CONTENTS

Page I I TRAUMA (MULTI-SPECIALTY APPROACH) 22

II II APPROACH TO ABDOMINAL PAIN 1010 Notes IIIII A PPROACH TO ABDOMINAL MASSES 1111 IV IV OESOPHAGEAL DISEASES 1212

For the M.B.B.S. V UPPER BLEEDING GIT AND ITS CAUSES 2121

V I VI COLORECTAL DISEASES 1919 By Andre Tan VII DISEASES 3939

VIII PANCREA TIC DISEASES 4545

IX IX BILIARY TRACT DISEASES 5151

X X BREAST DISEASES 6060

X I XI HEAD AND NECK MASSES 6969

XII SWELLINGS 7474

XIII THYROID DISEASES 7878

XIV PERIPHERAL ARTERIAL DISEASE 8585

XV ABDOMINAL AORTIC ANEURYSM 9393

XVI PERIPHERAL VENOUS DISEASE 9595

XVII UROLOGICAL DISEASES 9999

XVIII SURGICAL INSTRUMENTS 110110

TRAUMA (MULTI-SPECIALTY APPROACH) Management o f breathing -- Supplemental oxygen -- Ventilate as required if patient requires assistance with breathing AADVANCED TTRAUMA LLIFEIFE SSUPPORT ALGORITHM -- Needle thoracotomy for tension pneumothorax, followed by chest tube MAIN PRINCIPLES: -- Occlusive dressing for open pneumothorax -- Treat greatest threat to life first -- Definitive diagnosis is less important 3.3. CIRCULATION -- Time is important – – the “golden hour” after trauma is when 30% of trauma deaths Assessment of organ perfusion occur, and are preventable by ATLS -- Level of consciousness -- Skin colour and temperature, capillary refill -- Pulse rate and character – – all major pulses APPROACH -- Blood pressure 1.1. Primary survey and Resuscitation with adjuncts 2.2. Re-evaluation of the patient Classes of haemorrhagic shock 3.3. Secondary survey with adjuncts I II III IVIV 4.4. Post-resuscitation monitoring and re-evaluation Bld loss 5.5. Optimise for transfer and definitive care Amt (ml) <750 750-1500 1500-2000 >2000 Percentage <15<15 15-30 30-40 >40>40 Ht rate <100 >100 >120 >140 PRIMARY SURVEY – – ABCDE BPBP Normal Normal Decreased Decreased Cap refill Normal Prolonged Prolonged Prolonged 1.1. AIRWAY Resp rate 14-20 20-30 30-40 >35 Assessment of airway patency Ur output (ml/h) >30 20-30 5-15 Oliguric-anuric -- Is patient alert, can patient speak? Mental state Sl anxious Mild anxiety Anxious- Confused- -- Gurgling, stridor confused lethargic -- Maxillofacial injuries Fluid Crystalloid Crystalloid Crystalloid ++ Blood replacement blood -- Laryngeal injuries -- Caution: C-spine injury

Establishing patent airway Management -- Chin-lift or modified jaw thrust (protect C-spine) -- Sources of bleeding  apply direct pressure or pressure on proximal pressure -- Remove any foreign objects in the mouth where possible point -- Oro/nasopharyngeal airway -- Be suspicious about occult bleeding e.g. intraperitoneal, retroperitoneal (pelvic -- Definitive airway – – endotracheal tube, cricothyroidotomy, tracheostomy fracture), soft tissue (long bone fracture) -- Venous access – – large bore, proximal veins 2.2. BREATHING -- Restore circulatory volume with rapid crystalloid infusion – – Ringer’s lactate Assessment of breathing -- Blood transfusion if not responsive to fluids or response is transient -- Look, listen, feel: chest rise, breath sounds – – rhythm and equality bilaterally -- Reassess frequently -- Rate of respiration -- Effort of respiration -- Colour of patient -- Percuss chest -- Look for chest deformities e.g. flail chest 33 4.4. DISABILITY SECONDARY SURVEY

-- Glasgow coma scale When to do secondary survey Eye Verbal Motor -- Primary survey and resuscitation completed Spontaneous opening 44 Oriented speech 55 Obeys 66 -- ABCDEs reassessed Opens to voice 33 Confused 44 Purposeful 55 -- Vital functions returning to normal i.e. no need for active resuscitation at the moment Opens to pain 22 Inappropriate 33 Withdraws 44 No response 11 Incomprehensible 22 Flexion response 33 No verbal response 11 Extension response 22 1.1. AMPLE HI HI STORY Y No response 11 -- AAllergy -- MMedications GCS: 14-15 (minor); 8-13 (moderate); 3-7 (severe) -- PPast history -- LLast meal -- AVPU score: Alert, Verbal stimuli (responds to), Pain stimuli, Unresponsive -- EEvents leading to injury, Environment in which trauma occurred -- Pupillary reactivity 2.2. C C OMPLETE HEAD - - TO TO - - TOE EXAMINATION -- Call for neurosurgical consult as indicated Head -- Complete neurological examination 5.5. EXPOSURE -- GCS or AVPU assessment -- Remove all clothes -- Comprehensive examination of eyes and ears for base of skull fractures -- Check everywhere for injuries (log-roll to look at the back) -- Caution: unconscious patient; periorbital oedema; occluded auditory canal -- Prevent hypothermia Maxillofacial 6.6. ADJUNCTS TO PRIMARY SURVEY -- Bony crepitus/deformityity Monitoring -- Palpable deformity -- Vital signs – – BP, pulse rate, saturation (pulse oximeter) -- Comprehensive oral/dental examination -- ECG monitoring -- Caution: potential airway obstruction in maxillofacial injury; cribriform plate  -- Arterial blood gas fracture with CSF rhinorrhoea do not insert nasogastric tube

Diagnostic tools Cervical spine -- Screening X-ray films (trauma series): CXR, AP pelvis, lateral C-spine -- Palpate for tenderness, any step deformity -- Focused abdominal sonography in trauma (FAST) -- Complete neurological examination -- Diagnostic peritoneal lavage -- C-spine imaging -- Caution: Injury above clavicles; altered consciousness (cannot assess Urinary catheter accurately); other severe, painful injury (distracts from cervical spine pain) -- Functions: decompress bladder, measurement of urinary output -- Caution in urethral injury: blood at urethral meatus, perineal Neck (soft tissues) ecchymosis/haematoma, high-riding prostate -- Blunt versus penetrating injuries -- Airway obstruction, hoarseness Gastric catheter (orogastric or nasogastric) -- Crepitus (subcutaneous emphysema), haematoma, stridor, bruit -- Function: decompress , look at aspirate (bloody? bilious?) -- Caution: delayed symptoms and signs of airway obstruction that progressively -- Caution in base of skull fracture: CSF otorrhoea/rhinorrhoea, periorbital develop; occult injuries ecchymosis, mid-face instability (grab the incisors and rock), haemotympanum  insert orogastric tube instead of nasogastric Chest ABDOMINAL TRAUMA - Inspect, palpate, percuss, auscultate - Re-evaluate frequently TYPES OF INTRA-ABDOMINAL INJURY IN BLUNT TRAUMA - Look at CXR - Solid organ injury: spleen, liver – bleeding (may be quite massive) - Caution: missed injury; increase in chest tube drainage - Hollow viscus injury with rupture - Vascular injury with bleeding Abdomen - Inspect, palpate, percuss, auscultate - Abrasions and ecchymosis – “seat- belt sign” INDICATIONS FOR IMMEDIATE - Lower rib fractures  liver and spleen injury - Evisceration, stab wounds with implement in-situ, gunshot wounds traversing - Re-evaluate frequently abdominal cavity - Special studies: FAST, DPL, CT scan - Any penetrating injury to the abdomen with haemodynamic instability or peritoneal - Caution: hollow viscus and retroperitoneal injuries; excessive pelvic irritation manipulation - Obvious or strongly suspected intra-abdominal injury with shock or difficulty in stabilising haemodynamics Perineum - Obvious signs of peritoneal irritation - Contusions, haematomas, lacerations - Rectal exam reveals fresh blood - Urethral blood - Persistent fresh blood aspirated from nasogastric tube (oropharyngeal injuries - DRE: Sphincter tone, high-riding prostate, pelvic fracture (may feel fragments of excluded as source of bleeding) bone); rectal wall integrity; blood - X-ray evidence of pneumoperitoneum or diaphragmatic rupture - Vaginal examination: blood, lacerations

Musculoskeletal – extremities INVESTIGATIONS - Contusion, deformity - If patient is stable: FAST and/or CT scan - Pain - If patient is unstable: FAST and/or DPL - Perfusion - Peripheral neurovascular status FOCUSED ABDOMINAL SONOGRAPHY IN TRAUMA (FAST) - X-rays as appropriate - Ultrasonographic evaluation of four windows: Pericardial, right upper quadrant, left - Caution: potential blood loss is high in certain injuries (e.g. pelvic fracture, upper quadrant, pelvis femoral shaft fracture); missed fractures; soft-tissue or ligamentous injuries; examine patient’s back - Advantages  Portable 3. ADJUNCTS AND SPECIAL DIAGNOSTIC TESTS  Can be done quickly in <5min - As required according to suspicion, but should not delay transfer  Can be used for serial examination  Does not require contrast, no radiation risk 4. F REQUENT RE - EVALUATION - Disadvantages - Have a high index of suspicion for injuries to avoid missing them  Does not image solid parenchymal damage, retroperitoneum, diaphragmatic  - Frequent re-evaluation and continuous monitoring rapidly recognise when defects or bowel injury patient is deteriorating  Compromised in uncooperative, agitated patient, obesity, substantial bowel gas, subcutaneous air 5. P AIN MANAGEMENT  Less sensitive, more operator-dependent than DPL and cannot distinguish blood - Intravenous analgesia as appropriate from ascites  Intermediate results require follow-up attempts or alternative diagnostic tests 5

CT SCAN CARDIOTHORACIC TRAUMA - Only suitable for stable patient as quite long time involved in imaging with only patient in the room  can collapse There are 5 clinical scenarios in chest trauma where bedside procedures are lifesaving: cardiac tamponade, airway obstruction, flail c hest, haemothorax, and pneumothorax. - Advantages  Able to precisely locate intra-abdominal lesions preoperatively  Able to evaluate retroperitoneum CARDIAC TAMPONADE  Able to identify injuries that can be managed non-operatively - High index of suspicion required  Not invasive - Clinical features - Disadvantages  Chest trauma and hypotension  Expensive  Beck’s triad (hypotension, muffled heart sounds, distended neck veins) – only  Time required to transport patient seen in 50% of cases as hypovolaemia may prevent neck vein distension; muffled  Use of contrast heart sounds are least reliable  Pulseless electrical activity  Kussmaul’s signs (increased neck distension during inspiration, pulsus paradoxus) DIAGNOSTIC PERITONEAL LAVAGE (DPL) - Involves making a cut in the infraumbilical region and inserting a catheter into the - Diagnostic clues peritoneal cavity, aspirate, then instillation of saline and re-aspiration  Enlarged cardiac shadow in CXR (globular heart – very rarely seen)  Small ECG voltages, electrical alternans (uncommon) - Positive DPL  Pericardial fluid demonstrated on FAST or 2D-echo - definitive  Frank blood (>5ml) or obvious bowel contents aspirated  Lavage fluid seen to exit from chest drain or urinary catheter - Management  RBC >100,000 per mm3, WBC >500, Gram stain positive for bacteria in effluent  Aggressive fluid resuscitation – helps maintain cardiac output and buys time  Pericardiocentesis: ECG lead-guided or 2D-echo guided - Indications:  Any unstable patient with suspicion of abdominal trauma or where clinical exam is difficult or equivocal AIRWAY OBSTRUCTION  Unexplained hypotension in multiple trauma - Chin lift or jaw thrust  Patient requiring immediate surgery for extra-abdominal injuries - Remove any foreign body manually, suction blood/secretions - Definitive airway – ETT, cricothyroidotomy, tracheostomy - Contraindications  Absolute indication for laparotomy already exists  Previous abdominal surgery or FLAIL CHEST  Gravid uterus - When 2 or more ribs are fractured at 2 points forming a flail segment that moves  Morbid obesity paradoxically with breathing  Coagulopathy - Results in hypoxaemia mainly due to underlying pulmonary contusion, contributed to by pain with restricted chest wall movement - Advantages  Can promptly reveal or exclude the presence of intraperitoneal haemorrhage - Management: ensure adequate oxygenation and ventilation; judicious fluid therapy  Valuable in discovery of potentially lethal bowel perforation (avoid fluid overload); adequate intravenous analgesia - Consider mechanical ventilation in high risk patients: shock, severe head injury, - Disadvantages previous pulmonary disease, fracture of >8 ribs, age > 65, >3 associated injuries  Morbidity involved – wound complications (haematoma, ); intraperitoneal injury  False negative rate of 2% when there is failure to recover lavage fluid, early hollow viscus injury, diaphragmatic injuries, injuries to retroperitoneal structures HAEMOTHORAX (b) Subdural haemorrhage - Chest tube insertion in the triangle of safety (bound by the lateral border of the  Crescent shaped haematoma under the dura (between the dura and the pectoralis major medially, a line just anterior to the mid-axillary line laterally, and the arachnoid) upper border of the fifth rib inferiorly)  More severe than EDH (usually due to nature of injury that causes SDH to - Be wary of sudden cessation of chest tube drainage as tube can get blocked by clot occur – associated with higher impact, thus brain has other injuries)  - If blood >1500mls  massive haemothorax, call urgent cardiothoracic consult Pathology: underlying brain damage in addition to expanding SOL  Removal of blood does not solve underlying brain damage  poorer results

PNEUMOTHORAX (OPEN/TENSION) (c) Traumatic subarachnoid haemorrhage   - Tension pneumothorax is a clinical diagnosis (CXR will only delay treatment, and Usually only small amount of blood conservative treatment sufficient may cause death) – signs of pneumothorax, hypotension, neck vein distension, severe (d) Intraparenchymal haemorrhage respiratory distress  Any shape, size, location - Immediate needle thoracotomy in second intercostal space in mid-clavicular line  If large haematoma, will require evacuation - Followed by chest tube insertion 4. Diffuse axonal injury - Open pneumothorax occurs in a large chest wall defect with equilibration between - Global injury of axons intrathoracic and atmospheric pressure, producing a “sucking chest wound” - Arises from injury that causes rotational and shearing forces (high impact - Cover defect with a sterile dressing, taping it down on 3 sides to produce a flutter- injury) – rapid acceleration and deceleration of brain in the intracranial cavity valve effect, letting air out of the pleural cavity but not back in against relatively fixed points of attachment at the falx and tentorium - Insert chest tube (not through the wound) - Maximal effects at corpus callosum and brainstem - If severe, will see punctate haemorrhages at the grey-white border

5. Cerebral oedema (2 types) NEUROSURGICAL TRAUMA (a) Hypoxic (cellular)  Decreased blood supply (oxygenation)  loss of function of Na-K pump as AIM in management of head injuries is the prevention of secondary brain injury (from ATP decreases  increased intracellular sodium  cellular swelling hypotension, hypoxaemia, increased ICP etc) since neuronal death is irreversible. (b) Interstitial  Breakdown of blood-brain barrier  proteins enter interstitial space  PATHOLOGIES: oedema 1. Concussion - Physiological dysfunction without anatomical or radiological abnormality PATHOPHYSIOLOGY - (Physiological dysfunction is the first step towards cell death, but is reversible if no further insult occurs) 1. Monro e-Kellie doct rine - Usually recovers in 2-3 hours - Intracranial cavity is of fixed volume and its contents (brain, CSF, blood) are relatively incompressible 2. Contusion - Thus increase in intracranial volume  raised ICP - Small haematoma <1cm Cerebral perfu sion pressur e = Mean arteri al pr essur e – I ntracranial pressure 3. Intracranial haemor rhage (a) Extradural haemorrhage - Compensatory mechanisms:  Lens-shaped haematoma outside the dura (between skull and dura) (a) Hyperventilation  vasoconstriction of cerebral vessels due to increased  Pathology: expanding space-occupying lesion partial pressure of carbon dioxide  decrease in blood volume  20% of patients with EDH are alert and well; underlying brain is minimally (b) CSF pushed into spinal canal (but limited volume available) damaged, thus drainage gives good results - Removal of any reversible cause of raised ICP will improve cerebral perfusion 7 2. Fixed dilated pupil - Constrictor fibres to the pupil run in the oculomotor nerve, which exits the 3. Moderate head injury brainstem at the upper midbrain – nerve fibres lie just under the tentorium - All will be CT-scanned at ED  NES will operate if any indication to do so - Uncus of the temporal lobe sits on the tentorium - In ward: as per mild head injury - In raised ICP, the uncus herniates over the edge of the tentorium, compressing the fibres of the oculomotor nerve just below 4. Severe head injury - Thus a fixed dilated pupil occurs on the side of the compression due to - Must scan to look for reversible causes of raised ICP but stabilise patient first unoppressed sympathetic supply (dilates the pupil) - Medical methods to lower ICP 3. Cushing’s reflex (a) Intubate and hyperventilate - A triad of: (b) IV mannitol (must catheterise patient also; do not give if patient is unstable) (a) Raised ICP - Screen for other life-threatening injuries (likely to be multi-trauma patient) (b) Hypertension - Achieve haemodynamic stability (c) Bradycardia (a) Check for long bone fractures - From Monroe-Kellie doctrine, an increase in mean arterial pressure helps to (b) FAST for bleeding into abdominal cavity maintain cerebral perfusion pressure when ICP is raised (c) ABG to detect acidosis - Increase in mean arterial pressure achieved by sympathetic overdrive: (d) Keep monitoring patient and re-investigate where appropriate (a) Increased heart rate - Operate if reversible cause found (b) Increased contractility (a) Craniectomy (i.e. bone flap not replaced) or craniotomy (bone flap replaced (c) Increased vasoconstriction – increased total peripheral resistance after blood evacuated) [Burrhole usually not big enough to drain an acute (a) and (b) increase cardiac output  increased BP; (c) increases BP bleed] - Baroreceptors detect abnormally raised blood pressure and try to decrease it  (b) Evacuate clot heart rate falls (c) Insert endoventricular drain (EVD) if there is hydrocephalus - Total sedation after operation, ward in ICU MANAGEMENT  Prevents patient from struggling which will raise ICP 1. Assessment - 3 important parameters: ABCs, GCS, pupil size 5. Depressed skull fractur e - Glasgow coma scale (see above) – Minor head injury: 14-15; moderate injury: 8- - Can leave alone unless depression is greater than the thickness of the skull bone 13; severe injury: 3-7 6. Compound depressed fracture 2. Minor head injury - There is through-and-through skin laceration over the fracture - Most common - Always explore to ensure underlying dura is intact, and repair if dura is torn - Indications for admission: (since meningitis can occur with a torn dura)  Persistent headache and/or vomiting  CSF leak  Neurological deficit  Skull fracture  History of loss of consciousness  Amnesia - In ward: NBM, IV drip (no dextrose saline!), no sedation, monitor GCS - If patient deteriorates  CT scan, exclude metabolic causes (e.g. hypoglyc), do septic workup (exclude sepsis) MUSCULOSKELETAL TRAUMA Wound care - Swabs of the wounds for culture and sensitivity GENERAL POINTS - IV antibiotic prophylaxis - Extremity trauma tends not to be life-threatening - Tetanus toxoid cover - But occult blood loss can occur in large volumes especially in certain types of - Photograph wound (to prevent re-opening of wound by every doctor that comes to injuries – pelvic fracture (up to 3L), femoral shaft fracture (up to 2L) see patient) - Need to have high level of suspicion and treat with urgency - Betadine dressing - Look out for any tachycardia, early signs of shock - In OT: generous debridement, irrigation (within 4-8 hours, especially in open - Prepare to resuscitate patient fractures), fracture stabilisation (internal or external fixation depending on Gustilo classification) - Leave wound OPEN ASSESSMENT OF THE EXTREMITY - Perfusion: colour, pulses, skin temperature, c apillary refill - Deformity MANAGEMENT OF FRACTURES - Wounds – open or closed injury; abrasion over a fracture is considered open fracture - Recognise fracture and/or dislocation - Soft tissue assessment - Complete neurovascular examination of the limb involved before reduction - Abnormal joint mobility – ligamentous injury around the joint; if in the knee, highly - Appropriate X-rays (at least 2 planes) likely that the popliteal artery is injured as well - Analgesia - Neurological assessment - Correction of deformity - Viability of the limb - Temporary immobilisation – backslab, malleable splint - Neurovascular examination; examine for compartment syndrome - Circulation chart THE PULSELESS EXTREMITY Things to consider - Is pulselessness due to shock? OPEN FRACTURES - Arterial or venous compromise? Definition: there is communication between the fracture or fracture haematoma and the - Is there compartment syndrome (pulselessness is a very late sign) external environment - Any pre-existing vascular disease? Gustilo-Andersen classification Physical examination Type I  <1cm AND clean - Any limb deformity (can result in kinking of vessels)? - Any joint instability (dislocation of a joint can result in intimal tear in the major Type II  >1cm AND no extensive soft tissue damage, avulsions or flaps vessel running across it, with thrombosis and occlusion)? Type II IA  Extensive soft tissue damage, avulsions or flaps but adequate soft - Skin colour/temperature tissue coverage of bone OR - Post-reduction tibial pulse in knee dislocation – if still absent, do an urgent  High-energy trauma cause irregardless of size of wound angiogram! Type II IB  Extensive soft tissue loss with periosteal stripping and exposure of bone. SOFT TISSUE INJURIES  Massive contamination common Types - Open: laceration, abrasion Type III C  Arterial injury requiring repair - Crushing - Degloving: open or closed - Closed 9 - Grade I  Low velocity injury, prognosis similar to closed fracture  Treat with ORIF within 6 hours - Grade II  Moderate velocity, more trauma - Grade IIIA  Skin graft usually possible - Grade IIIB  Skin graft alone often not adequate  Local and free flaps will be necessary  Secondary bone procedures - Grade IIIC  Neurovascular injuries present in addition to musculoskeletal injuries

Management of open fractures - Recognise an open fracture - Stabilise patient first - Pain relief and analgesia - Cover the wound with moist gauze - Temporary immobilisation and splinting - IV broad spectrum antibiotics - Appropriate X-rays - Nil by mouth - Pre-op investigations: FBC, U/E/Cr, PT/PTT, GXM, ECG, CXR - Arrange for emergency operation - Angiogram if needed

Surgery involves: (a) Generous debridement of the wound with irrigation to decrease bacterial load (b) Treat any soft-tissue injuries (c) Stabilise fracture – usually using external fixator ABDOMINAL PAIN RHC Epigastric LHC Thoracic Hepatic Thoracic Others Thoracic Others  Pneumonia  Hepatitis (viral, autoimm etc)  MI   Pneumonia  Subphrenic abscess  Pleural effusion  Hepatomegaly  Pericarditis  Pleural effusion  Splenomegaly  Abscess  Aortic aneurysm  MI  Pancreatitis Biliary  Cholangitis Others Gastrointestinal Gastrointestinal  Cholecystitis  Subphrenic abscess  Oesophagitis  PUD  Gallstone disease  Pancreatitis  GERD  Diverticulitis  PUD  PUD  Mesenteric ischaemia  Appendicitis  Gastric outlet obstructn  CA stomach

Rt Loin Periumbilical Lt Loin Biliary (see RUQ) Gastrointestinal Others Spleni c disease Obstruction  Appendicitis (early)  Aortic Aneurysm  Hydronephrosis Urological  I/O  Pancreatitis Urological (see Rt Loin)  Nephrolithiasis Infection  Mesenteric ischaemia  Ureteral obstruction  Pyelonephritis  Colitis  Abscess CA  IBD  RCC Others  TCC renal pelvis  PKD  Bladder ca (ureteral obstructn)  Renal cyst  Angiomyolipoma Others  Infarction  Appendicitis

RIF Hypog astric LIF Gastrointestinal O&G Gastrointestinal O&G Orthopaedics  Appendicitis  Ovarian cyst  Colorectal CA  Ectopic pregnancy Infection Paediatric ortho condition s  Terminal ileitis  Ovarian torsion  Abortion  Septic hip arthritis  Transient synovitis  Meckel’s diverticulitis  Ectopic pregnancy Urological  PID  TB hip  Perthes’ dz  Mesenteric ischaemia  PID  ARU  Uterine rupture Degeneration  SCFE  Mesenteric adenitis  Bladder calculi  Fibroid complications  OA hip  IBD Orthopaedics (See LIF)  Cystitis / UTI  Adenomyosis Gastrointestinal Inflammation  Colitis  Endometriosis  Diverticulitis  RA hip  Colorectal CA  IBD  Ankylosing spondylitis   Colitis  Reiter’s syndrome  Colorectal CA Inflitration  Hernia  1o bone tumour (hip)  Metastasis to hip O& G (see RLQ) Destruction  # - NOF, pubic rami Radiation  Back pathologies (referred pain) 11 ABDOMINAL MASS RHC Epigastrium LHC Liver Li ver (see RHC) Stomach Spleen Stomach Massive  Pancreatic/periampullary ca  Cancer Massive  Cancer: HCC  Acute cholecystitis  Distension (GOO)  Infxns Descending colon Metastases  Hydrops  Pseudocyst  CML  Cancer Myeloprolftve dz  Empyema  Tumour Aorta  Myelofibrosis  Diverticular mass/abscess  Alcoholic liver dz  Mirizzi syndrome  Aortic aneurysm  Faeces Moderate  Rt ht failure/tricuspid regurg Tr ansverse colon  Above causes Ascendin g colon  Cancer Retroperiton eal lNpathy Left kidney(see Rt lumbar) Moderate  Portal hypt  Cancer  Diverticular mass/abscess  Lymphoma  Above causes  Lymphoprolftve dz  Diverticular mass/abscess  Faeces  Teratoma Left adrenal gland  Lymphoprolftve dz (lymphoma, CLL)  Faeces  Other malignancies  Haemochromatosis  H’lytic anaemia (thal, HS)  Amyloidosis  Storage dz (Gaucher’s) Right adrenal gland Mild Mild  Above causes Right k idney(see Rt lumbar )  Above causes  Infxns: Viral – Hep, IMS  Infxns: Viral hep, IMS Bacterial – abscess Endocarditis Parasitic – hydatid  Autoimm – SLE, RA, PAN cyst, amoebic abscss  Myeloprolftve dz – PRV,  Biliary obstruction essential thrombocytopaenia  Cirrhosis  Infiltratn – sarcoid, amyloid

