behavior of the disease (Vienna classification A L B system) as shown in Table 2).

Table 2: Vienna classification A L B system

Age Location Behavior

A1 <40 y L1: Terminal ileum B1: Non-stenosing, Non-penetrating A2 >40 y L2: Colon B2: Stricturing L3: Ileo-colic B3: Penetrating - other features of CD.

298

Intestinal Manifestations The following are the most common 1. Chronic regional enteritis: The lesion is usually in the ileo-cecal area and causes: (a) chronic ill-health, (b) abdominal pains due to obstructive attacks, (c) anemia, (d) change of bowel habits, attacks of obstruction and others of diarrhea, and (e) a mass may be palpable 2. Acute regional ileitis: simulates acute appendicitis 3. Enteric fistula: It is common to occur if deep fissures penetrate through the gut wall- giving rise to entero-enteric, or entero-colic, or entero-cutaneous or entero-vesical fistulae 4. Peri-anal CD: Edematous cyanotic skin tags (elephant ears), eccentric multiple fissures, painful cavitating ulcers, complex anal fistulae, recurrent perianal suppurations and strictures 5. Acute intestinal obstruction: There is always a history of recurrent similar attacks, which recover with or without treatment. 6. Colonic CD: Bleeding and mucus per rectum, diarrhea, abdominal pains and weight loss.

Extra-Intestinal Manifestations Extra intestinal manifestations are however, more common than UC; they may be associated with disease activity or not (Table 3)

Table 3: Extra-Intestinal manifestations of Crohn‘s disease

Associated with Disease Activity Not Associated with Disease Activity

- Skin - Joints o Erythema nodosum o Sacroilitis o Pyoderma gangrenosum o Ankylosing spondylitis - Joints - Hepatobiliary conditions o Asymmetrical non- o Primary sclerosing cholangitis (PSC) deforming arthropathy o Cholangiocarcinoma - Eyes o Chronic active hepatitis o Anterior uveitis o Gallstones: due to interrupted entero-hepatic o Episcleritis circulation at the terminal ileum o Conjunctivitis - Amyloid disease - Hepatobiliary conditions - Nephrolithiasis: Obstructive uropathy due to retro- o Acute fatty liver peritoneal infiltration and ↑ renal calculi specially Ca - Thrombo-embolic disease oxalate due to ↑ oxalate absorption - ↑ incidence of cancer colon and small bowel cancer

Investigations

Laboratory (same as UC) Endoscopy (same as UC with some differences) - Rectum is spared in 50% hence colonoscopy is preferred to sigmoidoscopy & barium enemata

299

- The presence of skip lesions and terminal ileal involvement mandates thorough complete colonoscopy and multiple biopsies - Colonoscopy is done with caution in case of the presence of deep fissures. Radiology - Barium enema, small bowel enema (superior to follow-through), CT and sinography are all used - The useful diagnostic signs in barium studies are edema with separation of the affected loops, string sign of Kantor, spike or thorn sings and fistulae with skip lesions. - In CT scan, abscesses and fistulae with the presence of inflammatory masses and bowel obstruction are the main features

Treatment

General Rules - Neither medical nor surgical treatment is curative - Medical treatment is employed initially and continued until the medications fail to alleviate symptoms or produce unacceptable side effects or a complication arises that requires surgery - Objectives of surgery are to: 1. Deal with the immediate adverse effects of the disease, 2. Restore health and function 3. Permit withdrawal of steroids or immunosuppressive agents

Induction of Remission Mild-to-moderate CD (ileal, ileo-colic and colonic) - Oral 5 ASA: Mesalazine (3-4 g/d) - Sulfasalazine (3-6g/d) - Alternative treatment (antibiotics are specially used in peri-anal CD): Metronidazole (10- 20 mg/kg/d) - Ciprofloxacin (1 g/d) - Controlled ileal release: Budesonide (3-9 mg /d) Moderate-to-severe CD - Prednisone 40-60 mg /d or - Budesonide (9 mg/d) until symptom resolution or weight gain resumption (usually 7-28 d) - Appropriate antibiotics or drainage for inflammation or abscess - Infleximab: Effective adjuvant treatment - possible alternative to steroids when not tolerated or ineffective and better to use in presence of fistulae Severe-to-fulminate CD - Hospitalization - CT and/or US ±PAD (percutaneous abscess drainage) and appropriate antibiotics - Surgical consultation for drainage in abscess formation. - If abscess is excluded: Parental steroids equivalent to 40-60 mg prednisone and TPN Peri-anal CD - Acute suppurating drainage with or without a Seton - Non-suppurating fistula or fissures: (1) Antibiotics (Ciprofloxacin and/or Metronidazole), (2) Immuno-modulators: AZA/6-MP, (3) Infleximab.

311

CD in Remission - Never use steroids - The use of 5-ASA is following induction with 5-ASA or post-operative, not effective after steroid induction (in general it is of unproven benefit as maintenance of remission). - Immunotherapy with AZA/6-MP after steroid induction in steroid-dependent disease is the usual way of maintaining a remission. - The use of antibiotics to maintain remission is in peri-anal disease and post-operatively. - Infleximab is used also to maintain remission - 5-ASA or AZA/6-MP are used after ileo-colonic resection to reduce the risk of symptomatic recurrence

Nutritional Support - Defective absorption and fear of food are among causes of malnutrition and anemia, and sometimes electrolyte imbalance that should be corrected. - TPN indications 1. Intestinal failure with gross nutritional deficiencies and severe hypo-albuminemia 2. Mal-nourished patients prior to surgery 3. In obtaining remissions in severe active cases 4. In patients with entero-cutaneous fistulae (30% heal, 6 weeks is the time limit) 5. As long-term TPN in short bowel syndrome (SBS)

Surgical Treatment of Crohn’s Disease

Indications of Surgery (Table 4)

Table 4: Absolute and relative indications of surgery for treatment of Crohn‘s disease

Absolute Indications Elective Indications

1. Perforation with 1. Chronic obstructive symptoms generalized peritonitis 2. Chronic ill-health or debilitating diarrhea 2. Massive hemorrhage 3. Intra-abdominal abscess or fistula 3. Carcinoma 4. Complications of peri-anal disease 4. Fulminant or non- 5. Growth retardation in children or amyloidosis responsive acute 6. Failure of medical treatment with persistent symptoms severe colitis 7. Complications of medical treatment (side effects of steroids or AZA), with inability to stop treatment 8. Surgery should be as conservative as possible 9. No evidence that increased resection margins reduces risk of recurrence 10. If possible, improve pre-operative nutritional state

311

Preparation of the Patient for Surgery

- Patient preparation is similar no matter the location of the disease. - Pre-operative counseling regarding the nature and course of disease with discussion about the rationalization of surgery, hospitalization, peri-operative morbidity including the possibility of ileostomy and future implications of recurrence is important. - Comprehensive assessment with endoscopic and radiological evaluation of the small and large bowel is important to define the road map of the diseased areas and the operative strategy. - Correction of restorable metabolic defects (anemia, electrolyte deficiencies, dehydration, and coagulation defects) is important. Pre-operative optimization of the nutritional status with TPN is controversial. In our practice, we use pre-operative hyper-alimentation for 5 to 7 days, if surgery is elective or semi-elective and malnutrition is considered severe. - Cessation of immuno-suppressive drugs (AZA, 6-MP and Cyclosporine) 3-4 weeks prior to elective procedure is advised because of concerns about tissue or anastomotic healing. - Stress-dose steroids are administered peri-operatively if the patient has been treated with steroids within 6 m prior to operation. - Additional preparation includes IV antibiotics with post-operative antibiotic selection based on operative findings, likely flora, pending cultures and sensitivity. After anesthesia, a nasogastric tube is passed and Foley catheter is placed in the bladder. Central venous lines & peripheral arterial lines may be placed on the likely difficulty and duration of the operation. Ureteric stents are not used routinely but can be helpful with re-operative pelvic surgery. Finally, prophylaxis against DVT is employed using pneumatic stockings with or without mini-dose heparin.

Surgical Strategy

- Conservation in surgical resection: Small bowel conservation in CD is imperative to minimize the risk of excessive bowel resection, which can cause disability from malabsorption, including short bowel syndrome (SBS). Thus, segments of small bowel affected by non-obstructing, non-hemorrhaging CD do not warrant resection. - Resected margins: Important to the concept of conservation in surgery is the issue of resected margins. Extended resection margins (12 cm) are unnecessary and have no advantage over limited (2-cm) margins in reducing cumulative recurrence rates of the disease. - Regarding re-operation: • Surgery is deferred for any elective indication until at least 3m, preferably 6m, since the last laparotomy. Otherwise, formidable adhesions may be encountered resulting in increased risks of inadvertent enterotomy. • A small window of time (7-12 d) exists following laparotomy during which reoperation for complications such as entero-cutaneous fistula can be attempted with reasonable success. • Reoperation is justifiable for complications of intestinal ischemia, peritonitis & major hemorrhage between 12-90 d after the initial laparotomy.

312

• For patients with post-operative ileus/bowel obstruction, it is safer to provide long-term TPN and a percutaneous gastrostomy than to risk enteric injury, fistulas or substantial loss of intestinal length in a well-intentioned, but dangerous venture. If an entero- cutaneous fistula complicated with intra-abdominal sepsis or pouching and skin excoriation problems occur during the high-risk period, a small laparotomy in the left upper quadrant with a proximal high-loop jejunostomy isolates the septic source. Home TPN is continued for 4-6 m to maintain nutrition, pending restoration to correct the fistula & restore intestine continuity. - The use of temporary ostomy may be indicated in situations where anastomotic healing is compromised such as excessive blood loss during a long operation, severe hypo- albuminemia, incompletely drained sepsis, or when a phlegmonous pyogenic membrane is in proximity to an anastomosis. These are not necessarily absolute indications or even the only indications for such diversion, but in the examples mentioned, temporary ostomies appear reasonable.

Surgical Procedures - Several types of operations are used in the surgery of CD. These may be classified as: 1. Resection with or without anastomosis. 2. Bypass procedures (internal and external such as gastro-duodenal bypass or ileostomy. 3. Stricturoplasty. - The surgical procedure depends on the site of the disease.

Ileo-colic Disease (40% of patients undergoing surgery)

- Resection of the diseased bowel with ileo-ascending anastomosis is the preferred procedure. As much as normal right colon as possible is preserved for increased water absorption and avoiding anastomosis sitting over the duodenum with the risk of fistula development. Wrapping the anastomosis with omentum may avoid this problem. - If disease is associated with perforation and peritonitis, creation of diverting ileostomy proximal to the ileo-colic anastomosis decreases the morbidity associated with the next procedure.

Jejuno-ileal Disease (Small Bowel) (10-20% of patients)

- Surgical resection • The most common surgical procedure is resection with end-to-end anastomosis (EEA), keeping as much of the bowel as possible. The leakage rate is > 15% - Stricturoplasty  Since CD is a pan-intestinal disease, surgical treatment has shifted toward conservative approaches. Stricturoplasty has become a valuable option for treatment of small bowel CD. • Types of stricturoplasty: Heinecke-Mikulicz stricturoplasty (most commonly used), is suitable for short strictures up to 10 cm. Finney stricturoplasty is used for longer strictures.

313

• Indications of stricturoplasty 1. Diffuse involvement of the small bowel with multiple strictures 2. Stricture(s) in a patient with previous major resection(s) of small bowel (>100 cm) 3. Rapid recurrence of CD manifested as obstruction. 4. Stricture in a patient with short bowel syndrome (SBS) 5. Non-phlegmonous fibrotic stricture • Contraindications of stricturoplasty 1. Free or contained perforation of the small bowel 2. Phlegmonous inflammation, internal fistula, or external fistula involving the affected site 3. Multiple strictures within a short segment 4. Stricture in close proximity to a site chosen for resection 5. Colonic strictures 6. Hypo-albuminemia (< 2.0 g/dl) 7. Rigid thickened non-supple bowel segments, either short or long 8. Extensive ulceration of mesenteric margin especially bleeding or friable 9. Suspicion of cancer in a strictured-segment.

Colonic Disease (20-30% of patients)

- The choice of operation for colonic CD depends on age, disease distribution, extent of involvement, previous resections, rectal compliance and adequacy of fecal continence. - Segmental resection with ileo-colic or colo-colic anastomosis and abdominal colectomy with ileo-proctostomy are the procedures most commonly performed for Crohn's colitis. - For pan-colitis a total procto-colectomy with permanent ileostomy is the operative choice. - Although isolated centers are offering procto-colectomy and ileal pouch-anal anastomosis with reasonable results, patients requiring surgical treatment of proctitis ± accompanying colitis usually undergo procto-colectomy and end-ileostomy. - Indications of ileo-rectal anastomosis (IRA) 1. The rectum should be spared when at least it has no major features of the disease (ulcerations and strictures) 2. Normal anal sphincter function 3. No active perianal disease 4. No rectal dysplasia or malignancy 5. Properly distensible rectum (assessment of rectal compliance is essential) 6. Despite the high rate of reoperation, IRA is important in Crohn‘s colitis because it postpones the need for proctectomy with the risk of sexual dysfunction and need for a permanent external appliance - The role of ileostomy alone in CD Ileostomy alone has a limited role in CD 1. Temporary diversion as with repair of anal sphincters or advancement flaps in repair of peri-anal or recto-vaginal fistulae 2. End or loop ileostomy is done for patients destined for procto-colectomy because of severe perianal sepsis to avoid later unhealed perineal wound complication

314

3. In cases with toxic colitis and patients who cannot tolerate subtotal colectomy or segmental colectomy or proctectomy. - Treatment of colonic fistulas in CD 1. Internal fistulas occur in CD in 25-40% of cases depending on the site of the disease, highest in the ileo-colic CD. 2. Ileo-sigmoid fistulas are the most common. The procedure of choice is resection of the ileo-cecal area involved + with ileo-colic anastomosis with separate segmental resection of the sigmoid with colo-colonic anastomosis. A covering temporary loop ileostomy is a must if there is associated pelvic sepsis or small bowel obstruction 3. Bladder fistula is treated with closure after dealing with the gut CD

Perineal Crohn's Disease

- The perianal abnormalities associated with CD include edematous skin tags or hemorrhoids, cyanotic, discoloration, fissures or canal ulceration, abscesses, fistula and anorectal stricture. - Patients with colonic disease are more likely to have perianal manifestations than those with ileocolic or small bowel disease patterns. - Surgery is reserved for symptomatic problems. If the patient is too uncomfortable for an adequate evaluation, a well-conducted examination under anesthesia (EUA) is mandated. - Simple incision and drainage of the abscess adequately relieves acute symptoms and allows resolution of inflammation. Controlling the proximal disease improves perianal disease.

Duodenal Crohn's Disease (1-2% of cases)

- The most common problem is stricture leading to obstructive symptoms. - Most patients can be managed successfully with medical therapy. - Surgical approaches are used when obstructive symptoms remain disabling despite medical treatment. Gastro-jejunostomy is the simplest and most widely used operation for relief of obstruction.

Main Differences between UC and CD (Table 5)

Table 5: Main differences between ulcerative colitis and Crohn‘s disease

Main Differences between UC and CD - UC affects the colon; CD can affect any part of the GIT, but particularly the small and large bowel - UC is a mucosal disease whereas CD affects the full thickness of the bowel wall - UC produces confluent disease in the colon and rectum, whereas CD is characterized by skip lesions - CD more commonly causes stricturing and fistulation - Granulomas may be found on histology in CD but not in UC - CD is often associated with peri-anal disease, whereas this is unusual in UC - CD affecting the terminal ileum may produce symptoms mimicking acute appendicitis, but this does not occur in UC - Resection of the colon and rectum (procto-colectomy) cures the patient with UC, whereas recurrence is common after resection in CD

315

CHAPTER 4

Rectal Prolapse and Intussusception

RECTAL PROLAPSE

Definition

- Prolapse means descent of part of the wall or full thickness wall of the rectum into the anal canal (during straining). It is associated with fecal incontinence in 50-70% of cases.

Predisposing Factors

- Prolonged straining - Vaginal delivery - Neurological - Dementia - Chronic constipation - Hyper motile sigmoid colon - Bilharziasis.

Classification

Partial (Incomplete or Mucosal) Prolapse - Definition: Partial prolapse means a ―1-4 cm protrusion of rectal mucosa and submucosa outside the anus‖ (Figures 1 and 2). - Age It occurs in the extremes of age (children and elderly): In children, the prolapse is usually mucosal and should be treated conservatively. - Predisposing factors 1. In infants: Underdeveloped sacral curve - low anal store 2. In children: diarrhea – whooping cough – loss of weight. 3. In adults: Hemorrhoids - prolonged straining - perineal tears (more among females) - secondary to surgery (due to damage of sphincters).

316

Figure 1. Patient with partial rectal Figure 2. Diagram illustrating mucosal (partial) prolapse showing prolapse of rectal rectal prolapse. mucosa

Complete Prolapse - Definition: Full thickness prolapse of the rectum. It contains the two layers of the rectal wall and has an intervening peritoneal sac. - Age: It occurs in older adults and is often associated with incontinence. - Gender: It occurs in females > males (6:1) worldwide; however, in Egypt, this ratio is reversed with a high incidence in young males. - Associated with weak pelvic and anal musculature. Sigmoid & rectum are floppy a redundant. - Surgery is necessary. The operation is performed either via the perineum or abdomen - Grades of complete rectal prolapse: Grade 1: Occult (internal) prolapse It is internal intussusceptions of upper into lower rectum. (Figure 3) Grade 2: Concealed prolapse: Prolapse to but not through the anus i.e. does not emerge from the anus (Figure 4 A,B). May be associated with solitary rectal ulcer (SRU). Grade 3: Complete rectal prolapse through the anus (Figure 5A-C).