Right Lumbar Umbilical Left Lumbar Right Kidney Right adrenal gland Li ver (see RHC) Pancr eas (see Epi gastriu m) Spleen (see LH C) Descending colon  Hydro/pyonephrosis  Cancer  Cancer – RCC Li ver (see RHC) Stomach(see Epi gastrium) Aorta Left ki dney (see righ t lumbar)  Diverticular mass/abscess  Polycystic dz  Aortic Aneurysm  Faeces  Single cyst Ascendin g colon mass Small in testine  Amyloidosis  Cancer  Obstruction Retroperiton eal lNpathy  Tuberous sclerosis, VHL  Diverticular mass/abscess  Lymphoma  Faeces M esenteri c cyst  Teratoma  Other malignancies

RIF Hypogastrium LIF Gastrointestinal O&G Bladder Uterus Gastrointestinal O&G  Appendiceal mass/abscess  Ovarian cyst/tumour  Acute retention of urine  Gravid uterus  Diverticular mass/abscess  Ovarian cyst/tumour  TB gut  Fibroids  Chronic retention of urine  Fibroids  Ca colon/sigmoid  Fibroids  Ca caecum  Tumour  Crohn’s dz (terminal ileitis)  Distended caecum (due to Others Anal /rectal mass  Faeces Others distal obstruction)  Transplanted kidney  Cancer Ovary  Transplanted kidney  Crohn’s dz (terminal ileitis)  Iliac artery aneurysm  Cyst Orthopaedics  Iliac artery aneurysm  Psoas abscess  Tumour  Chondroma/sarcoma of ilium  Psoas abscess Orthopaedics  Iliac lymphadenitis  Bony metastasis  Iliac lymphadenitis  Chondroma/sarcoma of ilium  Malignant change in undesc  Malignant change in undesc  Bony metastasis testis testis OESOPHAGEAL DISEASES - Pharyngeal muscles contract to propel food bolus past the relaxed cricopharyngeus into the oesophagus ANATOMY - Once in the oesophagus, involuntary contractions of the muscularis propria form - Oesophagus is a muscular tube that is 25cm (10 inches) long peristaltic waves to propel food bolus into stomach - Starts at the cricoid cartilage (C6 vertebra) from the oropharynx and continues into the stomach at the level of T10 APPROACH TO DYSPHAGIA - Upper oesophageal sphincter is formed by cricopharyngeus muscle - Lower sphincter is not an anatomical sphincter, but physiological: CAUSES OF DYSPHAGIA (i) Increased tone of the muscularis propria at the lower oesophageal sphincter - Dysphagia can be divided into oropharyngeal and oesophageal dysphagia (ii) Fibres of the right diaphragmatic crus loop around the cardio-oesophageal - In each anatomic region the dysphagia can be caused by neuromuscular dysfunction junction and ontract during coughing, sneezing etc when intra-abdominal (impaired physiology of swallowing) or mechanical obstruction to the lumen pressure increases, thus preventing reflux Oropharyngeal Oesophageal (iii) Angle of His where the oesophagus joins the stomach – acts as a valve Neuromuscular diseases Neuromuscular diseases (iv) Intra-abdominal pressure being higher than intra-thoracic pressure Stroke Achalasia - 3 narrow points along the course of the oesophagus Parkinson’s disease Spastic motor disorders Brain stem tumours Diffuse oesophageal spasm (i) Cricopharyngeus muscle (15cm from incisor teeth) Degenerative conditions e.g. ALS, MS Hypertensive lower oesophageal sphincter (ii) Carina where the left bronchus crosses the oesophagus (27cm from incisors) Peripheral neuropathy Nutcracker oesophagus (iii) Where the oesophagus passes through the diaphragm (40cm from incisors) Myasthaenia gravis Scleroderma Myopathies e.g. myotonic dystrophy - Structure: mucosa, submucosa, muscularis propria, adventitia (no peritoneal lining except for a short segment of intra-abdominal oesophagus) Obstructive lesions Obstructive lesions Muscularis propria is composed of striated muscle in the upper one-third, striated and Tumours Intrinsic structural lesions smooth muscle in the middle third, and smooth muscle in the lower third Inflammatory masses e.g. abscess Tumours Oesophageal webs Strictures: Peptic (reflux oesophagitis) - Blood supply (roughly divided into thirds): Inferior thyroid artery to upper third, Pharyngeal pouch (Zenker’s divert) Radiation oesophageal branches of the aorta to the middle third, oesophageal branches of left Anterior mediastinal mass Chemical (caustic ingestion) gastric artery to lower third Medication - Venous return also divided into thirds: Brachiocephalic veins (upper), azygos veins Lower oesophageal rings (Schatzki’s ring) (middle), left gastric vein (lower) --- a portosystemic anastomosis exists at the lower Oesophageal webs (Plummer-Vinson) Foreign bodies oesophagus thus leading to formation of varices in Extrinsic structural lesions Vascular compression (enlarged aorta or PHYSIOLOGY OF SWALLOWING left atrium) - Process of mastication forms a food bolus on the dorsum of the tongue Mediastinal masses – retrosternal thyroid, - The tongue then contracts upwards and backwards pushing the food bolus against the lymphadenopathy hard palate Others - Soft palate elevates (contraction of palatoglossus) to close off nasopharynx Oesophagitis: Reflux - Further elevation of tongue pushes food bolus into oropharynx Infectious (candida, herpes) - As the base of the tongue is elevated posterior, the epiglottis falls back; at the same Radiation-induced Medication-induced time, the pharyngeal muscles contract to bring the posterior surface of the larynx Chemical-induced (alcohol) upwards to make the laryngeal inlet smaller  closed off by the epiglottis 13 HISTORY: - Medication history 1. Is there odynophagia (pain associated with difficulty swallowing)? - Symptoms of systemic disease e.g. stroke (focal neurological deficits), scleroderma (telangiectasia, sclerodactyly, calcinosis, Raynaud’s), Parkinson’s - Signifies some form of oesophagitis : infectious (candida, herpes), post-radiation, chemical-induced (usually alcohol), reflux oesophagitis 5. Systemic review - Oesophageal spasm - Loss of weight occurs in cancer and achalasia, but of much later onset in - Scleroderma achalasia compared to cancer - Pain occurs late in achalasia and oesophageal cancer (not painful from the start) - Symptoms of anaemia (bleeding from tumour, or as part of Plummer-Vinson syndrome) 2. Differentiating oropharyngeal from oesophageal dypshagia - Symptoms of aspiration pneumonia – fever, cough, shortness of breath (i) Oropharyngeal - Presenting complaint is usually of difficulty in initiating swallowing 6. Tumour spread - May be associated with choking, coughing, nasal regurgitation - Hoarseness (recurrent laryngeal nerve) - Voice may sound nasal (bulbar palsy) - Fever, cough and haemoptysis (tracheo-oesophageal fistula) - Cause of oropharyngeal dysphagia is usually neuromuscular rather than - Haematemesis (invasion into aorta) mechanical; stroke is the most common cause - Neck lump (lymph node) (ii) Oesophageal - Presenting complaint is that of food “getting stuck” in the throat or chest PHYSICAL EXAMINATION - Patient’s localisation of the symptom often does n ot correspond to actual site of pathology 1. General condition - Can be due to either neuromuscular dysfunction or mechanical obstruction - Vitals: the patient may be hypovolaemic from vomiting/decreased intake - Nutrition: presence of cachexia 3. Differentiating mechanical obstruction from neuromu scular dysfunction - Conjunctival pallor : bleeding from tumour, oesophagitis ulcerations, or (i) Mechanical associated with P-V syndrome - Patient complains of more difficulty swallowing solids than fluids - Scleral icterus: metastases to liver - May have regurgitation of undigested food - Dehydration (mucous membranes, skin turgor, etc) - Recent onset dysphagia that is progressively worsening, with loss of weight 2. Disease  high suspicion of oesophageal cancer - Presence of cervical lymph nodes (esp Virchow’s node) - Intermittent symptoms are suggestive of webs, rings - Scars/marks over the chest and abdomen suggesting previous surgery, radiation (ii) Neuromuscular - Palpable mass in abdomen (not likely) - Patient complains of more trouble swallowing fluids than solids - Hepatomegaly - Dysphagia more long-standing, slowly progressive - Ascites - Intermittent symptoms suggestive of diffuse oesophageal spasm, nutcracker - PR examination for malaena oesophagus - May have history of stroke, neuromuscular disease 3. Complications of disease - Signs of pneumonia: patient febrile, may be toxic, lung crepitations, decreased 4. History of predisposing conditions air entry usually over right lower lobe - Reflux symptoms e.g. retrosternal burning pain (heartburn), sour fluid reflux into mouth (acid brash), excessive salivation (water brash), postural aggravation on 4. Treatment lying down - Tube feeding through NG tube, / – if aspirates seen, - Caustic chemical ingestion in the past what is the colour? - Smoking, chronic alcohol intake - Total parenteral nutrition - Radiation to the chest MANAGEMENT 1. Stabilise patient 3. Manometry - Resuscitate if patient is haemodynamically unstable - Gold standard for diagnosing achalasia: - IV fluids (correct fluid deficits and also any electrolyte derangements) (i) Absence of peristalsis - Consider feeding with fluids if patient can tolerate it (only having problems with (ii) Very high pressures at the lower oesophageal sphincter solid food) otherwise consider tube feeding or TPN  need to correct patient’s (iii) Absence of relaxation at the LES on swallowing food nutritionally debilitated state 4. Videofluroscopic examination of swallowing (VFES) or flexible-endoscopic - Keep NBM if patient cannot tolerate even fluids examination of swallowing (FEES) - Treat any aspiration pneumonia – NBM, IV antibiotics - Used to assess oropharyngeal dysphagia (neuromuscular causes) by looking for 2. Investigate for underlying cause and treat it penetration and aspiration of various consistencies of food during swallowing

INVESTIGATIONS Supportive 1. Blood investigations: Diagnostic - Full blood count – Low Hb (anaemia from chronic blood loss) 1. Barium swallow High TW (aspiration pneumonia) - Advantage of barium swallow is that it is less invasive than OGD, especially - Urea, electrolytes, creatinine – electrolyte disturbances from vomiting, poor when suspecting webs, diverticula in the oesophagus where OGD may cause intake; raised creat and urea in dehydration (creat will be raised more than urea if perforation; however if patient is at high risk of aspiration, barium swallow is patient has prerenal failure from dehydration) dangerous. - Liver function tests – low albumin with nutritional deprivation - Visualisation of obstructive lesions: o Shouldering of a stricture (benign strictures form a smoother contour 2. CXR whereas malignant strictures form a more right-angled contour) - Consolidation (aspiration pneumonia) o Bird’s beak sign of achalasia 3. 24-hour pH probe monitoring - If patient complains of reflux symptoms and no signs are seen on OGD (see later section on Gastro-oesophageal reflux disease)

Achalasia Benign stricture Carcinoma - Visualisation of pharyngeal pouch or oesophageal diverticulum - Diffuse oesophageal spasm gives a corkscrew appearance

2. Oesophagogastroduodenoscopy (OGD) - Advantage is direct visualisation of the lesion and ability to take tissue biopsy (especially useful in malignancy), may also be therapeutic (stopping bleeding from a tumour, stenting the lumen, etc) 15

CANCER OF THE OESOPHAGUS STAGING T Tis High-grade dysplasia/carcinoma in-situ Stage T N M EPIDEMIOLOGY T1a Tumour invading lamina propria or 0 is 0 0 - Third most common cancer in Singapore muscularis mucosa - Male predominance T1b Tumour invading submucosa but does I 1 0 0 - Increasing incidence with age not breach submucosa IIA 2 / 3 0 0 T2 Tumour invades the muscularis propria IIB 1 / 2 1 0 T3 Tumour invades adventitia RISK FACTORS III 3 1 0 T4 Invasion of surrounding structures 4 any 0 - Smoking (100x increased risk for SCC, 10x for adenocarcinoma) N N1 Regional node involvement (1-3 nodes - Alcohol (2x increased risk) involved =1a; 4-7=1b; >7=1c) IVA any any 1a - Obesity (related to reflux, increases adenocarcinoma incidence) M M1a Nonregional lymph node involvement IVB any any 1b M1b Other distant metastases - Diet: Hot beverages, preserved foods (nitrosamines), betel nuts; vitamin and mineral deficiencies (selenium, vitamin E, beta-carotene) PRESENTATION - Tylosis (autosomal dominant disorder with keratosis of palms and soles Usually of insidious onset, with earliest symptoms being non-specific e.g. retrosternal - Barrett’s oesophagus (intestinal metaplasia of oesophageal mucosa due to reflux; discomfort, “indigestion”, and most patients already have advanced disease when they increased risk of cancer due to metaplasia-dysplasia-carcinoma sequence; risk is 30- are diagnosed – 75% have lymph node involvement at time of diagnosis. 40x higher than in individual without Barrett’s, and is about 1% per year) 1. Dysphagia - Achalasia (2-8% incidence of SCC) - Present in 80% of patients – most common presentation - Caustic injury (ca occurs at site of scar/stricture, mostly middle third of oesophagus) - Pain develops late and is usually due to extra-oesophageal involvement - Plummer-Vinson (or Paterson-Brown-Kelly) syndrome – Post-cricoid oesophageal 2. Weight loss web and iron deficiency anaemia. (10% develop cancer in upper third of oesophagus) 3. Regurgitation 4. Anaemia (with or without malaena/frank haematemesis – bleeding is usually occult) PATHOLOGY 5. Vocal cord paralysis (left more than right) - 70% squamous cell carcinoma, 30% adenocarcinoma 6. Aspiration pneumonia - SCC can arise anywhere in the oesophagus while adenocarcinoma occurs in lower 7. Tracheo-oesophageal or broncho-oesophageal fistula third and gastro-oesophageal junction (related to reflux and Barrett’s oesophagus) - Overall: 10% of cancers occur in the upper third, 60% in the middle third, 30% in the INVESTIGATIONS lower third Diagnosis - Three growth patterns: 1. Barium swallow  Fungating (60%) - 92% accuracy in showing mucosal irregularity and annular constrictions but not  Ulcerative (25%) able to diagnose malignancy with confidence  Infiltrative (15%) - Tumour spread: direct extension into surrounding structures, vascular invasion, 2. Oesophagogastroduodenoscopy  lymphatic spread - Allows biopsy of the lesion confirmatory histological diagnosis - Common sites of metastases: liver, lung, bone Staging 1. Endoscopic ultrasound - If endoscope can pass around the lesion, the EUS is good for T staging, and also to identify enlarged regional lymph nodes 2. Chest X-ray - Endoluminal surgery – for early lesions; no attempt to remove any LNs (usually no - Presence of any lung metastases LN involvement) - Aspiration pneumonia - Oesophagectomy - Pleural and/or pericardial effusion - Tracheal deviation or extrinsic compression of tracheobronchial system (i) Ivor-Lewis - Widened superior mediastinum in an upper oesophagus tumour Two-stage procedure involving gastric mobilisation (first stage, done through - Raised hemidiaphragm with phrenic nerve involvement upper midline abdominal incision), oesophagectomy and gastro-oesophageal anastomosis in the chest (second stage, through right thoracotomy incision) 3. CT scan or MRI of the thorax with extension to include liver and adrenals (ii) Trans-hiatal - Can be used for T, N, and M staging Done via two incisions – one in the abdomen and one in the neck 4. Bronchoscopy Blunt oesophagectomy, gastric mobilisation, and gastro-oesophageal - Exclude bronchial involvement especially in tumours involving upper two-thirds anastomosis in the neck of oesophagus Less morbidity than Ivor-Lewis as the chest is not opened, but controversial

5. Bone scan for bony metastases (iii) Tri-incisional Three incisions – abdominal, chest, and also left neck incision for gastro- 6. Laryngoscope to assess for vocal cord paralysis oesophageal anastomosis in the neck

 Supportive Performed with two-field lymphadenectomy (upper abdominal and mediastinal)  1. Full blood count – Low Hb (anaemia from chronic blood loss) No difference in survival between trans-hiatal and I-L modalities; the stage of the High TW (aspiration pneumonia) cancer when the operation is performed is a greater factor influencing survival  2. Urea, electrolytes, creatinine – electrolyte disturbances from vomiting, poor intake; Radical en-bloc dissections not shown to improve survival raised creat and urea in dehydration (creat will be raised more than urea if patient  Oesophagectomies have high mortality (5%) and morbidity (25%) rates, thus has prerenal failure from dehydration) patients have to be carefully selected in order to maximise survival benefit from surgery 3. Liver function tests – low albumin with nutritional deprivation  Complications of surgery dependent on extent of surgery and incisions used - Intraoperatively, injury to lung, thoracic duct, RLN can occur TREATMENT - Respiratory complications higher in thoracotomies – atelectasis, pneumonia Principles - Anastomotic leak and resultant mediastinitis (for chest anastomosis) most feared - Three modalities available – surgery, chemotherapy, radiotherapy – used singly or in - Reflux can result in the long term due to loss of the LES combination - Anastomotic stricturing can also occur - Aims of treatment: Curative or palliative (50% of patients have unresectable cancer - Palliative debulking for obstructive symptoms on presentation) - Surgical treatment is usually performed with curative intention, but can also achieve Radiotherapy good long-term palliation of symptoms - Usually given in combination with chemotherapy - Choice of treatment depends on several patient factors: age, co-morbidities, - Primary treatment for poor-risk patients; palliation for unresectable lesions with nutritional state, life expectancy, and prognosis of cancer obstructive symptoms, pain and bleeding - SCCs are radiosensitive Surgery - Curative in early lesions (in-situ, T1a) and part of multimodal therapy in more - Modalities: External beam radiation or brachytherapy advanced stages - Obstructive symptoms may worsen temporarily after radiotherapy due to oedema - Resection should not be done in patients with distant metastases or contraindications - Complications: tracheo-oesophageal fistula, stricture to surgery 17

Chemotherapy GASTRO-OESOPHAGEAL REFLUX DISEASE (GORD) - Current regimen: 5-Fluorouracil and cisplatin - Addition of chemotherapy to external beam radiation for unresectable cancers shown EPIDEMIOLOGY to have improved survival compared to EBRT alone Incidence in Singapore not known - Chemotherapy given preoperatively and postoperatively improves survival Increasing prevalence, more common in males than females

Overall curativ e treatment PATHOPHYSIOLOGY Preoperative neoadjuvant chemoradiotherapy (increases rates of complete resection), - Lower oesophageal sphincter is a physiological sphincter with various mechanisms oesophagectomy, and postoperative adjuvant chemoradiotherapy for responsive that help to prevent reflux (see above, Anatomy of the oesophagus) tumours - Some physiological reflux occurs that is rapidly cleared by peristaltic movements in the oesophagus Palliative treatment - Surgical debulking - GORD results from various pathophysiological factors (loss of the normal protective - Bypass surgery rarely done nowadays mechanisms, or the mechanisms are overwhelmed) singly or in combination:  - Endoscopic laser fulguration to relieve obstruction Loss of LES function – decreased tone, hiatal hernia, iatrogenic injury - Photodynamic therapy is a new treatment option  Delayed gastric emptying - Stenting to maintain lumen patency  Increased intra-abdominal pressure – obesity, tight garments, large meal  Motor failure of oesophagus with loss of peristalsis Feeding in oesophageal obstruction - Acid incites inflammation in the lower oesophagus – extent of inflammation - Feeding via oropharyngeal route is preferred unless the passage is obstructed or it is increases with increasing duration of contact with acid unsafe for the patient to feed via that route (i.e. risk of aspiration) - Chronic inflammation results in complications of GORD: oesophagitis, stricture,  - If still able to pass NG tube around tumour feed via NG (but also consider Barrett’s oesophagus complications with long-term NG placement e.g. erosions around nasal area, sinusitis); consider PEG placement for long-term feeding if able to get scope around CAUSES/RISK FACTORS tumour - Malfunction of LES - If unable to pass tube or scope around tumour, consider open gastrostomy - Motility disorder of oesophagus e.g. scleroderma - Total parenteral nutrition is another option but has more complications, more costly - Relief of obstruction via various techniques as listed above help to enable oral - Hiatal hernia (loss of normal LES mechanisms) feeding, but most techniques are not long-lasting and dysphagia will return with - Chronically increased intra-abdominal pressure – pregnancy, chronic cough, obesity, tumour growth constipation, etc - Drugs that cause smooth muscle relaxation e.g. calcium channel blockers, sedatives, PROGNOSIS beta agonists, anticholinergics, etc. Coffee and smoking also cause LES relaxation. - 80% mortality at 1 year, overall 5-year survival <10% - Eating habits – lying down after a heavy meal - Any cause of decreased gastric emptying

PRESENTATION - Heartburn: retrosternal pyrosis - Acid brash: reflux of sour gastric juices into back of mouth i.e. regurgitation - These symptoms occur usually after food , particularly a heavy meal, and are aggravated by lying flat ( posturally related ) - Long-standing disease can lead to dysphagia due to stricture formation; dysphagia can also result from an underlying oesophageal motility disorder ; odynophagia suggests oesophagitis with ulceration - Reflux can also lead to pulmonary symptoms: chronic cough, chest infections - Can detect motility disorders that cause reflux, and also pick up oesophageal (aspiration) ulceration and stricturing resulting from reflux - Other symptoms: globus (feeling of a lump at the throat), chest pain (can mimic - Can sometimes see reflux of barium contrast into oesophagus anginal pain with radiation to neck, jaw, arm), nausea, water brash (hypersalivation in 5. Manometry response to reflux) - No value in reflux except for detecting motility disorder