A B

Figure 3. Grade 1 prolapse Figure 4. Grade 2 prolapse (does not pass through the anus)

317

A B C

Figure 5. Grade 4 prolapse (passes through the anus)

The 6 typical anatomical features are: 1. The intussusception itself 2. Deep cull-de-sac or pouch of Douglas 3. Absent fixation of the rectum to the sacrum 4. Redundant rectum and sigmoid colon 5. Weakness of the pelvic floor and/or the anal sphincters 6. Possibly, the presence of a rectocele

Presentation - Usually occurs after defecation & may reduce spontaneously or be reduced manually - Bleeding, mucus discharge or incontinence may be troublesome - PR Examination usually shows poor anal tone - Prolapse may be visible on straining. Most prolapses that are > 5 cm are complete (Figure 6) - Differential diagnosis in adults includes (1) Large hemorrhoids (Figure 7), (2) Prolapsing rectal tumors and (3) Prolapsing anal polyp.

Figure 6. Folds in prolapsed of the rectal wall. Figure 7. (C) Radial folds of prolapsing internal (A) Concentric folds of rectal procidentia. (B) hemorrhoids. (D) Bowel wall with only mucosal Bowel wall layers seen with complete rectal prolapse or prolapsing internal hemorrhoids. procidentia.

318

In adults Internal or occult prolapse - This may cause complete prolapse - There are symptoms of outlet obstruction with urgency, straining, sense of incomplete evacuation and digitated defecation - This may lead also to solitary rectal ulcer syndrome, which is a precursor of procidentia (prolapse) and is caused by injury & ischemia caused by the internal prolapse and straining, which force the anterior wall of the upper rectum into the anal canal (serpiginous or polypoidal ulcer) with passage of excess mucus and blood-tinged mucus per rectum Complete prolapse - The prolapse itself is the main complaint - There is soiling of the underwear by blood, mucus and feces - Some degree of fecal incontinence may be present (can be the start of the intussusception or the effect due to the stretch of the sphincter). - Generalized perineal descent with stretch of the pudendal nerves can also contribute in long-standing cases to the incontinence of the patient - On PR examination, the anus is patulous, with deficient sphincter tone and little discomfort. - On straining, the prolapse is seen (>5 cm is definite complete and < 5 cm can be complete or partial) and needs bidigital examination. In children - It is the feature of the first 2 years being more common in boys - Parents complain of the passage of red area around the anus of the child after defecation. - Excessive mucus, and traces of blood is a very common presentation - Inserting a suppository & putting the child on a pot may be necessary to precipitate the prolapse because usually examination is negative.

Complications of Prolapse

1. Ulceration (Figure 8) 2. Strangulation (Figure 9) 3. Spontaneous rupture with evisceration (Figure 10 and 11) 4. Incontinence (fecal and urinary)

Figure 8. Figure 9. Ulcers on Rectal prolapse top of rectal with prolapse strangulation of bowel

319

Figure 10. Figure 11. Rectal Rectal prolapse prolapse with with spontaneous spontaneous rupture and rupture and evisceration evisceration of of normal strangulated bowel bowel (note the dark color)

Investigations (in Elective Cases)

1. To detect the precipitating factor → At least a flexible sigmoidoscopy 2. Assessment of surgical risk (no effective non-operative treatment) 3. Anorectal manometry, pudendal nerve test (predicts functional outcome after surgery).

Treatment

In Children (Partial or Complete) - Alleviation of straining (constipation, diarrhea/tenesmus) and training of regular bowel habits. - Buttocks are strapped together after defecation after spontaneous or manual reduction - The body of the child and fat reservoirs should be built-up - Sclerosant injection (phenol in almond oil for submucous injection in partial prolapse and alcohol for retro-rectal injection in complete prolapse) - In case of failure, one of the operations described below is resorted to

In Adults - In partial prolapse mucosal hemorrhoidectomy will often suffice to deal with the condition. Recently, Longo‘s procedure (PPH stapler is used to induce anal lift and refixation of the prolapsed mucosa back to the rectum and anal canal). - In complete prolapse, the final functional result is dependent on the degree of continence rather than simple control of the prolapse.

Operations of Prolapse - The choice of the operation depends on: 1. Degree of prolapse present 2. Associated disorders (cystocele, rectocele, incontinence or constipation) 3. Co-morbid conditions (spinal cord lesion and mental, psychic or vital system problems) 4. The main symptoms of presentation

311

- Goals of surgery are 1. Resection of redundant colon 2. Fixation of the rectum to the sacrum 3. Improving symptoms of fecal incontinence and constipation

Perineal Procedures 1. Thiersch Procedure: Obsolete nowadays! 2. Altemeier Operation: (Perineal Procto- sigmoidectomy) (Figure 12) - Mortality rate (MR) = 0-5% - Complications: pelvic sepsis, leakage - Recurrence rare (RR) = 0-16% - Best if combined with posterior levatorplasty - Ideal for incarcerated or strangulated prolapse Figure 12. Altemeier operation - Difficult to perform for small prolapse (Perineal procto-sigmoidectomy) 3. Delorme Procedure - It is the minimum procedure that should be done!!! (Figure 13) - Mortality rate = 0-4% - Recurrence rate is high = 4-38% (At St. Marks Hospital-UK = 12.5%) - Good for short prolapse - Can be repeated if necessary

Figure 13. Delorme’s operation for rectal prolapse. (a) The mucosa has been removed from the prolapse. (b) Interrupted sutures have been placed in the underlying muscle so that, when tied, the muscle is plicated.

Abdominal Procedures Rectopexy 1. Sutured Posterior Rectopexy (Ripstein Procedure) 2. Prosthesis or Mesh Rectopexy a. Anterior ventral rectopexy b. Posterior rectopexy (Wells operation) 3. Resection rectopexy (Frykman-Goldbery procedure)

311

Laparoscopic versus open procedure

1. Sutured Rectopexy (Ripstein Procedure)

- The procedure entails posterior mobilization of the rectum to the tip of the coccyx and division of the lateral ligaments on either side (Figure 14). - No reported mortality - RR = majority 0-8% (range: 0- 28%) A B - There is a variable response to Figure 14. A: Mobilization (dotted line), B: Division of lateral constipation ligaments

2. Prosthesis or Mesh Rectopexy a. Anterior ventral rectopexy (Figure 15) b. Posterior rectopexy (Wells operation) (Figure 16). Disadvantages: Constipation following rectopexy is a real problem that may be caused by: - Redundant sigmoid kinked in the Douglas pouch - Division of the parasympathetic nerves during rectal mobilization

Figure 15. Figure 16. Anterior Posterior ventral rectopexy rectopexy (using (using mesh) mesh) (Wells operation).

3. Resection-rectopexy (Frekman Repair) - It is the best-used operation for adults now. - It entails, sigmoidectomy & posterior rectopexy (Figure 17) - The operation added 1% to MR - Recurrence rate = 0-5% - Improved constipation. Other forms of posterior rectopexy cause severe constipation.

312

Figure 17. Frekman repair A. Rectal mobilization B. sigmoid resection C. Colorectal anastomosis and rectopexy

Comparison between Operations for Rectal Prolapse (Table 1 below)

Criteria Sutured Rectopexy Mesh Rectopexy Resection-Rectopexy - Mortality 0% 0-2.8% 1-4% - Recurrence 0-8% 0-13% 0-5% - Complications Rare 8-52% Up to 30% - Continence Improved Improved Improved - Constipation Variable Up to 42% Improved

Laparoscopic Approach for Rectopexy - It includes the following procedures for rectopexy (sutured, stapled, posterior mesh, resection) - It is as effective as the open approach (no long-term difference) - Recurrence 0-10% - Benefits include: 1. Shorter hospitalization 2. Overall decrease in cost 3. Less morbidity and earlier recovery 4. Earlier return to work. - Laparoscopic approach is desirable because of: 1. The benign nature of the condition 2. Patients are often at high surgical risk for laparotomy.

The Choice of Operation should be Individualized - Abdominal procedures are ideal for young fit patients & provide the best chance of cure - Sutured rectopexy gives good results - Combination of a resection reduces constipation - Laparoscopic approach provides similar results with less morbidity - Perineal procedures are indicated for frail patients with extensive co-morbidity, not fit for major abdominal surgery - Perineal recto-sigmoidectomy, combined with levatorplasty gives better result than Delorme‘s operation. 313

INTUSSUSCEPTION

Definition

- Intussusception is a process in which a segment of intestine invaginates into the adjoining intestinal lumen (Figure 18) (causing bowel obstruction) - As a common cause of abdominal pain in children, intussusception is suggested readily in pediatric practice based on a classic triad of signs and symptoms (vomiting, abdominal pain, and passage of bloody red currant stools per rectum) Figure 18. Intussusception of the intestine Classification

According to Location - Ileo-colic (the most common) (Figure 19) - Colo-colic - Ileo-ileo-colic - Ileo-ileal. - Jejuno-jejunal

According to Etiology - Primary (idiopathic) Intussusception - Secondary Intussusception Figure 19. Primary ileo-colic intussusception

Etiology

Primary Intussusception - In most infants and toddlers with intussusception, etiology is unclear. This group is believed to have idiopathic intussusception. - One theory about the etiology of idiopathic intussusception is that it occurs because of enlarged Peyer‘s patches (following viral upper respiratory tract infection or gastroenteritis) starting the process of intussusception.

Secondary Intussusception - In approximately 2-8% of children with intussusception, a surgical lead point is found. - Occurrence of surgical lead points increases with age and indicates that the probability of non-operative reduction is highly unlikely. - Examples of lead points are: 1. Meckel‘s diverticulum or appendix 2. Benign tumor of malignant tumor of the mesentery or intestine e.g. lymphoma, polyps (Figure 20) and hamartomas associated with Peutz-Jeghers syndrome

314

3. Mesenteric or duplication cysts. 4. Cystic fibrosis 5. Ectopic pancreatic and gastric rests 6. Foreign body (FB) 7. Following abdominal operations and trauma.

Figure 20: Secondary (polyp) ileo-ileal intussusception

Pathophysiology

- The pathogenesis of intussusception is believed to be 2ry to an imbalance in the longitudinal forces along the intestinal wall. - This imbalance can be caused by a mass acting as a lead point or by a disorganized pattern of peristalsis (eg, an ileus in the post-operative period). As a result of the imbalance, an area of the intestinal wall invaginates into the lumen, with the rest of intestine following. - The invaginating portion of the intestine (intussusceptum) completely invaginates into the receiving portion (intussuscipiens) (Figure 21). This process continues & more proximal areas follow, allowing the intussusceptum to proceed along the lumen Figure 21. Ileo-colic intussusception showing of the intussuscipiens. the intussusceptum and intussuscipiens - Early in this process, lymphatic return is impeded, then, with the rise in the pressure within the wall of the intussusceptum, venous drainage is impaired. Finally, the pressure reaches a point at which arterial inflow is inhibited& infarction ensues (Figure 22). - The mucosa is most sensitive to ischemia because it is furthest away from the arterial supply. Ischemic mucosa sloughs off, which initially →the classic "red currant jelly stool" (a mixture of sloughed mucosa, blood& mucus – Figure 23). If untreated, the process → transmural gangrene & perforation of the leading edge of the intussusceptum.

315

Figure 22. Pathophysiology of intussusception Figure 23. Red current jelly

Incidence

- General incidence: 1-4 cases / 1000 live births. - Gender: Slight preponderance of males (3:2). - Age: o 2/3 of children with intussusception are <1 year; most commonly, in infants aged 5-10 months. Intussusception has been very rarely reported in the neonatal period. o Intussusception can account for as many as 25% of abdominal surgical emergencies in children <5 years, exceeding the incidence of appendicitis. o Intussusception is the commonest cause of intestinal obstruction between 5 months-3 years.

Clinical Features

History (Symptoms) - History of preceding upper respiratory tract or GIT infection. - Pain is colicky, severe and intermittent, and occurs in 100% of cases. The parents or caregivers describe the child as drawing the legs up to the abdomen and kicking the legs in the air. - Initially, vomiting is non-bilious and reflexive, but when intestinal obstruction occurs, it becomes bilious. It occurs in 80% of cases. Any child with bilious vomiting is assumed to have a condition that must be treated surgically until proven otherwise. - Parents also report the passage of stools that look like redcurrant jelly. This is a mixture of mucus, sloughed mucosa& shed blood, and is seen in 90% of cases. - Lethargy is relatively common with intussusception, especially after attack of colic.

316

Physical Signs - On clinical examination, the patient is usually plump & in good health. Intussusception is uncommon in children who are malnourished. - The child is found to have periods of lethargy alternating with crying spells, and this cycle repeats every 15-30 minutes. The infant can be pale, sweaty and hypotensive if shock has occurred. - The hallmark findings are a right hypochondrium sausage-shaped mass (Figure 24) and emptiness in the right lower quadrant (Dance sign). This is hard to detect and is palpated best when the infant is quiet between spasms of colic.

Figure 24. The site of sausage shaped intussusception in the abdomen, shown schematically in and clinically in a female child.

- Abdominal distention is frequently found if obstruction is complete. - If intestinal gangrene and infarction have occurred, peritonitis can be suggested on the basis of rigidity and involuntary guarding. - Classic red currant jelly stools appear. - Fever and leukocytosis are late signs and can indicate transmural gangrene and infarction. - A rare presentation is prolapse of the intussusceptum through the anus: This prolapse can be confused with rectal prolapse, in which the anal crypts are everted with prolapse (not with intussusception). Moreover, an examining finger can pass between the prolapse and the anus in patients with intussusception but not in patients with rectal prolapse. - Complications o These include gangrene and perforation. o In complicated cases, vomiting becomes bilious and physical findings include lethargy, increased abdominal distention, tender and rigid abdomen.

Investigations - Imaging Studies

1. Plain Radiograph - It may be normal early in the course of intussusception. - As the disease progresses, earliest radiographic evidence includes an absence of air in the right lower and upper quadrants (radiological Dance‘s sign) (Figure 25) - Visible abdominal mass - Meniscus sign (Figure 26) - Late cases show an obvious pattern of small bowel obstruction, with small bowel dilatation and air-fluid levels (Figure 27) or free intra-abdominal air

317

Figure 25. Plain X-ray Figure 26. Plain X-ray showing Figure 27. Plain X-ray showing showing paucity of air in the meniscus sign multiple fluid levels right side (obstruction)

2. Contrast Enema (Barium, Saline or Air) - Contrast enema is a quick and reliable, and has the potential to be therapeutic. - Exercise caution when performing contrast enema in patients >3 years because most of them have a surgical lead point in the small bowel and the diagnostic and therapeutic yield of the enema is lower in these patients. - The classic appearance of the head of intussusception in contrast enema is Cobra head sign or Napoleon cap sign (Figure 28). It results from arrest of the barium in the colon with crescentic outline (cobra-head appearance) around the apex of intussusception. - Barium flowing between 2 layers of intussusception (spring coil appearance) (Figure 29) - Appearance of the rounded apex of the intussusceptum protruding into a column of contrast material (meniscus sign) (Figure 30).

Figure 28. Barium enema Figure 29. Barium enema Figure 30. Barium enema showing Copra-head showing Spring coil showing meniscus sign appearance appearance

318

3. Ultrasonography(US) - It is a very accurate and non-invasive method of diagnosis. - The characteristic target sign (Figure 31) represents transverse section through the mass while the pseudo-kidney sign represents a more oblique cut.

4. CT Scan - Target sign (Figure 32) and to detect the cause in 2ry intussusception

Figure 31. Ultrasound showing target sign Figure 32.CT scan showing target sign (arrow)

Principles of Treatment

- Early cases: Reduction of the invaginating segment, either non-operatively or operatively. - Neglected cases: Resection of the strangulated bowel & restoring continuity of the bowel. - For all children, start IV fluid resuscitation and naso-gastric (NG) decompression as soon as possible. - The presence of peritonitis and any evidence of perforation revealed on plain X-ray are the only 2 absolute contra-indications to an attempt at non-operative reduction with a therapeutic enema.

Medical Treatment 1. Hospitalization 2. Nasogastric tube 3. IV fluids. 4. Antibiotics

Non-surgical Reduction (Therapeutic enemas) - Can be hydrostatic, with either (1) barium or (2) water-soluble contrast, or (3) pneumatic, with air insufflation. Therapeutic enemas must be performed under fluoroscopic or ultrasonic guidance. - Criteria of successful reduction = the flow reaches the small bowel + disappearance of the target sign - A systematic approach to hydrostatic reduction of intussusception, the reported success rate of this non-operative intervention varied widely from <40% to >90%.

319

- When performing a therapeutic enema, the recommended pressure of air insufflation

should not exceed 120 cmH2O. When using barium or water-soluble contrast, the column of contrast should not exceed 100 cm above the level of the buttocks. - An attempt is not considered successful until the reducing agent, whether air, barium, or water-soluble contrast, is observed refluxing back into the terminal ileum.

Surgical Reduction - Indications 1. Failure or contra-indications of hydrostatic reduction. 2. Above the age of 2 years (2ry cases). 3. Previous abdominal operations. 4. Recurrent intussusception. - Surgical options include simple manual reduction or resection and anastomosis according to severity of presentation - Simple manual reduction: Traditional Figure 33. entry into the abdomen is through a Transverse right upper abdominal transverse right upper abdominal muscle muscle cutting cutting incision (Figure 33). Deliver the incision (red line) intussusception into the wound & attempt simple reduction. Milking the intussusceptum out of the intussuscipiens is important. Sustain gentle manual pressure rather than pulling out the intussusceptum to avoid risk of iatrogenic perforation (Figure 34).

- Resection with an end-to-end anastomosis (R-EEA) o Indications of R-EEA 1. Failed simple reduction. 2. Gangrenous bowel loops. 3. Perforation. 4. Secondary causes (eg. Meckel‘s) Diligent search for any lead points is advised especially if the patient is >2-3 years. - Post- operative course o After manual reduction (or hydrostatic reduction) the child is observed and starts oral feeding after passage of stools, usually after 6 hours and is usually discharged after 24 hours. A sure sign of complete release of intestinal obstruction after manual or hydrostatic reduction is the passage of a normal motion. o After R-EEA, the child receives IV fluids and Figure 34. Manual reduction antibiotics & oral feeds are withheld for 4-5 days.

321

Prognosis

- Early diagnosis, appropriate fluid resuscitation and treatment reduced the mortality rate from intussusception to <1%. If left untreated, this condition is uniformly fatal in 2-5 days. - The morbidity rate is very low after treatment of intussusception. - Recurrence o Risk of recurrence of the intussusception after operative reduction is 5-10 %. o Most of recurrences occur within the first 72 hours. o Multiple recurrences indicate a pathological lead point that necessities surgery

SUMMARY OF INTUSSUSCEPTION

Age (3-12m) Etiology(1ry or 2ry) Clinical (pain, vomiting, red current jelly stools) Test (US target sign) Treatment (Hydrostatic or surgical reduction)

321

CHAPTER 5

Hemorrhoids

Definition

Anal fibrovascular cushions (or hemorrhoids) are part of the normal anatomy within the anal canal and are believed to be important in maintaining continence. The term hemorrhoidal disease means engorgement or enlargement of the normal fibrovascular cushions lining the anal canal.

SURGICAL ANATOMY AND CLASSIFICATION

- External hemorrhoids arise from the inferior hemorrhoidal plexus & are covered by modified squamous epithelium distal to the dentate line (Figure 1). - Internal hemorrhoids originate from the superior hemorrhoidal plexus & are covered by mucosa proximal to the dentate line (Figure 1).

- Internal hemorrhoids are classified by history and not by physical examination. - They are graded as shown in Table 1 as follows: Figure 1. External and internal hemorrhoids

Table 1: Grading of hemorrhoids into 4 degrees (1-4)

Degree Description

First degree ………….. Bleeding per rectum only, no prolapse. Second degree ……….. Prolapse but reduce spontaneously. Third degree …………. Prolapse & have to be manually reduced Fourth degree ………... Permanently prolapsed

322

PATHOPHYSIOLOGY

1. Mechanism of Bleeding (Venous Obstruction Theory) - Repeated straining to evacuate a fecal bolus impedes the venous drainage of the anal cushions. Consequently, the anal cushions become engorged the overlying mucosa becomes thin & more friable with subsequent bleeding at defecation. 2. Mechanism of Prolapse (Cushions Sliding Theory) - Repeated straining & passage of hard fecal bolus cause stretching & disruption of the elastic fibers holding the cushion to the underlying internal sphincter.

CLINICAL PICTURE AND DIAGNOSIS

Clinical Features

- External hemorrhoids may swell or become thrombosed causing pain or they may ulcerate with subsequent bleeding. Thrombosis can ultimately resolve or a skin tag may remain which affords the possibility of itching, burning and soiling. - Internal hemorrhoids may cause burning, swelling, itching, pain, protrusion, discharge, bleeding &/or soiling. - Hemorrhoidal Bleeding: occurs with, or following, bowel movements, is almost universally bright red, very commonly drips into the toilet water and not mixed with the stool, dark, or melanotic in nature. Rarely, may present with anemia 2ry to chronic blood loss. - Hemorrhoidal Prolapse usually occurs in association with a bowel movement, particularly when straining is present. Hemorrhoids may also prolapse during walking or heavy lifting as a result of increased intra-abdominal pressure. The prolapse is associated with a full, uncomfortable feeling, which resolves when the prolapse reduces.

Summary of Symptoms of Hemorrhoids - Hemorrhoids or piles are symptomatic anal cushions. They are more common when intra- abdominal pressure is increased e.g. in obesity, constipation and pregnancy

- Classically, they occur in the 3, 7 and 11 O‘clock positions with the patient in the lithotomy position - Symptoms may include: 1. Bright red bleeding. 2. Prolapse that may cause a protruding mass (Figure 2). 3. Mucoid discharge with prolapse. 4. Itching (if hygiene is poor). 5. No pain unless external and thrombosed or prolapsed Figure 2. Prolapsed hemorrhoids (protruding mass)

323

Evaluation of Per-anal Bleeding

Rigid Sigmoidoscopy - It is indicated in young individuals with bleeding associated with hemorrhoids and no other systemic symptoms and no family history of colorectal cancer (CRC). Colonoscopy - It is indicated in older patients with a family Hx of CRC or change in bowel habits

TREATMENT

- Treatment of symptomatic internal hemorrhoids varies from simple reassurance to operative hemorrhoidectomy. - Treatments are classified into three categories: (1) Dietary and lifestyle modification, (2) Non-operative/office procedures and (3) Operative hemorrhoidectomy.

Dietary and Lifestyle Modifications

- Avoid prolonged attempts at defecation - Increasing fluid and fiber in the diet - Exercise - Stool modifiers - Warm sitz baths and topical creams (prolonged use of topical steroids is harmful)

Non-operative/Office Procedures (Grade I or Grade II)

1. Rubber Band Ligation (RBL) - It is useful for Grade I or Grade II hemorrhoids and, in some circumstances, Grade III hemorrhoids. - It involves strangulation of the redundant mucosa proximal to the internal anal cushions by using tight rubber bands, the resulting ulcer heals with fixation of the mucosa to the

underlying muscle wall (90% success Figure 3. Rubber band ligation (RBL) rate) (Figure 3) 2. Infrared Coagulation - It is useful for Grade I or Grade II hemorrhoids; however, it may need repetitive treatment sessions, thus, alternative methods may be more efficacious (i.e. RBL). 3. Sclerotherapy - It involves the injection of an irritating material into the submucosa in order to reduce vascularity and increase fibrosis. - This, in theory, reduces blood entering the hemorrhoidal bundle, also fixes the hemorrhoidal bundle to the underlying internal sphincter and ∴ r bleeding a prolapse. - Injecting agents have traditionally been Phenol in oil or Sodium Morrhuate.

324

Operative Procedures (Grade III or Grade IV)

Hemorrhoidectomy Principle - Various types of hemorrhoidectomy have developed throughout the years, but they are all associated with the same basic principle. As hemorrhoids are thought to be redundant fibrovascular cushions, most treatments reduce blood flow and remove redundant tissue. Additionally, mucosal and submucosal blood flow is interrupted by a circular staple line Techniques 1. Open (Milligan and Morgan) hemorrhoidectomy - It is most commonly used. This technique involves ligation and excision of the hemorrhoids while leaving the wound open. - One, two or three hemorrhoids may be treated in this manner. 2. Closed (Ferguson) hemorrhoidectomy - This technique involves excision of the hemorrhoid and the pedicle is then sutured with a suture ligature. The wound is then closed with a running suture beginning at the apex and extending at the anoderm. - As with open hemorrhoidectomy, one, two or three hemorrhoidal bundles may be excised in this fashion. It is essential in this procedure that only minimal amounts of anoderm are excised, otherwise, closing the anal wounds can result in significant post- operative pain and perhaps long-term anal stenosis. Complications of open & closed hemorrhoidectomy - Severe post-operative pain (avoid intra-anal packing after surgery) - Acute urine retention (conservative treatment, if failed a urinary catheter should be inserted) - Reactionary or 2ry hemorrhage (needs re-admission for surgical control of bleeding) - Anal fissure (associated with stenosis due to excessive excision of anal verge skin and pectin mucosa) - Abscess or fistula - Skin tags - Anal stenosis (avoidable by leaving adequate islands of mucosa between hemorrhoidectomy wounds) - Pseudo polyps - Incontinence 3. Diathermy Hemorrhoidectomy / LigaSure - Both open and closed techniques can be done using diathermy electro-coagulation. - This technique results in less post-operative bleeding and pain as no intra-anal pack is needed. Recently, vascular-sealing devices are used e.g. LigaSure 4. Stapled Hemorrhoidectomy - This technique involves trans-anal, circular stapling of redundant anorectal mucosa with a standard circular stapling instrument. - No incisions are made in the somatically innervated, highly sensitive anoderm theoretically resulting in significantly less post-operative pain, short hospital stay and early return to normal activity.

325

- It is a promising new technique available to patients otherwise requiring surgical hemorrhoidectomy. It is associated with significantly less pain and similar complication rates when compared to conventional surgery.

Less Invasive Operative Techniques 1. Pile Sutures - This technique involves three levels suture ligation of perforating veins at the base of vascular cushions 2. Ligation Anopexy - This technique allows reduction of the redundant mucosa, reduction of the blood flow to the hemorrhoidal cushions, re-setting of hemorrhoidal cushions in their normal position, and fixation of hemorrhoidal cushions to the underlying internal sphincter

COMPLICATIONS OF INTERNAL HEMORRHOIDAL DISEASE

1. Thrombosis / Strangulation / Ulceration/ Gangrene Pathogenesis - Prolapse of internal hemorrhoid, which becomes gripped by the sphincter thus interfering with venous return (causing strangulation) and so it becomes tense, very painful and if strangulation is not relieved in few hours, the pile becomes dark purple or black and feels solid, with considerable edema around the anus and persistence of tenderness rather than pain (thus become thrombosed). - Superficial ulceration is the 3rd step in the events after strangulation and thrombosis, and this occurs in the exposed mucosa Ulceration is usually associated with bleeding that may not stop except with urgent surgery. - Thrombosis with bacterial invasion and/or gangrene of the cushion or the whole anal canal can occur. This is due to occlusion of the arterial blood supply or superadded infections with anaerobic bacteria. This is rare now and responds to antibiotic. Portal pyemia is history. Conservative Treatment - Bed rest - Stool modifiers - Warm sitz baths - NSAIDs and analgesics - Venotonic such as Diosimin - Antibiotics: Metronidazol & broad spectrum antibiotics - Local decongestant applications (e.g. jell, ointment, or lead sub-acetate compresses) Indications of early surgery (open hemorrhoidectomy under antibiotic cover). - In case of very early diagnosis (rare) with no extensive edema & a single prolapsed, strangulated, thrombosed pile - Ulceration with partial extrusion causing bleeding or infection.

326

2. Anemia - Anemia may develop secondary to chronic blood loss. 3. Perianal dermatitis - It results from mucus leakage, skin tags, bad hygiene, and sensitivity to drugs and additives to sitz baths. - It is treated by taking care of the perianal skin hygiene, excision of prolapsed piles, skin tags, and stoppage of use of unnecessary local applications 4. Fibrosis - Fibrous tissue polyps of the anal lining may develop as a result of resolution of thrombosis. 5. Suppuration - Suppuration may occur due to bacterial invasion of ulcerated hemorrhoids (anaerobic or portal pyemia) 6. Partial rectal mucosal prolapse

COMPLICATIONS OF EXTERNAL HEMORRHOIDS

Thrombosis of external vascular channels

- It is incorrectly termed peri-anal hematoma. - It presents as a tense swelling that feels indurated and painful, smooth and tender, with blue coloration seen below the skin with variable amount of edema. - Self-resolution is the rule, but, it may persist and need surgical excision for extrusion with continuous bleeding or for persistence

327

CHAPTER 6

Ano-rectal Abscess and Fistula-in-Ano

ANO-RECTAL ABSCESS

Etiology

- Ano-rectal abscesses start as an infection in the anal glands at the base of the anal crypt. The abscess develops into the plane between the IAS & EAS (i.e. inter-sphincteric), and then extends to adjacent areas as the abscess expands as shown in Figure 1.

Figure 1. Spread of anorectal sepsis from infected anal glands in the inter- sphincteric space (1) downwards toward the perineum, (2) upwards to supra-levator space, (3) through the EAS either peri-anal or ischio-rectal, and (4) internally into the anal canal along the duct to dentate line.

Classification

- The classification is based on the predominant area of expansion: perianal, ischio-rectal, low inter-sphincteric, high inter-sphincteric, submucous, or pelvirectal, in addition to the posterior space of Courtney, which is located posterior to the rectum in the midline loose areolar tissue joining the 2 ischiorectal fossa (Figure 2) Figure 2. Different sites of ano-rectal abscess

- The spaces, which allow the pus to spread in a circumferential pattern, are spaces connected posteriorly to each other with no septae in between. These are mainly 3 spaces: (1) the ischio-rectal spaces, (2) the pelvi-rectal spaces, and (3) the low inter-sphincteric spaces.

328

Clinical Picture

- Severe throbbing pain (worse on sitting and coughing) and dyschasia (painful defecation). - Systemic symptoms (fever, malaise, etc) (especially in ischio-rectal and pelvi-rectal collections) - Local area of tenderness and induration (around the anus, or felt by PR examination). - Quite often pus is seen (discharging from the anus or a spontaneous external opening). - Determine if the patient is immuno-compromised (diabetic or on steroids or immunosuppressants).

Differential Diagnosis

- An ano-rectal (or peri-anal) abscess should be differentiated from other painful peri-anal conditions, which include: 1. Anal fissure 2. Peri-anal hematoma 3. Strangulated or thrombosed internal piles 4. Peri-anal fistula 5. Anal carcinoma 6. Pruritis ani 7. Proctalgia fugax.

Treatment

Conservative Treatment - Very early in the course of the disease, where only a localized area of tenderness is found, massive doses of antibiotics with gram negative spectrum and anaerobes efficiency can be used (Quinolones and Metronidazole) with analgesics, sit baths and close follow-up.

Surgical Treatment (Incision and Drainage - I&D) - The sign for incision drainage is the presence of induration (do not wait for fluctuation), and this can be easily detected in suppuration of spaces close to the perianal skin e.g. low inter-sphincteric, perianal, and ischio-rectal spaces suppuration. - I&D is done under general anesthesia to ensure complete evacuation of pus.

Summary: Ano-rectal abscess

 They usually produce a painful, throbbing swelling in the anal region.  The patient often has swinging pyrexia.  They are subdivided according to anatomical site into perianal, ischiorectal, submucous and pelvirectal  Underlying conditions include fistula-in-ano (most common), Crohn‘s disease, diabetes, and immunosuppression.  Treatment is drainage of pus in first instance, together with appropriate antibiotics  Always look for a potential underlying problem

329

FISTULA-IN-ANO

Definition

- A fistula is a tract between 2 epithelial-lined surfaces AND has 2 openings, an internal and an external. - Fistula-in-ano opens deeply in the anal canal (or rectum) and superficially on the skin around the anus.

Etiology

- All fistulae arise from pyogenic abscesses, which have been allowed to develop to the point of spontaneous discharge or surgical drainage especially if inadequate. The point of debate thereafter is to explain why the condition has persisted. - This may be due to: 1. Persistence of the internal opening (recurrent activation of infection). This is the most accepted explanation nowadays. 2. The presence of anal glands, which act as a reservoir for infection or as foreign bodies 3. Inflammatory bowel disease (IBD) is very important association: Crohn's disease (CD) in 20% of cases, ulcerative colitis (UC) to a lesser extent and indeterminate colitis in between. 4. Active TB is present in 8-16 % (one half shows evidence of healed pulmonary TB). 5. Very rarely, higher carcinoma is coexistent.

Classification

It is necessary to consider the outline of the track in 2 planes: horizontal & vertical.

Horizontal Plane (Goodsall’s Rule) - Fistulae with their external opening in the anterior half of the anus have their internal opening opposite the external one, while those with the external opening in the posterior half have their internal opening in the midline i.e. have curved tracks. - Lateral extension on both sides may take place leading to the characteristic horse-shoe Figure 3. Goodsall's rule of fistula-in-ano fistula (Figure 3).

331

Vertical Plane (Park’s Classification) Alan Park's classification of anorectal fistulae is demonstrated in Figure 4 and includes the following types:

Figure 4. Alan Park's classification of ano-rectal fistulae.

1. Subcutaneous (SC) - Rare, just below the perianal skin (superficial to the SC part of the EAS) near the pectinate line or at the base of a fissure 2. Inter-sphincteric - Low inter-sphincteric: The track runs from the region of the pectinate line through the lower part of the IAS and skirts underneath the SC part of the external anal sphincter (EAS). - Inter-sphincteric with an internal opening located at the dentate line or higher a track is extending in the inter-muscular plane. - Inter-sphincteric with upward blind extension. - Inter-sphincteric with blind upward extension and a supralevator pocket. - Inter-sphincteric with no external opening, and only an internal opening located at the dentate line with blind upward inter-sphincteric tract (a sinus rather than a fistula). 3. Trans-sphincteric - Trans-sphincteric: the internal opening may lie at or higher than the pectinate line but below the ano-rectal ring (ARR). The tract extends through the EAS. - Trans-sphincteric with occasional side track extending blindly upwards between the two sphincters or in the ischio-rectal fossa. - Trans-sphincteric with a blind upward extension in the ischio-rectal spaces with a supra-levator pocket. 4. Extra-sphincteric - Iatrogenic or due to pelvic pathology of the rectum. 5. Supra-sphincteric - It is originally an inter-sphincteric fistula with a blind upward extension & supralevator pocket, which has penetrated through the levator to present as an ischio-rectal abscess with an exit to the ischio-rectal skin.

331

Fistulae may thus be classified as: 1. Inter-sphincteric (70%) 2. Trans-sphincteric (25%) 3. Supra-sphincteric (5%) 4. Extra-sphincteric (<1%) Extra-sphincteric fistulae are usually not associated with inter-sphincteric sepsis. In such cases, IBD or neoplasia should be considered.