COMPLICATIONS GRADING OF OESOPHAGITIS 1. Pain and spasm 1. Savary-Miller classification 2. Stricture Grade I: One or more supravestibular non-confluent reddish spots, with or 3. Haemorrhage (occult more common than frank) without exudates 4. Shortening of oesophagus Grade II: Erosive and exudative lesions, may be confluent but not circumferential 5. Ulceration Grade III: Circumferential erosions covered by haemorrhagic and 6. Barrett’s oesophagus (see below) pseudomembranous exudate 7. Dysmotility Grade IV: Presence of chronic complications such as deep ulcers, stenosis, or 8. Schatzki’s ring (constrictive ring at the squamocolumnar junction composed of scarring with Barrett’s metaplasia mucosa and submucosa) 9. Malignancy (adenocarcinoma arising from Barrett’s oesophagus) 2. Los Ang eles classification Grade A: one or more mucosal breaks, each <5cm in length DIAGNOSIS Grade B: at least one mucosal break >5cm long, but not between the tops of adjacent mucosal folds 1. History is important as most patients with reflux are seen in the primary setting Grade C: at least one mucosal break that is continuous between the tops of adjacent with no facilities for detailed investigation mucosal folds, but which is not circumferential - Exclude cardiac cause of chest pain, and exclude malignant cause of dysphagia Grade D: mucosal break that involves at least three-quarters of the luminal 2. Oesophagogastroduodenoscopy circumference - Cannot actually diagnose reflux - Relevance of classification schemes: subjective and dependent on assessment by the - Can visualise and grade oesophagitis if present, and take biopsy specimens for endoscopist; also, due to the multitude of classification schemes available, just confirmation (see below) mentioning a grade may not have any meaning if the actual abnormalities are not - May see a hiatal hernia which is associated with reflux (though not all patients described with hiatus hernia will have reflux) TREATMENT 3. Oesophageal pH probe - Confirmatory test for reflux is the ambulatory 24hr oesophageal pH probe Lifestyle especially if oesophagitis is not seen on OGD 1. Diet and eating habits  Avoid coffee, chocolate, fatty foods, or anything that worsens symptoms - Antimony probe most commonly used; alternative is the Bravo capsule (a  Do not eat 2 hours prior to sleeping wireless capsule that is temporarily attached to the oesophageal wall)  Walk after eating - The probe is placed 5cm above the manometrically-determined upper limit of the  Avoid excessive eating; eat small meals LES (for the wired probe), or 6cm above the endoscopically-determined 2. Avoid drugs that relax LES e.g. anticholinergics, muscle relaxants, etc. squamocolumnar junction (for the wireless capsule) 3. Weight reduction if obese - Diagnosis based on the percentage of time in 24hrs the pH reading is below 4 4. Elevate head of bed 4. Barium swallow and follow-through 5. Smoking and alcohol intake cessation - Not of much value in diagnosing reflux 19 Medication 1. Acid suppression therapy: proton pump inhibitors or H2-receptor antagonists - Outcome of surgery 2. Prokinetics to increase LES pressure e.g. domperidone, metoclopramide  80-90% Excellent to good (no symptoms, no medications and lifestyle changes required) Surgical  10-15% Satisfactory (some residual symptoms) - Indications:  <5% Unsatisfactory  Failure of medical therapy (or incomplete resolution of symptoms)  <1% Mortality  Oesophagitis with frank ulceration or stricture  5-40% need for acid suppression therapy at 5 years due to symptoms  Complications of reflux oesophagitis – respiratory complications, Barrett’s oesophagus - Management of stricture  Severe symptoms or progressive disease  Rule out malignant cause of stricture by taking biopsy  Compliance problems - patient does not want to be on medication for life  Dilatation (variety of means available – balloon, dilators, etc) (despite good results)  Treatment of underlying reflux  If resistant to dilatation  resection and reconstruction - Goal of surgery:  Increase pressure at the gastro-oesophageal junction but not so much that it prevents food from entering the stomach (too tight  dysphagia) BARRETT’S OESOPHAGUS - Surgery versus conservative treatment  Surgery has higher rates of cure and better long-term results Features  No need to adhere to strict lifestyle and diet change as well as long-term - Intestinal metaplasia of the oesophageal epithelial lining (stratified squamous medication epithelium converted to mucus-secreting columnar epithelium with goblet cells)  Disadvantage of surgery is the associated morbidity and mortality - Associated with long-term reflux – an adaptation mechanism where intestinal - Fundoplication is the mainstay of surgical therapy epithelium withstands exposure to acidic reflux better than oesophageal epithelium  Can be done via open surgery or laparoscopic surgery (most laparoscopic now) - Diagnosed on and histology:  is the most commonly done – a 360 degree (total) wrap of  The squamocolumnar junction (or Z line) is visible on endoscopy as gastric and the fundus around the gastro-oesophageal junction intestinal type epithelium is pink and granular in appearance, but stratified squamous epithelium is smooth and pale  Partial fundoplications can also be done in patients where oesophageal motility is poor or the oesophagus is foreshortened; anterior 90 degrees, anterior 180 deg,  The gastro-oesophageal junction is defined as the point where the gastric folds and posterior 270 deg fundoplications are various options available begin  If the squamocolumnar junction is above the gastro-oesophageal junction (i.e. - Complications of surgery they do not align) and biopsy of the junction shows intestinal metaplasia, the  Perforation of the oesophagus – most feared complication, may result in patient is diagnosed to have Barrett’s oesophagus mediastinitis if not promptly detected and repaired intraoperatively - Short segment Barrett’s is defined as the squamocolumnar junction being <3cm  Excessively tight wrap resulting in dysphagia above the gastro-oesophageal junction, while in long segment Barrett’s the distance  Excessively loose or short wrap – reflux recurs (failure of treatment) between the two junctions is >3cm.  “Slipped- Nissen” occurs when the wrap slides down, the GE junction retracts - Long segment Barrett’s is associated with more severe reflux, as well as higher risk into the chest, and the stomach is partitioned; usually due to a foreshortened of dysplasia and subsequent adenocarcinoma development than short segment oesophagus unrecognised in the first operation Barrett’s  “Gas bloat syndrome” – patient experiences difficulty burping gas that is - Risk of development of adenocarcinoma is about 10-15% in 10 years swallowed Management ACHALASIA 1. Treatment of underlying reflux - Lifestyle changes, acid suppression, surgery etc FEATURES - Not shown to decrease risk of cancer  still requires surveillance - Abnormal peristalsis secondary to absence or destruction of Auerbach’s (myenteric) plexus and failure of the LES to relax; affects body and distal oesophagus 2. Endoscopic surveillance - Aetiology unknown - Not certain regarding benefit for surveillance if patient has Barrett’s but no - Patients present with dysphagia, regurgitation, weight loss, retrosternal chest pain, dysplasia  if 2 scopes in a year reveal no dysplasia, repeat OGD once every 3 and recurrent pulmonary infections years - Main purpose of surveillance is to pick up dysplasia - Barium swallow demonstrates “bird’s beak” narrowing of distal oesophagus with - If patient has high grade dysplasia, it should be treated (see below), otherwise to proximal dilatation undergo intensive surveillance (q3mths for at least one year) to detect cancer - Manometric studies (required for diagnosis) show abnormally high pressures at the development LES, with incomplete LES relaxation on swallowing, and lack of progressive peristalsis (often aperistaltic) 3. Treatment of high-grade dysplasia - 1-10% of patients develop SCC after 15-25 years of disease - Endoscopic therapies to ablate the dysplastic tissue e.g. photodynamic therapy, laser therapy, argon plasma coagulation  will not remove all dysplastic cells TREATMENT thus potential for malignancy still remains - Mainly palliative in nature - Oesophagectomy is the only definitive treatment to remove all dysplasia, but is - Non surgical treatment: associated with high morbidity and mortality (worth it?)  Injection of botulinum toxin (problem is that it is not long lasting and only used - Possibility of endoscopic mucosal resection as a treatment modality (research in patients not fit for surgery) still undergoing)  Pneumatic balloon dilatation (about 65% of patients improve, 40% response rate at 5 years) - Surgical treatment  Laparoscopic Heller cardiomyotomy (much like Ramstedt for pyloric stenosis) – good results with 85% symptom-free after 5 years; there is a 3% chance of developing reflux  addition of fundoplication helps prevent this 21 UPPER BLEEDING GIT AND ITS CAUSES 3. Aetiolog ical clues Gastric ulcer/gastritis/erosions APPROACH TO BLEEDING UPPER GIT - Any history of dyspepsia, gastric ulcer (any OGD done in the past showing these problems? On any “gastric” medications?) CAUSES - Any drugs that may predispose – NSAIDs, antiplatelets, steroids, anticoagulants, 1. Peptic ulcer disease (bleeding peptic ulcer) TCM 2. Gastro-oesophageal varices Varices 3. Gastritis, gastric erosions - Any history of chronic liver disease 4. Mallory-Weiss tear Mallory-Weiss tear 5. Gastric malignancy - Binge-drinking with subsequent severe retching and vomiting leading to 6. Rare causes: AV malformation (Dieulafoy lesion), aortoenteric fistula haemetemesis Malignancy HISTORY (if patient is stable) - Recent constitutional symptoms e.g. LOA, LOW, malaise 1. Nature of bleeding - Early satiety - Dyspepsia Haematemesis - Can be fresh red blood as in variceal bleeding, Mallory-Weiss tear, AV 4. Complications malformation - Symptoms of anaemia: postural giddiness, shortness of breath, lethargy, - Coffee grounds vomitus is altered blood (due to gastric acid) and can come from decreased effort tolerance, palpitations, chest pain gastric ulcer, gastritis/erosions, or variceal blood that has entered the stomach - May even be having AMI if it’s an old patient with history of IHD Malaena 5. Comorbidities - Altered blood; malaena indicates bleeding from the upper GIT i.e. above the  ligament of Treitz - Elderly patient (>60) high risk - Other comorbidities: liver disease, renal disease, IHD  high risk - Different types of malaena: (a) Fresh malaena – jet black with sheen, tarry, non-particulate (almost liquid in consistency) PHYSICAL EXAMINATION (b) Stale malaena – black-grey, dull, mixed with normal stool, occasionally 1. Vitals! particulate - Blood pressure, heart rate stable? Any postural hypotension? (Tachycardia is an (c) Iron stool – greenish hue on rubbing between gloved fingers, particulate. early sign of shock) - If gloved f inger is stirred in a cup of water, malaena will “dissolve” completely - Patient’s conscious level – confused? with no sedimentation and turn the water black, but iron stool will sedimentate - Compare current vitals with vitals in ambulance, ED – is there a worsening trend? and turn the water green 2. General inspec tion Frank PR bleeding - Pallor - Very brisk upper GI bleed can present as frank PR bleeding as blood passes - Cold clammy peripheries  impending shock down so fast it doesn’t get altered - Stigmata of chronic liver disease

2. Amoun t of blood 3. Abdomen - If patient is having haematemesis, ask how much blood  Cup? Bowl? - Any tenderness (not very helpful) 4. Digital rectal examinatio n - Malaena or frank blood IMMEDIATE MANAGEMENT VARICEAL BLEEDING 1. Resuscitation PATHOPHYSIOLOGY - Protect airway, supplemental oxygen, 2 large-bore IV cannula in antecubital A result of portal hypertension (i.e. portal venous pressure >20 cmH O or >12 mmHg – fossa 2 normal should be 7-14 cmH O or 5-10 mmHg) - Take blood for investigations: FBC, U/E/Cr, PT/PTT, LFT, GXM 4 pints 2 - ECG to detect any acute myocardial ischaemia/infarction - 1 pint N/S over half to one hour if patient is in shock, followed by more fluids if WHEN TO SUSPECT VARICEAL SOURCE IN UBGIT necessary (be wary in patients with renal failure, heart failure) - Previous history of variceal bleed - Packed cells if Hb is less than 10, to keep Hb above 10g/dL - Chronic alcohol intake - Jaundice or stigmata of chronic liver disease - May consider platelets if patient is on antiplatelet medication (qualitative defect in platelets) - FFP if patient is on anticoagulants or PT/PTT prolonged (+ vitamin K) MANAGEMENT OF VARICES can be divided into three categories: 1. Acute bleeding 2. Adjuncts 2. Prophylaxis - NG tube if patient is having haematemesis – prevents aspiration, allows gastric 3. Chronic management lavage prior to OGD (DO NOT insert if suspecting varices) - Catheterisation – monitor input/output balance especially in elderly patient or I. ACUTE BLEEDING – MANAGEMENT when large amount of fluid resuscitation required, or anticipating surgery 1. Resuscitate - IV omeprazole 80mg bolus (increases stomach pH and stabilises clot formation) - Airway, breathing, circulation - If suspecting varices – IV somatostatin/octreotide, IV antibiotics, vitamin K - If patient appears well, look for early signs of shock – tachycardia, postural hypotension 3. Close monitoring - Look at hydration status - Monitor for:  Increase in heart rate 2. Ass ess mental state  Decrease in BP - If patient has altered mental state (encephalopathy)  need to protect airway  Decrease in urine output (may require intubation)  Increasing confusion and lethargy 3. Vascular access, fluids/blood resuscitation, and blood investigations 4. Emergency oesophagogastroduodenoscopy - 2 large-bore IV cannula in proximal veins (cubital, EJV, IJV) - Indications: - Send bloods – GXM, FBC, U/E/Cr  Shock (resuscitated) - Infuse fluids  Ongoing BGIT - Under-resuscitate in variceal bleed (cf ulcer bleed) to keep Hb around 9, as  Suspected variceal bleed enthusiastic transfusion can increase portal pressure and cause more bleeding

- Role of endoscopy 4. Management of severe bleeding  Identify source of bleeding, confirm diagnosis - If patient is hypotensive and bleeding is still continuing – may require use of  Therapeutic interventions – injection of ulcer, ligation/sclerotherapy for Sengstaken-Blakemore tube. varices - Protect airway before inserting tube. - Inflate gastric balloon and pull upwards against cardioesophageal junction (balloon will press on perforator veins entering oesophagus from stomach, and thus decrease oesophageal variceal bleeding); oesophageal balloon is not inflated nowadays 23 5. IV som atostatin/oc treotide If bleeding is no t remediable by endoscopic intervention: - Not given in ulcer bleed; mode of action is as a splanchnic vasoconstrictor which - Insert Sengstaken-Blakemore tube (only temporary) and repeat endoscopy 10-12  decreases portal blood flow and hence portal pressures decreased variceal hours later bleeding - Also acts indirectly to inhibit secretion of gut hormones that increase portal - Radiologically guided insertion of transjugular intrahepatic porto-systemic shunt blood flow (TIPSS)

6. Acid suppression - Shunt surgery - Increasing intragastric pH increases clot stability, aids haemostasis  Portocaval shunts (joining portal vein to IVC) – side-to-side, end-to-side - Agents available: omeprazole, esomeprazole, pantoprazole, etc.  Mesocaval shunts (joining superior mesenteric vein to IVC)  Proximal splenorenal shunt (splenectomy with end-to-side anastomosis of portal 7. Antibiotics side of splenic vein to left renal vein) - Not given in ulcer bleed  Distal splenorenal shunt (Warren-Zeppa shunt – splenic vein divided and splenic - Studies have shown that cover with broad spectrum antibiotics (with Gram neg side anastomosed end-to-side to left renal vein) cover) decreases infectious complications, possibly mortality, and also risk of recurrent bleed - Sugiura procedure (last resort): splenectomy, proximal gastric devascularisation, - Preferably started before endoscopy (procedures increase bacteraemia) selective , pyloroplasty, oesophageal devascularisation, oesophageal transection 8. Endoscopy - Purpose: confirm diagnosis and institute management II. PRIMARY PROPHYLAXIS OF VARICEAL BLEEDING - Needs to be done emergently (on that night of admission) as soon as patient is stabilised since bleeding can be torrential and life-threatening Use of non-selective beta-blocker e.g. propranolol can be used to prevent development - Banding is the best modality for stopping oesophageal variceal bleeding of varices in patients without varices, and can decrease the size of and prevent bleeding (sclerotherapy is associated with higher morbidity e.g. mucosal ulceration) from varices in patients who already have them. In patients with small varices with no - Gastric varices are usually too large to be banded, sclerotherapy used instead risk of bleeding, the use of propranolol is of questionable benefit – repeat OGD to monitor varices. 9. Observation - Continue antibiotics and omeprazole Predictors of variceal haemorrhage: - Continue somatostatin up to the point where haemostasis is achieved or 5 days 1 Site: varices at the gastro-oesophageal junction have the thinnest coat of supporting (exact ideal duration not well studied) tissue and are at highest risk of rupture a nd bleeding - Anticipate complications: (a) encephalopathy – fleet and lactulose, treat hypokalaemia from vomiting 2 Size: F1: Small straight varices (b) aspiration – protect airway; ?benefit of gastric decompression using NG tube F2: Enlarged tortuous varices that occupy less than one-third of the lumen (c) risks of procedure – OGD-related risks F3: Large coil-shaped varices that occupy more than one-third of the lumen 3 Child’s score – patients with higher Child’s score have higher risk 10. Secondary prophylaxis - Best option is combination of band ligation and non-selective beta-blockers e.g. 4 Red signs: Red wale marks (longitudinal red streaks) propranolol unless propranolol is contraindicated Cherry red spots (flat discrete spots) Haematocystic spots (raised discrete spots – resemble “blood blisters”) 11. Management of possible precipitants Diffuse erythema - NSAIDs; Hepatic vein thrombosis 5 Previous variceal haemorrhage: 70% of patients will have further variceal bleeds after an index bleed 30% rebleed within 6 weeks (risk highest in first 48 hours after first bleed); 30% rebleed after 6 weeks III. CHRONIC MANAGEMENT - H. pylori causes a local inflammatory reaction and secretes enzymes that break down the gastric mucosal barrier, and also enhances gastric acid secretion and decreases - Start patient on an ablation regimen (endoscopy with initial ligation/sclerotherapy bicarbonate production and subsequent endoscopic monitoring and repeated ligation/sclerotherapy as required to completely ablate varices) - NSAIDs impair mucosal prostaglandin production (through non-selective COX inhibition)  prostaglandins are important for mucosal bicarbonate and mucin - If patient bleeds again  failed ablation  consider surgery (as above – shunts, or production and inhibiting gastric acid secretion, as well as maintaining mucosal blood Sugiura) flow

PRESENTATION 1. Incidentally detected on OGD PEPTIC ULCER DISEASE 2. Symptoms of dyspepsia EPIDEMIOLOGY (a) Ulcer-like dyspepsia: pain in the upper abdomen is the predominant symptom - Incidence about 100 per 100,000 per year (b) Dysmotility-like dyspepsia: non-painful discomfort in the upper abdomen, - 68% of patients are over 60 years of age associated with upper abdominal fullness, early satiety, bloating, belching, - Overall mortality is 7-10%, unchanged for last 2 decades – mostly due to ulcer nausea bleeding especially in elderly with significant comorbidities (c) Unspecified dyspepsia - Pain is usually worse with food in a gastric ulcer, while it is relieved by food in a MAIN AETIOLOGICAL FACTORS duodenal ulcer

H. pylori 3. Bleed - 60% of population are positive for H. pylori by age 21 - As above, presenting with haematemesis (coffee-grounds vomitus) or malaena - About 10-20% of infected patients develop an ulcer - Accounts for 90-95% of duodenal ulcers, and 50% of gastric ulcers 4. Perforation - Patient presents with sudden generalised abdominal pain that is aggravated by NSAIDs even the slightest movements - Accounts for most of the rest of ulcer disease not caused by H. pylori - Board-like rigidity, guarding will be present on examination (signs of peritonism) - 30% of patients on NSAIDs will get an ulcer, of which one-fifth will have a clinically - Erect CXR will show air under diaphragm significant ulcer i.e. symptomatic, bleeding

Other factors ENDOSCOPY (OGD) - Cigarette smoking - The most important and valuable investigation - Alcohol - Steroids and anticoagulants do not increase the risk of ulcer formation, but increase - Roles of endoscopy: the risk of bleeding in an existent ulcer (a) Diagnosis  Confirmation of ulcer disease PATHOGENESIS  Location of ulcer - An imbalance between mucosal protective mechanisms against acid, and aggressive  Biopsy to rule out malignancy (usually 6 bites) forces that damage the gastric mucosa  Biopsy of antral tissue for CLO (Campylobacter-like organism) test for H. - Aggressive forces: gastric activity and pepsin activity pylori - Protective mechanisms: mucus secretion, bicarbonate secretion into mucus, robust mucosal blood flow to remove protons, epithelial regenerative capacity, prostaglandin secretion by mucosa to maintain blood flow 25 (b) Pro gnos tication of bleeding risk (in UBGIT) SURGICAL MANAGEMENT  Forrest classification (or endoscopic stigmata of recent haemorrhage – ESRH) D UODENAL ULCER Forrest grade Bleeding risk Indications for surgery: 1a Spurting (arterial) 90% 1. Persistent bleeding (e.g. erosion of a posterior duodenal ulcer into gastroduodenal 1b Non-spurting, ooze (venous) 20% artery) 2a Non-bleeding ulcer with visible vessel 40% 2. Perforation 2b Non-bleeding ulcer with adherent clot 20% 2c Ulcer with haematin-covered base (flat spot) 10% 3. Gastric outlet obstruction (patient presents with vomiting of undigested food not 3 Ulcer with clean base 5% long after meal, succussion splash, air-fluid levels on AXR; characteristic electrolyte abn of hypokalaemic hypochloraemic metabolic alkalosis with paradoxical aciduria) (c) Endot herapy (in UBGIT) 4. Failure of medical management (ulcer does not heal)  Injection with adrenaline (1:10,000) or absolute alcohol  Thermal coagulation (heater probe) Surgery:  Haemostatic clipping (endoclip) 1. Oversewing the bleeding vessel  Argon plasma coagulation 2. Vagotomy with gastric drainage procedures - Rationale for vagotomy is to eliminate direct cholinergic stimulation to gastric CONSERVATIVE MANAGEMENT secretion; parietal cells also become less responsive to histamine and gastrin, and vagal stimulus for gastrin release is abolished 1. Gastroprotection - Vagotomy can be truncal, selective, or highly selective (a) Standard dose proton pump inhibitor - Drainage procedures usually done with vagotomy as gastric emptying is  20mg OM decreased with denervation  gastrojejunostomy or pyloroplasty  Promotes ulcer healing even with ongoing NSAID use 3. Antrectomy with truncal vagotomy (b) Double dose famotidine 4.  40mg BD  Inferior to omeprazole as famotidine only promotes ulcer healing if NSAIDs 5. Omental patch repair is sufficient for small perforated ulcer are stopped; ulcers will not heal with ongoing NSAID therapy Perforated ulcer: IV fluids, IV antibiotics, PPI, surgery (patch repair)

2. H. pylori eradication  First line triple therapy: omeprazole 20mg BD, amoxicillin 1g BD, G ASTRIC UL CER clarithromycin 500mg BD for 7 days Indications for surgery  In penicillin-allergic patients, substitute amoxicillin with metronidazole 400mg 1. Failure to heal after 3 months of conservative therapy BD 2. Dysplasia or carcinoma  Document eradication by endoscopy with CLO test, or stool 3. Recurrence serology testing 4. Perforation, persistent bleeding  Treatment failure occurs in up to 20% - treat with quadruple therapy: colloidal Surgery bismuth subcitrate 120mg QDS, tetracycline 500mg QDS, metronidazole 400mg 1. Oversewing the bleeding vessel BD, omeprazole 20mg BD for 7 days 2. Gastrectomy 3. If prepyloric ulcer, can treat similar to duodenal ulcer Re-scope in 6 weeks to docum ent ulcer healing If ulcer still present, biopsy ulcer again (exclude malignancy for gastric ulcer) and also do antral biopsy for CLO test (to confirm eradication of H. pylori) GASTRIC CARCINOMA 5. H. pylor i infection - 3-6X increased risk of gastric cancer EPIDEMIOLOGY - Fourth most common cancer in males, sixth most common in females in Singapore HISTOLOGY - Female to male ratio 2:1 - Incidence 10-18 per 100,000 per year Adenocarcinomas - Incidence increases steeply after 50 years old - Make up 90-95% of stomach tumours - Lauren classification: RISK FACTORS (a) Intestinal type (most common overall) – occurs in high risk population, distal 1. Environmental third of the stomach, in older men; associated with erbB2 and erbB3 receptor - Diet: preserved foods (nitrosamines), smoked foods, polycyclic hydrocarbons stimulation - Smoking (b) Diffuse type – occurs in low risk population, proximal third and cardio- - Alcohol oesophageal junction, in younger and female patients; more aggressive, present - Occupational exposure: asbestos, heavy metals, rubber later, worse prognosis; associated with K-sam oncogene - Low socioeconomic status - Early gastric cancer  2. Genetic Confined to mucosa and submucosa - Blood type A  Good survival and prognosis regardless of size, lymph node status, histological - HNPCC – Lynch syndrome II grade - P53 mutation Non-adenocarcinoma - Germline mutation of e-cadherin - Make up less than 10% of stomach tumours - Family history of gastric cancer - Types: SCC, neuroendocrine tumour, leiomyosarcoma, GIST, primary gastric non- Hodgkin’s lymphoma (MALT, linitis plastica ) PRECURSOR CONDITIONS 1. Partial gastrectomy for benign disease with Bilroth II reconstruction - Usually occurs >15 years after surgery MORPHOLOGY - Due to chronic exposure of gastric mucosa to biliary, pancreatic and intestinal Borrmann’s classification: secretions at the anastomotic zone - Type I (3%): Nonulcerated, polypoid, growing intraluminally

2. Gastric polyps - Type II (18%): Ulcerated, circumscribed with sharp margins - Highest risk in inflammatory polyps: 75-90% - Type III (16%): Ulcerated, margin not sharply circumscribed - 10-20% risk in adenomatous polyps especially in large polyps (>2cm) or those - Type IV (63%): Diffuse, infiltrating, may be ulcerated; may diffuse entire stomach with villous histology (linitis plastica) - Also increased risk of adenocarcinoma elsewhere in the stomach