Clinical Features and Differential Diagnosis

History of abscess (spontaneous or surgical drainage) or recurrent abscesses or discharge

A. Symptoms - Discharge: Continuous or intermittent sero-purulent discharge with pruritis & soiling. Sometimes it stops due to obstruction of the external opening. - Pain: due to abscess formation or obstructed internal opening. B. Examination - Inspection: The following should be defined: (1) number, (2) site (anterior or posterior), (3) distance from the anal canal (the nearer the external opening from the anus, the lower is the fistula), (4) discharge, and (5) granulation tissue or temporary healing. - Palpation: The fistulous tract is felt and its relation to the ARMR is determined. - PR Exam: the internal opening may be felt, induration of the track may be identified and other pathologies excluded e.g. cancer. - Proctoscopy or sigmoidoscopy is a must before embarking on the treatment. - Hidradenitis suppurativa, pilonidal sinus, dermal suppurative sinuses, infected sebaceous cysts and urethra-perineal fistulas must be differentiated from cryptogenic fistulas.

Investigations

Endo-anal/Endo-rectal Ultrasound (US) - Passage of a 7 to 10-MHz transducer into the anal canal helps define the muscular anatomy to differentiate inter-sphincteric from trans-sphincteric lesions. A standard water-filled balloon transducer can help evaluate the rectal wall for any supra-sphincteric extension. - This modality has not been used widely for routine clinical fistula evaluation.

Anal Manometry - Pressure evaluation of the sphincter mechanism is helpful in certain patients. - Decreased tone observed during preoperative evaluation may be due to: 1. Previous fistulotomy or obstetrical trauma 2. High trans-sphincteric or supra-sphincteric fistula (if known) 3. Very elderly patients - If reduced, surgical division of any portion of the sphincter mechanism should be avoided

332

Magnetic Resonance Imaging (MRI) - Magnetic resonance imaging may be used in difficult cases.

Examination under Anesthesia (EUA) - An examination of the perineum, digital PR and anoscopy are performed under anesthesia. - This EUA is necessary before surgical intervention, especially if outpatient evaluation causes discomfort or has not helped delineate the course of the fistulous process. - Several techniques have been described to help locate the course of the fistula and, more importantly, identify the internal opening. 1. Hydrogen peroxide, milk, or dilute methylene blue (MB) is injected into the external opening & its egress at the dentate line is observed (The MB often obscures the field rather than helping to identify the opening). 2. Traction (pulling or pushing) on the external opening may also cause a dimpling or protrusion of the involved crypt. 3. Insertion of a blunt-tipped crypt probe via the external opening may help outline the direction of the tract. If it approaches the dentate line within a few millimeters, a direct extension likely existed. Avoid using excessive force and creating false passages.

Treatment

- The Puborectalis is the key to future continence - Low fistulas: Lay open with either fistulotomy or fistulectomy - High fistulas: Require two stage surgery 1. Setons - loose or tight. 2. Anorectal advancement flap (may be considered).

Fistula Lay-Open - The main target of the operation is to lay open the tract from its external opening to the internal opening in order to allow granulation of the wound to close the defect created. - The main problem is cut through a significant part of the EAS especially if the deep part is involved or more seriously the puborectalis, thus compromising continence. - In these cases, the use of a Seton is an acceptable technique to avoid the occurrence of this dreadful complication

Seton Placement - A Seton can be placed alone, combined with fistulotomy, or in a staged fashion. This technique is useful in patients with the following conditions: 1. Complex fistulae (high Trans-sphincteric, supra-sphincteric, extra-sphincteric) or multiple fistulae. 2. Recurrent fistulae after previous fistulotomy. 3. Anterior fistulae in female patients. 4. Poor preoperative sphincter pressures. 5. Patients with Crohn's disease (CD) or patients who are immunosuppressed.

333

- Setons can be made from large silk suture, Silastic vessel markers, or rubber bands that are threaded through the fistula tract. They have 2 purposes beyond giving a visual identification of the amount of sphincter muscle involved; namely: (1) To drain and promote fibrosis and (2) To cut through the fistula. - Single-stage seton (cutting) o The Seton is passed through the fistula tract around the deep EAS after opening the skin, SC tissue, IAS muscle and subcutaneous EAS muscle. o The Seton is tightened down and secured with a separate silk tie. o With time, fibrosis occurs above the Seton as it gradually cuts through the sphincter muscles and essentially exteriorizes the tract. o The Seton is tightened on subsequent office visits until it is pulled through over 6-8 weeks. o A cutting seton can also be used without associated fistulotomy - Two-stage Seton (draining/fibrosing) o The Seton is passed around the deep portion of the EAS after opening the skin, SC tissue, IAS muscle & subcutaneous EAS muscle. o Unlike the cutting Seton, the Seton is left loose to drain the inter-sphincteric space and to promote fibrosis in the deep sphincter muscle. o Once the superficial wound is healed completely (2-3 m later), the Seton-bound sphincter muscle is divided. o Two studies (74 patients combined) support the 2-stage approach with a 0-nylon Seton. o Once wound healing is complete, the Seton is removed without division of the remaining encircled deep EAS muscle. Eradication of the fistula tract occurs in 60- 78% of cases.

Mucosal Advancement Flap (MAF) - The MAF is reserved for patients with chronic high fistula, but is indicated for the same disease process as Seton use. - Advantages include a one-stage procedure with no additional sphincter damage. - A disadvantage is poor success in patients with Crohn's disease or acute infection. - This procedure includes total fistulectomy with removal of the 1ry and 2ry tracts, in addition to complete excision of the internal opening. - A rectal mucomuscular flap with a wide proximal base (2 times the apex width) is raised. - The internal muscle defect is closed with an absorbable suture, and the flap is sewn down over the internal opening so that its suture line does not overlap the muscular repair.

Post-operative Complications

Early Post-Operative Complications - Urinary retention - Bleeding - Fecal impaction - Thrombosed hemorrhoids

334

Delayed post-Operative Complications - Recurrence - Incontinence (stool) - Anal stenosis: The healing process causes fibrosis of the anal canal. Bulking agents for stool help prevent narrowing. - Delayed wound healing: Complete healing occurs by 12 weeks unless an underlying disease process is present (ie, recurrence, Crohn's disease).

Complication Fistulotomy Seton Mucosal Advancement Flap Recurrence rate 0-18% 0-17% 1-10% Fecal incontinence 3-7% 0-17% 6-8%.

Causes of recurrent fistula 1. Inadequate excision or missing side tracks 2. Specific infection e.g. TB. 3. Presence of active disease e.g. Crohn‘s disease. 4. Malignancy. 5. Epithelization of the track (with incomplete excision).

Future Perspectives

- Recent biotechnology has led to the development of many new tissue-adhesive materials. - Reported series exist of fibrin glue treatment of fistula-in-ano, with one-year follow-up success rates approaching 60%. By its less invasive nature, this therapy leads to decreased postoperative morbidity. However, information on long-term success rates and modification of techniques will have to await further studies.

Summary: Ano-rectal Fistulae

 Anorectal fistulae are common and may be simple or complex  Anorectal fistulae are classified according to their relationship to the anal sphincters  They may be associated with underlying disease such as TB or Crohn‘s disease  Laying-open is the surest method of eradication, but sphincter division may result in incontinence

335

CHAPTER 7 Pilonidal Disease and Fissure-in-Ano

PILONIDAL DISEASE

Definition

- Common infection around hair containing sinuses in the natal cleft. - Pilus means ‗hair or tuft of hair‘, nidus means ‗nest‘.

Etiology

- Congenital: sinus of ano-coccygeal area - Acquired: Most common, supported by : 1. Inter-digital pilonidal sinus (PNS) in hair dressers 2. The age incidence is between 20-29 years 3. Hair follicles are demonstrated in the wall of the sinus 4. The disease usually hairy men 5. Recurrence is common

Pathology

- Abscess formation - Sinus formation: the sinus extends into the subcutaneous planes. Branching channels are not infrequent with stratified squamous epithelial lining. Hair shafts are found lying loose in the sinus and embedded in granulation tissue. In 3/4 of the cases, foreign body giant cells are common.

Clinical Picture

1. Acute presentation - The patient presents with an abscess in the natal cleft. 2. Chronic presentation - Midline sinus at the first piece of coccyx - Tuft of hair projecting from its mouth - Discharge, mostly blood-stained, and contains foul sebum and sometimes hair. - Secondary openings may be present on either side of the midline. - Age: third decade - Gender: male: female = 4:1

336

Treatment

Conservative Treatment - Cleaning out of the openings - Shaving of hair - Frequent washing by detergents and water - Avoiding long sittings

Treatment of Acute Exacerbation (Abscess) - Incision-drainage - Broad spectrum antibiotics - Instillation of pure phenol solution

Treatment of Chronic Sinus - Local excision and healing by secondary intention (lay-open) - Lateral drainage and midline pit excision (Buscom) - Excision and closure - Rhomboid excision and flap repair - Karydakis procedure: sound closure to the side of midline.

Causes of Recurrence

- Over-looked diverticulum in the primary operation - New hair enters the skin or the scar - Deformity of natal fold by scarring (the least trauma causes tearing of the scar).

FISSURE-IN-ANO

Definition

- An anal fissure is an elongated ulcer in the long axis of the anal canal.

Location

- Midline posterior - Midline anterior - Lateral

Etiology

- Lack of support of the anal mucous membrane - High anal pressure - Trauma and ischemia - Inflammatory bowel disease (IBD) - Anal stenosis (post-operative)

337

Pathology

Acute Fissure - It is a deep tear of anal margin skin extending to the dentate line - The edges are inflammatory and edematous - There is spasm of anal sphincter

Chronic Fissure - It is characterized by more inflamed, indurated margins and a base consisting of scar tissue. - A skin tag can be present at the inferior extremity of the ulcer called ―sentinel pile‖ - The muscle becomes contracted by infiltration of fibrous tissue. - Chronic fissure- in-ano may have a specific cause e.g. Crohn‘s disease.

Clinical Presentation

- Pain during defecation - Bright red bleeding - Constipation - Puckered anus

Treatment

Conservative Treatment - Glyceryl trinitrates --- nitric acid - Laxatives - Xylocaine ointment

Operative Treatment - It is rarely needed for acute anal fissure - For chronic anal fissure, anal sphincterotomy is done (lateral or dorsal sphincterotomy)

338

CHAPTER 8 Malignancy of Colon, Rectum and Anus

COLO-RECTAL CANCER (CRC)

Incidence

- In Egypt, colorectal cancer (CRC) is the 4th most commonly diagnosed cancer in both men and women, with a median age of 48 years and a male to female ratio of 1.1.

Risk Factors for CRC

1. Hereditary CRC Syndromes a. Adenomatous polyposis syndromes - Familial adenoamtous polyposis (FAP) - MYH-associated polyposis (MAP) b. Non-polyposis syndrome - Hereditary non-polyposis CRC (HNPCC) c. Hamartomatous polyp syndromes - Peutz-Jeghers syndrome (PJS) - Juvenile polyposis syndrome (JPS) - Cowden disease (Bannayan-Ruvalcaba-Riley) 2. Inflammatory Bowel Disease (IBD): Ulcerative Colitis 3. Personal History (Hx) of CRC 4. Family Hx of CRC 5. Ethnic background: Ashkenazi Jews 6. Age: above 50 y in the Western community. In Egypt the median age of CRC is <50 y. 7. Environmental Factors a. Increased Risk of CRC ↑ with a diet high in red meat & animal fat, and low-fiber diet (low overall intake of fruits & vegetables). b. Lifestyle choices that are associated with ↑ risk for CRC include alcohol & tobacco consumption, obesity & sedentary habits

Factors Associated with Lower Risk Include: 1. Folate intake 2. Calcium intake 3. Estrogen replacement therapy

339

Genetics of CRC

- Genetics does not mean inherited, genetics means pathogenesis. Genetically, CRC represents a complex disease, and accumulation of genetic alterations is associated with progression from normal epithelial cells or premalignant lesion (adenoma) to invasive adenocarcinoma. Colorectal cancers have several gene expression profiles with different pathogenic (genetic) pathways (Table 1). - The major known mechanisms of genetic Table 1. Types of Colorectal Cancer (CRC) instability are chromosomal instability Type % (CIN), which constitutes 85% of colon Sporadic 75 cancer and the other 15% is due to Family History of CRC 18 microsatellite instability (MSI) mismatch HNPCC 5 repair (MMR) pathway. FAP 1 MAP 1 Pathway I CIN (Chromosomal Instability) PJS & JP <1 IBD 1 - Acquired (somatic) or inherited (germline) macrogenetic alterations at chromosomal level either in the form of abnormal number (aneuploidy) or gross changes i.e. loss of heterozygosity (LOH). - The important genes involved in these chromosome losses are APC (5q), DCC (18q) and TP53 (17p). This pathway is seen in FAP and adenoma-carcinoma model.

APC K-ras DCC p53

Normal Early Intermediate Late Cancer Epithelium Adenoma Adenoma Adenoma

Pathway II Microsatellite Instability (MSI) Mismatch Repair Pathway (MMR) - Germline or somatic microgenetic (intragenic) mutations of DNA damage-repair genes result in microsatellite instability (MSI) i.e. insertion or deletion of nucleotides in one or more alleles. MSI means alterations in repeating units of DNA that occur normally throughout the genome. - The important genes involved in this MSI are MMR genes (hMSH2, hMSH6, hMLH1, hMLH3, hPMS1, hPMS2) - This pathway is seen in 95% of HNPCC and 18% of sporadic CRC.

Alternative Pathways - Alternative pathways are involved in up to 30% of CRC, precursors not easily seen with rapid evolution following genetic instability. Alternative pathways include: 1. Flat and depressed adenoma 2. De Novo CRC 3. Colitis induced CRC 4. Serrated Pathway

341

Colitis-induced CRC - This pathway is seen in ulcerative colitis and schistosomiasis Japonicum. Chronic

inflammation results in free O2 radicals that cause DNA damage (dysplasia-carcinoma model)

Aneuploidy K-ras p53 p53 MSI DCC

Normal Indefinite Low Grade High Grade Cancer Dysplasia Dysplasia Dysplasia Epithelium

Serrated Pathway: CpG Island Methylator Phenotype (CIMP) - Epigenetic factors are mechanisms outside the gene such as a cell‘s exposure to carcinogens or hormones, or genetic variations that modify a gene or its protein by methylation, demethylation, phosphorylation, or dephosphorylation. Genes that must be expressed in all tissues have unmethylated regions, called CpG islands. On the other hand, genes that must be turned off in differentiated tissues have these islands methylated. - In this pathway type C tumor suppressor genes silenced by promoter region CpG methylation. - This pathway is seen in juvenile polyposis, Peutz-Jegher‘s syndrome & serrated adenomas

Screening for CRC

- The life-time risk of CRC is summarized in Table 2.

Table 2: Life-Time Risk of CRC

Group Life-time Risk of CRC

General Population 5% Ashkenazi Jews 6-10% Family Hx of CRC 10-15% Personal Hx of CRC 15-20% IBD 15-40% PJS 50% JP 50% HNPCC 70-80% FAP >95%

341

342

High-Risk Group - Patients with a 10-100% lifetime risk according to family history criteria, pathological criteria and pathogenic gene mutation. - Screening Method: in Genetics Centre for formal counseling & mutation analysis

Moderate-Risk Group - First-degree relative (FDR) with CRC aged <45 years or two FDRs: 1. Single colonoscopy at age 55yrs. 2. Polyps snared: If adenomatous, then adenoma surveillance 3. Colon is clear of neoplasia: Reassured & shift to population risk fecal occult blood test (FOBT) screening.

Low-Risk Group - No family history of CRC - Population-based screening 1. Annual (FOBT) 2. Flexible Sigmoidoscpy / 5 years 3. Colonoscopy / 10 years

Clinical Picture of CRC

- Patients with CRC may be asymptomatic (detected by screening programs mentioned above) or present with manifestations of the primary lesion, metastases or complications of the disease. 1. Asymptomatic (detected by screening) 2. Primary Lesion 3. Metastases 4. Complications

Clinical Picture (C/P) of Primary CRC - The sites of primary CRC are demonstrated in Figure 1. As may be seen, the most common site is the rectum (38%) followed by the sigmoid colon (20%). The least commonly affected site is the appendix (0.5%) followed by the splenic flexure (2%) - The most common presenting symptoms associated with CRC are abdominal pain, followed by change in bowel habits, rectal bleeding and occult blood in the stool. Table 3 and Figure 2 summarize the patient's

symptoms according to the location of the tumor. Differences are shown in Table 4. Figure 1. Sites of primary CRC

343

Table 3: Clinical presentation according to tumor location

Cancer Right Colon Cancer Left Colon Cancer Rectum 1. Change in bowel 1. Change in bowel habits: 1. Bleeding /rectum habits: Diarrhea Increasing constipation 2. Mucous/ rectum 2. Vague upper 2. Colicky pain with 3. Tenesmus (sense of incomplete abdominal pain distension evacuation) 3. Iron deficiency 3. Weight loss 4. Change in bowel habits: anemia 4. Mass (advanced or fecal Spurious morning diarrhea 4. Weight loss matter) 5. Pain: Colicky pain in recto- 5. Mass (advanced 5. Obstruction: Common or sigmoid lesions. Severe pelvic cases) 1st presentation pain in advanced cases due to 6. Obstruction: Rare 6. Bleeding /rectum infiltration. 7. Mucous/rectum 6. Weight Loss 7. Obstruction: Rare, in recto- sigmoid lesions.

Figure 2. Clinical presentation of primary CRC according to location of the tumor

Table 4: Comparison between symptoms according to tumor site

Symptom Right Side Left Side Rectum Constipation Change in bowel habits Diarrhea Constipation spurious morning diarrhea Constipation Change in bowel habits Diarrhea Constipation Spurious morning diarrhea Bleeding/rectum Altered Dark Fresh Colicky pain + ++++ upper 1/3 Mass +++ ++ - Mucous/rectum - + ++++ Tenesmus - - +++++ Incomplete evacuation Weight loss +++ + ++ Iron deficiency anemia ++++ + +

344

Spread of CRC

1. Direct spread: (a) intramural (circumferential – longitudinal), and (b) transmural 2. Lymphatic spread 3. Hematologic spread: liver and lung metastases 4. Trans-peritoneal spread: Malignant ascites, Krukenberg‘s tumor, etc.

Complications of CRC

1. Intestinal obstruction 2. Penetration (fistula) 3. Bleeding (anemia or hypovolemia) 4. Intussusception: incomplete obstruction 5. Perforation: tumor perforation or cecal perforation caused by a left-sided colon cancer

Diagnosis (Dx)

An individualized approach to diagnosis considers the following: 1. Patient‘s symptoms 2. Age 3. Personal history of IBD, colon polyps, or CRC. 4. Family history of CRC or predisposing genetic syndromes (e.g., FAP or HNPCC).

Diagnostic Evaluation 1- Colonoscopy: Evaluation of the patient‘s entire colon and rectum for the presence of synchronous neoplasms. Colonoscopy allows biopsy (Bx) and histological confirmation of diagnosis. It also allows for identification and endoscopic removal of synchronous polyps. Pre-operative histology should be done in all rectal tumors. 2- Depending on the patient‘s age and health status, a variety of laboratory, radiologic, and cardiorespiratory tests may be appropriate to assess the patient‘s operative risk.

Pre-operative Assessment 1- Pre-operative, carcinoembryonic antigen (CEA) level. Beneficial for 2 reasons: a. Post-operative return to normal of an elevated pre-operative CEA is associated with complete tumor resection, whereas persistently elevated values indicate the presence of visible or occult residual disease. b. Elevated preoperative CEA level is an independent prognosticator of poor outcome. CEA is not useful as a screening tool as it increases in many conditions eg, CRC, proximal GIT cancers, lung & breast cancers, benign inflammatory conditions of the GIT, and smoking. 2- Pre-operative CT scanning: CT scans can be used to evaluate local extension of the tumor & regional lymphadenopathy, as well as for the presence of hepatic metastases. 3- Preoperative chest x-rays or CT scan to evaluate the lungs for evidence of metastatic disease. 4- PET–CT scan is not routinely indicated.

345

Staging of CRC

TNM Staging T Tx: Incomplete information Tis: It involves only the mucosa T1: Extends into the submucosa T2: Extends into the muscularis propria T3: Extends into subserosa T4a: Penetrates visceral peritoneum T4b: Tumor invades other organs or structures N Nx: Incomplete information N0: No LN involvement N1: In 1- 3 regional LNs. - N1a: in 1 regional LNs - N1b: in 2- 3 regional LNs - N1c: Tumor Deposits in subserosa, mesentery, nonperitonealized pericolic or perirectal tissues without regional LNs metastasis N2: In 4 or more regional LNs - N2a: in 4-6 regional LNs - N2b: in 7 or more regional LNs M M0: No distant Metastasis M1: Distant Metastasis - M1a: Metastasis confined to one organ or site (Liver, lung, ovary, non-regional LNs) - M1b: Metastasis in more than one organ/site or peritonium

Stage grouping of CRC and the 5-y SR according to stage are shown in Tables 5 and 6, respectively

Table 5. Stage grouping of CRC Table 6. 5-y-Survival Rate (SR) of CRC

Stage TNM Duke’s Stage 5-Y-SR 0 Tis N0 M0 - I T1 N0 M0 A T2 N0 M0 A I 92% IIA T3 N0 M0 B IIB T4a N0 M0 B II 73% IIC T4b N0 M0 B IIA 85% IIIA T1-2 N1/N1c M0 C IIB 72% T1 N2a M0 C III 56% IIIB T3-4 N1/N1c M0 C IIIA 83% T2-T3 N2a M0 C T1-T2 N2b M0 C IIIB 64% IIIC T4a N2a M0 C IIIC 44% T3-T4a N2b M0 C IV 8% T4b N1-N2 M0 C IVA Any T Any N M1a - IVB Any T Any N M1b -

346

Dukes Staging of Rectal Cancer

Dukes classified carcinoma of the rectum into three stages (Table 7)

Table 7: Dukes Staging (Figure 3)

Stage Description

A The growth is limited to the rectal wall (15%): Prognosis excellent

B The growth is extended to the extra-rectal tissues, but no metastasis to the regional LNs (35%): Prognosis reasonable.

C There are secondary deposits in the regional LNs (50%). These tumors are further subdivided into: - C1, in which the local para-rectal LNs alone are involved. - C2, in which the LNS accompanying the supplying blood vessels are implicated up to the point of division. This does not take into account cases that have metastasized beyond the regional LNs or by way of the venous system: Prognosis is poor.

D It is often included, which was not described by Dukes. This stage signifies the presence of widespread metastases, usually hepatic.

Figure 3. The three cardinal stages of progression of the neoplasm (After C. Dukes, FRCS).

347

Treatment of CRC

Preparation for Operation

A. Informed consent All patients who are to undergo surgery for colon cancer need to be clearly informed of the reasons for and the extent of the proposed resection, the likely outcome of the surgery, the pertinent complications and their likelihood of occurring, expected length of hospitalization and recovery, alternatives to the proposed surgery and prognosis. B. Mechanical bowel preparation Despite its nearly universal use, the literature does not support a defined benefit for preoperative mechanical bowel preparation. The persistence in using it may be justified by the advantages it affords in ease of handling the prepared colon, the proven safety of the methods used for bowel cleansing and the relatively low cost. Outpatient bowel preparation (the day before surgery) is generally safe and cost effective. C. Prophylactic antibiotics Prophylactic antibiotics have proven effectiveness in decreasing the rate of infection, mortality and cost of hospitalization after colonic resection. Regardless which parenteral antibiotic regimen is selected, it must be given before the start of the operation. D. Blood cross match & transfusion The need for transfusion is based on the starting hemoglobin (Hgb), patient‘s physiologic status & extent of intra-operative blood loss. The immunosuppressive effect of transfusion increases the incidence of infection, but not the rate of cancer recurrence. Other factors (extent of resection, tumor location and experience of surgeon) in patients requiring transfusion may actually be the cause for the increased recurrence rate (RR). E. Thrombo-embolism prophylaxis All patients undergoing surgery for colon cancer should receive prophylaxis against thrombo-embolic disease as they have a high incidence of venous thrombo-embolism, including deep venous thrombosis (DVT) and pulmonary embolism (PE).

Operative Issues

A. Operative Technique - The determinant of adequate bowel resection for colon cancer is resection of the 1ry feeding arterial vessel and its corresponding lymphatics. Tumors located in border zones should be resected with the neighboring lymphatic regions to encompass both possible directions of spread. The length of bowel resected is usually governed by the blood supply to that segment. Ligation of the origin of the 1ry feeding (Figure 4) vessel ensures the inclusion of the apical LNs, which may affect prognosis. - There is much concern regarding intra-operative manipulation of the tumor with shedding of cancer cells into the portal circulation. However, the value of the ―no touch‖ technique has not been proven, although there is a theoretic basis for its use. - Operative procedures according to tumor location are summarized in Table 7

348

Table 7: Operative procedures according to site of the lesion

Site of the Lesion Operation Vessels Excised Parts

Appendix, cecum, Right hemicolectomy - Ileocolic Cecum, appendix, or with ileo-transverse - Right colic ascending colon and ascending colon anastomoses (Figure 5) - Right branch of right 1/3 of transverse middle colic colon Hepatic flexure Extended right hemi- - Ileocolic Cecum, appendix, colectomy with ileo- - Right colic ascending colon and transverse anastomosis - Middle colic right 2/3 of transverse colon Transverse colon Transverse colectomy Middle colic Transverse colon with anastomosis including both between the ascending hepatic and splenic and descending colons flexures ± greater omentum in T4 lesions Splenic flexure Extended left Hemi- - Inferior mesenteric Descending colon colectomy with - Left branch of and left half of the anastomosis between Middle colic transverse colon transverse and sigmoid colons

Descending colon Left hemicolectomy - Inferior mesenteric Descending colon with anastomosis and left third of the between transverse and transverse colon ± sigmoid colons or sigmoid colon (rectum) (Figure 6)

Sigmoid colon Sigmoidectomy - Inferior mesenteric Sigmoid colon with anastomosis between left colon and upper rectum (Figure 7)

349

Figure 4: Arterial supply of the colon and rectum

Figure 5. Right hemicolectomy with ileo-colic anastomoses for a tumor in the cecum

351

Figure 6: Left hemicolectomy with anastomosis between transverse and sigmoid colons for a tumor in the descending colon.

Figure 7: Sigmoidectomy with colo-rectal anastomoses for a tumor in the sigmoid colon.

351

B. Synchronous colon cancer - Synchronous colon cancers can be treated by 2 separate resections or subtotal colectomy. C. Contiguous organ attachment (15% of cases) - Colon cancers adherent to adjacent structures should be resected en bloc. At the time of surgery, it often is impossible to distinguish between malignant & inflammatory adhesions. - Because these adhesions harbor malignant cells in 40% of the time, an en bloc excision is necessary to achieve a tumor-free resection. D. Synchronous resection of liver metastases (10-20% of cases) - Resection (or ablation) of synchronous liver metastases should be performed at the time of the initial colon resection because it remains only means of obtaining long-term survival in this group of patients. - Removal of the metastasis (metastatectomy) can proceed if the following conditions are met: 1. Colon resection has proceeded with minimal blood loss or contamination 2. The medical condition of the patient permits combining both procedures. 3. Resection can be done with at least 1-cm margin 4. The incision is appropriate for hepatic resection. 5. The surgeon is comfortable with performing the hepatic resection. E. Role of oophorectomy - Bilateral oophorectomy is advised when one or both ovaries are grossly abnormal or involved with contiguous extension of the colon cancer. However, prophylactic oophorectomy is not recommended. F. Role of laparoscopic resection - Laparoscopic resection of colon cancer is feasible but requires specific surgical expertise. Adherence to oncologic principles is possible and adequate lymphadenectomy with disease-free margins can be achieved comparable to open surgery.

Operative Issues - Emergency

A. Obstructing colon cancer a. Obstructing right or transverse colon cancer: Right or extended right colectomy with a 1ry ileo-colic anastomoses. b. Left-sided colonic obstruction: The procedure should be individualized. - Resection with end colostomy and Hartmann‘s pouch - Resection with on-table colonic lavage and 1ry anastomosis - Subtotal colectomy with ileo-rectal anastomosis. - Insertion of colonic Stent to relieve the acute obstruction thereby permitting an elective colonic oral lavage, colonoscopy and then resection with 1ry anastomosis. The 3-stage approach of performing "proximal diversion, then resection, then colostomy closure" is less advantageous because of its high mortality and morbidity rates.

352

B. Colonic Perforation The site of colonic perforation caused by colon cancer should be resected if at all possible. a. Right-sided colon perforation from a right colon cancer should be resected. If there is a free perforation with peritonitis, an anastomosis may be unwise & the patient is best left with an end ileostomy. The distal end may be brought out as a mucous fistula or stapled off as a Hartmann‘s pouch. Alternatively, if there is limited fecal spillage, the surgeon may choose to reanastomose the bowel ± fecal diversion. b. When a left colon cancer perforates resulting in peritonitis, a Hartmann‘s resection is mostly indicated. If there is massive proximal colonic distention and/or ischemia, a subtotal colectomy may be the best choice. If peritoneal contamination is limited, an ileo-rectal or ileo-sigmoid anastomosis with a diverting loop ileostomy may be done. c. In the case of a right colon perforation caused by a left-sided colon cancer, most experts advocate a subtotal colectomy. Whether an anastomosis or a loop ileostomy to protect the anastomosis is performed, it depends on the surgeon‘s judgment. C. Massive Colonic Bleeding a. Acutely bleeding colon cancers that require emergent resection should be removed following the same principles as in elective resection. Because of the cathartic effect of the bleeding, the bowel has been effectively cleansed of the bulk of fecal matter and a 1ry anastomosis can be considered. Whether to proceed with an anastomosis or an end stoma & mucous fistula (or Hartmann‘s pouch) is based on the surgeon‘s judgment. b. If the site of the bleeding cannot be identified, a subtotal colectomy is preferred.

Post-operative Staging of Colon Cancer - It is important that accurate pathologic evaluation of the radial margin of resection be performed. Each operation is given a resection code to denote completeness of resection: R0: Complete tumor resection with all margins negative R1: Incomplete tumor resection with microscopic involvement of the margin R2: Incomplete tumor resection with gross residual tumor that was not resected. - Other factors that have an impact on the patient‘s risk of recurrence and survival. 1. Microscopic venous or lymphatic invasion within the specimen worsen the prognosis for every stage. 2. Histologic grade & histologic type 3. Serum CEA - Lymph node (LN) number o To be properly evaluated, one should strive to have a minimum of 15 LNs examined microscopically. o The accuracy of colon cancer staging improves with increasing the number of LNs evaluated microscopically. Ten or more LNs can be found in 98%t of colon specimens and 13 or more LNs can be found in 91% of specimens without using fat- clearing techniques.

353

Adjuvant Therapy A. Chemotherapy - Administration of 5-Fluorouracil (5-FU)/Leucovorin for 6 m post-operatively, in patients with stage III, may ↓ RR and ↑ SR. - Patients with Stage II who are considered at higher risk for recurrence include those with one or more of the following: tumor perforation, adherence, or invasion of adjacent organs; non-diploidy by flow cytometry; poorly differentiated tumor; or venous, lymphatic and peri-neural invasion. These patients may receive adjuvant chemotherapy using Capecitabine, which is an oral Fluoropyrimidine Carbamate preferentially converted to 5-FU in tumor cells. B. Immunotherapy - Its value is undetermined and its use is recommended in the setting of clinical trials. C. Intra-peritoneal/Intra-portal chemotherapy - Intra-peritoneal and intra-portal infusion of chemotherapy is limited to clinical trials. D. Radiation Therapy (RT) - The role for RT in colon cancer is limited. It is rarely used in the treatment of colon cancer. Radiation‘s potential for injury to the abdominal viscera limits its usefulness.

Surveillance 1. History and Physical Examination: Every 3-6 months for 2 years, then every 6 month for a total of 5 years. 2. Serum CEA: Every 3-6 months for 2 years, then every 6 months for a total of 5 years. 3. CT scan of abdomen and pelvis: Annually for 3 years. 4. Colonoscopy: At one year, then as clinically indicated.

Management of Rectal Cancer

- Anatomically, the rectum is the distal 15-cm of the large bowel leading to the anal canal. Thus, any cancer whose distal margin is 15 cm or less from the anal verge using a rigid sigmoidoscope should be classified as rectal. - Rectal cancer comprises about 25% of the malignancies arising in the large bowel. - Cancers of the intra-peritoneal rectum behave like colon cancers regarding recurrence and prognosis. - The extra-peritoneal rectum resides within the confines of the bony pelvis; it is this distal 10 to 12 cm that constitutes the rectum from the oncologic standpoint. - Rectal cancer is a challenging disease because: 1. It is a common malignancy 2. Difficult dissection 3. Treatment not infrequently entails a permanent stoma 4. Sexual and urinary morbidity 5. Local recurrence

354

Pre-operative Assessment An individualized approach to the diagnosis considers: 1- Patient‘s symptoms 2- Age 3- Personal history of IBD, colon polyps, or CRC 4- Family history of CRC or predisposing genetic syndromes (eg, FAP and HNPCC).

Diagnostic evaluation 1- Colonoscopy: Evaluation of the patient‘s entire colon and rectum for the presence of synchronous neoplasms. Colonoscopy allows biopsy (Bx) and histological confirmation of Dx. It also allows for identification & endoscopic removal of synchronous polyps. 2- Depending on the patient‘s age & health status, a variety of laboratory, radiological and cardio-respiratory tests may be appropriate to assess the patient‘s operative risk. 3- When an ostomy is a consideration, preoperative counseling with an entero-stomal therapist should be offered when available. 4- Digital rectal examination and rigid procto-sigmoidoscopy a. Digital rectal examination enables detection and assessment of the size and degree of fixation of mid and low rectal tumors. b. Rigid procto-sigmoidoscopy and digital rectal examination allow the most precise assessment of tumor location and the distance of the lesions from the anal verge. 5- Abdominal and pelvic CT scans often used to evaluate local extension of the tumor, regional LNs and liver metastases. However, its role in local staging is limited. 6- Trans-rectal ultrasound (TRUS) is the diagnostic modality of choice for preoperative local staging of mid and distal rectal cancers. It may be more accurate in defining T1, T2. 7- Pelvic MRI is more accurate in assessing T3 and T4 lesions, offers a multiplanar & larger field of view of the mesorectal fascia and more accurately predicts obtaining a tumor-free circumferential resection margin. 8- Preoperative routine chest X-ray or chest CT scanning: Rectal cancer is more likely than colon cancer to be associated with lung metastases without liver metastases. 9- CEA level is most useful when it is elevated pre-operatively and then normalizes after tumor resection. Subsequent increase suggests recurrence or metastases. CEA is not used as a screening test. 10- PET-CT scan is not routinely indicated

Treatment Considerations - Informed opinion of the multidisciplinary team (MDT) (radiologist, pathologist, medical oncologist and colorectal surgeon) should be obtained by the patient and family. The patient should have an access to treatment within 31 days of discussion with MDT. - Surgery is the mainstay of treatment for rectal cancer. The risk of recurrence depends on TNM stage. Early stage cancer can be treated by surgical resection alone. More advanced lesions require adjuvant therapy to increase the probability of cure. - The surgeon is a critical variable regarding morbidity, sphincter preservation as well as local recurrence.