3. Chronic atrophic gastritis LOCATION - Hypertrophic gastritis (Menetrier’s disease) – inflammatory disease of gastric - 30% in pyloric channel or antrum epithelium, up to 10% risk of malignant change - 20% in body - Pernicious anaemia – autoantibodies to parietal cells with achlorhydria, 2-10% - 37% in cardia risk of gastric cancer - 12% in entire stomach 4. Peptic ulcer disease - <1% risk of malignant change 27 SPREAD 6. CT scan – good for T and N staging - Direct extension to neighbouring organs 7. Staging prior to operation – picks up small peritoneal metastases that - Lymphatic spread are occult on CT scanning (up to one-fifth of patients whose disease was thought to (a) Regional nodes be resectable)  change in stage of disease (b) Supraclavicular nodes (Virchow’s node) (c) Umbilical (Sister Joseph’s node) STAGING - Haematogenous spread – liver, lung, bone, brain Tis Carcinoma in situ N0 No regional LN - Peritoneal seeding to omentum, parietal , ovaries (Krukenberg’s tumour), T1 Tumour limited to mucosa and submucosa N1 1-6 regional LN involved or cul-de-sac (Blumer’s shelf) T2 Tumour invades muscularis mucosa N2 7-15 regional LN involved T3 Tumour penetrates serosa N3 >15 regional LN involved PRESENTATION T4 Tumour invades adjacent structures Very non-specific symptoms and signs: - Abdominal pain 60% CURATIVE TREATMENT - Weight loss 50% - Nausea/vomiting 40% S URGERY - Anaemia 40% Principles of surgery: - Palpable mass 30% - Wide resection of the tumour to negative margins (at least 6cm margins) - Haematemesis/malaena 25% - En-bloc excision of regional lymph nodes - Early satiety 17% - Choice between total gastrectomy and subtotal gastrectomy - Metastatic symptoms late (bony tenderness, neurological deficits, etc)  Subtotal gastrectomy leaves a small portion of proximal stomach – easier to  New onset dyspepsia at age>35 years old should cause concern anastomose to jejunum than oesophagus since oesophagus does not have serosa (higher risk of leak)  COMPLICATIONS Subtotal gastrectomy is associated with less morbidity, better functional outcome (some residual reservoir function preserved) - Bleeding  Total gastrectomy is the resection of choice for proximal tumours (fundus, cardia, - Gastric outlet obstruction  vomiting (dehydration, hypokalaemic metabolic body) as well as diffuse-type tumours and cardio-oesophageal junction tumours alkalosis, aspiration) - Perforation - Reconstruction - Malnutrition  Bilroth I (end-to-end gastroduodenostomy) – rarely done as it is difficult to mobilise duodenum up to anastomose with residual stomach  INVESTIGATIONS Bilroth II/Polya (gastrojejunostomy) – no protection against biliary reflux into stomach Diagnosis by OGD – best for visualisation and biopsy (usually an ulcer with heaped-  Roux-en-Y – to prevent biliary reflux; but involves 2 anastomoses, higher up edges) chance of leak  Supportive/staging investigations Oesophagojejunostomy (after total gastrectomy) 1. FBC – low Hb Complications of gastrectomy: 2. U/E/Cr – if vomiting, low potassium, low chloride, alkalosis Early 3. LFTs – albumin as a marker of nutritional status (alb<35 is poor); liver mets 1. Bleeding 4. CXR – lung mets 2. Infection 5. Endoscopic ultrasound – gold standard for T staging and good for N staging 3. Anastomotic leak Late PALLIATIVE THERAPY 1. Early satiety - For palliation of symptoms such as pain, bleeding, obstruction 2. Retained antrum syndrome - Endoscopic laser ablation for obstruction - Not enough antrum removed leads to increased acidity in residual stomach, with - Embolisation for bleeding formation of marginal ulcers on the jejunal side of the anastomosis - Surgical options: subtotal gastrectomy (6-15% mortality), total gastrectomy (20-40%), gastrojejunostomy for obstruction 3. Intestinal hurry - External beam radiotherapy for pain, low-level ongoing bleeding (not for heavy  - Inadequate reservoir function leads to poor digestion may have phytobezoar bleeding as it takes weeks to cause fibrosis) formation 4. Dumping syndromes PROGNOSIS - Early dumping syndrome: due to increased osmotic load in bowel occurring half to one hour after meal, resulting in flushing, palpitations, dizziness, nausea; - Stage I 90% 5-year survival treat by eating small frequent meals with low carbo and high protein/fat - Stage II 70% - Late dumping syndrome: reactive hyperinsulinaemia with hypoglycaemia; treat - Stage III 40% by eating more carbohydrates - Stage IV 0% 5. Biliary/intestinal reflux into stomach - Leads to symptoms of dyspepsia 6. Afferent limb syndrome - Occurs in Bilroth II/Polya reconstruction - Mechanical obstruction of the afferent jejunal loop due to kinking, anastomotic narrowing, or adhesions  postprandial epigastric pain with non-bilious vomiting - Can be decreased by doing Roux-en-Y surgery (but may still occur) 7. Nutritional deficiency - Iron deficiency – mixed picture (a) Loss of intrinsic factor  B12 deficiency (b) Decreased conversion of iron from Fe3+ to Fe2+ by gastric acid  decreased iron absorption in terminal ileum - Need to supplement with B12 injections and iron supplements

C HEMOTHERAPY /R ADIOTHERAPY Adjuvant therapy 5-fluorouracil with chemotherapy 5-fluorouracil with epirubicin for advanced disease

Neoadjuvant therapy - 5-FU and cisplatin can be used to downstage unresectable, locally advanced disease with a significant increase in resectability (61%  79%) - For resectable disease: preoperative 5-FU, cisplatin, doxorubicin, methotrexate, followed by intraperitoneal 5-FU  improved resection rates, response rates, median survival 29 COLORECTAL DISEASES 2. Defects in DNA mismatch repair - Involved in 10-15% of sporadic cases COLORECTAL CARCINOMA - Like the APC pathway, there is accumulation of mutations, but due to a different EPIDEMIOLOGY mechanism, and without clearly identifiable morphologic correlates i.e. no Commonest cancer in Singapore men, number 2 cancer in Singapore women adenomas Peak incidence at 60-70 years of age - Due to mutations in one of the five DNA repair genes (MSH2, MSH6, MLH1, PMS1, PMS2) of which MSH2 and MLH1 are the most commonly involved in sporadic colorectal carcinomas PATHOLOGY - Almost all tumours are adenocarcinomas - Loss of DNA mismatch repair results in microsatellite instability which affects - 90% of tumours are sporadic coding or promoter regions of genes involved in cell growth such as the BAX - 8% arise in association with hereditary non-polyposis colon carcinoma (HNPCC) and gene and the type II TGF-β receptor 1% in association with familial adenomatous polyposis (APC) - Tumours that arise from this pathway have a better prognosis than tumours that - 1% arise in association with long-standing ulcerative colitis (>10 years) arise from the APC pathway

PATHOGENESIS SITE: There are 2 pathways for cancer development in the colorectal mucosa: - 25% in caecum and ascending colon - 25% in transverse colon 1. APC pathw ay (adenoma-carcino ma sequenc e) - 25% in descending colon and proximal sigmoid - Accounts for 80% of sporadic colorectal carcinomas - 25% in distal sigmoid and - Most are left-sided though there is an increasing incidence of right-sided tumours - Characterised by chromosomal instability - Stepwise accumulation of mutations in a series of oncogenes and tumour MORPHOLOGY suppressor genes: 1. Polypoid – more common in the right colon as there is more space to grow  Loss of the APC suppressor gene on 5q21 (congenitally absent in patients 2. Scirrhous – annular “apple-core” lesions, more common in the left colon with familial adenomatous polyposis – APC) is the earliest event in adenoma 3. Ulcerated formation 4. Nodular  APC is required to break down beta-catenin; with the loss of APC, beta- catenin accumulates and activates various genes in the nucleus (such as MYC and cyclin D1) which promote cell proliferation SPREAD  K-RAS (12p12) mutation follows the loss of APC – an activating mutation 1. Intramural – along bowel wall that causes the RAS to keep delivering mitotic signals and prevent apoptosis 2. Direct extension into surrounding tissues e.g. small bowel, ovary  Loss of tumour suppressor gene at 18q21 3. Intraluminal 4. Lymphatic  Loss of p53 late in carcinogenesis 5. Haematogenous – to liver, lungs - The molecular evolution of colon cancer through this pathway occurs through a 6. Transcoelomic series of morphologically identifiable stages: localised epithelial proliferation  small adenoma  large, more dysplastic adenoma  carcinoma in-situ  invasive cancer RISK FACTORS HISTORY 1. Age >50 years A) 5 things to ask about bow el habits – FACCE

2. Environmental factors - Frequency - >3x/day=diarrhoea; <2x/week=constipation - Diet: high in red meat, preserved foods (nitrosamines), low in fibre, vitamins, - Associated secretions minerals  Blood - NSAIDs may be protective against CRC o Mixed in stool? o Separate from stool? 3. Genetic predisposition  Mucus (a) Polyposis syndromes – FAP and its variants, Gardner’s and Turcot’s - Colour – black, clay, brown syndromes are associated with near 100% risk of cancer formation; other - Consistency – pellet, normal, soft, watery polyposis syndromes such as Peutz-Jegher’s, Cronkhite-Canada have a small increased malignant potential - Effort – tenesmus (b) HNPCC – more common than FAP, accounting for 8% of cancers Stool Changes 4. Ulcerative colitis - Bloody diarrhoea – ddx: - Increased risk after 10 years of disease if the patient has pancolitis; after 15-20  IBD years if the patient has disease limited to the left colon  Infective e.g. amoeba, TB, hookworm  Antibiotic Associated Colitis (C. difficile) 5. Adenomatous polyps - Increased risk if there is a personal history of large (>1cm )adenomatous polyps, - Haematochezia – ddx:  and polyps with tubulovillous or villous histology, particularly if multiple (3-6X Diverticulitis  increased risk) Angiodysplasia (AVM, common in old)  Massive upper GI - Small (<1cm) tubular polyps do not have increased risk  Hemorrhoids 6. Family history or personal history of colorectal carcinoma - Progressively worsening constipation requiring chronic laxatives - Metachronous colorectal cancers occur at a rate of 3-5% in the first five years - Spurious diarrhoea after resection of a primary CRC, while metachronous adenomas occur at a rate - Mucoid stools of 25-40% - Progressive intestinal obstruction symptoms - Family history of one first-degree relative with CRC increases risk of CRC 1.7X, - Tenesmus (rectal ca) and risk is further increased if there are 2 first-degree relatives with CRC, or if the relative had CRC before the age of 55 Other Symptoms - Family history of colonic adenoma appears to have the same significance as - Iron deficiency anaemia – fatigue, decreased effort tolerance, family history of colonic ca rcinoma palpitations, postural giddiness, shortness of breath, chest pain  Anyone with one first degree relative diagnosed with CRC younger than 45 - Abdominal mass years old, or two first or second degree relatives from the same side of the - Metastatic symptoms – bone pain family with CRC at any age, should be screened starting at age 45, or 10 years - Constitutional symptoms – LOA, LOW, malaise earlier than the youngest cancer in the family Note: Site of CA - Ascending: anaemia, no obstructive symptoms usually - Lower down: I/O, bleed, tenesmus 31 B) High-risk factors INVESTIGATIONS Positive Family history Aims - 1 or more 1st deg relative with CC at age <40 - Diagnose colorectal cancer - 2 or more 1st/2nd deg same side relative with CC at any age - Stage the cancer - Personal or family history of breast, ovarian cancer - Investigate for complications of cancer FAP history I . D IAGNOSIS Amsterdam Criteria for Hereditary Non-Polyposis Colorectal Ca (HNPCC) – (a) – first-line investigation [3, 2, 2, 1] - Can visualise lesion and have an idea of which part of the colon it is in - 3 or more family members from the same side with CRC, - Can take biopsies of the lesion - At least 2 of which must be first-degree relatives - Enables detection of synchronous lesions – synchronous polyps in 30%, - 2 successive generations synchronous cancer in 3-5% - 1 of the CC must occur prior to age 50 - Enables therapeutic procedures e.g. polypectomy, stenting of obstructed colon - FAP is excluded  Family history alone considered high-risk; FAP and HNPCC considered very (b) Double-con trast barium enema (barium + air) high-risk - Not adequate for diagnostic purposes, may miss small lesions - Classically can see an apple core lesion with barium enema C) Other risk factors (c) Carcinoem bryon ic antigen level (CEA) - Low fibre diet - A tumour marker for colorectal carcinoma – >90% of tumours produce CEA - Obesity - Red meat - Measured pre-operatively as a baseline level – if the CEA is raised pre-op and - Ulcerative colitis falls to within normal range post-op, it is likely tumour has been totally removed - Follow-up after surgery with CEA levels to detect tumour recurrence D) Complications - Causes of false positive raised CEA: smoking, pregnancy, bronchitis, cholangitis - Haemorrhage (usually occult)  anaemia and cancers of the stomach, lung, breast, pancreas, cervix, bladder and kidney - Obstruction (left sided lesion)

- Perforation I I . S TAGING - Fistula – colovesical fistula causing faecuria, pneumaturia, recurrent UTI - Infection – abscesses, peritonitis (a) CT scan of the abdom en and pelvis - Local T staging - Staging of regional and no-regional lymph note involvement PHYSICAL EXAM - Metastases to the liver 1. Abdominal mass 2. Mass on DRE – hard, non-tender, polypoid, irregular, contact bleeding (b) Endoscopic ultrasound, or transrectal ultrasound for rectal tumour - Very good for T staging to determine depth of involvement by tumour 3. Complications, Metastases - Can also assess local lymph node status - Cachexia - Anaemia (c) CXR + CT scan of the chest - Jaundice, hepatomegaly - Lungs (d) MRI of the tumour - Brain - Superior to CT for delineating fat planes in T staging especially in rectal cancer - Bone tenderness (e) B one scan if approp riate - Cervical lymphadenopathy – Virchow’s node III. C OMPLICATIONS  While segmental resection (excision of only the segment of colon containing the (a) FB C for low Hb, together with iron studies tumour) is sufficient for primary tumour removal, a wider resection is often required to achieve sufficient lymphadenectomy (b) Urea, electrolytes and cr eatinine in patient with obstruction – may be vomiting,  may have third space losses (intraluminal) with fluid and electrolyte abnormatlities; Adequate clearance of the draining lymphatics involves excision of the vascular creatinine may be elevated due to pre-renal failure arcades supplying the segment of involved colon back to their origin (from the SMA or IMA) as lymphatics follow the arteries generally (c) Liver function tests for derangements caused by metastasis (though these changes will only occur late) – raised bilirubin, ALP - Obstructed left sided carcinoma  (d) If patient presented with symptoms of intestinal obstruction – erect and supine No difference shown for doing a staged procedure (i.e. tumour removed with AXR can help in diagnosis and location of obstruction proximal end of colon brought out as a ) as compared to creating a primary anastomosis (e) Erect CXR in perforated tumour to detect air under diaphragm  On-table bowel decompression is equivalent to irrigation for clearance of faecal material DUKE’S STAGING  Segmental for the tumour with intraoperative decompression is Stg Description 5yr surv comparable to subtotal/total colectomy without decompression with regard to A Tumor confined to bowel wall with no extension into extrarectal/ 75% bowel function and rates of complications extracolic tissue, no LN mets - Site of surgery for colonic carcinoma B Invades past muscularis propria into extrarectal/ extracolic tissue, 55% no LN C LN mets present  C1: only nearby nodes involved (paracolic LNs) C1:40%  C2: continuous string of LN involved up to proximal resection C2:20% (LN at base of mesentery) D Distant mets/ extensive local mets such that surgically incurable poor

TREATMENT

S URGERY Pre-operative measur es - Bowel prep  Modification of diet – 3 days low residue diet, NBM day before operation)  Bowel clearance with polyethylene glycol - Prophylactic antibiotics  ampi/ genta/ metronidazole at induction of anesthesia

Principles of surgery for colonic carcinomas - En-bloc resection of tumour with adequate margins  For colonic tumours, a margin of 5 cm proximally and distally is adequate 33 Tumo ur site Surgery Structures involved - Reconstruction - Caecum Right - Excision of caecum and ascending colon  Formation of a straight coloanal anastomosis in anterior resections is associated - Ascending colon hemicolectomy - Division of ileocolic and right colic arteries, and with poor function due to the lack of reservoir function the right branch of the middle colic artery  - Hepatic flexure Extended right - Excision of caecum, ascending colon, and Creation of a colonic J-pouch using the proximal end of colon (the end of the - Transverse colon hemicolectomy proximal transverse colon colon is folded back on itself to form a J, and the two limbs opened and stitched near the hepatic - Division of ileocolic and right colic arteries, and together to form a pouch, the apex of the J being anastomosed to the anus) is flexure the middle colic artery at its origin associated with improved post-operative function - Mid-transverse Transverse - Excision of transverse colon  Coloplasty is another alternative that is equivalent to colonic J-pouch (the distal colon colectomy - Division of middle colic artery colon is cut longitudinally but sewn transversely, widening the diameter at that - Transverse colon Left segmental - Excision of distal transverse colon and proximal segment to form a small pouch), done when there is difficulty creating the near splenic colectomy descending colon colonic J-pouch flexure - Division of supplying vessels – left branch of middle colic artery, left colic artery - Mesorectal excision - Descending colon Left - Excision of descending colon  Proximal rectal tumours – 5cm distal margin of mesorectal excision hemicolectomy - Division of left colic and inferior mesenteric arteries  Mid-rectal tumours – wide mesorectal excision of at least 4cm distal to the - Sigmoid colon Sigmoid - Excision of sigmoid colon tumour colectomy - Division of inferior mesenteric artery and/or  Lower rectum tumours – total mesorectal excision required (complete excision sigmoid branches of the intact visceral mesorectal tissue to the level of the levators)

- Extended resections  Principles of surgery for rectal carcinomas For locally advanced, adherent tumours (T4), multivisceral resection of organs involved (pelvic exenteration) is associated with improved local control and - En-bloc resection with adequate margins overall survival compared with standard resection, though high morbidity of 25-  For rectal tumours a margin of 5 cm proximally and 2 cm distally is adequate (as 50% is associated it has been found that lymphatic spread of rectal tumours is predominantly in the  Consider neoadjuvant chemoradiotherapy prior to surgery to downstage disease proximal direction)  Radial margins are also important as there is a zone of downward spread within - Stoma creation the mesorectum (peritoneal investment of the upper rectum) – for upper rectal  A defunctioning loop (or loop c olostomy) is usually created during an tumours, mesorectal excision of 5 cm distal to the distal margin of the tumour is anterior resection as the manipulation of the colon deep within the pelvic cavity adequate (see Mesorectal excision below) causes increased risk of an anastomotic leak  - Sphincter-sparing versus loss of sphincter A defunctioning stoma does not protect against anastomotic leak, but mitigates against disastrous complications should a leak occur  The anal sphincter can be spared if the distal margin is >2cm above the level of the sphincter complex, usually taken to be at the level of the dentate line (which - Neoadjuvant chemoradiotherapy is 5cm above the anal verge) i.e. distal margin of the tumour must be >7cm from  Neoadjuvant therapy with radiotherapy in combination with 5-fluorouracil can the anal verge downstage tumour significantly  ability to preserve sphincter, ability to resect  Sphincter-sparing surgery involves a low anterior resection previously unresectable tumour, etc  If the tumour is so low that it cannot be resected without removing the sphincter complex, then an abdominoperineal resection is performed where the entire anus and sphincter complex is dissected, with the creation of an end colostomy - Surgical treatment according to stage Surgery for m etastases - Isolated liver metastases (synchronous or metachronous) may be resected with Stage of disease Treatment survival benefit; neoadjuvant chemotherapy can be given to downstage the T1 Involvement of submucosa, but no Local excision (AR or APR) metastases if they are initially resectable penetration through muscularis propria - Lung metastases usually indicate systemic dissemination of disease, but in the rare T2 Invasion into, but not penetration a) Local excision + adjuvant setting that there is an isolated lung secondary, resection can provide survival benefit through, muscularis propria Chemo/RT OR b) radical resection Surgery for recurrence T3 Penetration through muscularis propria Neoadjuvant chemo / RT before - Loco-regional recurrence, if detected early with adequate resection, can confer into subserosa (if present), or pericolic radical resection survival benefit fat, but not into peritoneal cavity or

other organs R ADIOTHERAPY T4 Invasion of other organs or - Role as neoadjuvant therapy in rectal cancer to downstage tumour involvement of free peritoneal cavity - Post-operative adjuvant therapy in stage II or III rectal cancer

C HEMOTHERAPY Operative com plications Adjuvant therapy Immed iate (<24h) Damage to other organs e.g. ureters - Colon cancer: 5-FU + folinic acid (leucovorin) for 6 months, or 5-FU + levamisole for 12 months in Duke’s C cancer (node positive); not recommended in Duke’s B or Early (<30 days) Wound infection less Bleeding - Rectal cancer: post-operative adjuvant therapy in combination with radiotherapy in Abscess stage II or III disease (5-FU based regimen used) Anastomosis breakdown/leak Early stoma complications Palliative therapy Late (>30 days) Diarrhoea - 5-FU in combination with folinic acid is first-line therapy Impotence (damage of pelvic nerves) - Alternatives for first-line therapy: Raltitrexed when 5-FU is not tolerated; Adhesions (I/O) capecitabine or UFT (uracil combined with tegafur) plus folinic acid Anastomotic stricture Late stoma complications FOLLOW UP - Follow-up visits 3-monthly for the first 2 years, then 6-monthly for the next three years, and subsequently yearly, measure CEA at each visit Surgery for palliation - Yearly colonoscopy - Resection of primary for palliation of symptoms such as bleeding, perforation, - CXR and liver ultrasound to detect metastases (recommended frequency not known) obstruction or pain - Resection of asymptomatic primary is controversial, but may confer survival benefit in a select group of patients where metastatic tumour burden is restricted to one side and liver involvement is not extensive 35 STOMA PRINCIPLES Stoma Complications

Nursing intervention Early - Necrosis of terminal bowel (stoma appears dusky; double check by passing a - Stoma nurse to counsel, discuss best site for stoma placement proctoscope into the stoma to look at colour of mucosa)  refashion stoma  Stoma siting - Obstruction e.g. fecal impaction explore with finger, enema - Siting of a stoma over the rectus sheath decreases the risk of prolapse, but care must - Leakage between skin and appliance causing skin erosion  resite also be taken not to site it too near a midline surgical incision due to fears of wound Late contamination and infection - Prolapse of bowel  refashion - Should be sited away from skin creases or bony prominences such that stoma wafer - Parastomal hernia  refashion can be attached flush with the skin (otherwise there are gaps between skin and wafer - Stenosis  refashion  leakage of fluid) - Retraction  refashion - Sited away from old surgical scars (hernia risk) - Psychological problems - Sited where the bag will be easily accessible and visible to the patient i.e. not under a large fold of abdominal fat ASSOCIATED CONDITIONS - Intra-operatively, the bowel that forms the stoma must not be overstretched – tension causes decreased vascularity of the stoma and may cause stoma necrosis I. Familial adenomatou s polypo sis (FAP)

Types of stomas - 1 in 10,000, autosomal dominant inheritance - Germline mutation of APC gene on 5q21 Permanent (end colostomy) - >100 adenomatous polyps all over colon; polyps take 5-6 yrs to turn malignant - When no distal bowel remaining - 50% patients will have polyps by 16yrs; 90% will have colorectal CA by 45yrs  Low rectal/ anal tumor requiring abdomino-perineal resection  Panproctocolectomy without ileal pouch anal anastomosis e.g. FAP - Other sites for polyps: stomach, duodenum  Usually sited on the left side with a single opening - Extraintestinal manifestations  Epidermoid cysts Temporary  Lipoma - Decompression – relief of large bowel obstruction causing proximal dilatation  Osteoma of skull, mandible - Defunctioning – to protect a distal bowel anastomosis  Dental abnormalities  Previously contaminated bowel  Congenital hypertrophy of retinal pigment epithelium (CHRPE)  Technical considerations e.g. after low anterior resection, where risk of  Desmoid tumours (intraabdominal tumours, treated with chemo, RT or hormonal anastomotic leakage is high Rx. Not treated by surgery)  Usually loop or c olostomies with 2 openings (ileostomies usually on  Follicular or papillary thyroid cancer the right side, in the epigastric/hypochondriac [transverse colostomy]  Periampullary CA – requires 5 yearly OGD for surveillance of CA. or left side) - Diagnosis - To rest an inflamed distal portion e.g. acute Crohn’s  Colonoscopy showing >100 polyps  Genetic testing Note: Colonic stoma is usually flushed with skin, while ileal stoma protrudes 3cm (a ‘spout’, as ileal contents are corrosive, to prevent contact with skin) - Surveillance  Yearly colonoscopy for at-risk family members from 12y onwards  Genetic testing of at-risk family members  Affected members should undergo prophylactic proctocolectomy with ileal pouch anal anastomosis (IPAA – involves folding loops of ileum back on themselves and stitching or stapling them together to form a reservoir pouch PATHOGENESIS which is them anastomosed to the anus) at ~ 20 YO 1. Increased intralum inal pressu re  Subtotal colectomy is an option if the rectum is relatively spared of polyps - Associated with lack of dietary fibre, constipation 2. Degenerative chang es in colon ic wall Hereditary Non-Polypo sis Colorect al CA (HNPCC) - Usually at point of entry of terminal arterial branches where serosa is weakest - Associated with weakening of collagen structure with age - Divided into Lynch syndrome I or Lynch syndrome II based on clinical features

- Tumours usually proximal to splenic flexure (~70% proximal to splenic flexure) PRESENTATIONS - Tumours tend to arise from polyps which are commonly flat, with villous histology 1. Acute diverticulitis - Resultant tumour is often poorly differentiated - LLQ pain - Lynch syndrome type II is differentiated from Lynch syndrome I as it is associated - Tender palpable mass with increased risk of cancer elsewhere, most commonly endometrial cancer, and - Low grade fever also gastric, ovarian, small bowel, hepatobiliary, and renal pelvis/ureter cancers - N/V - Constipation / diarrhoea - Diagnosis is based on the Amsterdam criteria – see above -  WBC - Surveillance – 1-3yrly colonoscopy starting at 20 years old 2. Chronic diverticulitis - Recurrent LIF pain Ulcerative colitis - Irregular bowel habit - Passage of mucus PR - Screening – yearly colonoscopy starting after 10 years of UC 3. Complicated diverticulitis a. Perforation b. Paracolic abscess / inflammatory mass – 2o to localized perforation c. Bowel obstruction – 2o to structure or adherence to a diverticular mass d. LGIT haemorrhage – ulcerated vessel @ neck of diverticulum o DIVERTICULAR DISEASE e. Fistula formation (commonest: colovesical fistula) – 2 to pericolic abscess discharging, operation or drainage of pericolic abscess. May present with PATHOLOGY – acquired herniation of colonic mucosa through muscular wall, with a urinary symptoms. Others – colo-cutaneous, colo-uterine, colo-enteric, colo- covering of colonic serosa vaginal