355

Surgical Therapy

Resection Margin - The proximal resection margin is determined by blood supply considerations. For upper third rectal cancers both the rectum and meso-rectum are divided not <5 cm below the lower margin of the tumor. - A 2-cm distal margin is adequate for most low rectal cancers. In smaller cancers of the low rectum without adverse histologic features, a 1-cm distal margin is acceptable.

Operative Procedures - Operative procedures advocated according to rectal tumor locations are listed in Table 8.

Table 8: Operative procedures according to tumor location

Site of the Lesion Operation Vessels Excised Parts

Upper 1/3 rectum Tumor specific meso-rectal Inferior Sigmoid colon and excision (Figure 8) with mesenteric upper third rectum anastomosis between with distal safety descending colon & middle margin of 5 cm 1/3 rectum. Anastomosis above the peritoneal reflection = anterior resection, while below it = low anterior resection (LAR)

Middle and lower Total meso-rectal excision - Inferior Sigmoid colon & 1/3, Rectum down (TME) (Figure 9) with mesenteric total proctectomy to > 2 cm from the anastomosis between - Middle rectal with distal safety dentate line descending colon & anal margin at least 2 cm canal (colo-anal anastomoses = ultra-LAR )

< 2 cm from the TME with abdomino- - Inferior Sigmoid Colon, dentate line or perineal excision of the mesenteric total proctectomy, lesion infiltrating rectum (APER) with - Middle rectal anal canal, anal the anal sphincter permanent colostomy - Inferior rectal sphincters and or the pelvic floor (Figure 10). pelvic floor muscles muscles

356

Figure 8. Tumor Specific Meso-rectal Excision Figure 9. Tumor Meso-rectal Excision (TSME) (TME)

Level of proximal vascular ligation - Proximal lympho-vascular ligation at the origin of the superior rectal artery is adequate for most rectal cancers. There is no demonstrable survival advantage for a high ligation of the inferior mesenteric artery (IMA) at its origin. - In patients with LNs thought to be involved clinically, removal of all suspicious nodal disease up to the origin of IMA is recommended. - High ligation of the inferior mesenteric vessels may be helpful to provide additional mobility of the left colon, as

often is required for a low colorectal Figure 10. TME with APER anastomosis. Circumferential resection margin - For distal rectal cancers, TME is recommended. For upper rectal cancers, TSME is adequate. - The meso-rectum is the fatty tissue that encompasses the rectum. It contains lympho- vascular and neural elements. Surgical excision of the meso-rectum is accomplished by sharp dissection in the plane between the fascia propria of the rectum and the pre-sacral fascia. Radial clearance of meso-rectal tissue enables the en-bloc removal of the 1ry rectal cancer with any associated lymphatic, vascular, or perineural tumor deposits. TME is associated with the lowest local recurrence rates. - Pathologic assessment of rectal cancer specimens suggests that distal meso-rectal spread may occur up to 4 cm away from the 1ry tumor. Thus, a cancer in the distal rectum should be treated with a TME in most cases. Upper rectal cancers may be treated with a TSME with distal safety margin of 5 cm.

357

- Circumferential margin involvement in the presence of an intact mesorectal specimen is a strong predictor for local recurrence. A margin of ≤ 2 mm between tumor and the mesorectal fascia was considered positive and is associated with a higher local recurrence rate. Furthermore, patients who had a margin ≤1 mm had an increased risk of distant metastases En Bloc Resection of Adherent (T4) Tumors - Rectal cancers with adjacent organ involvement should be treated by en bloc resection. - Tumors may be adherent to adjacent organs by malignant invasion or inflammatory adhesions. Locally invasive rectal cancer (T4) is removed by an en-bloc resection to include any adherent tissues. If a tumor is transected at the site of local adherence, resection is deemed incomplete, because it is associated with a higher incidence of local recurrence rate. Inadvertent Perforation - It worsens oncologic outcome and should be documented. - It must be considered in postoperative adjuvant treatment decisions and outcome measurements.

Other Operative Considerations 1. Role of oophorectomy Bilateral oophorectomy is advised when one or both ovaries are grossly abnormal or involved with contiguous extension of the colon cancer. However, prophylactic oophorectomy is not recommended. 2. Laparoscopic-assisted resection of rectal cancer It is feasible but requires specific surgical expertise. 3. Emergency intervention: Hemorrhage, obstruction and bowel perforation are the most common indications for emergency intervention for rectal cancer. Appropriate treatment must be individualized with options, including resection with anastomosis and proximal diversion, or diversion alone followed by radiation. Self-expandable metallic stents can be used to relieve obstruction by a proximal rectal cancer.

Pre-operative Clinical Staging 1. T1-2,N0 2. T3, N0 OR T any, N1-2 3. T4 &/or locally unresectable primary 4. T any, N any, M1 resectable metastases 5. T any, N any, M1 unresectable metastases or medically in operable

Treatment of T1-2, N0, M0 Surgery A. Trans-anal Resection (conventional resection or trans-anal microsurgery) Full thickness excision is performed through the bowel wall into the peri-rectal fat. Indications include:

358

1. T1N0 2. Small (<3 cm) 3. Grade 1-2 4. within 8 cm of AV 5. < 30% of rectal circumference 6. Negative (> 3 mm) deep and mucosal margins 7. No Tumor fragmentation According to histopathology of the resected specimen, either Surveillance would be sufficient or further radical surgery might be indicated B. Trans-abdominal radical resection is indicated in unfavorable histological features: 1. LVI + PNI+ 2. Grade 3-4 3. Positive Margins 4. T2 C. Trans-abdominal radical resection: 1. Mid to upper rectum: LAR 5 cm below distal edge of tumor + colorectal anastomosis 2. Low rectal lesions a. Tumor within 2 cm above the dentate line: APER or ISRR if Ta and T2 b. Tumor above 2 cm from the dentate line: i. TME with colo-anal anastomosis (CAA) ii. Spare autonomic nerves Adjuvant Chemotherapy - If the pathology review after trans-abdominal resection revealed T3N0 or T1-3/N1-2 - Regimens over 6 months 1. 5-FU +/- LV or FOLFOX or Capecitabine ( 1 Cycle) 2. Concurrent 5-FU/RT or Capecitabine/RT 3. 5-FU +/-LV or FOLFOX or Capecitabine (2 Cycles)

Treatment of T3, N0 / T any, N1-2, M0 - Pre-operative (Neoadjuvant) therapy is a MUST: Continuous 5-FU/RT or bolus 5- FU/LV/RT or Capecitabine/RT - Operative treatment: Trans-abdominal approach only, performed 5-10 weeks after completion of neoadjuvant therapy - Postoperative adjuvant therapy: 5-FU +/- LV or FOLFOX or Capecitabine. All patients receiving preoperative (Neoadjuvant) therapy should receive postoperative adjuvant therapy for 6 months.

Treatment of T4 and/or Locally Unresectable Primary Resectable case = anticipated negative (R0) microscopic circumferential resection margin - Pre-operative (Neoadjuvant) therapy: Continuous 5-FU/RT or bolus 5-FU/LV/RT or Capecitabine/RT. - Operative treatment: More radical (multi-organ) approach, complete removal of tumor & involved viscera with R0 margins.

359

- Post-operative adjuvant therapy: 5-FU +/- LV or FOLFOX or Capecitabine. All patients receiving pre-operative (Neoadjuvant) therapy should receive postoperative adjuvant therapy for 6 months.

Treatment of T any, N any, M1 Resectable Metastases 1. Combination chemotherapy: a. FOLFOX/FOLFIRI/capeOX +/- Bevacizumab b. FOLFIRI/FOLFOX +/- Cetuximab (KRAS wild-type gene only) 2. Staged or synchronous resection of metastases and rectal lesion 3. Adjuvant Therapy: a. Continuous IV 5FU/ RT b. Bolus 5FU+leucovorin/ RT c. Capecitabine/RT

Treatment of T any, N any, M1 Unresectable Metastases or Medically Inoperable 1. Role of palliative surgery: Only if obstruction, perforation or bleeding 2. Symptomatic R/ a. Chemotherapy alone b. Combined modality: 5-FU/RT or Capecitabine / RT. c. Resection of involved rectal segment + CT d. Intra-luminal Stent + CT e. Diverting colostomy + CT

Pathological Evaluation of Rectal Cancer 1. Gross: Tumor and Specimen 2. Grade 3. LVI and PNI 4. T Stage 5. Number of regional LNs evaluated 6. N Stage: Number of +ve LNs 7. M Stage: Other organs – peritoneum – nonregional LNs. 8. Proximal, distal, and CRM 9. ―p‖ = pathologic staging 10. ―yp‖ = pathologic staging following neoadjuvant therapy

Surveillance 1. History and physical examination: Every 3-6 months for 2 years, then every 6 months for a total of 5 year. 2. CT scan of abdomen and pelvis Annually for 3 years 3. Colonoscopy At one year then as clinically indicated

361

Adjuvant Therapy

Neoadjuvant Therapy (pre-operative chemoradiation) should be offered to patients with stage II and III rectal cancers. - It is given as long course (45-50 Gy in 25-28 fractions) - 5-FU based chemotherapy should delivered concurrently with radiation - All patients receiving preoperative (Neoadjuvant) therapy should receive postoperative adjuvant therapy for 6 m

Rationale for neoadjuvant therapy 1. It avoids irradiation of neo-rectum & small bowel 2. It provides early systemic therapy i.e. less burden of disease and better drug perfusion (non-surgically manipulated - well vascularized oxygenated tissue). 3. It acts as a Radio-sensitizer: It thus enhances rates of down-staging and pathological complete response (ypCR)

Treatment according to site is summarized in Table 9.

Table 9: Treatment of rectal cancer according to "T" and 'tumor location".

Site At DL < 2cm DL > 2 cm DL Mid-Rectum Upper Rectum

Pedunculated Polypectomy and observe Polyp LVI-, G1-2

Sessile Polyp or Radical Surgery

LVI+, G3-4

T1, LVI-, G1-2 SCRT +APR APR/ Local Local Excision/ TSME CRT+ISRR Excision TME

T1, LVI+, G3-4, SCRT+ APR APR/ TME TME TSME or T2 CRT+ISRR

Good T3 CRT + APR CRT+APR TME TME TSME

Bad T3 CRT+ APR CRT+APR CRT+TME CRT+TME CRT+TSME

T4a CRT+APR CRT+APR CRT+TME CRT+TME CRT+TSME

T4b CRT+APR CRT+APR CRT+TME CRT+TME CRT+TSME

361

NEOPLASTIC DISEASE OF THE ANAL CANAL

Pathology

- These tumors are classified into squamous (SCC), basaloid, or muco-epidermoid types. It is almost agreed that the dentate line separates the anal margin (the area below) from the anal canal (the area above up to the ano-rectal ring). - SCC (30%) (keratinizing or not) tend to arise from skin of anal margin. On the other hand basaloid cell tumor (50%) (cloacogenic cell tumor) arise from the anal canal (transitional zone above the dentate line). - The muco-epidermoid type is rare, and it produces both keratin and mucus.

Spread

- Direct spread, usually towards the rectum in an upward direction in the submucous layer. - Lymphatics can spread to both inguinal & towards lymphatics of the rectum - Hematogenous: Very rarely it can give blood borne metastasis

Clinical Picture

- The presence of the ulcer, with all its consequent symptoms of pain, discharge, itching etc is complained of. - Inspection and PR examination will reveal the diagnosis.

Management

- It usually start with biopsy of the lesion, which when proved to be malignant is treated recently with NEGRO regimen; a pre-operative course of chemo-radiotherapy, which can be followed by either, local excision, no surgery or abdomino-perineal resection according to the response to the initial treatment. - Inguinal LNs are only dissected as a block excision 4-6 weeks after anal lesion treatment if they proved to be involved

362

CHAPTER 9

Colo-rectal Emergencies

VOLVULUS

- The word volvulus derives from the Latin ‗to twist‘. A volvulus is the twisting of a loop of intestine around its mesenteric attachment, resulting in a closed loop bowel obstruction. The affected bowel can become ischemic due to a compromised blood supply, rapidly leading to bowel necrosis and perforation. - Most volvuli occur at the sigmoid colon and are a common cause of large bowel obstruction in many countries (after malignancy and diverticular disease). They can also occur at the stomach, cecum, small intestine, and transverse colon, but are much rarer. - The long mesentery of the sigmoid colon (which increases with age) means that this segment bowel is more prone to twisting on its mesenteric base (Figure 1) to form a volvulus than any other region.

Figure 1: A volvulus occurs when bowel twists on its own mesentery

363

Etiology

A long narrow based mesentery predisposes to volvulus. 1. Congenital anomaly of long and mobile mesentery (neonatal midget volvulus). 2. Acquired: - Mesenteric adhesions producing narrowing of the base, or causing fixation of the loop at its top - Overloading of one limb of the gut as in sigmoid colon

Pathogenesis of Volvulus Sigmoid

- Anti-clockwise torsion of 180ₒ is physiological and can be reverted spontaneously. If the posterior loop gradually fills with gas and stools, and become heavier, it then changes its position and becomes anterior, thereby completing the 360ₒ anti-clockwise torsion as shown in Figure 2.

Figure 2: Pathogenesis of volvulus of the sigmoid

Risk Factors

The risk factors for developing a volvulus include: - Increasing age - Neuropsychiatric disorders - Chronic constipation or laxative use - Male gender - Previous abdominal operations

Clinical Features

- Patients with a volvulus will present with the clinical features of bowel obstruction. - As the sigmoid colon is located distally in the gastro-intestinal tract (GIT), vomiting is usually a late sign, whilst the colicky pain, abdominal distension, and absolute constipation occur earlier in the clinical course.

364

- Particularly noteworthy in cases of volvulus, compared to other causes of bowel obstruction, is the rapidity of onset (over a few hours) and degree of abdominal distension. - On physical examination, the abdomen is often very tympanic to percussion. Ensure to examine for signs of perforation or peritonism, as this indicates ischemia or perforation and is a surgical emergency.

Differential Diagnosis (DD)

The main DD to consider are the alternative causes of bowel obstruction, as well as severe constipation, pseudo-obstruction, and severe sigmoid diverticular disease.

Investigations

All patients with clinical features of bowel obstruction should be investigated accordingly. - Routine blood tests should be taken, including electrolytes, calcium, and thyroid function tests (TFTs) to exclude any potential pseudo-obstruction. - CT scan abdomen-pelvis with contrast, as this is much more sensitive and specific for bowel obstruction and identifies the site and cause. CT imaging classically will demonstrate a severely dilated sigmoid colon with a ‗whirl sign‘, from the twisting mesentery around its base (Figure 3).

Figure 3: CT scan reveals characteristic mesenteric whirl sign.

- Abdominal radiograph (plain X-ray) will classically show (in around 60-75% cases) a ―coffee-bean sign‖ arising from the left iliac fossa; if the ileocecal valve is incompetent, the abdominal X-ray will also show signs of small bowel dilatation (Figure 4).

365

Figure 4. Plain X-ray abdomen: characteristic coffee bean appearance, with the convexity of the loop lying in the right upper quadrant (opposite the site of obstruction).

- Gastrografin enema shows a narrowing at the site of the volvulus and a pathognomonic ‗bird's beak‘ sign (Figure 5)

Figure 5. Gastrografin enema shows ‗bird's beak‘ sign.

Management

All patients admitted with suspected sigmoid volvulus should be managed initially as per any patient with bowel obstruction. They should be examined for any signs of ischemia and given fluid resuscitation.

366

Conservative Treatment - Most patients with sigmoid volvulus are treated conservatively initially with decompression by sigmoidoscope and insertion of a rectal tube (Figure 6).

Figure 6. sigmoidoscope (left) and rectal tube (right)

- In sigmoidoscope decompression, the patient is placed in the left lateral position and a lubricated sigmoidoscope gently guided into the rectum. It is maneuvered to locate the twisted bowel and once the sigmoidoscope is in the correct position, there will be a rush of air and liquid feces as the obstruction is relieved. - A flatus tube is often left in-situ for a period (up to 24 hours) after initial decompression to allow for the continued passage of contents and aid recovery of the affected area. In 25-50% of patients, this approach is unsuccessful and a formal decompression with a flexible sigmoidoscope is required.

Surgical Management - The indications for surgery (which is usually a laparotomy for a Hartmann‘s procedure) are: 1. Colonic ischemia or perforation 2. Repeated failed attempts at decompression 3. Necrotic bowel noted at endoscopy. - The decision on which operation to perform will depend on the patient‘s nutritional status, adequacy of blood supply, hemodynamic stability, and the presence of any perforation or peritonitis. - Patients with recurrent volvulus who are otherwise healthy may choose to have an elective procedure (most commonly sigmoidectomy with primary anastomosis) to prevent further recurrence.

Complications

- The main immediate complication of a sigmoid volvulus is bowel ischemia and perforation. Longer term complications are mainly the risk of recurrence (occurring in up to 90% of patients) and complications from a stoma if placed.

367

- Overall mortality from surgery in these patients is high as they are generally old, frail and with multiple co-morbidities. There is also often a delay in getting these patients to theatre which contributes to their physiological compromise and high mortality.

Cecal Volvulus

- The second most common site for a volvulus to occur is at the cecum (Figure 7), accounting for around 25-40% of all colonic volvulus. There is a bimodal age of onset, in the 10–29-year group and then again in the 60-79-year group. - Those in the younger group may have intestinal malformation or excessive exercise as the predisposing cause, whilst in older patients it is more associated with chronic constipation, distal obstruction, or dementia.

Figure 7. Cecal volvulus is second most common site of volvulus

- Diagnosis is once again made on CT imaging, which shows a distended cecum, a mesenteric ―swirl‖ and small bowel obstruction. - The management of a cecal volvulus is always laparotomy and ileo-cecal resection (assuming the patient is well enough to tolerate the surgical procedure)).

ISCHEMIC COLITIS

Ischemic colitis (colonic ischemia): hypoperfusion of the large bowel, which is mostly transient and self-limiting (non-gangrenous form), but can also lead to severe acute ischemia with bowel infarction (gangrenous form)

Types

1. Acute mesenteric ischemia: acute inadequate blood flow to the intestine (arterial or venous) that can result in bowel infarction. 2. Chronic mesenteric ischemia: constant or episodic hypoperfusion of the small intestine, usually due to atherosclerosis.

368

Epidemiology

- Ischemic colitis is the most common form of intestinal ischemia. - Mainly occurs in adults > 60 years. - Mild, non-gangrenous form occurs in 80–85%.

Etiology

- It is usually caused by transient hypoperfusion - Thrombo-embolism - Hypotension, hypovolemia (e.g., sepsis, dehydration, hemorrhage) - Cardiovascular surgery (especially aortic repairs or cardiac bypass) - Vaso-constrictive drugs - Thrombophilia (e.g., anti-phospholipid syndrome) - Colonic obstruction from, e.g., tumors, adhesions

Pathophysiology

- Intestinal blood flow of the superior mesenteric artery (SMA) and/or inferior mesenteric artery (IMA) is suddenly compromised → intestinal hypoxia → intestinal wall damage → mucosal inflammation and possibly bleeding → may progress to infarction and necrosis (gangrenous type) → disruption of mucosal barrier and perforation → release of bacteria, toxins, vasoactive substances → life-threatening sepsis. - The splenic flexure and the recto-sigmoid junction are at high risk for colonic ischemia because they are ‗watershed areas‘.

Clinical Features

Typically presents with 3 clinical stages. 1. Hyperactive phase: - Sudden onset of crampy abdominal pain (usually left lower quadrant) - Bloody, loose stools - Most patients recover and do not progress beyond this phase. 2. Paralytic phase: - Pain more diffuse - Bowel sounds become absent. - Bloating - Bloody stools. 3. Shock phase: - Acute abdomen with abdominal guarding and rebound tenderness. - Signs of septic shock.

369

Diagnosis

Laboratory Studies 1. No specific laboratory findings in mild ischemic colitis 2. In severe ischemic colitis: - ↑ Lactate, ↑ LDH, ↑ creatine kinase - Leukocytosis - Metabolic acidosis

Imaging - Plain abdominal radiograph: insensitive, unspecific (air-filled, distended bowel), but helps exclude other disorders (Figure 8)

Figure 8. Plain X-ray abdomen air-filled, and distended left colon

- Selective angiography can demonstrate blood flow in celiac trunk and mesenteric arteries (Figure 9)

Figure 9. Mesenteric Angiogram. Note complete lack of contrast in mesenteric vessels in AP view (left). The occluded origins of the celiac axis and superior mesenteric artery are demonstrated in the lateral view (right).

371

- CT scan with oral contrast may show increase in wall thickness, pneumatosis intestinalis, and edematous thickening of the mucosa causes indentations in the large bowel wall (Thumbprint sign). (Figures 10 and 11)

Figure 10. CT scan: increase wall thickening, Figure 11. Thumbprint sign pneumatosis intestinalis

Colonoscopy - A pale mucous membrane and a loss of vascular pattern can be seen.

Treatment

Mild to Medium-Severe Forms - Supportive care (IV fluids, bowel rest, nasogastric tube in case of an ileus) - Anti-platelet drugs - Reduce risk of atherosclerosis (anti-coagulation therapy)

Severe Forms (Signs of Peritonitis, Sepsis): Surgical intervention - Pre-operative preparation - Assure adequate resuscitation. - Monitoring - Foley Catheter - Urgent exploration 1. Surgical embolectomy. 2. Bowel resection.

371

INJURY (TRAUMA)

Etiology

Penetrating Trauma - This is the most common type of trauma seen and is usually due to: 1. High-velocity missiles (75%) - stab wounds (20%). 2. Iatrogenic injuries due to uterine perforation during curettage of the uterus, use of endoscopies whether diagnostic or during polypectomies, or other rectal instrumentations and/or during surgical operations for urological or gynecological operations. 3. Rectal impalement injuries that result when a patient falls on a penetrating object or using foreign bodies for sexual satisfaction.

Blunt Trauma - The commonest cause is motor vehicle accidents followed by falls and crush injuries. - This is rare due to the protected situation of the anus and rectum. - It is usually associated with fracture pelvis.

How to Suspect Large Bowel Injuries? How to Investigate?

- Intra-abdominal injuries are diagnosed as any abdominal trauma by the presence of manifestations of peritoneal irritation, free intra-peritoneal fluid, or air. - Pain x-ray may show air under diaphragm (Figure12). - CT scan indicates free intra-peritoneal fluids and pneumo-peritoneum (Figure 13).

Figure 12. Plain X-ray abdomen Figure 13. CT scan of the abdomen showing showing air under diaphragm (arrows) pneumo-peritoneum and free fluid

- Diagnostic peritoneal lavage (DPL): It will not evaluate the retro-peritoneum. Bacteria / vegetable matter suggestive - The focused assessment with sonography in trauma (FAST) - Rigid procto-sigmoidoscopy

372

- However, rectal, anal canal and perineal trauma are diagnosed by proper inspection and PR examination. Presence of bleeding PR is a very important sign. The presence of different types of urethral injuries or fracture pelvis should stimulate the surgeon to properly examine and even sigmoidoscope (the preferred method of investigation) the rectum and the pelvic colon. - If enema is to be used, water-soluble contrast (gastrographin) is a must and barium should never be used. Again, a CT scan abdomen and pelvis with double or at least IV- contrast is indispensable for proper diagnosis.

Grading

According to AAST (American Association for Surgery of Trauma Organ Injury Scale) Colon Injury - Grade I – Contusion or hematoma; partial thickness laceration - Grade II – Full-thickness laceration <50 percent of circumference - Grade III – Full-thickness laceration ≥50 percent of circumference - Grade IV – Transection - Grade V – Transection with tissue loss; DE vascularized segment Rectum and Recto-Sigmoid Injury - Grade I – Contusion or hematoma; partial thickness laceration - Grade II – Full-thickness laceration <50 percent of circumference - Grade III – Full-thickness laceration ≥50 percent of circumference - Grade IV – Full-thickness laceration with perineal extension - Grade V – DE vascularized segment

Treatment Options

Direct laceration sutures closure (Figure14) - Criteria and contraindications of primary closure are summarized in Table1. - In practice, direct laceration sutures closure this is only valid in situations where the colon and/or rectum are injured in a patient whose large bowel is prepared as in operative or endoscopic iatrogenic injuries.

Figure 14. Sutured closure of a laceration in the colon

373

Table 1: Direct laceration sutures closure.

Criteria for Primary Closure Contraindications of Primary Closure

No hemodynamic shock. Patient has been in shock (systolic < 80 mm Hg) Interference in a time < 8 hours from wound The interval between injuries and closure is > 8 injury. hours. Small tear < 2 cm large tear > 2 cm Minor spillage reaching to a distance < 5 cm Massive contamination with loaded colon. with unloaded colon. No other large bowel injuries Injuries at two different locations of large bowel No other organ injuries More than one organ injured. Absent of prosthetic material or the necessity of Presence of prosthetic material or the necessity of its insertion its insertion

Resection + Ileo-transverse Anastomosis - Resection of the injured area with direct anastomosis of the small bowel to the transverse colon (Figure 15) is only valid in right-sided lesions where direct closure is contraindicated.

Figure 15. Resection with ileo- transverse anastomosis.

Double-Barrel Colostomy - Double-barrel colostomy at the site of injury (instead of exteriorization of the repaired injured colon) is done in the transverse or sigmoid colon (mobile area with long mesentery) (Figure16).

Figure 16. In mobile segments of the colon, the mesentery allows the injured segment to be exteriorized as a double barrel colostomy.

374

Resection with End-Colostomy and Mucosal Fistula or Hartmann Pouch (Figure 17) - This is done if the injury is at a site where mobility of the distal limb is limited while mobility of the proximal limb is free. - One condition is a must. This is to ensure evacuation and emptiness of the distal limb of the large bowel. This is especially applicable in the following situations: 1. Injury near the splenic flexure of the colon 2. Injury at the distal region of the sigmoid colon.

Figure 17. An injury of the distal sigmoid is managed by proximal colostomy & Hartmann closure of the distal rectum.

Suture Closure with Proximal Diversion (Figure18) - It means 1ry closure of the laceration (even if some conditions prohibit that closure) with protection of the 1ry repair with proximal fecal diversion, by ileostomy or colostomy with the following conditions: 1. Diversion should be complete with no chance of any fecal matter passage to the distal limb. 2. Assurance of removal of all fecal residue in the distal limb - This is suitable in (1) descending colon injuries, (2) distal sigmoid lesions and (3) proximal intra-peritoneal rectal injuries.

Figure 18. Suture closure with protective colostomy

375

Principles of Management of Rectal Injuries

1. The injury in the rectum is detected by a through endoscopic examination preferably done by a rigid sigmoidoscope in the left lateral or lithotomy position to determine the injury's location whether in the intra-peritoneal segment or extra-peritoneal one. The extra-peritoneal space for the rectum is divided into retro-peritoneal high up in the abdomen & sub-peritoneal low in the pre-sacral space. Also using the scope, removal of the retained feces with irrigation is done. 2. Direct per rectal repair is done in low injuries (sub-peritoneal spaces) with possible drainage of the pre-sacral space through an incision situated midway between the coccyx and the anus. This is specially indicated in posterior injuries. 3. Direct repair through abdominal exploration is done in injuries of the intra-peritoneal segment or in the retro-peritoneal segment, with possible use of suction drainage of the pre-sacral space if the injury is posteriorly located, and drainage of the Douglas pouch if it is anteriorly located as is usually the case. 4. A proximal complete fecal diversion is a must in all situations. 5. Removal of all retained feces should be ensured by irrigation through either the distal limb of the colostomy, or better still through the rectum.

Principles of Management of Perineal and Anal Canal Injuries

1. In perineal and anal canal injuries, no attempt should be done for 1ry sphincteric or tissue repair. Only debridement and hemostasis are done. 2. It is however, mandatory to divert the fecal stream totally from the wound in the perineum if the lesion is even moderately extensive and specially if involving the sphincteric complex of the anal canal. 3. After healing of the wound and before any colostomy closure, sphincteric repair can be done under cover of the diversion usually with satisfactory results. Treatment options of sphincter injury repair are summarized in Table 2.

Table 2: Treatment options of sphincter injury repair according to presentation

Presentation Treatment

No associated injuries – no delay in treatment Primary repair Significant associated injuries - minor sphincter Delayed repair injury Significant associated injury - major sphincter Fecal diversion followed by delayed injury repair No significant associated injuries - Delayed Fecal diversion followed by delayed presentation (inflammation and edema) repair

376

BLEEDING

Definitions

- Bleeding per rectum (hematochezia) is the passage of altered maroon-colored or reddish- dark, brown-colored offensive fluidly stool motions with possible clots. The nature of blood is evident to the patient. - Fresh bleeding per rectum is the passage of fresh blood during passing a stool motion, and it takes several forms as splashing, dripping, streaking of stools or as terminal drops. Sometimes it stains the underwear after defecation. It may be associated with mucus &/or pus.

Massive (Serious) Lower GIT Bleeding

- Passage of a large volume of red or maroon blood through the rectum - Hemodynamic instability and shock (systolic blood pressure of < 90 mmHg) - Initial decrease in hematocrit level of 6 g/dL or less - Transfusion of at least 2 units of packed red blood cells (RBCs) - Bleeding that continues for 3 days - Significant rebleeding in 1 week

Causes of Lower GIT Bleeding:

Causes of lower GIT bleeding by source are summarized in Table 3, whereas common causes of lower GIT bleeding by age are listed in Table 4,

Table 3: Causes of Gastrointestinal Bleeding by Source

Etiology Upper GIT Small Bowel Sources Colonic Sources

Common - Esophageal varices - Meckel‘s - Angiodysplasia causes - Ulcers (DU & GU) diverticulum - Diverticula - Gastritis - IBD and vasculitis - Neoplasms - Gastric tumors - Lymphoma - IBD - Polyps - Hemorrhoids

Less - Aorto-enteric fistula - Mesenteric ischemia - Solitary rectal ulcer common - Deulafoy‘s lesion - Radiation enteritis syndrome causes - GERD - Intussusceptions - Radiation proctitis - Mallory Weiss - Endometriosis syndrome - Rectal varices

377

Table 4: Common Causes of Lower Gastrointestinal Bleeding by Age

Adolescents and young Adults to 60 years of age Adults > 60 years adults - Meckel‘s diverticulum - Diverticula - Angiodysplasia - IBD - IBD - Diverticula - Polyps - Neoplasms - Neoplasms

IBD: Inflammatory bowel disease

Differential Diagnosis

Diagnosis of anal conditions, which present with the following:

Pain and Bleeding - Fissures

Pain, Lump and Bleeding - Prolapsed hemorrhoids - Carcinoma of the anal canal - Prolapsed rectal polyp or carcinoma. - Prolapsed rectum

Diagnosis of Conditions Presenting with Rectal Bleeding but No Pain - Blood mixed with stool  colon carcinoma. - Blood streak on stool anal fissure & rectal carcinoma - Blood after defecation hemorrhoids - Blood and mucus colitis - Blood alone diverticular disease - Melena peptic ulcer

Investigations

Laboratory Investigations - Complete blood count (CBC) count - Serum electrolytes - Blood urea nitrogen, creatinine - Blood grouping and cross matching - Coagulation profile including: Partial thromboplastin time (PTT), platelet count, bleeding time

378

Colonoscopy

Colonoscopy has diagnostic and therapeutic capabilities. The patient must be in stable condition. - It can be used even with ongoing massive bleeding - Timing ideally 6-24 hours post-presentation - Increased risk of bowel perforation (2% risk of perforation) - Should not be attempted with ischemia or severe mucosal inflammation. - Endoscopic coagulation treatment of choice for bleeding AVMs (Arterio-venous malformations) - Diverticular bleeding cannot usually be endoscopically coagulated (technically challenging)

Nuclear Scintigraphy

It can detect bleeding at a rate of ≥0.05-0.1mL/minute.

Technetium 99m (99mTc) Sulfur-Colloid - Requires no preparation → inject immediately - Short half-life - Enhances liver and spleen → can obscure bleeding sites - Able to detect slower rates of bleeding

99mTc-labeled RBCs – Preferred method (Figure 19)

Figure 19. Active bleeding from ascending colon

379

– Good for intermittent bleeds – Able to visualize bleeds near liver/spleen – 73-98% sensitive – False localization rate ~20%1 (3-59%) – Blood does not remain stationary within bowel lumen: Reflux into small bowel - rapid transit through bowel with pooling in other areas – Institution dependent: Not available in all hospitals - Variability in quality

Angiography - Selective mesenteric angiography (Figure 20) o Femoral artery punctured o Evaluates superior mesenteric (SMA), then inferior mesenteric artery (IMA), then the celiac axis o Positive test if extravasation of contrast into bowel lumen - Can detect hemorrhage rates ≥0.5mL/min - Diagnostic and potentially therapeutic: Adrenaline infusion - embolization - Sensitivity 40-86% - Complication rate ~ 2%

Figure 20. Normal selective inferior mesenteric artery (IMA) angiogram

CT Angiography - New and evolving technology - Requires modern CT scanner - Fast (< 15 minutes) - Non-invasive - Identifies large and small bowel hemorrhage - Sensitivity and specificity 72-80%

381

Treatment

An algorithm for management of lower GIT bleeding is depicted in Figure 21.

Figure 21: Algorithm for management of lower GIT bleeding

- Resuscitation with crystalloid solutions, preparing for blood transfusion & diagnostic nasogastric (NG) intubations are important steps. The presence of bile in the aspirate is the only sure sign that there is no active upper GIT source of bleeding. Upper endoscopy can diagnose more accurately the upper GIT source. - It should be recognized that about 85% of bleeding from lower GI sources stops spontaneously; however, the presence of large amounts of blood in the colon can give the impression of a continuous bleeding process. That is why in monitoring bleeding, one should depend more on the hemodynamic figures rather than frequent passage of bloody motions.

381

- PR examination, anoscopy and possibly rigid sigmoidoscopy are imperative to rule out palpable neoplasms and other anal and rectal conditions. It is important, even if detection of a lesion is not possible due to bad preparation, to ascertain a healthy lower 15-20 cm for a possible use of subtotal colectomy. Treatment of a simple visible cause like piles can be done at the same setting by injection or ligation. - Colonoscopy offers the chance of proper diagnosis and sometimes-non-surgical stoppage of bleeding. Mechanical preparation can be done. It should be done as soon as the patient is stabilized and better under general anesthesia. Diverticulosis, IBD, polyps & neoplasms are confidently diagnosed; however, AVMs are sometimes difficult to diagnose, but if seen can be treated with injection of diluted adrenaline or photocoagulated by laser therapy. Colonoscopy can also diagnose the source of bleeding as small bowel if the cecum is reached & more fresh blood is coming out of the ileo-cecal valve. This will indicate small bowel site (refer to the Table above for causes). - Arteriography (by selective catheterization of all mesenteric vessels) and 99mTc-labeled RBC scanning are only of use if active bleeding is present with a high rate (2ml/minute or higher), which is not usually the case on admission of the patient. Their use is more or less of academic interest only. Selective vasopressin infusion can help, as well as peripherally used vasopressin.