STAGING TERMS - Hinchey classification of acute diverticulitis – need for surgery is reflected by - Diverticulosis coli – presence of acquired pseudodiverticula degree of infective complications - Diverticular disease – symptomatic diverticulosis coli - Diverticulitis – inflammation of diverticula Stage 1 Pericolonic / - ABx, NBM, IV fluids Mesenteric abscess - Consider 1 stage surgery after acute episode – resection of affected bowel segment with primary EPIDEMIOLOGY anastomosis - Increases with age Stage 2 Pelvic / retroperitoneal - Percutaneous drainage - 10-30% of diverticulosis coli are symptomatic abscess - Elective 1 stage surgery - Risk factors – dietary fibre & genetics Stage 3 Purulent peritonitis - 2 stage operation – Hartmann’s procedure (partial - Site – majority are in the sigmoid colon (dec. diameter, inc. pressure) Stage 4 Faecal peritonitis colectomy + diverting end colostomy & rectal stump formation) + secondary re-anastomosis 3 months later 37

Presentation Clinical features Investigations Differentials Management Acute - LIF pain – colicky, progressing to - FBC – leucocytosis - Acute salpingitis Conservative Diverticulitis constant, relieved by defecation - ↑ ESR - Acute appendicitis - Bed rest - LIF tenderness - AXR – ileus, air-fluid level w/in an abscess - GE - NBM, IV fluid - Palpable LIF mass - Barium enema - Irritable bowel syndrome - Broad-spectrum antibiotics – - Nausea - ± CT or U/S: thickened bowel wall, pericolic fat augmentin or metronidazole or - Pyrexia inflammation, extraluminal gas & contrast, abscess, ciprofloxacin - Increase pulse rate free fluid & gas - Antispasmodics - Laparoscopy – if diagnosis is in doubt - CT scan w triple contrast is gold std for diagnosis After acute phase has settled  Contrast: IV for vascular lesions, oral for small - Ba enema &/or Colonoscopy – bowels, enema for large bowels confirm dx & exclude CA colon  Features – diverticula, mesenteric fat infiltration, concentric bowel thickening, pelvic abscess

Chronic - Recurrent LIF pain - Rigid – oedematous mucosa & - CA colon – may coexist. Hard Conservative – see above Diverticulitis - Irregular bowel habits – constipation rigidity of rectosigmoid junction to differentiate – therefore, Surgical & bouts of diarrhoea - Flexible sigmoidoscopy – diverticular orifices ALWAYS exclude CA colon Indications: - Passage of mucus PR - Barium enema – ‘saw-tooth’ appearance, e.g. histology after bowel - Severe / recurrent attacks of diverticula, strictures resection diverticulitis - Colonoscopy – exclude differentials (i.e. Ca colon) - Ischaemic colitis - Possible CA colon - Radiation colitis - Segmental resection of affected - Colonic endometriosis colon + anastomosis

Generalised - Acute onset abdominal pain – severe - FBC – ↑ TW, ↑Hb (dehydration) - Other causes of peritonitis – Mgmt as for acute abdomen peritoniti s / & continuous - U/E/Cr – dehydration & ARF perforated PUD, appendicitis - Resuscitate perforation - Abdominal guarding & rigidity - CXR – free gas under diaphragm etc - Surgical - Vomiting  Peritoneal toilet - Tachycardia  Resection of affected segment - Pyrexia  End sigmoid colostomy (Hartmann’s procedure)

Pericolic - May follow acute diverticulitis - FBC – ↑ TW - CT/US guided percutaneous abscess - LIF tenderness & guarding - CT – differentiate between inflammatory phlegmon aspiration - LIF mass – may be detected on DRE & pericolic abscess - Surgery – evacuation of pus ± - Swinging fever resection of affected segment

Persistent - LIF pain, tenderness & palpable mass inflammatory - Fever mass - Malaise

Small bowel - Usually temporary, due to I/O attachment of enteric loop against area of acute diverticulitis - Surgery if does not resolve Presentation Clinical features Investigations Differentials Management Lar ge bowel - PHx of recurrent acute diverticulitis - AXR – dilated bowels proximal to stenosis - CA colon - NBM, Drip & suck I/O or irregular bowel habit - Water soluble contrast enema - Surgery – Resection ± primary - Colicky abdominal pain, constipation anastomosis & abdo distension

H emorrhage - Usually in the elderly who have - Invx as for LGIT bleed – resus, invesigations + - Anorectal bleed - Resuscitate & correct higher density of sigmoid diverticula colonoscopy & angiography to locate site of bleed - Angiodysplasia coagulopathy - Massive bleed usually right-sided - ± on-table if required - Ischaemic colitis - Surgery – resection or radiologic - ± tagged RBC scan (not as sensitive compared to - UGIT bleed embolisation of site of bleeding – angiogram) - Colorectal CA done while doing angiography. - IBD Hence, angiogram is the preferred - Other colitis invx as it is of diagnostic AND - Coagulopathy therapeutic value

Vesicocolic - PHx of chronic diverticulitis & UTI - UFEME – pus, faecal debris, intestinal organisms - Other causes of fistula – CA - Surgery – Resection of affected fistula - Hx of dysuria, freq, haematuria, - Cystoscopy – cystitis colon, CA bladder, Crohn’s colon + anastomosis + closure of pneumaturia, faecaluria - Sigmoidoscopy – usually normal disease, post-irradiation bladder fistula opening - KUB – air in bladder necrosis - Barium enema – diseased diverticular bowel segment

Indications fo r emergency op eration 1. Sepsis 2. Perforation 3. Diverticulitis not responding to conservative management 4. Emergency bleed a. Haemodynamically unstable b. Need > 4 units of PCT c. Previous bleed 5. Obstruction – need to rule out cancer at the same time

Indications for elective operation 1. Stricture 2. Fistula 3. Recurrent attacks – occurs in 30% of patients after 1 st episode. a/w higher mortality & complication rates 4. Young patientss <40YO – high recurrence rates 5. Immunocompromised patients (e.g. renal transplant) – may not show S/S of acute attack or complications

Advice to patients: - 70% of patients will not have recurrence after first attack - Advise high fibre diet & to drink lots of fluid 39 SURGICAL LIVER PROBLEMS - Segment I is the caudate lobe - Segments II to VIII are named clockwise, while facing the patient, starting from ANATOMY OF THE LIVER the upper right corner (i.e. the upper left segment of liver) – see picture - The liver is divided into two lobes, right and left - Segment IV can be further divided into IVa and IVb, where IVa is the superior subsegment and IVb is the inferior subsegment - The anatomical division of the liver lobes is demarcated by the falciform ligament - The functional division (more practical in surgery) is demarcated by the plane of the - The liver has two blood supplies – portal vein (formed from the joining of the splenic gallbladder and inferior vena cava (also by the plane in which the middle hepatic vein and superior mesenteric vein) and hepatic artery (a branch of the coeliac trunk) vein runs) - Drainage is via the three hepatic veins into the inferior vena cava

CAUSES FOR A LIVER NODULE ON IMAGING

Benign Cyst - Single - Multiple – familial (polycystic) or non-familial Haemangioma - Small - Big Adenoma Fibronodular hyperplasia

Malignant Secondary Colorectal, stomach, pancreas, breast, urogenital tract, lung Primary Hepatocellular carcinoma (or hepatoblastoma in children) Cholangiocarcinoma (only 10% intrahepatic) – presents like HCC except no background of cirrhosis

HEPATOCELLULAR CARCINOMA

EPIDEMIOLOGY - The liver can be further divided into 8 functional segments (Couinaud segments) that - Incidence in Singapore is 18 per 100,000 per year in males, and 4.6 per 100,000 in each have their own vascular inflow, outflow, and biliary drainage, independent of females the other segments - Third most cancer among males, overall fourth most common cancer - The segments are divided by one transverse plane and three sagittal planes as shown - More common in men, with a ratio of 3:1 in the picture - Peak age of onset: 30-40 years old - The transverse plane is at the level of the main branches of the portal vein, and - Primary cancers of the liver are mainly hepatocellular carcinomas (85%), with a divides the liver into an upper half and a lower half small proportion of intrahepatic cholangiocarcinoma (6%) - The sagittal planes are formed by the three main hepatic veins (right, middle and left) AETIOLOGY AND RISK FACTORS 6. Other manifest ations - Hepatitis B infection (high HBV DNA load, HBeAg positivity increase the risk) - Budd-Chiari syndrome (occlusion of portal vein, resulting in portal hypertension) - Hepatitis C infection - Pyrexia (central tumour necrosis) - Cirrhosis (of which cirrhosis resulting from hep B, hep C and haemochromatosis are - Paraneoplastic syndromes – hypoglycaemia (high metabolic demands of tumour), associated with the highest risk) erythrocytosis (tumour produces erythropoietin), hypercalcaemia, watery diarrhoea, etc. - Aflatoxin ingestion

PATHOLOGY INVESTIGATIONS

- Pathogenesis involves a chronic inflammatory process or ongoing hepatocellular D IAGNOSIS damage with high cellular regeneration, which leads to increased rates of genetic Biopsy is usually not performed due to risk of tumour seeding (1-2% risk) along the mutation in the cells and accumulation of these mutations leading to carcinoma needle track – diagnosis is based on clinical, biochemical and radiological tests formation - Two histological subtypes: WHO criteria for HCC:  Nonfibrolamellar – associated with hep B and cirrhosis (a) Risk factors for HCC e.g. hep B/C carrier  Fibrolamellar – associated with younger patients, more common in females, no (b) Characteristic CT findings (of a hypervascular lesion) association with hep B or cirrhosis, 70% resectable, good prognosis (c) Raised AFP (>400) - Metastasises to lymph nodes, bones, lungs and adrenals

1. Alph a-foetoprotein (AFP) PRESENTATION - Elevated in 80% of hepatocellular carcinoma 1. Asymptomatic - During screening (ultrasound) for chronic hepatitis B carrier 2. Triphasic CT scan is the gold standard investigation - Investigations for liver cirrhosis - Triphasic CT involves scanning the liver at three different times after - Incidentally found on imaging of the abdomen intravenous contrast: 2. Local sympto ms (a) Arterial phase – aorta lights up as contrast fills arteries; IVC and portal - Upper abdominal pain vein are dark - Hepatomegaly found on examination (b) Portal venous phase – contrast enters portal system so portal vein is as - Obstructive jaundice due to invasion of intrahepatic biliary tree, compression of bright as the aorta a major duct (c) Delayed phase – contrast drains out, so none of the vessels in the liver are lighted up 3. Liver decompensation (on top of underlying cirrhosis) - Characteristic feature of HCC is enhancement in the arterial phase (as HCCs - Worsening liver function have a rich arterial supply derived from the hepatic arterial system) with rapid - Ascites contrast washout in the portal venous phase (hypodense) - Variceal bleeding - Encephalopathy - In a patient with hepatitis B/C and raised AFP, a liver lesion that is not a haemangioma on imaging should be considered HCC until proven otherwise 4. Tumour rupture - CT can also look for nodal involvement, and metastases to the adrenals - Abdominal pain (peritonitis) - Shock 3. Dynamic MRI scan of the liver 5. Metastases - Adjunct investigation done when CT findings are equivocal - Bone pain - Images liver in greater detail, can be used to exclude benign conditions - Dyspnoea 41 4. Hepatic angiogram with lipiodol and post-lipiodol CT scan 4. Residual liver function post-operatively - Lipiodol will be retained in HCC even after many days as the HCC does not - Dependent on tumour size and how much of the liver it takes up, because tumour contain Kupffer cells to ingest lipiodol is non-functional - Hepatic angiogram may reveal abnormal blood vessels within the HCC - A large tumour taking up most of the liver segments being resected translates to - CT scan of the liver weeks after lipiodol ingestion will pick up the areas of smaller amount of functional liver tissue being resected, while a small tumour tumour (where the pre-lipiodol CT may not have demonstrated the tumour means that more functional tissue is removed with the same resection margins clearly) 5. Degree of portal hypertension - Resection of the liver results in worsened portal hypertension since the effective 5. If indicated, investigations to look for GI primary portal venous capillary bed has decreased  increased resistance to flow - CEA, CA 19-9, endoscopy 6. Location of tumour S TAGING (looking for metastases) - Has to be located in a suitable location for resection 1. Lung – chest X-ray, CT thorax 2. Adrenals – CT abdomen - Problem in patients with cirrhosis is that there is already a “field-change” effect in 3. Bone – bone scan if clinically indicated the liver, thus a new tumour can still develop in the remnant liver - Requires a fine balance between adequate resection margins and preservation of TREATMENT sufficient functional liver to prevent liver failure - Good immediate and short-term results, but not long term (<30% 5-year survival) S URGERY due to occurrence of new primaries in the cirrhotic liver - The only curative treatment for HCC is surgical removal of the tumour - Only about 10-20% of patients with HCC will have disease amenable to surgery Transplantation - 2 surgical possibilities: - for transplantation (>75% 5-year survival if followed) (a) Resection of tumour (partial hepatectomy) (a) Single tumour 5cm or smaller, or 3 or less tumours none larger than 3cm (b) (b) No evidence of gross vascular invasion (c) No regional nodal or distant metastasis Factors affecting resectability: - Problems with availability of donor organ – the disease might have progressed past 1. Stage of disease being suitable for transplant by the time donor organ is available - Metastatic disease is not suitable for resection  Possibility of “bridging therapy” such as radiofrequency ablation to shrink - Multicentric disease affecting both lobes is a contraindication to hepatectomy disease and prevent progression until donor liver is available 2. General fitness for operation - In hepatitis B carriers, there is a risk for reinfection of the donor liver (high risk factors are HBeAg positivity, high HBV DNA levels) – can be aggressively treated 3. Liver function pre-operatively with anti-viral drugs 2 months before transplant and anti-HBV immunoglobulin long- - Cirrhotic patients have higher risk of post-operative mortality (4-14%) compared term after transplant to non-cirrhotic patients (0-4%) due to complications such as liver failure,

bleeding and infection P ALLI ATIVE THERAPY  - If patient has cirrhosis, assess the Child’s status Child’s C and most of Child’s Loco-regional ablation B will not be fit for operation; only Child’s A and good Child’s B (a) Radiofrequency ablation – best results for locoregional strategies - Use of indocyanin green (ICG) – the percentage of ICG remaining in the liver (b) Percutaneous ethanol injection after 15 minutes indicates the level of liver function. If >15% remains after 15 (c) Cryotherapy minutes, the patient cannot tolerate major liver resection (>3 segments removed) Intra-arterial therapy TRIPHASIC CT (a) Transarterial chemoembolisation - Hypodense on arterial phase (as metastases are usually hypovascular compared to (b) Transarterial embolisation hypervascular HCC) (c) Radioactive isotopes – Yttrium-90 - Increasing contrast uptake on portal venous and delayed phases

Systemic therapy – limited results ROLE OF SURGERY - Promising results with colorectal and neuroendocrine metastases if isolated SCREENING FOR CHRONIC HEPATITIS CARRIERS resectable metastatic disease – 5-yr survival >50% - Increasing role in urogenital, breast mets - Combination of six-monthly to yearly ultrasound with AFP levels - Poor results for stomach, oesophageal mets - Ultrasound alone is not very sensitive or specific as it is operator dependent and may - Palliation for symptoms in neuroendocrine metastases miss certain areas of the liver where imaging is difficult, but it is not associated with radiation exposure - AFP is also not a perfect screening test as 20% of HCC will not have raised AFP - Thus the combination of ultrasound and AFP can increase the sensitivity and LIVER HAEMANGIOMA specificity of screening EPIDEMIOLOGY - Frequency of screening is controversial, but should be increased in patients at - Prevalence 0.4-20% increased risk – HbeAg positivity, high HBV DNA levels - Female to male ratio 3:1 - Important to also screen family for chronic hepatitis B carrier status especially if there is a family history! – e.g. mother had hep B/HCC, sibling has hep B, etc PATHOGENESIS - Vascular malformation that enlarges by ectasia, congenital in origin

PRESENTATION 1. Usually small and asymptomatic, found incidentally LIVER METASTASES 2. Mass effects compressing on surrounding organs - Still more common than primary liver tumour for malignancy occurring in the liver 3. Pain from liver capsule stretch - Primaries: Colorectal, gastric, pancreatic, urogenital, breast, lung 4. Rupture (<1%) 5. Kassabach-Merritt syndrome for large haemangiomas – consumptive coagulopathy, thrombocytopaenia PRESENTATION DEPENDS ON SITE OF METASTASIS 6. Heart failure from large arteriovenous shunt Mets to liver parenchyma Mets to porta hepatis LN Hx - Incidentally found on follow up - Symptoms of obstructive jaundice DIAGNOSIS (for cancer)  Yellow sclera - Radiological - Hard mass  Tea-coloured urine Characteristic features on triphasic CT – slow enhancement of the rims on arterial - Heaviness  Pale stools and portal venous phase, brightest in the delayed phase - Pain from rupture - DO NOT BIOPSY P/E - Hard, irregular nodular hepatomeg - Jaundice early, progressive - Jaundice is a late sign - Hepatomegaly may not be present TREATMENT Invx - Both obstructive and transaminitis - Obstructive picture in early stages - Only for symptomatic or complicated lesions picture - Possible role for prophylactic surgery in large, lateral, inferior lesions since there is higher risk for rupture 43

SIMPLE LIVER CYSTS HEPATIC ABSCESS

EPIDEMIOLOGY 2 types depending on aetiology: pyogenic or amoebic - 50% of cysts are single - Prevalence 1-3% PYOGENIC ABSCESS - 9:1 female predominance for symptomatic cysts - More common than amoebic abscess locally

PATHOGENESIS - Causative organism s: Klebsiella pneumoniae, Enterococcus, Enterobacter , E. coli, - Congenital malformation when an aberrant loses communication with the Staph aureus rest of the biliary tree and becomes progressively dilated (fluid within the cyst is not bilious) - Routes of infection: - No solid component and not septated (mixed cysts with septations are suggestive of (a) Ascending infection from biliary system (ascending cholangitis) malignancy) (b) Intra-abdominal source through portal vein – acute appendicitis, diverticulitis, - [Cysts that communicate with the biliary system are called choledochal cysts] inflammatory bowel disease, pancreatitis, pelvic abscess PRESENTATION – WITH COMPLICATIONS (c) Contiguous spread – from gallbladder empyema - Bleeding (d) Haematogenous spread in sepsis e.g. infective endocarditis - Rupture (e) External inoculation – iatrogenic, traumatic - Mass effect - Torsion - Presentation: Spiking fever with chills, rigors. 50% of patients have jaundice, and - Compression of inferior vena cava one-third have hepatomegaly - Fistulation into duodenum - Cholestasis due to compression of CBD - Investigations: - Portal hypertension  FBC, U/E/Cr - Carcinoma (rare)  Blood cultures  Melioidosis serology TREATMENT (IF SYMPTOMATIC)  Tumour markers: AFP, CA 19-9, CEA (may resemble tumour on imaging) - Aspiration  Stool ova, cysts and parasites - Ethanol sclerotherapy (painful)  Ultrasound - Fenestration (open or laparoscopic)  CT scan to exclude liver tumour (KIV endoscopy to rule out GI malignancy) - Excision/resection  If any aspiration done, aspirates for histology, stains, cultures

- Imaging: Irregular lesion with central area of necrosis, air-fluid levels, may be multiloculated. Rim-enhancing appearance on triphasic CT scan.

- Treatment 1. Antibiotics  Empirical antibiotics – Ampicillin/Gentamicin/Metronidazole  Change to definitive antibiotics when blood c/s results return  Total duration of 6 weeks – first 2 weeks intravenous, next 4 weeks oral 2. Drainage Drainage if >3cm – open drainage or percutaneous aspiration

Percutaneous aspiration Open drainage - Minimally invasive, performed under - Invasive procedure, done under GA radiologic guidance - Shorter hospital stay - Can be done under LA - Single procedure - Longer stay for patient as drainage - Not dependent on location tube stays in patient for a longer time - Indications - May require multiple attempts if  Concomitant pathology requiring unable to completely drain pus surgery - Contraindications:  Multiple abscesses or  Ascites (pus can leak into multiloculated abscess peritoneal cavity)  Immunocompromised patient  Uncorrected coagulopathy  Failed percutaneous drainage (tube  Proximity to vital structures blocked, or pt not getting better)  Ascites  Ruptured abscess

AMOEBIC ABSCESS

- Causative organis m : Entamoeba histolytica (infects the gut, forming ulcers in the colon, then spreads to the liver through the portal vein)

- Transmission is faecal-oral

- Presentation  Usually single abscess  No sepsis, jaundice  Hepatomegaly often present  Complications: rupture into pleural/peritoneal spaces

- Treatment :  Metronidazole  Aspiration if amoebic serology inconclusive; pregnancy (metronidazole contraindicated); suspicion of secondary infection; severe symptoms from distension or fever; impending rupture 45 PANCREATIC DISEASES - Gallstones are thought to cause due to obstruction of the pancreatic duct causing interstitial oedema which impairs blood flow to the ACUTE PANCREATITIS pancreatic cells  ischaemic cellular injury  predisposition to enzyme activation DEFINITION - The mechanisms in which alcohol cause pancreatitis are not known, though it is An acute inflammatory process of the pancreas with variable involvement of regional believed alcohol itself results in injury to pancreatic cells through generation of free tissues or remote organ systems radicals during its metabolism, and may sensitise the pancreas to injury by other agents EPIDEMIOLOGY Incidence is difficult to measure accurately as many patients with mild pancreatitis may ATLANTA CLASSIFICATION (FOR SEVERITY) not be diagnosed. Patients with acute pancreatitis make up 7-10% of those presenting with abdominal pain. 1. Mild acute pancreatitis - Interstitial oedema - Minimal organ dysfunction CAUSES (I GET SMASHED) - Uneventful recovery 1. Idiopathic 2. Gallstones 2. Severe acute pancreatitis (any 1 of the follow ing) 3. Ethanol - Pancreatic or peripancreatic necrosis 4. Trauma - Associated with organ failure 5. Steroids - May be associated with local complications 6. and other infections (VZV also) 7. Autoimmune – SLE, PAN 8. Scorpion toxin PRESENTATION 9. Hypercalcaemia, hypertriglyceridaemia, hypothermia - Symptoms (generally non-specific): 10. ERCP  Abdominal pain (most consistent, in >90% of patients) – constant epigastric 11. Drugs (SAND: Sulphonamides, azathioprine, NSAIDs, diuretics) pain, classically radiating to the back (in 50%), maximal intensity within several 12. Rare causes: Cystic fibrosis, cancer of the head of the pancreas, severe blunt trauma, hours of onset; usually occurs after a heavy meal; alleviated by sitting up, worse pancreas divisum on movement Gallstones and alcohol are the most common causes (>90% of acute pancreatitis)  Nausea and vomiting  Anorexia PATHOPHYSIOLOGY - Signs (also non-specific) - The final common pathway of pancreatitis involves inappropriate activation of  Epigastric tenderness (less than one third have signs of peritonism) proenzymes stored within zymogen granules in the pancreatic cell – trypsin is implicated in this mechanism as it activates most of the proenzymes secreted by the  Tachycardia, low grade fever pancreas when they are secreted into the duodenum  Abdominal distension with diminished or absent bowel sounds (paralytic ileus) - The activated lytic enzymes destroy the pancreatic acinar cells resulting in release of  Ecchymoses: flank (Grey-Turner’s sign) or umbilical (Cullen’s) – formed by potent cytokines that attract neutrophils and macrophages, which themselves secrete blood-stained peritoneal fluid tracking to the flank or umbilicus; suggest severe pro-inflammatory cytokines haemorrhagic pancreatitis associated with profound fluid loss (third-spacing) - The cytokine cascade amplifies the local inflammatory response and also results in a systemic inflammatory response (resulting in systemic complications of acute pancreatitis such as ARDS and multiorgan dysfunction) MANAGEMENT STRATEGY 3. Urinary diastase - Similar function to serum lipase, used when serum amylase is equivocally raised Diagnosis or normal, as urinary diastase will be elevated for a longer time after onset of symptoms

Severity stratification 4. X-rays of the chest, abdomen - Erect CXR may show pleural effusion, elevated hemidiaphragm, pulmonary Assess for aetiology infiltrates; in severe cases, there will be signs of ARDS – complete whiteout - Abdominal X-ray may show the “sentinel loop sign” (dilated proximal jejunal loop near the pancreas) or “colon cut-off sign” (distended colon from ascending Supportive Monitor for Treat aetiology to mid-transverse with no air distally) – these occur in more severe disease and treatment complications (reverse / cont rol) are due to functional spasm of the bowel around the pancreas resulting from inflammation

Manage Prevent future 5. Ultrasound complications recurrence - Preferred over CT scan - Good for visualising biliary tree and picking up gallstones - Pancreas may be diffusely enlarged and hypoechoeic – often difficult to INVESTIGATIONS visualise due to overlying bowel gas

D IAGNOSTIC 6. CT scan - Not the first investigation of choice unless considering pathologies other than 1. Serum amylase pancreatitis, since CT may worsen pancreatitis - Serum amylase is raised within 12 hours of onset of acute pancreatitis, usually - Value of CT is at a later point in the disease time course to look for more than 1000 or 3 times normal complications such as fluid collections; IV contrast needs to be given to detect - High sensitivity and moderate specificity (specificity increased when cut-off necrosis taken at 3 times normal upper limit) - Returns to normal level 48 to 72 hours after onset – not useful in late diagnosis P ROGNOSTIC / S UPPORTIVE / L OOKING FOR AETIOLOGY - Other causes of elevated serum amylase: 1. FB C (TW for Ranson, Glasgow; haematocrit for Ranson) GI sources - Ischaemic bowel (can cause elevated amylase in the thousands) 2. U/E/Cr (urea and glucose for Ranson and Glasgow) - Cholecystitis, cholangitis 3. LFTs (AST for Ranson and Glasgow; albumin for Glasgow; obstructive picture in - Peptic ulcer disease gallstone pancreatitis) - Kidney stone - Ectopic pregnancy 4. Lactate dehydrogenase (for Ranson and Glasgow) - Intestinal obstruction, perforation 5. AB G (PaO for Ranson and Glasgow; base excess for Ranson) Non-GI - Salivary gland injury or inflammation 2 sources - Macroamylasaemia 6. Ca/Mg/PO4 with albumin (hypercalcaemia – aetiology) - Impaired clearance due to chronic renal failure 7. Fasting lipids (hyperlipidaemia – aetiology) 2. Serum lipase - Rises within 8 hours of onset of symptoms and returns to normal after 7-10 days 8. EC G (rule out AMI as a cause of epigstric pain) - Thus more useful in late diagnosis of acute pancreatitis 47 SEVERITY STRATIFICATION III. Glasgow/Imrie sco re

I. Danger signs in the first few hours 1. Age >55yrs 2. White cell count >15x109/dL - Encephalopathy 3. Glucose >10mmol/L - Hypoxaemia 4. LDH >600U/L - Tachycardia >130/min 5. AST >100/L - Hypotension <90mmHg 6. Urea >16mmol/L - Haematocrit >50 7. Calcium <2mmol/L

- Oliguria <50mls/hr 8. PaO2 <60mmHg - Azotemia 9. Albumin <32g/L >3 criteria severe - Presence of Gray Turner’s/Cullen’s sign - Preferred over Ranson’s scoring in certain centres

II. Ranson’s criteria IV. C-reactive peptid e Present at admission Within 48 hours of admission - As a single prognostic marker 1. Age >55 yrs 1. Fall in Hct >10% 9 - If CRP is >210mg/dL at 48 hours, the pancreatitis is more likely to be severe 2. White cell count >16x10 /dL 2. Rise in urea >0.9mmol/L - No relevance beyond three days of onset as other confounding factors come into the 3. Fasting bld gluc >11.2mmol/L 3. Calcium <2mmol/L picture 4. LDH >600U/L 4. PaO <60mmHg 2 - Combination of Glasgow score and CRP improves overall prognostic value 5. AST >120U/L 5. Base excess >4mmol/L 6. Neg fluid balance >6L V. Balthazar’s CT severi ty index - Ranson’s criteria prognosticates mortality according to score - Grades severity of disease according to CT findings - Any patient with a score of 3 and above is considered to have severe pancreatitis - Not very useful as CT is not usually done in the first week in local context, and disease is still evolving (CT findings lag behind) in the early stages - Mortality: <3 0.9% 3-4 15% 5-6 50% COURSE OF DISEASE >6 90% - 75% of patients have a mild course of disease , and will recover without much - Shortfalls of Ranson’s: treatment unless comorbidities cause deterioration  Was originally validated in patients in whom the aetiology was mostly alcohol, - 20-25% have more severe outcome – one-third of these patients will ultimately die thus questionable to apply it in pancreatitis secondary to other conditions, such - Overall mortality rate <10% as gallstones - Death is bimodally distributed:  A revised Ranson’s score was created for gallstone pancreatitis, but it is difficult to tell aetiology in acute setting (a) Early  Within first week of disease  Cumbersome to wait for 48 hours, and difficult to assess for negative fluid  Occurs due to severe organ failure, SIRS balance  Very little can be done in terms of treatment (b) Late  Most common cause is infection with resultant sepsis  Multi-organ failure can be the course of death SUPPORTIVE TREATMENT 7. Endoscopic retrograde cholangiopancreatography (ERCP) - No benefit in mild biliary pancreatitis 1. Monitoring - In general ward for patients with mild pancreatitis - Indications: - Patients with severe pancreatitis will require HD/ICU monitoring  Severe pancreatitis  Evidence of ductal stones 2. Nil by mouth (bowel rest) and intravenous fluid replacement  Cholangitis - May include gastric decompression if there is persistent vomiting, significant  No response to treatment within 48 hours gastroparesis, or intestinal obstruction (ileus) - ERCP should be done within first 48-72 hours for maximum benefit - Acid suppression does not change course of disease, but protects against stress ulcer formation MONITORING FOR COMPLICATIONS AND TREATING - Fast patients for at least 48 hours until more stable Local complications: - May start feeding early with fluids in mild pancreatitis unless symptoms do not permit (e.g. vomiting, pain) - Acute fluid collections  Due to increased vascular permeability; 70-80% resolve spontaneously - Prolonged NBM results in poorer recovery due to nutritional debilitation – think about NJ feeding, or open jejunostomy creation early in patients with severe - Pancreatic necrosis pancreatitis; if not tolerable, then consider TPN  Areas of no contrast uptake on CT with intravenous contrast - Pseudocyst 3. Analgesia  Persistent fluid collection walled off by fibrosis and not an epithelium-lined - Do not give NSAIDs as they can worsen pancreatitis, and cause renal failure surface (can only be called a pseudocyst after 4 weeks) (since there is already decreased renal perfusion in acute pancreatitis) - Abscess - Use opioid analgesics other than morphine (morphine causes increased tone of  Infection of fluid collection (not necrosis) sphincter of Oddi) - Infected necrosis 4. Treatment of fluid and electrolyte abnormalities such as hypocalcaemia Surgery for local complications 5. Antibiotics - CT-guided aspiration of pancreatic necrosis - Either prophylactic or therapeutic  Can help differentiate between sterile and infected necrosis - Not shown to have any benefit in patients with mild pancreatitis unless  Consider surgery if patient doing poorly patient is having cholangitis secondary to a biliary obstruction that also caused the pancreatitis - Necrosectomy for infected necrosis  - Prophylactic in severe acute pancreatitis to prevent infection of necrosis Some kind of lavage and drainage procedure is done after necrosectomy to (infection will occur in 40-70% of patients with necrosis and increases the decrease infective load mortality rate from 12 to 33%) - Pseudocyst - Therapeutic in cholangitis, pancreatic infection  Operate if larger than 6cm and persisting for more than 6 weeks as the chance - 3 main regimens to choose from: of spontaneous resolution is low and risk of complications (infection, (a) Third-generation cephalosporin e.g. ceftriaxone, with metronidazole haemorrhage, rupture) is high (b) Fluoroquinolone e.g. ciprofloxacin, with metronidazole  Surgery can be open, laparoscopic, percutaneous (radiologically guided) or (c) Carbapenem on its own (e.g. imipenem rarely used due to inability to endoscopic escalate therapy in situation of resistance)  Endoscopic – internal drainage via a cystogastrostomy, cystoduodenostomy or cystojejunostomy - Use for 14-28 days

6. Support for organ failure 49 MANAGEMENT OF AETIOLOGY & PREVENTION OF RECURRENCE PRESENTATION for biliary pancreatitis May be asymptomatic, picked up on imaging for some other purpose - 18-21% of patients with biliary pancreatitis will have another episode Pancreatic head or periamp ullary Pancreatic body /tail nd - Among these, 25-65% will develop the 2 episode within 30 days of the initial one - Obstructive jaundice (painless Late presentation - Cholecystectomy can be done in the same admission for pts with mild pancreatitis obstructive jaundice with palpable - Coeliac and mesenteric plexus - In patients with severe pancreatitis, there is reluctance to do the surgery early, as the GB) + cholangitis invasion – dull constant pain in the patient may develop complications that require surgical intervention – better to do all - Duodenal obstruction epigastrium radiating to the back surgery in the same operation instead of opening the patient twice - Bleeding upper GIT (haematemesis - Malaise, weight loss, anorexia, nausea and/or malaena) - Exocrine insufficiency with duct - Malaise, weight loss, anorexia, nausea obstruction  steatorrhoea, malabsorption PANCREATIC CANCER - Metastatic symptoms: ascites, bone pain, CNS symptoms, dyspnoea EPIDEMIOLOGY - Paraneoplastic syndromes – migratory - Incidence about 3-5 per 100,000 per year in each gender thrombophlebitis in 6% - Eighth cause of cancer death in Singapore - 1.7:1 male to female ratio - Very poor prognosis – median survival for unresectable disease is 6 months (80% of IMMEDIATE MANAGEMENT patients have unresectable disease at presentation); overall 5-year survival <3% - Treat any life-threatening complications such as cholangitis, pancreatitis, bleeding

ASSOCIATIONS - Cigarette smoking (most clearly established – 2-5X increased risk) INVESTIGATIONS - Industrial carcinogens – benzidine, betanaphthylamine D IAGNOSTIC - Lower socioeconomic class 1. CA 19-9 - Diabetes mellitus - Not a screening test for pancreatic cancer as it can be false positive - Chronic pancreatitis - Can act as a prognostic marker: high CA 19-9 levels usually associated with - Genetic factors (mutations in K-ras gene, p16 gene) unresectable disease with poorer prognosis - Familial cancer syndromes e.g. Peutz-Jeghers - Can be used as a marker for tumour recurrence during post-op follow-up 2. CT scan PATHOLOGY - Better sensitivity (85-90%) and equal specificity (90-95%) in diagnosing - Most common histology is ductal adenocarcinoma (90% of tumours) pancreatic cancer - Anatomic distribution: 75% in the head, 20% in the body, 5% in the tail - Features: Mass lesion within pancreas, bile and pancreatic duct dilatation in head of pancreas tumours (double duct sign) - Distinct category of tumours collectively called periampullary tumour: - Any evidence of extra-pancreatic spread: Involvement of regional lymph nodes,  Malignant cells arise from one of a few cells: liver metastases, ascites (a) Duodenal epithelium (best outcome out of all three) (b) Biliary ductular epithelium 3. MRI pancreas with MRCP – MRI pancreas is not superior to CT scan; MRCP is (c) Ampullary ductular epithelium useful in delineating biliary system anatomy especially if the system is not  The periampullary tumours have better tumour biology than pancreatic adenoca obstructed and there are no therapeutic indications for ERCP (since there are  Prognosis is also better as they present earlier with obstructive jaundice considerable risks with ERCP) 4. ERCP with stenting to relieve obstruction (in cholangitis) - Complications of Whipple’s operation  Mortality rate is 2-7%, with a morbidity rate of up to 20-30% (mostly mild 5. Endoscopic ultrasound + FNA biopsy complications) - Can be used to stage tumour and nodal involvement - FNA with EUS guidance is preferred to transcutaneous biopsy as there is less  Intraoperative/early complications risk of tumour seeding (a) Injury to other organs – liver, kidney, bowel (b) Bleeding S TAGING (c) Infection  sepsis 1. CT/MRI of the abdo men – T, N stage; metastasis to the liver (d) Pancreatitis (e) Pancreatic anastomotic leak (5-20%) 2. Endoscopic ultrasound – T, N stage (f) Biliary anastomotic breakdown 3. Lungs – CXR + CT thorax (g) Fistulation, pseudocyst formation may occur due to anastomotic leaks 4. Bones – bone scan when suspicion is high  Late 5. Staging laparoscopy – for peritoneal metastases, just before definitive operation (a) Long-term exocrine insufficiency resulting in malabsorption and for a resectable tumour (since CT/MRI may miss small peritoneal deposits)  if no steatorrhoea peritoneal disease found, continue with surgery, otherwise, close up and abort (b) Gastric stasis with pylorus- preserving Whipple’s surgery (c) Diarrhoea resulting from autonomic nerve injury during lymph node dissection TREATMENT (d) Endocrine insufficiency  DM

S URGERY Palliative surg ery Curative resection Surgical bypass of obstruction - Improves chances of survival - However, recurrence rates after surgery are high – 5 year survival only 10 to 30% - Triple bypass involving anastomosis between - Only about 15-20% of patients will have resectable disease at presentation – usually (a) Stomach and jejunum (gastrojejunostomy) in periampullary or head of pancreas tumours; tumours of the body and tail present (b) Biliary system and jejunum (choledocho-/hepatico-/cholecysto-jejunostomy) too late to be resectable (c) Jejunum and jejunum, to prevent reflux of food into biliary tree – essentially a - Resectable disease: Roux-en-Y loop (jejunojejunostomy)  No metastases (lung, liver, bone, peritoneum)  Patent superior mesenteric vein and portal vein N ON - SURGICAL PALLI ATIVE MEASURES  Definable tissue plane between tumour and superior mesenteric artery as well as 1. Endoscopic stenting coeliac axis - Stenting of obstructed biliary duct - Stenting of obstructed duodenum - Whipple’s operation  for head of pancreas or periampullary tumour 2. Coeliac plexus block for pain  Usually preceded by a staging laparoscopy to confirm absence of peritoneal 3. Palliative chemo therapy/radio therapy/chem oradiot herapy metastases - Not shown to provide good outcomes  Removal of the head of the pancreas, duodenum, proximal 15cm of jejunum, common bile duct, gallbladder, and distal part of the stomach  Common hepatic duct and pancreas are then anastomosed to the jejunum, 45- 60cm proximal to the gastrojejunostomy 51 DISEASES OF THE BILIARY SYSTEM 2. Aetiology – benign or m alignant - Recurrent spikes of similar jaundice that resolve on their own with time suggest APPROACH TO OBSTRUCTIVE JAUNDICE benign obstruction e.g. stones, strictures - A young patient with painful jaundice  usually benign cause CAUSES - Previous history of gallstone disease or biliary colic symptoms Intraluminal Benign - Previous history of surgery to the biliary tract or ERCP - Gallstones - Malignancy is suggested if the patient is old, jaundice is of new onset and - Parasitic infections (recurrent pyogenic cholangitis) progressively worsening, and there is no associated pain (i.e. painless progressive jaundice) Mural Benign - Post-instrumentation strictures (ERCP, operation) - Constitutional symptoms: loss of appetite, loss of weight, malaise - Strictures from other causes (gallstones, chronic pancreatitis) - Metastatic symptoms: bone pain, neck lump, dyspnoea, etc - Primary sclerosing cholangitis - Pain is a late symptom of pancreatic cancer and tends to be constant and - Choledochal cyst relentless compared to biliary colic which subsides after a few hours Malignant 3. Complications - Cholangiocarcinoma (distal) - Symptoms of cholangitis: fever, chills, rigors with RHC pain and jaundice Extramural Benign - Fat malabsorption: steatorrhoea, fat-soluble vitamin deficiency (A, D, E, K) – - Mirizzi syndrome especially coagulopathy (very unlikely in acute setting) Malignant - Liver decompensation: encephalopathy - Head of pancreas cancer - Pruritus as a result of bile salt retention - Periampullary cancer - Metastases to the porta hepatis PHYSICAL EXAMINATION 1. Vitals: Is patient haemodynamically stable? Any fever? HISTORY 2. General inspect ion: Jaundice. Pallor? Any abdominal distension, leg swelling? 1. Confirm obstructive jaundice 3. Peripheries: Stigmata of chronic liver disease? Scratch marks? Conjunctival pallor? - Confirm jaundice – patient’s sclera are yellow 4. Abdomen - Establish obstructive jaundice – tea-coloured urine, pale stools - Any scars of abdominal surgery? - Exclude pre-hepatic and hepatic jaundice (more importantly hepatic jaundice - Generalised distension? (ascites could be due to malnutrition, peritoneal since it can also cause tea-coloured urine) malignancy, or obstruction of portal vein by cancer)  Symptoms suggestive of viral hepatitis: prodrome of fever, malaise, - Hepatomegaly? (Could be due to metastatic disease, or primary liver pathology) arthralgia, myalgia, nausea/vomiting, etc. - Enlarged gallbladder ? (Recall Courvoisier’s law – if the gallbladder is palpable  Risk factors for viral hepatitis: travel history, ingestion of seafood, family in a person with painless obstructive jaundice, the cause is unlikely to be stones) history of hepatitis (esp mother, siblings), blood transfusions, drug - Splenomegaly? (Portal hypertension – think prehepatic, hepatic, posthepatic) abuse/needle sharing, needlestick injuries, sexual contact 5. DRE: Pale stools?  Alcohol intake  Drug history: any TCM intake recently, any new medications taken 6. Cervical and supraclavicular lymph nodes  History of chronic liver disease 7. Bony tenderness 8. Respiratory examination INVESTIGATIONS (guided by clinical suspicion after Hx and P/E) STONE COMPOSITION AND PATHOPHYSIOLOGY

Bloods Cholesterol sto nes 1. FBC – any infection, anaemia - More common in older patients (peak at 40-50 years) 2. U/E/Cr - Cholesterol stones are hard and faceted 3. LFT – bilirubin raised, more direct bilirubin than indirect in obstructive jaundice; - Cholesterol stones results from disruption in the solubility equilibrium of bile ALP and GGT should be raised more than AST and ALT in an obstructive picture - Risk factors for cholesterol stones formation: 4. PT/PTT – any prolonged PTT from vitamin K malabsorption, liver dysfunction 1. Increased cholesterol secretion in bile 5. Tumour markers – CA 19-9, CEA (cholangioca and pancreatic ca)  Obesity  Hyperlipidaemia Imaging  Increased oestrogens: female, pregnancy, exogenous administration - Ultrasound versus CT 2. Decreased emptying of the gallbladder  Both useful in demonstrating dilated biliary system and site of obstruction as  Gallbladder malignancy is an important cause to exclude well as the cause of obstruction  Truncal vagotomy  Ultrasound is sufficient if malignancy is unlikely, but CT is preferred if there is a  Spinal cord injury suspicion of malignancy as it can define the tumour better and also have a staging function at the same time to determine involvement of nodes and other Pigment stones organs - More common in younger patients - Pigment stones are soft stones and crumble easily MANAGEMENT - Can be divided into black pigment stones and brown pigment stones The patient is managed as for the causative aetiology (see relevant sections) - Black pigment stones are composed of mostly calcium salts and bilirubin – predisposing factors include increased secretion of bilirubin into bile (e.g. chronic haemolysis, chronic liver disease, TPN), decreased bilirubin solubilisers, and gallbladder stasis GALLSTONE DISEASE - Brown stones are composed of calcium salts, bilirubin, and more cholesterol than black pigment stones; they form in the biliary ducts due to infection with bacterial DEFINITION degradation of biliary lipids , the degradation products of which then precipitate Gallstone is a generic term for any kind of stone (cholesterol, pigment) in any part of the biliary system (gallbladder, cystic duct, hepatic duct, common bile duct, etc) Biliary sludge - Microlithiasis suspended in bile; a milieu that predisposes to stone formation EPIDEMIOLOGY - Can be visualised on the ultrasound scan as layering in the biliary tree - Exact incidence in Singapore not known - Sludge is a pre-stone condition, but not all sludge becomes stones - In the West: overall 10-15%; 20% in women and 10% in men - 20% of biliary sludge will disappear, 60% recur, and 10% form stones - Consistent 2:1 female to male ratio - Typical picture (the F’s): Fat, female, forty, fertile, flatulent CLINICAL COURSE

NORMAL PHYSIOLOGY OF BILE Asymptomatic - Normal bile contains bile salts (primary and secondary), phospholipids, cholesterol, - 80-95% of patients will have asymptomatic gallstones protein, and bilirubin - Risk of symptom occurrence is 1 to 2% per year, of which the greatest risk is - Bile salts and phospholipids are amphiphatic and help to solubilise cholesterol within the first 5 years of diagnosis – 10% at 5 yrs, 15% at 10 yrs, 18% at 15-20 yrs 53 - Of those who develop symptoms, 7-10% will have moderate symptoms, and 3-5% INVESTIGATIONS FOR GALLSTONE DISEASE severe; the rest will have minor symptoms 1. Plain abdom inal X-ray - Thus the majority of patients do not require removal of the stones or the gallbladder  expectant management - Pickup rate for gallstones is less than 10% since most stones are radiolucent

- Role of surgery in the asymptomatic patient: 2. Ultrasound of the hepatobiliary system (a) Predisposing cause for gallbladder stasis that should be surgically treated e.g. - Investigation of choice for gallstones gallbladder mass suspicious of malignancy, or in patients with high risk of - Even more sensitive than CT scan for stones since CT may miss small stones due malignancy (gallbladder polyp, porcelain gallbladder)  prophylactic surgery to the spacing of the cuts taken (b) Immunocompromised patient where presentation of gallstone disease is not - Features of stone on ultrasound: strong echogeneic rim around the stone, with like the normal patient posterior acoustic shadowing  (c) Patients with chronic haemolytic disease (e.g. sickle cell anaemia, thalassaemia) - Bile should appear as black patch in gallbladder; if not homogeneous sludge – as high as 50-60% will develop symptomatic disease in their lifetime 3. CT scan Symptom atic sequlae - Usually not done to diagnose stones (as mentioned above) - Usually done in symptomatic patient where it is uncertain what is the cause of 1. Biliary colic symptoms  looking for other possible causes as well - Typically epigastric or right hypochondriac pain - Radiation to the inferior angle of the right scapula, or tip of right shoulder 4. Magnetic resonan ce chol angiopanc reatography (MRCP) - Waxing-waning in character but rarely have any pain-free intervals between - MRCP is not the same as MRI liver/pancreas – only selected cuts taken in order waves of pain (unlike ureteric colic where pain will resolve completely between to reconstruct the biliary tree, so the resolution is not as good as MRI waves) - Comparable to ERCP, and also minimally invasive  preferred to ERCP if - Often triggered by meals – binge-eating, fried oily foods, dehydration patient does not require any therapeutic intervention that ERCP provides - Lasts for minutes to hours, often resolves spontaneously - Associated with nausea and vomiting (patient gets better after vomiting), 5. Endoscopic retrograde cholangiopancreatography (ERCP) bloating, abdominal distension - The largest value of ERCP lies in its therapeutic potential  Stone removal (using balloon catheter, or Dormia basket) - Biliary colic is a “herald” symptom that indicates risk of further sequelae  Sphincterotomy (in order to relieve obstruction or facilitate removal of stone) 2. Acute cholecystitis (see below)  Stenting 3. Empyema of the gallbladder (see below) - High level of complications  Pancreatitis in 2-3% 4. Mucocoele of the gallbladder or hydrops (see below)  Cholangitis 1-2%, haemorrhage 2-3% 5. Choledocholithiasis with obstructive jaundice (see below)  Perforation into bile duct, duodenum 0.5-1%  Overall risk of complications is 10-15% 6. Cholangitis and septic sequelae (see below) - Before doing ERCP, need to assess the benefits and risks, and select patients 7. Acute pancreatitis (see above) carefully 8. Mirizzi syndrome with obstructive jaundice (see below) 9. Fistulation and passage into gut resulting in gallstone ileus – subacute IO 6. Percutaneous transhepatic (PTC) /biliary drainage  Infection (PTBD) - Non- surgical means of stone treatment - PTC involves a tube being inserted into the liver under radiologic guidance into  Chemodissolution one of the biliary ducts (must be dilated duct)  Liver diet - Rarely done now; main indications: 1) high obstruction not well visualised in  Shockwave – more morbidiy cf renal lithotripsy as less fluid around ERCP; 2) therapeutic purpose of drainage for an obstructed system that cannot to dampen waves; good results only for cholesterol stones be drained from below   All not shown to work for long-term - Mostly for therapeutic rather than diagnostic purposes - Complications: bleeding; leakage of bile when tube is removed