382

CHAPTER 10 Vermiform Appendix

Epidemiology

- Acute appendicitis affects 6-7 % of the population. - Peak incidence in adolescents and young adults, with a slight male predominance in this age group. - Infants, elderly, pregnant women and immuno-compromised patients tend to have atypical presentations and have higher morbidity and mortality

Anatomical Positions (Figure 1)

- Retro-cecal: 64% - Pelvic position: 32% - Other positions: 5%

Figure 1: Different anatomical positions of the appendix. The- retro-cecal is the commonest.

Position of the appendix also changes according to the position of the cecum, which can be sub-hepatic, or pushed up by pregnancy

383

Figure 2: Anatomy of the pelvic appendix. The second most common site.

Pathophysiology

- More than two-thirds of the cases are due to obstruction of the lumen by 1. Fecolith 2. Lymphoid tissue in response to viral infection 3. Kink or adhesion 4. Parasite or tumor - This will lead to a period of appendicular colic, with accumulation of secretion inside the lumen that will be infected causing inflammatory appendicitis, which easily perforates - Less than one-third of the cases is due to non-obstructive acute appendicitis. Inflammation is usually catarrhal and stays like that with little liability to perforate

Fate of the Inflammatory Process

1. Resolution with or without future recurrent attacks 2. Gangrene and perforation (either at the tip or at the site of the fecolith impaction) 3. Walling-off by omentum, and small bowel loops forming a phlegmon (inflammatory mass), also known as appendicular mass 4. Generalized peritonitis in extremes of age and in patients with bad immunological defenses 5. Localized perforation within the appendicular mass leading to appendicular abscess, which can later perforate giving rise to generalized peritonitis 6. Portal pyemia with septicemia is rare

384

Clinical Presentation

Symptoms - Pain that shifts is the presenting symptom. - Visceral type of pain in the peri-umbilical region shifts to somatic type of pain localized to the right iliac fossa (RIF). - Anorexia and nausea are constant, while vomiting is early and preceded by pain. - Constipation is usual; however, diarrhea can be the start of the condition (usually viral), then acute appendicitis starts due to hypertrophy of the lymphoid follicles

Physical Signs - Temperature and pulse are only slightly raised early in the disease, higher elevations indicate complications. - Localized pain that increases with coughing. - Localized tenderness and rebound usually over the McBurney‘s point (Figure 3) with guarding. - Rigidity and restricted respiratory abdominal movements are present with local peritonitis. - PR or PV examination can elicit tenderness in pelvic positions.

Figure 3: McBurney‘s point at the junction of the lateral third and medial two-thirds of the line between the umbilicus and the anterior superior iliac spine.

Special Signs - Rovsing sign: contra-lateral pressure cause right-sided pain. - Psoas sign: extension of the hip cause pain. - Obturator sign: internal rotation of the hip cause pain.

Atypical Clinical Features - Deep pelvic appendix causes tenesmus + dysuria and PR tenderness. - Sub-hepatic cecum gives manifestations that simulate cholecystitis. - Long retro-cecal appendix, gives pain simulating right colonic pain with minimal abdominal signs. - A retro-ileal appendix can present with diarrhea.

385

Special Age Groups

Children - Approximately, 80% of cases lead to early generalized perforation due to: 1. Lack of well-developed omentum 2. Lack of precise diagnosis due to difficulty in examining that age group 3. Mis-interpretation of the case as gastroenteritis due to frequent vomiting Elderly - Acute appendicitis in the elderly also gives rise to early generalized perforations due to: 1. Weak immune system 2. Signs are not prominent although the case can be perforated Child-Bearing Age / Pregnancy - In such cases, early generalized perforation may occur due to 1. The usual site is different (higher) 2. Mis-diagnosis as pyelitis 3. Weak omental action 4. Plus the seriousness of the 50% abortion or the 30% premature labor

Differential Diagnosis

Thoracic Problems - Right-sided pneumonia or pleurisy mimic the condition, yet the marked physical signs of the chest clinches the diagnosis

Upper Abdominal Problems - Perforated peptic ulcer specially the leaking one; however, a long history of upper abdominal troubles and the free air under the diaphragm may help in diagnosis. - Acute cholecystitis, yet the prominent vomiting, and the ultrasound (US) confirmation of the possibly palpable gall bladder (GB) usually differentiate between both conditions. - Acute pancreatitis especially the edematous type, yet the type of pain and lack of localized tenderness and other special signs may clinch the diagnosis, which is confirmed by amylase.

Lower Abdominal Problems - Non-specific lymphadenitis: it usually affects children, with higher fever, and may follow a sore throat, yet differentiation is difficult - Meckel‘s diverticulum: it is almost impossible to diagnose except after exploration - Crohn‘s disease is also impossible to diagnose before operation in the patient firstly presenting to the surgeon - Cancer cecum: this is the usual differential diagnosis of an appendicular mass rather than appendicitis.

Pelvic Problems (Ultrasound is Usually Conclusive) - Rupture Graffian follicle (mid-cyclic pain) is very common differential diagnosis - Disturbed ectopic pregnancy: pallor and history of amenorrhea should elicit diagnosis

386

- Complicated ovarian cyst (infected or twisted), where the presence of a mass is the clue to diagnosis, this is not usually palpable due to the severe overlying rigidity - Pelvic inflammatory disease (PID): it is characterized by bilateral tenderness, in addition to the vaginal discharge and higher degrees of fever. - Red degeneration of fibroid: this is usually the differential diagnosis of appendicitis in pregnant women.

Urologic Problems - Ureteric colic: this is typical visceral pain, with prominent vomiting, and lack of rigidity and rebound, with no high temperatures, and may be hematuria - Right pyelonephritis: the temperature is very high with rigors, and pain is more localized to the loin except if it is ectopic kidney, and dysuria is very common

Neurologic Problems - Herpes Zoster gives pain similar to appendicitis, usually the age is advanced, and the eruption that occurs after 36 to 48 hours clinches the diagnosis. No fever, no vomiting, and no rigidity or rebound tenderness

Investigations

Laboratory Investigations - Complete blood picture: showing leukocytosis with shift to the left - Urine analysis: for RBCs and pus cells (hematuria excludes non-ureteric problems) - Pregnancy test: in females in childbearing age

Imaging Studies Ultrasound (US) examination - It is operator-dependent, yet it is very useful in children and in females in child-bearing age - Signs if significance are: 1. Non-compressible appendix 2. Appendix > 6 mm in diameter 3. Detection of a fecolith 4. Presence of peri-appendicular fluid collection 5. Probe tenderness 6. Exclusion of tubo-ovarian pathology CT with or without IV contrast - It approaches a 97% accuracy rate, and is the main mode of investigating acute abdominal conditions - Signs of significance are: 1. Enlarged distended appendix > 6 mm 2. Appendiceal wall thickening > 2 mm 3. Peri-appendiceal fat stranding 4. Focal cecal apical thickening 5. Appendicolith (seen in 20-40% of patients with appendicitis)

387

6. Target structure (concentric thickening of the inflamed appendiceal wall) 7. Extra-luminal air 8. Collections of fluid in the RIF or pelvis Magnetic resonance imaging (MRI) - It is only used if ultrasound was not conclusive in a pregnant woman that cannot be exposed to irradiation of the CT scan and needs definitive diagnosis.

Types of Patients

- Patients with acute appendicitis are categorized as high-risk and non-high-risk patients. - High-risk patients: 1. Children due to high risk of perforation 2. Elderly patients due to high risk of perforation and the existence of co- morbidity 3. Women in the child-bearing period due to the presence of tubo-ovarian pathology that mimics appendicitis, and the high rate of complications in pregnancy 4. Immuno-compromised patients due to high mortality and high morbidity

Perforation of Appendix in the Pediatric Age - In children below two years, perforation approaches 100% - From three to five years, perforation is around 70% - From six to ten years, perforation is around 40%

Risk in Elderly Patients - Elderly patients have higher morbidity and mortality rates 1. Less typical presentation 2. Cancer may be a possibility as an underlying cause. 3. Perforation of 50% and mortality of 20% has been reported

Pregnancy with Acute Appendicitis - 1: 2000 pregnancies. - More common in the first two trimesters of pregnancy - The appendix is pushed superiorly and laterally - WBC > 15,000/mm3 - Premature labor: 10-15% with surgery - Perforated appendix leads to fetal death in 20% of cases - Rapid diagnosis and treatment is advised.

In Immuno-Compromised Patients - Be aware of CMV or Kaposi sarcoma as the underlying cause - WBC may not rise

388

Treatment

In Non-Complicated Acute Appendicitis - Treatment is by appendectomy - No delay is allowed specially in extremes of age, pregnancy and diabetics.

In Appendicular Mass - It is recommended to start conservative treatment (Oschsner–Sherren regimen) although some surgeons use midline incisions to dismantle adhesions and remove the appendix - Oschsner-Sherren regimen 1. Bed rest 2. Nil by mouth for few days then liquids for another few days 3. IV fluids and antibiotics (double or triple regimen attack) 4. Follow up as regards pain, mass size, temperature and pulse - Further Treatment: Interval appendectomy is done after 3 months if the mass resolved, or otherwise one is dealing with appendicular abscess.

In Appendicular Abscess - Ultrasound, or more accurately, CT scan confirms the presence of pus inside the mass, and can be used as guide to insert a pigtail catheter for drainage in case there is a single loculus. - Surgical extra-peritoneal drainage is the classical treatment; all loculi are opened and an overflow or suction drainage is used - No attempt is made to remove the appendix except if it is available without risk to the inflamed tissues. Interval appendectomy is otherwise done after 3 months to avoid recurrent inflammation like in appendicular mass.

In Generalized Peritonitis - Urgent surgery with appendectomy and peritoneal toilet and drainage is the sole line of treatment

Different Situations in Management

HIGH-RISK PATIENTS

389

NON-HIGH-RISK PATIENTS

POTENTIAL COMPLICATED APPENDICITIS IN ANY PATIENT

- Delayed presentation, high fever, palpable mass, toxic look and signs (SIR) - Prompt evaluation with US and CT is mandatory to evaluate the condition whether ―pus‖ is present or it is only a ―phlegmon‖. - Surgery through a midline exploration is a must if pus is present, while Oschsner technique is advised if no pus is present with interval appendectomy done after 3 months

Complications of Appendectomy

Mortality - Death following appendectomy done for perforated appendix is usually attributable to sepsis. Delay by the patient in seeking medical attention or delay on the part of the physician in instituting appropriate treatment is an important factor in allowing perforation to occur and results in increased morbidity and mortality. Perforation can often be diagnosed pre-operatively. It should be suspected when the duration of symptoms exceeds 48 hours. A temperature higher than 38℃ and a WBC count of >15,000 are both uncommon in non- perforated appendicitis. A history of diffuse abdominal pain following symptoms confined to the right lower quadrant accompanied by signs of diffuse peritonitis on physical examination indicates that free perforation has occurred. If perforation has been walled off into an appendiceal abscess, a tender mass can often be palpated in the right lower quadrant. - Diagnosis should be suspected in a patient with a history suggestive of acute appendicitis of longer than 48 hours duration, who also has fever, leucocytosis with or without a tender mass in the right lower quadrant of the abdomen. - General awareness of symptoms of appendicitis and a more aggressive surgical approach should reduce morbidity and mortality by decreasing the incidence of perforation. - Age is also an important contributing factor to mortality, as death following appendectomy is more common in the very young and the elderly, with a mortality rate of 15% in patients above 70 years. This is most probably due to the presence of multiple associated medical problems, delay in seeking medical advice, and the surgeon‘s reluctance to operate until diagnosis is 100% certain.

391

The Most Important Complications are 1. Wound infection 2. Pelvic and other intra-abdominal abscesses 3. Appendiceal stump abscess and other problems of the stump 4. Fecal fistula 5. Intestinal obstruction, mainly adhesive intestinal obstruction, can be due to injury of the mesentery of the terminal ileum and gangrenous small bowel consequence. 6. Adhesions, sterility and increased rate of ectopic pregnancies or abortion if appendectomy is done in a pregnant woman. 7. Hemorrhage intra-peritoneal (slipped meso-appendiceal ligature) hypovolemic shock with urgent surgical intervention. 8. Bleeding per rectum (usually inverted non ligated or sloughed stump) 9. Right inguinal hernia (may be due to nerve injury (ilio-hypogastric nerve) or transversalis fascia or transverses aponeurosis injury during McBurney‘s grid iron incision) 10. Hepatic abscess and portal pyelo-thrombophlebitis 11. Intussusceptions of the appendiceal stump

Wound Infection - It is the most common complication following appendectomy, occurring in up to 30% of patients. As expected the severity of the appendiceal inflammation correlates well with the incidence of wound infection, as it most common following appendectomy for perforated appendices (dirty wound) and least common after removal of a normal appendix (clean contaminated wound). It is usually a subcutaneous infection only; the muscles are really very strong barrier to infection on the condition of being viable. - Frequently overlooked is the importance of the use of meticulous surgical technique in the prevention of postoperative wound infection. Care should be taken to prevent contact of the inflamed appendix with the wound, and the operative area should be carefully packed off to prevent inoculation of the wound when the appendix is removed. If perforation has occurred closure of the skin and subcutaneous tissue is an invitation to infection. These wound are best managed by packing open with Betadine –soaked gauze that is changed three times daily until 72 hours, when delayed closure is done if the wound appeared to be clean otherwise it is left to granulate and close by secondary intention. - It is suspected in patients who has post-operative fever or complains of inordinate incisional discomfort .The signs of wound infection are well known and include erythema, tenderness, and purulent discharge. This is the clinical picture of the usual type of wound infection that appears as fever from the third postoperative day on and the usually offending organism is E. coli or mixture of E.coli and staphylo- or strepto-cocci. However, a severe form of wound infection starts as early as the first 24 hours postoperative and it affects patient with some sort of immune compromise, and its offending organism is streptococci with anaerobes and cause non-clostridial gas producing infection like necrotizing fascitits. Clostridia can also affect these wound early post-operatively with frank gas gangrene. 391

- Treatment consists of opening the wound and packing it with Betadine-soaked gauze to be changed three times daily. Adequate debridement of necrotic tissue is also necessary. Antibiotics are given for coexisting cellulites.

Pelvic Abscess - It is not uncommon following appendectomy and its incidence also increases with the degree of appendiceal inflammation and is most common after perforation. Owing to its dependent position and proximity to the appendix, the pelvis is a frequent site of abscess formation. It usually becomes apparent 5-10 days after operation. - Signs and symptoms include fever, abdominal discomfort, frequent loose bowel movement (irritative), lower abdominal deep tenderness and most importantly P.R tenderness and bogginess. Diagnosis is easily confirmed by US and/or CT scan through either of them PAD (per cutaneous abscess drainage) is done for treatment. Patients with multiple abscesses, or difficult locations of abscesses operative drainage is used. - Abscesses may develop elsewhere in the abdomen according to the location of the appendix, most commonly the Morrison pouch and right sub-hepatic spaces.

Appendiceal Stump Problems - Three basic methods have been advocated for handling the appendiceal stump: simple ligation, ligation and inversion, and inversion without ligation. The most commonly practiced technique is to ligate the appendix at its base and then invert the stump into the cecum using a purse string suture. - The theoretical advantages of the method include 1. Good control of hemorrhage from the stump 2. Secure closure of the cecal wall 3. Minimal contamination of the peritoneum by burial of the transected stump 4. Serosal-to serosal approximation that minimizes the extent of raw surface exposed to create adhesions - Simple ligation alone, on the other hand, can cause increase chance of peritoneal contamination, by exposure, sloughing of the stump or loosening of the ligature. Inversion is designed to prevent this possibility. - Inversion is also criticized by being able to compromise the cecal wall vitality, and hence cause necrosis and /or perforation of the cecum with all imagined consequences. It can also cause formation of abscess in the wall of the cecum rupture of which can cause either generalized or localized peritonitis. - Abscess formation due to appendicular stump rupture is uncommon 0.5% or even less. It typically occurs from the fifth day on. The patient who has been discharged from the hospital comes back with picture of generalized or localized peritonitis; US will demonstrate free fluid, which is usually a turbid offensive one, with fecal matter only present if sloughing of the cecal wall is present. Treatment is by re-exploration and refreshing of the edges of the cecum with closure in layers, cecostomy, or limited right hemicolectomy according to the anticipated healing power of the patient and tissues involved.

392

Fecal Fistula - This complication is usually following the operation on appendicular abscess through a small McBurney‘s incision with too much manipulations of the inflamed tissues endangering the vitality of the cecal wall which if get adherent to the wound area will cause wound infection opening of which will disclose the presence of feces in the wound. - A cecal fistula usually closes with conservative treatment provided that the patient is anabolic, the skin is healthy, the fistula is small, there is no associated intra-abdominal abscess and there is no distal obstruction or foreign bodies inside. - If conservative treatment (NPO, IV alimentation and antibiotics, and somatostatin subcutaneously) surgery with limited right hemicolectomy or simple cecal wall closure after refreshment is indicated.

Intestinal Obstruction - This is usually small bowel obstruction due to adhesions between a loop of the small bowel and the appendiceal stump if not inverted. Conservative treatment can usually help the patient to avoid an exploratory operation. However sometimes these adhesions cannot be treated except surgically. The typical mechanical small bowel obstruction with repeated copious vomiting as the patient tries to drink or eat with distension and multiple fluid levels in the plain standing X-ray clinches the diagnosis (with no fever, and no ileus). - Another more serious type of intestinal obstruction is due aggressive manipulations in delivering the cecum out of the abdominal cavity with multiple injuries to the mesentery of the terminal ileum causing gangrenous and/or ischemic insults to the ileum giving clinically a picture of strangulated not pure mechanical obstruction, with ileus and signs of peritoneal irritation, together with a bad general condition of the patient. Surgery is a must in these cases to resect the non-viable bowel with end-to-end anastomosis.

393

394