7. HIDA scan ACUTE CHOLECYSTITIS - No longer used commonly, except in biliary atresia PATHOPHYSIOLOGY 5 criteria for a normal cholangiopancreatogram - Gallstone gets stuck in the cystic duct causing obstruction of biliary flow (a) Normal intrahepatic ducts - Gallbladder becomes distended and inflamed (b) No filling defects (c) Smooth common bile duct PRESENTATION (d) No stricture/narrowing of the common bile duct - Constant, severe RHC pain (less commonly epigastric) - Radiates to the inferior angle of the scapula (e) Good and free flow of contrast into duodenum - Associated with fever, nausea, vomiting - RHC tenderness with guarding found on clinical examination; Murphy’s sign positive TREATMENT - Gallbladder may be palpable – omentum wrapping around GB; worst case scenario is Asymptomatic empyema - No surgery required unless patient has indications for surgery (see above) - LFTs usually normal; no jaundice - Expectant management and close follow-up - Counsel patient about symptoms – biliary colic, acute cholecystitis, obstructive ULTRASOUND FEATURES OF ACUTE CHOLECYSTITIS jaundice, etc - Presence of gallstones in biliary system - Contracted gallbladder (from chronic gallstone disease) Symptomatic - Pericholecystic fluid (oedema of gallbladder wall) - Cholecystectomy is the only way to treat gallbladder stones that are symptomatic - Sonographic Murphy’s positive - Can be open or laparoscopic – laparoscopic is preferred as it is associated with - (Fat stranding around gallbladder not seen on ultrasound but on CT) shorter hospital stay, less pain, less complications post-operatively - Risks of laparoscopic cholecystectomy MANAGEMENT  Conversion to open operation up to 5% (due to abnormal anatomy; difficult or - Resuscitate the patient complicated dissection; iatrogenic injury); conversion rate is higher if there is ongoing infection e.g. cholecystitis – up to 1 in 3 to 1 in 4 - Septic workup  Injury to bowel - Bowel rest and intravenous fluids  Injury to biliary structures e.g. CBD - Analgesia  Spilled bile  peritonitis, sepsis  Haemorrhage - Empirical intravenous antibiotics – IV ceftriaxone and metronidazole 55 - Definitive treatment – laparoscopic cholecystectomy - Patient is usually toxic, requiring urgent surgery Timing of cholecystectomy 3. Gangrene and perforatio n - Dependent on several factors:   Severity of illness - Localised perforation abscess that is confined by the omentum  Response to resuscitation and antibiotic therapy - Free perforation  generalised peritonitis and sepsis, requiring emergency  Logistical considerations (availability of OT, surgeon etc) laparotomy

- Possibilities available: 4. Cholecys tenteric fistula i. Emergency (immediate; in very sick patients who are not doing well/not - Most commonly occurs in duodenum, then colon, and stomach; after repeated responding to treatment) attacks of cholecystitis ii. Early (within few days of onset) - Usually asymptomatic iii. Delayed/interval (after 6-8 weeks) - On AXR, aerobilia is seen in 40% of cases Early Delayed - Symptomatic fistulas should be treated with cholecystectomy and fistula closure Advantages Advantages - Everything done in one admission - Lower risks 5. Gallstone ileus - Easier to operate as the gallbladder is - Better laparoscopic success - Stones causing cholecystenteric fistula pass into the enteric lumen causing oedematous intermittent bouts of small bowel obstruction - Accounts for 1-2% of IO overall Disadvantages Disadvantages - Most common site of obstruction is terminal ileum   - Ongoing inflammation higher risk - Fibrosis difficulty mobilising - Small stones (<2-3cm) usually pass spontaneously without problems of bleeding gallbladder - Mortality is 10-15%, mostly in elderly patients in whom gallstone ileus is more - Higher risk of injuring some other - Need for another admission common structure due to difficulty in - Chance of recurrence during the time - Small bowel enterotomy proximal to the point of obstruction is usually required visualisation to remove the stone - Higher conversion rate to open chole - Immediate cholecystectomy not warranted as <4% of patients will have further - Increased risks of post-op infection symptoms Early surgery has been found to be more beneficial than delayed surgery

Cholecystostomy ACALCULOUS CHOLECYSTITIS - In moribund patients who are not fit for surgery - Occurs in very ill patients with prolonged stay in ICU – prolonged fasting, poor - Can be done under LA, or more commonly under radiologic guidance (percutaneous) nutrition, labile blood pressure, sepsis - Drains the gallbladder and alleviates the inflammation  better outcomes - Poor nutrition leads to biliary stasis, while dehydration and hypotension leads to formation of viscous bile and gallbladder ischaemia  bile may get infected  COMPLICATIONS OF ACUTE CHOLECYSTITIS cholecystitis 1. Hydrops - Treatment involves emergent cholecystectomy - Cystic duct obstruction leads to a tense gallbladder filled with mucus - May lead to gallbladder wall necrosis if pressure exceeds capillary bld pressure

2. Empyema - Gallbladder is filled with pus due to bacterial infection of the stagnant bile (cystic duct being obstructed by a stone) - Previous Bilroth II (unsuitable anatomy for ERCP) CHOLEDOCHOLITHIASIS CHOLANGITIS

PRESENTATION PRESENTATION - Obstructive jaundice – tea-coloured urine, pale stools - Classically Charcot’s triad: RHC pain, fever, jaundice (only 50-70% of patients - Biliary colic have the classic triad) - If infection sets in  cholangitis (see below) - Reynold’s pentad: Charcot’s triad plus mental obtundation and shock - A surgical emergency! BLOODS - FBC (check TW for any rise suggestive of infection) PATHOLOGY - Amylase (CBD stone may cause pancreatitis) - Usually results from obstruction to the biliary system with infection of stagnant bile - LFTs (raised bilirubin – direct; ALP raised more than transaminases) - Most common cause is choledocholithiasis (60%); also consider benign strictures and malignancy (pancreatic, biliary) - Common causative organisms are gram negative bacteria and anaerobes – Klebsiella, ULTRASOUND E. coli, Enterobacter, Enterococcus - Gallstones in gallbladder - Gallstone in CBD MANAGEMENT - Dilated CBD (normally <8-9mm)  >10mm is abnormal 1. Resuscitation - Anticipate rapid deterioration  In older patients, post-cholecystectomy, or patients on long-term opiates, the - Obtain good intravenous access and fluid resuscitate as appropriate CBD may be larger, up to 11-12mm in size - Take bloods for investigations – cultures especially - Close monitoring of vitals in HD/ICU MANAGEMENT - Catheterise and watch urine output - If unsure of presence of stone  less invasive investigation such as MRCP, EUS - CVP line insertion if patient has shock unresponsive to fluid resuscitation - If likelihood of CBD stone is high  ERCP with stone removal 2. Antibiotics ERCP successful Plan for lap cholecystectomy - IV ceftriaxone and metronidazole; imipenem if the patient is in shock ERCP failed If patient is well and can tolerate another ERCP, try again (+ in between to drain bile) 3. Biliary decompression and definitive treatment - Biliary decompression and definitive treatment can be combined or separate Operative removal – Open CBD exploration - Investigate for cause of obstruction – ultrasound or CT (depending on what – Lap CBD exploration facilities are available; ultrasound is preferred if suspecting stones) - If no facilities to do ERCP: open or laparoscopic cholecystectomy with CBD exploration - Decompression commonly performed using ERCP  Decompression is the primary objective – stenting or external drainage When to do operative removal of st ones (i.e. not suitable for ERCP) (nasobiliary drain) - Stone >25mm  If cause of obstruction can be treated in the same setting (e.g. stones to be - Intrahepatic stone removed) then treat the cause also - Large number of stones  Success rate 90% - Impacted stone - Dual pathology - Definitive treatment dependent on:  - Tortuous duct Medical condition of patient  Success of biliary decompression 57  Logistical considerations - Consider biliary bypass if there are multiple stones, the CBD is more than 2cm in - Choices for definitive treatment: diameter, or there are strictures (since the CBD has been chronically dilated, quite (a) Open cholecystectomy with CBD exploration unlikely that it will function normally even after removal of the obstruction) (b) Laparoscopic cholecystectomy MIRIZZI’S SYNDROME (c) Laparoscopic cholecystectomy with CBD exploration PATHOLOGY CBD EXPLORATION - Gallstone in the Hartmann’s pouch compressing the common hepatic resulting in obstructive jaundice Cholangiogram or choledochoscopy is performed - - Compression effect is not just physical (the stone) but also contributed by the  Cholangiogram involves injection of dye – can image higher ducts surrounding inflammation  Choledochoscopy involves using a scope to visualise the large biliary ducts – - One of the caveats to Courvoisier’s law cannot image higher ducts, thus not as sensitive, but can be used to remove stones visualised in the duct GRADING  Choice of imaging depends on site of obstruction and the cause - Grade I: No fistula; extrinsic compression on CHD - Removal of stones - Grade II: Fistulation into common bile duct with the fistula <1/3 diameter of the  Manual removal with stone-grasping forceps CHD  Flushing out stones - Grade III: Fistula 1/3 to 2/3 diameter of CHD  Dredging stones out using balloon catheter or Dormia basket - Grade IV: Fistula >2/3 diameter of CHD  Lithotripsy

- Consider use of biliary stent or T-tube after removal of stone(s) If there is a lot of instrumentation of the biliary system during the operation, one CHOLANGIOCARCINOMA should anticipate swelling and oedema of the biliary system resulting in post- operative obstruction and buildup of bile  higher risk of biliary leakage SITE (a) Stent – removed later by endoscopy - Intrahepatic/peripheral 10% - Distal 25% (b) T-tube  A T-shaped tube with its horizontal limb placed in the CBD and the vertical - Perihilar 65% (Altemeier-Klatskin tumour) limb leading out to drain bile Bismuth classification  Functions as a “pressure release valve” as most of the bile will flow through i. Type I: below confluence of hepatic ducts the horizontal limb of the tube into the distal part of the CBD; only when ii. Type II: tumour reaching confluence there is obstruction to flow will bile be diverted out through the vertical limb iii. Type IIIA/B: involving common hepatic duct and either right or left hepatic duct iv. Type IV: multicentric or involving confluence and both hepatic ducts  Allows for post-op cholangiogram to check for remaining stones (at POD 9- 10) before removal – free flow of contrast into duodenum, no residual stones, and no free leak of contrast into peritoneum ASSOCIATIONS  If all normal  release anchoring stitch and exert gentle traction on the tube; - Related to chronic cholestasis: the tube should slip out easily, if not, call for help  Primary sclerosing cholangitis / Ulcerative c olitis   If stones are present  leave tube in for 4-6 weeks to form a f ibrous tract  Hepatolithiasis  allows for instrumentation of tract with a scope to remove the stones Parasitic infection – Clonorchis sinensis, Opisthorchis viverrini  Caroli’s disease (multifocal segmental dilatation of large intrahepatic bile ducts) - Bile duct adenoma - Choledochal cyst - Thorotrast exposure

PRESENTATION R ECURRENT PYOGENIC CHOLANGITIS - Painless jaundice (painful if there is cholangitis) - Acholic stools BACKGROUND - Pruritus Cholangiohepatitis, or recurrent pyogenic cholangitis (RPC), is characterized by: - Advanced signs and symptoms: - Recurrent bacterial cholangitis  Abdominal pain - Intrahepatic pigment stones  Fatigue, malaise - Intrahepatic biliary obstruction.  Weight loss  Hepatomegaly PATHOPHYSIOLOGY Helminthic infxn (eg Ascaris lumbricoides, Clonorchis sinensis)  epithelial damage DIAGNOSIS  predispose to seeding of coliforms into biliary system  stone formation  - CA 19-9 >100μl/ml (good sensitivity of 89%, specificity 86%) recurrent cholangitis - Contrast CT - PTC (2 functions: 1) roadmapping for surgery; 2) drainage of obstructed system if ERCP cannot drain) HISTORY: - A history of recurrent attacks of cholangitis – typical hx:  1-2 episodes of fevers, jaundice, and RUQ abdominal pain per year CURATIVE TREATMENT  Hx of prev biliary surgery, endoscopic procedures, or percutaneous biliary - Surgery is the only chance of long-term cure drainage procedures. - Only 25% of tumours are resectable - Complications of pyogenic cholangitis - Contraindications to surgery  cirrhosis with portal hypertension  Bilateral or multifocal intrahepatic disease  cholangiocarcinoma  Invasion of portal vein trunk or hepatic artery  Bilateral involvement of hepatic arterial or portal venous branches PHYSICAL EXAMINATION  Unilateral hepatic vascular invasion with contralateral ductal spread No specific physical findings are evident in RPC. Dx based on history.  Distant metastases DIFFERENTIALS PROGNOSIS FOR RESECTABLE DISEASE (5-year survival) Primary Sclerosing Cholangitis - Intrahepatic: 35-45% - Distal 35-45% - Perihilar 10-30% (worse prognosis due to early lymphatic spread) INVESTIGATIONS For diagnosis PALLIATION For Complications - Endoscopic/percutaneous transhepatic biliary stenting Bloods - Bilateral drainage for hilar cholangiocarcinoma - FBC - If after opening up and finding that tumour is not resectable, can perform surgical - LFT with ALP>ALT, AST bypass - Prothrombin time: N/ (if prolonged cholestasis causes fat malabsorption and vitamin K deficiency)  Impt to exclude – correct with parenteral Vit K before invasive procedures 59 - Blood C/S: bacteremia – results help guide antibiotic choice. - Ova and parasites: RPC freq a/w Clonorchis infxn – look for it and treat when present.

Radiology - U/S HBS  segmental biliary dilatation  hepatolithiasis  liver abscesses  helps determine choice of supplemental axial imaging techniques. - ERCP or PTC – imaging modality of choice for delineating the biliary tree. - CT scan  centrally dilated bile ducts with peripheral tapering  bile duct stones  pyogenic liver abscesses.

TREATMENT PRINCIPLES: - Treat current infection - Biliary drainage - Management of other complications e.g. dehydration etc

Surgical - Usual surgical approach includes:  Initial biliary decompression – ERCP sphincterotomy / stent placement  Definitive biliary drainage procedure – e.g. Roux-en-Y choledochojejunostomy

PROGNOSIS - Death occurs in approximately 15-20% of patients over 5-6 years. DISEASES OF THE BREAST Level II: posterior to pectoralis minor Level III: medial to pectoralis minor, extending up to apex of axilla ANATOMY 2. Internal mammary nodes - The breast is a modified sweat gland that lies in the subcutaneous tissue of the  Account for about 20% of drainage from the ipsilateral breast – upper and anterior chest wall between the superficial and deep layers of the superficial fascia lower inner quadrants - The base of each breast extends from the lateral border of the sternum to the mid-  About 4 nodes per side, with one node in each of the first three interspaces axillary line, from the second to the sixth rib and one in the fifth or sixth interspace - The axillary tail pierces the deep fascia and enters the axilla 3. Interpectoral (Rotter’s nodes) – between pec major and pec minor muscles - Each mammary gland consists of 15-20 lobules that are drained by lactiferous ducts that open separately on the nipple PRESENTATION OF BREAST DISEASE - Fibrous septa (Cooper’s ligaments) interdigitate the mammary parenchyma and extend from the posterior capsule of the breast to the superficial layer of fascia within 1. Breast lump (painful vs painless) the dermis, and provide structural support to the breast (involvement of these 2. Pain with no lump (cyclical vs non-cyclical) ligaments by malignancy causes dimpling of the overlying skin) 3. Nipple changes or discharge

APPROACH TO BREAST LUMP DIFFERENTIALS Painless lump Painful lump 1. Carcinoma 1. Area of fibroadenosis 2. Cyst 2. Cyst 3. Fibroadenoma 3. Abscess (usually in lactating women) 4. Area of fibroadenosis (nodularity) 4. Galactocoele (lactating women) 5. Periductal mastitis 6. Fat necrosis 7. Carcinoma (rare; 10% present with pain)

HISTORY 1. History of lump - Site of the lump? Single or multiple? - When was it first noticed ? Why was it noticed (pain, self-examination, etc)? - Duration since first noticed - Lymphatic drainage: - Painful or painless? 1. Axillary nodes – 75% of ipsilateral breast drains to the axillary nodes - Overlying skin changes noted: erythema, warmth, dimpling, swelling? Any 1. 40-50 nodes in total, in 5 groups: Anterior , posterior , medial , lateral , general asymmetry of the breasts noticed? apical - Any increase in size from first noticed to now? 2. Drain secondarily into supraclavicular and jugular nodes - Any changes in the nipple e.g. retraction 3. Anatomic division into levels I, II and III by the pectoralis minor - Nipple discharge? If present, what is the colour and consistency? muscle: - Any other lumps elsewhere – other breast? Axilla? Neck? Level I: lateral to pectoralis minor 61 2. Oestrogen exposure history and other risk factors for cancer Palpation - Age of menarche (early menarche <12 years old  increased risk) - Patient should be lying down at 45 degrees to the horizontal with her hand tucked - Whether married, and if married, how many children (nulliparity) behind her head – this splays the breast out so it can be palpated properly - Age at which first child was born (>30 years old) - Start with the normal side first! - Whether patient breastfed her children, and if so, for how long after birth - Ask for any pain before starting to palpate - Is patient currently postmenopausal ? If so, how old was she when she became - Use one hand to retract and stabilise the breast and palpate with the other menopausal? (>55 years old) - Palpate in a systematic manner e.g. quadrant by quadrant from centre outwards - Use of hormonal replacement therapy and/or oral contraceptive pills - Be thorough and examine the entire breast including the axillary tail - Family history of breast cancer or ovarian cancer (BRCA gene) especially if cancer occurs in first degree relative below the age of 40, or in bilateral breasts - When the lump is located, check with the patient whether this is the same lump she detected on her own - Previous breast cancer that has been treated - Previous biopsy of the breast showing atypical ductal hyperplasia or LCIS - Characterise the lump:  Site (which quadrant) - Exposure to ionising radiation  Tender or non-tender - Alcohol intake, especially before age of 30  Warmth of overlying skin 3. Systemic review  Size - Loss of appetite, loss of weight  Shape - Fever (infective cause)  Surface (smooth or nodular/irregular) - Bone pain (metastasis)  Consistency (soft, firm, or hard)  Fluctuance PHYSICAL EXAMINATION  Margins (regular and smooth, or irregular and ill-defined)  Preliminaries Fixation to the skin – try to pick up the skin above the lump  - Introduce yourself to the patient, ask for permission to examine the breast Fixation to underlying muscle – ask patient to press her hands against her hips to contract the pectoralis major muscle, then try to move the lump in 2 - Always have a chaperone to accompany you if you are male perpendicular directions, then ask patient to relax and try to move the lump again - Expose patient adequately from the waist up with exposure of axillae - Good lighting - Don’t be happy just finding one lump, still examine carefully for other lumps (multiple lumps are unlikely to be malignant, usually fibroadenoma or fibroadenosis) - Position the patient at 45 degrees or sitting position if a bed is not available - If the patient complains of nipple discharge and none is visible, ask patient if she can Inspection show you the discharge by expressing it herself (NEVER squeeze the nipple - Start off with patient’s hands relaxed at her sides – look for any asymmetry in the yourself!); if patient cannot do it, then ask the chaperone to help breast contours, any obvious skin changes (peau d’orange, erythema, puckering) - Look for any scars of previous operation, or procedure e.g. punch biopsy Axillary lymph nodes - Then ask patient to raise her arms (to accentuate any tethering to the skin which - Palpate the normal side first shows up as dimpling) - Rest the patient’s right forearm on your right forearm and use your left hand to - Ask the patient to push her hands against her hips to contract the pectoralis major palpate the right axilla (vice versa for the left side) muscles – this may reveal a previously unnoticeable lump - Palpate gently, slowly, and systematically, covering the major groups of nodes: - Look for nipple changes (7 D’s): anterior, posterior, medial, lateral, and apical  Discolouration  Depression (retraction)  Destruction - If any lymph nodes are found to be enlarged, note the number of lymph nodes, their  Discharge  Deviation  (Duplication – unlikely) site, size, tenderness, consistency (firm, hard, matted), mobility  Displacement To com plete the examination - Stellate lesion is a localised distortion of the breast parenchyma without - Examine the cervical lymph nodes especially the supraclavicular nodes perceptible mass lesion – high chance of it being malignant - Examine the lungs for any pleural e ffusion - Causes: Invasive cancer, radial scar (a benign lesion), fat necrosis, - Percuss the spine for bony tenderness abscess, etc - Examine the abdomen looking for hepatomegaly (d) Architectural disortion - Look at the axilla on the MLO view for any enlarged lymph nodes FINDINGS FOR THE COMMON BREAST LUMPS - BI-RADS (Breast Imaging Reporting and Data System) classification Type of lump A ge Pain Surface Consistenc y Mobility Category 0: Need additional imaging evaluation Cyst 30-55 Occ Smooth Soft to hard Not fixed Category 1: Negative (nothing to comment on, 0.05% risk still present) Nodularity 20-55 Occ Indistinct Mixed, Not fixed Category 2: Benign fluctuant Category 3: Probably benign, short-term follow-up suggested (<0.2% risk) Fibroadenoma 15-25 No Smooth, bosselated Rubbery Very mobile Category 4: Suspicious, biopsy should be considered (25-74% risk) Cancer 35+ No Irregular Stony hard May be tethered or Category 5: Highly suggestive of malignancy (75-99% risk) fixed Category 6: Known malignancy

INVESTIGATIONS 2. Ultrasound - Usually used as the first investigation in young patients (<35 years old) or “The evaluation of a breast lump is via the TRIPL E ASSESSM ENT – (i) Clinical pregnant, lactating patients examination; (ii) Imaging; and (iii) Histology.” - Can be used to guide interventional procedures such as biopsy, localisation of a Imaging lump preoperatively, drainage of abscess, aspiration of cyst 1. Mammography - Evaluates consistency (solid vs cystic), margins - Most sensitive of the proven breast imaging modalities - Localisation of lesion seen in only one mammographic projection - Usually performed in older women (>40 years old) as the breast tissue in - Evaluation of a palpable mass with a negative mammogram younger women is denser, more difficult to pick up abnormalities on - Evaluation of mass in mammographically-difficult areas e.g. chest wall, axilla mammogram - Pitalls: Operator dependent, non-standardised techniques, poor resolution, - Normally, 2 views are done: craniocaudal (CC) and mediolateral oblique (MLO) “partially blind” to microcalcifications - Additional specialised views: magnification and coned compression; done on - Features of malignancy: request to help magnify areas of abnormality or help visualise breast better  Markedly hypoechoeic + thick echogenic halo  - Abnormal features: Irregular edges  Hypoechoeic shadowing (a) Neo-density or asymmetric density  Taller than it is wide (fir-tree appearance) (b) Microcalcifications  High central vascularity - Calcifications <0.5mm in size (if >0.5mm  macrocalcifications) - Sole feature of 33% of cancers detected on mammography 3. MRI of the breast - Causes: DCIS, invasive cancer, fibrocystic disease, papilloma - Rarely used due to high cost, but provides good soft tissue definition - Features of malignancy: pleomorphic microcals, heterogeneous - Indications: appearance, closely grouped or arranged in a linear pattern (ductal  Positive axillary lymph node but mammogram and ultrasound negative distribution), underlying density  Suspicion of multifocal or bilateral malignancy (esp ILC which has a high - Benign microcals are punctate, and may have a “tea-cup” appearance incidence of multifocality/bilaterality)  (c) Spiculated mass or stellate lesion Assessment of response to neoadjuvant chemotherapy  - 95% of spiculated masses on mammography are due to malignancy When planning for breast conservation surgery  Screening in high-risk patient? 63

Histology APPROACH TO NIPPLE DISCHARGE - Options available: CAUSES (a) Fine needle aspiration cytology (b) Core biopsy (Trucut) Colour of discharge Cause (c) Incisional biopsy Red or pink (blood + serum) Ductal papilloma (d) Excisional biopsy Ductal carcinoma Clear yellow (serous) Ductal papilloma - Mostly a choice between FNAC a nd core biopsy Duct ectasia (= periductal mastitis)  FNAC is less invasive, less painful, smaller wound, does not require any local Cyst  anaesthetic, but only cells are obtained with no histology cannot differentiate Ductal carcinoma between in-situ cancer and invasive cancer , requires skilled cytopathologist Green, brown, black (cell debris) Duct ectasia  Core biopsy is more invasive, requires local anaesthetic, will result in a larger Purulent, foul-smelling Mastitis/abscess wound, more painful, risk of complications higher (because biopsy needle is a Thin, white fluid (milk) Galactorrhoea/lactation spring-loaded firing mechanism, improper angling may result in puncture of the lung or heart), but can obtain tissue specimen, can stain for ER/PR status  better HISTORY: diagnostic value 1. Is the disch arge true? - Can be guided by clinical palpation (if there is a palpable mass) or radiologic - Exclude other conditions that can cause discharge but not from the nipple e.g. guidance if the mass is small or there is no palpable mass  more accurate but still eczema, Paget’s, etc not 100% 2. Is the discharge significant?  Ultrasound guidance - Spontaneous discharge or discharge only on pressing ( spontaneous is sig)  Stereotactic guidance (stereotactic mammotome) - Intermittent or persistent ( persistent is sig) - Relation to breastfeeding (significant if >1yr after stopping breastfeeding ) MANAGEMENT 3. Is the discharge worrisome? - If triple assessment suggests benign disease (i.e. all three aspects suggest benign - Unilateral or bilateral (unilateral more worrisome) nature of lump), follow patient up with physical examination for a year (q3-6mths) to - Discharge from multiple ducts or single duct ( single duct more worrisome) make sure the lump is stable or regresses - Nature of discharge (bloody more worrisome) - If all three aspects of triple assessment suggest malignancy  further staging and - Age of the patient (more worrisome in older patient >60) treatment 4. Is it troubling the patient? - If one or two out of three aspects suggest malignancy  further workup, may require excisional biopsy PHYSICAL EXAMINATION (as described above) INVESTIGATION 1. Discharge for cytology to detect malignant cells 2. Mammography/ultrasound of both breasts to detect any underlying malignancy 3. Histology of lesion if found on imaging 4. Ductography

MANAGEMENT - If malignancy found, manage malignancy - Microdochectomy for intraductal papilloma - Antibiotics for mastitis/abscess + incision and drainage for abscess - Conservative management for most other pathologies unless discharge persists and is troubling patient  microdochectomy of offending duct BREAST CANCER Lobular LCIS ILC - Malignant cells arise from terminal - 5-10% of invasive cancers EPIDEMIOLOGY duct lobular unit (like DCIS) but do - 10-20% multicentric and/or bilat - Most common cancer in females in Singapore not distort lobular architecture - Cells morphologically similar to - Age-standardised incidence 55 per 100,000 in 2002; incidence is half that of the West - Usually does not form palpable cells of LCIS: monomorphic, bland - Bimodal age distribution, one peak at 45-55 years and another in older women (>75) mass and not detected by mammo, round nuclei - Gender ratio is about 100-150:1 incidentally detected - Cells invade individually into - 60-80% multicentric and bilateral stroma (due to loss of E-cadherin, a RISK FACTORS - Not premalignant, but a marker for cell-adhesion molecule) 1. Age (increases with increasing age with two peaks as mentioned) increased risk of invasive disease in - Similar prognosis to IDC 2. Family history (breast or ovarian cancer, especially if first degree relative, young both breasts (7-10x increased risk) onset <40 years old, bilateral cancer in relative affected) - If ca develops, will be IDC usually, occurs >15 years after diagnosis 3. Genetic predisposition (BRCA1 on 17q, Li-Fraumeni syndrome involving p53 mutation) Others Specialised types 4. Previous breast cancer - Medullary, colloid (mucinous), 5. Alcohol consumption tubular, papillary 6. Oral contraceptive usage - Better prognosis than IDC 7. Hormonal replacement therapy Inflammatory carcinoma 8. Previous biopsy showing atypical ductal hyperplasia or lobular carcinoma in-situ - Presents as erythematous. enlarged, 9. High oestrogen exposure (early menarche <12y/o, nulliparity, late childbearing with swollen breast w/o palpable mass first child at >30y/o, late menopause >55y/o) - Histologically not specialised 10. Ionising radiation to breast - Diffuse invasion of breast parenchyma by ca cells blocking numerous dermal lymphatic spaces PATHOLOGY  swelling - WHO classification divides breast cancers into epithelial and non-epithelial tumours. - No histo features of inflammation - Non-epithelial tumours arise from supporting stroma (e.g. angiosarcoma, malignant - Very poor prognosis, rapidly fatal phyllodes tumour, primary sarcomas) and are very uncommon - Epithelial tumours arise from cells lining the ducts or lobules, and can be further divided into invasive and non-invasive based on invasion of the basement membrane PRESENTATION Non-invasive Invasive - Most patients present with self-detected lump in the breast (more than one-third of patients), other presentations include painful lumpiness, pain alone, discharge, nipple Ductal DCIS IDC retraction - Malignant cells arise from terminal - 70-80% of invasive breast cancer duct lobular unit, cause distortion - Includes all cancers that cannot be - In patients presenting late, there may be overlying skin changes e.g. peau d’orange, of lobules, but do not invade BM subclassified into a specialised type tethering (means mass is still mobile but overlying skin will be indented when - Non-palpable, detected on mammo  “no special type” moving the lump), fixation (means the mass is not mobile), even fungation as microcals - Poorer prognosis than a carcinoma - May have symptoms of metastatic spread e.g. bone pain (metastases from breast - 35% multicentric, occult invasive of specialised type cancer spread to lung, liver, lymph nodes, bone, brain) ca in 10-20% - Two-thirds express ER/PR, one- - Increasing number of patients with abnormalities detected on mammographic - Progress to ca within 10 yrs, ~30% third overexpress C-erbB2 screening but with no palpable lump risk; considered pre-malignant - Good prognosis if treated DIAGNOSIS – BY TRIPLE ASSESSMENT (see above) 65 STAGING Survival - Investigations: Stage I: 90% (70% in 10 years) (i) Chest X-ray (for lung metastases) Stage II: 60% (40-50%) (ii) CT chest Stage III: 30% (20-30%) (iii) CT abdomen Stage IV: 10% (<2%) (iv) Bone scan THERAPEUTIC OPTIONS T stage N stage M stage Tis: Carcinoma in-situ, Paget’s N1: Mobile ipsilat axillary nodes M1: distant mets Options can be divided into aims of control: with no tumour N2: Fixed/matted ipsilat axillary nodes (a) Lo coregional control: T1: <2cm Surgery N3: N3a – Ipsilat infraclav nodes T1a – 0.1 to 0.5 cm Radiotherapy N3b – Ipsilat int mammary nodes T1b – 0.5 to 1.0 cm N3c – Ipsilat supraclav nodes (b) Systemic control: T1c – 1.0 to 2.0 cm Chemotherapy T2: 2 to 5 cm Hormonal therapy T3: >5cm Targeted therapy T4: T4a – Chest wall involvmt Surgery T4b – Skin involvmt T4c – Both 4a and 4b 1. Wide excision (breast-conserving surgery) T4d – Inflammatory ca - Removal of tumour with clear margins, while achieving good cosmetic result - Criteria: Stage 0 Stage I Stage II Stage III Stage IV  Tumour <5cm in size, no skin or chest wall involvement (i.e. T2 or less) Tis  Only one tumour, not multiple (unless in the same quadrant) T1N0 T2N0, T3N0  No metastatic disease T0N1, T1N1, T2N1 T3 N1  Appropriate tumour size-to-breast ratio (to achieve good cosmetic result) T0N2, T1N2, T2N2, T3N2  Patient must agree to post-operative radiotherapy Any T, N3 T4, any N - Overall survival at 25 years for WEAC comparable to SMAC M1 - Slightly higher local recurrence rates for WEAC (1% per year, 4% in 5 years) Aaaaa – Stage Xb (e.g. IIb, IIIb) - Higher risk in younger patients as cancer tends to be more aggressive

2. Simple mastectomy PROGNOSIS - Removal of breast tissue, nipple-areolar complex, and overlying skin Prognostic factors: - Lower rates of local recurrence 1. Stage of disease – tumour size, lymph node involvement [Major prognostic factor] 2. Histological grade of tumour 3. Axillary clearance 3. Lymphovascular invasion - Performed for all invasive carcinoma (WEAC or SMAC) 4. Age (younger patient  higher chance of recurrence, progress of disease) - Not required for DCIS (because theoretically cancer cells are confined to the 5. C-erbB2/Her-2 positivity indicates more aggressive tumour  worse breast) 6. ER/PR positivity is good – more of a “predictive factor” because it predicts - Complications: numbness/pain along inner aspect of arm, shoulder stiffness, response to treatment with tamoxifen; also means tumour is less undifferentiated lymphoedema 7. p53 mutation - Sentinel lymph node biopsy is a new modality of treatment Radiotherapy  Principle: the sentinel lymph node, being the first lymph node draining the 1. Adjuvant breast, is representative of the rest of the axilla; if the SLN is negative for - Usually done after breast-conserving surgery tumour cells, then the rest of the axillary nodes should be negative as well - Regimen consists of 25 to 30 cycles in total, 1 cycle per day from Monday to  Use of blue dye (isosulphan blue, methylene blue) or radioactive isotope (Tc- Friday over five to six weeks 99 sulphur colloid or colloidal albumin) injected in the area of the breast just 2. Palliative before surgery  concentrates in the first lymph node (sentinel node) that drains the breast Chemotherapy  During the op, look for the SLN by colour, or using a Geiger-Muller counter 1. Neoadjuvant to detect the node with highest radioactivity - Given in locally advanced cancer to shrink the tumour before surgical resection  Send node for frozen section - 20% of tumours achieve complete clinical response (cCR) i.e. tumour is no  If negative, do not clear axilla; if positive, perform axillary clearance longer palpable - Of these tumours, a further 20% will achieve complete pathological response  False negative rate 8% (quite good) (cPR) i.e. no more tumour cells  No difference in axillary recurrence between full axillary clearance versus - Need to place a clip into the tumour before starting neoadjuvant therapy to guide only sentinel node biopsy surgery in case the tumour “disappears”  SLN biopsy is now standard of care in many hospitals in SG 2. Adjuvant 4. Palliative surgery - Given in all locally advanced cancers after resection, and in some early breast - Palliative mastectomy for symptoms (bleeding, fungating, infected tumour) cancers depending on stage (see below) - Surgery at other sites: Fixation of pathological fractures, decompression of - Premenopausal patients tend to have better response to chemotherapy than spinal cord compression, surgical excision of brain metastases hormonal therapy (and vice versa for postmenopausal patients) - Main active agents are the anthracyclines (e.g. doxorubicin, epirubicin) and the 5. Breast reconstruction taxanes (e.g. paclitaxel, docetaxel) - Safe, can be done during breast surgery or at a later time - Common regimens: AC (anthracycline, cyclophosphamide), FAC (5-FU, - No delay in subsequent treatment and no increase in rates of relapse anthracycline, cyclophosphamide), CMF (cyclophos, methotrexate, 5-FU) - Options: 3. Palliative (i) Prosthesis - Anthracyclines and taxanes are the mainstay (ii) Muscle flap from rectus abdominis or latissimus dorsi - Helps to reduce load of disease to alleviate symptoms, increase survival Complications of surgery Hormonal therapy Early Haemorrhage (POD1) Wound Infection (POD3) - Used in adjuvant setting Seroma formation (accumulation of serum) in 50% - For hormone receptor-positive disease Flap ischemia - Preferred for postmenopausal women as response to hormonal therapy is better than Late Cosmetic deformity to chemotherapy Complications of Axillary Clearance: - Mostly used as adjuvant therapy but can a lso be used as palliative treatment - Lymphoedema – RT is contraindicated with AC as it worsens oedema Classes - Cellulitis – even in minor trauma, due to lymphoedema. Need to clean even (a) Selective oestrogen receptor modulators (SERMs) minor wounds with antiseptic solution + prophylactic ABx vs staphstrep - Tamoxifen  50% reduction in recurrence, 25% reduction in mortality - Shoulder stiffness – require physiotherapy - Side effects: menopausal symptoms (hot flushes, etc), endometrial cancer (0.1% - Intercostobrachial nerve transection – numbness over inner aspect of arm. per year), deep vein thrombosis 67 (b) Aromatase inhibitors - Lanastrazole, letrozole, exemestase  Adjuvant therapy usually involves chemotherapy if tolerable and/or hormonal - Inhibit peripheral conversion of testosterone and androstenedione to oestradiol therapy if ER/PR positive - Only suitable for post-menopausal patients as use of these agents will cause  In general: In premenopausal pt  chemotherapy + hormonal overactivity of the HPA axis in premenopausal women In postmenopausal pt  hormonal + chemotherapy - Side effects: musculoskeletal pain, osteoporosis 3. Locally advanced breast cancer Targeted therapy - T3 or T4 (tumour >5cm or skin/chest wall involvement), N2 or N3 (fixed lymph node involvement or supraclavicular node involvement) - Main agent is Herceptin (trastuzumab) which targets Her-2-neu a.k.a. C-erbB2 - Surgical resection dependent on size of tumour and resectability (if tumour is too receptor (a type of epidermal growth factor receptor [EGFR] that is overexpressed in large, the skin defect will be very large  inoperable) 18-20% of cancers) - Systemic therapy: - Used in C-erbB2 positive tumours, early or late stage  Neoadjuvant therapy to downstage inoperable tumour (in addition, it helps by - Side effects of Herceptin: cardiomyopathy, pulmonary toxicity, infusion reactions, predicting the tumour response to chemotherapy before resection) febrile neutropaenia  Adjuvant therapy after resection – chemotherapy or hormonal (as above) - Other agents include Avastin (or bevacizumab, targets vascular endothelial growth factor [VEGF] receptor, used in advanced cancer); Lapatinib (targets Her-1 and Her- 4. Advanced breast cancer 2, used in advanced cancers) - Distant metastases - Minimal locoregional therapy except for palliative purposes - Systemic therapy is the mainstay of treatment – chemotherapy or hormonal TREATMENT BY TUMOUR STAGE therapy Tumour can be divided into in-situ cancer, early breast cancer, locally advanced breast cancer, or advanced breast cancer. FOLLOW-UP 1. DCIS - 3-monthly for first 2 years - Wide excision without axillary clearance - 6-monthly for the next 3 years (i.e. third to fifth years) - Usually no adjuvant therapy - Yearly for another 5 years (to tenth year) - Some patients given hormonal therapy to reduce recurrence at surgical site; - At each visit – ask about symptoms and do clinical examination tamoxifen reduces overall breast cancer risk by 50% (in contralateral breast as - Repeat mammo of same breast 1yr postop; then 2-yrly bilateral mammo subsequently well) – strictly not adjuvant therapy BREAST SCREENING 2. Early breast cancer - T2 or less (<5cm), N1 or less (no nodes or non-fixed nodes) Normal risk, 40-49 YO  Annual mammogram - Locoregional therapy: SMAC, or WEAC with postop radiotherapy asymptomatic 50-64 YO  Biannual mammogram (by invitation) - Adjuvant therapy >65 YO  Optional 2 yrly mammogram  Purpose of adjuvant therapy is to destroy systemic micrometastases  Likelihood of patient having micromets is deduced from T and N stage (major Increased risk Start screening 5 yrs before  Monthly BSE prognostic factors): onset of breast dz in  6 mthly CBE & U/S breast youngest family member  Annual mammography Chemo Intermediate No chemo T >20mm, N stage >1 11mm < T < 20mm, N=0 T <10mm, N=0 HRT therapy 40-49 YO Annual mammogram Look at histological grade 50-65 YO Biannual mammogram up to 5 yrs after cessation of (minor prognostic factor); if high grade  chemo HRT PAGET’S DISEASE OF THE NIPPLE - Presents as erythema and eczematous change of the nipple (not the areola) with crusting exudates, may develop into erosions and ulcerations - Often associated with intraductal carcinoma ( DCIS ) or invasive carcinoma just beneath the nipple - Malignant cells invade across the epithelial-epidermal junction and enter the epidermis of the nipple, breaking the normal epidermal barrier thus allowing fluid to be extruded onto the nipple - Examination and investigations should be targeted towards detecting an underlying tumour – may find a palpable mass and/or mammographic abnormalities - Punch biopsy of the nipple may be required - Prognosis of the underlying cancer is not altered by the presence of Paget’s disease of the nipple - Treatment should be planned according to the underlying cancer if found - If no palpable mass or mammographic abnormality is detected, wide excision is an adequate treatment 69 APPROACH TO NECK MASSES MASSES BY LOCATION Midline NECK MASSES 1. Submental lymph node 2. Thyroglossal cyst ANATOMY 3. Thyroid nodule in the isthmus - The neck is composed of two triangles on each side – anterior and posterior 4. Sublingual dermoid cyst triangles 5. Plunging ranula (retention cyst of the sublingual) - The anterior triangle is bounded by the lower border of the mandible superiorly, the 6. Rarely, hyoid pathology e.g. bursa midline anteriorly, and the anterior border of the sternocleidomastoid posteriorly - The posterior triangle is bounded by the posterior border of the sternocleidomastoid Anterior triangle 1. Lymph node – along anterior border of sternocleidomastoid (levels II, III, IV) anteriorly, the anterior border of the trapezius posteriorly, and the clavicle inferiorly 2. Thyroid nodule 3. Submandibular gland mass (see later section on Salivary gland swellings) 4. Branchial cyst + fistula 5. Chemodectoma (carotid body tumour) 6. Carotid aneurysm 7. Pharyngeal pouch 8. Laryngocoele (rare; an air-filled, compressible structure seen in glass-blowers)

Posterior triangle 1. Lymph node – level V and supraclavicular lymph node groups 2. Cystic hygroma 3. Cervical rib 4. Brachial plexus neuroma/schwannoma

CAUSES OF MIDLINE MASS

Approach: - Does it move with swallowing – divides the thyroglossal cyst and thyroid nodule from the other causes - If it moves with swallowing, does it move with tongue protrusion – thyroglossal cyst moves with protrusion but a thyroid nodule does not

- Masses in the neck region can be subdivided according to the triangle they occur in Thyroglossal cyst as there are pathologies peculiar to each triangle Epidemiology: - Locations: (i) Midline Equal in males and females. Occurs mostly in children and adolescents but up to one- (ii) Anterior triangle third occur in patients older than 20 years. (iii) Posterior triangle Pathology: - In general, enlarged lymph nodes are the most common cause of a lump in the neck, A cystic expansion of the remnant thyroglossal tract (the embryological origin of the regardless of location (see section on Lymph node enlargement) thyroid gland which descends from the foramen caecum on the tongue). Features: Plunging ranula Smooth, rounded, cystic lump. 75% are in the midline while 25% are slightly to the left Pathology: or right. Usually asymptomatic but may become infected with sinus formation and A pseudocyst associated with the sublingual glands and submandibular ducts. Ranulas seropurulent discharge (occurs with incision or rupture of cyst) can be congenital or acquired after oral trauma. A large ranula can present as a neck mass if it extends through the mylohyoid musculature of the floor of the mouth – termed Histology: a “plunging” ranula. Cyst with columnar or squamous epithelial lining which may be ciliated. The cyst may also contain thyroid and lymphoid tissue. If malignancy occurs (carcinoma of the Treatment: thyroglossal duct), it is usually a papillary carcinoma (~90%). - Complete resection if possible, often in continuity with the associated sublingual gland (but often difficult due to close association with the lingual nerve and Treatment: submandibular duct). Sistrunk procedure – resection of the cyst and mid-portion of the hyoid bone in - If complete resection not possible, marsupialisation and suturing of the pseudocyst continuity and resection of a core of tissue from the hyoid upwards towards the foramen wall to the oral mucosa may be effective. caecum.

Dermoid cyst CAUSES OF ANTERIOR TRIANGLE MASS

Pathology: Branchial cyst and/or fistula Can be congenital or acquired. (i) Congenital – developmental inclusion of epidermis along lines of fusion of skin Epidemiology: dermatomes (seen in younger patients, present since birth). Locations include: Affects both sexes equally, usually in young adults in their 20s. o medial and lateral ends of the eyebrows (internal and external angular dermoid Pathology: cysts) A branchial cyst is thought to develop because of failure of fusion of the embryonic o midline of the nose (nasal dermoid cysts) second and third branchial arches. It is lined by squamous epithelium. o midline of the neck and trunk (ii) Acquired – due to forced inclusion of skin into subcutaneous tissue following an Features: injury, usually on fingers. Seen in older patients, no previous history of mass, - Occurs anterior to the upper or middle third of the sternocleidomastoid muscle. history of trauma to area (may have associated scar). - Smooth firm swelling that is ovoid in shape, with its long axis running downwards and forwards. Histology: - May be fluctuant, usually not transilluminable (due to desquamated epithelial cell Cyst lined by epidermis, with evidence of adnexal structures such as hair follicles, contents). sebaceous glands and sweat glands. - Look for fistula in this area – a branchial fistula will run between tonsillar fossa and the anterior neck, passing between the external and internal carotid arteries. Features: Small non-tender mobile subcutaneous lump, may be fluctuant, skin-coloured or bluish. - Fine needle aspiration of the cyst will yield opalescent fluid with cholesterol crystals under microscopy. Management - May be complicated by recurrent infections – purulent discharge, fixation to - Imaging investigations (e.g. XR, U/S, CT) are important especially for cysts on the surrounding structures. skull as they can communicate with cerebrospinal fluid. - Complete surgical excision of the cyst. Management: - If fistula present, perform fistulogram to delineate course. - Surgical excision of the cyst where possible. If fistula/sinus present, inject Bonney’s blue dye into tract prior to surgery to allow accurate surgical excision. - Treatment of infection with antibiotics. - Complications: cyst recurrence; chronic discharging sinus. 71 Chemodectoma Features Pathology: - Occurs in older patients A chemodectoma is a tumour of the paraganglion cells (paraganglionoma) of the carotid - A cystic swelling low down in the anterior triangle, usually on the left body located at the bifurcation of the common carotid artery (into the internal and - Squelching sound on deep palpation external carotids). They are usually benign, but locally invasive; the risk of malignancy - Patient complains of halitosis, regurgitation of undigested food with coughing and is 10%, with metastasis to local lymph nodes (no histopathological features for dysphagia in the neck, hoarseness, weight loss malignancy, thus malignant nature can only be diagnosed by presence of metastasis). - Complications: chest infection (due to aspiration); diverticular neoplasm (<1%)

Features: Diagnosis by barium swallow - Solid, firm mass at the level of the hyoid bone (where the bifurcation is) – be gentle Treatment during palpation as pressure on the carotid body can cause vasovagal syncope. - Leave it alone if small and asymptomatic - Mass is pulsatile but not expansile, due to transmitted pulsation from carotids. - Minimally invasive treatment: endoscopic c ricothyroid myotomy - Due to close association with carotid arteries, lump can be moved side to side but not - Surgical approaches (several available) up and down. o Diverticulectomy + cricothyroidotomy (diverticulectomy associated with risk of - May be bilateral. mediastinitis, dangerous) - If suspecting aneurysm, listen for bruit, look for signs of Horner’s syndrome, o Diverticulopexy (done in high risk patients, involves suspending the lumen of examine the rest of the peripheral vascular system. the pouch in the caudal direction so that food and secretions cannot enter the pouch; as the diverticulum is still present, the risk for malignancy still remains) Differentials and investigation: - Main differential is carotid artery aneurysm; aneurysm can occur at any level but carotid body tumour occurs at the level of the hyoid bone. CAUSES OF POSTERIOR TRIANGLE MASS - DO NOT PERFORM FNA - CT and/or MRI can be used to delineate tumour anatomy in relation to surrounding Cystic hygroma structures; CT reveals homogenous mass with intense enhancement following IV Pathology: contrast administration. A cystic hygroma is a congenital cystic lymphatic malformation found in the posterior - Angiography is the gold standard investigation – shows a hypervascular mass triangle of the neck, probably formed during coalescence of primitive lymph elements. displacing the bifurcation. May also show vessel compromise by tumour invasion, It consists of thin-walled, single or multiple interconnecting or separate cysts which and undetected synchronous tumours. insinuate themselves widely into the tissues at the root of the neck.

Treatment: Features: - Surgical excision with pre-operative embolisation (reduces bleeding and - 50-65% present at birth, but occasionally may present later in childhood or adulthood complications, and facilitates resection); any enlarged ipsilateral lymph nodes are - Lobulated cystic swelling that is soft, fluctuant, and compressible (usually into also removed due to the small possibility of malignancy another part of the cyst), located in the posterior triangle at the root of the neck - Radiotherapy is an effective alternative for patients who are unfit for surgery or - Classically “brilliantly transilluminable” whose tumours are too large. - A large cyst may extend deeply into the retropharyngeal space

Complications: Pharyngeal pouch (also called Zenker’s diverticulum) - Cystic hygroma seen on prenatal ultrasound in the first trimester suggests Pathology: chromosomal abnormality (50% of foetuses, usually trisomy 21) or other structural A herniation of the pharyngeal mucosa (pulsion diverticulum) through its muscular coat abnormalities (33% of foetuses with no chromosomal abnormality, usually congenital at the weakest point – Killian’s dehiscence – between the cricopharyngeus muscle and heart anomalies) the lower inferior constrictor muscles. - May obstruct delivery - Compressive problems after delivery – respiratory, swallowing Management: CERVICAL LYMPHADENOPATHY - Radiological investigations e.g. CXR, CT to delineate extent of cyst - Non-surgical treatment – aspiration and injection of sclerosant (usually unsuccessful) ANATOMY: - Surgical excision – partial (to alleviate symptoms) or complete

Cervical rib Features: - Usually more symptoms than signs as it causes thoracic outlet syndrome - A hard mass in the posterior triangle at the root of the neck - Symptoms/signs: o Arterial: pallor, gangrene or necrosis of the tips of the fingers o Venous: oedema, cyanosis o Neurological: complaints of radicular symptoms (pain, paraesthesia), wasting of the small muscles of the hand - Adson’s test can be done – ask patient to extend neck and rotate it towards side of symptoms  radial pulse will be diminished, occasionally with reproduction of radicular symptoms in the limb - Diagnosis by CXR

Neuroma/Schwannoma Features: - Slow growing tumour arising from peripheral neural structures of the neck e.g. brachial plexus, cervical plexus, vagus nerve, phrenic nerve, etc. - Fusiform, is mobile in plane perpendicular to axis of nerve but not parallel - Usually benign - May be Tinnel’s positive – tap on the mass for any paraesthesia occurring in Levels distribution of the nerve There are six levels of lymph nodes in the neck, and different structures drain to - DO NOT PERFORM FNA – excruciatingly painful different groups of nodes: Level Ia – submental Ib – submandibular II – long internal jugular vein from skull base to bifurcation of carotids (includes jugulodigastric nodes) III – along internal jugular vein from carotid bifurcation to omohyoid IV – along internal jugular vein from omohyoid to clavicle Va – Posterior triangle Vb – Supraclavicular VI – Tracheo-oesophageal groove (not palpable) VII – Superior mediastinum 73 Drainage: PHYSICAL EXAMINATION  - Oral cavity and oropharynx levels I – III Inspection  - Thyroid and larynx levels II – VI - Location  - Nasopharynx II – V (usually upper neck – level II and high level V) - Any overlying skin changes e.g. erythema, discharging sinus (multiple lymph node enlargement with discharging sinuses can be TB or actinomycosis; sulphur granules CAUSES: seen in actinomycosis) Can be divided into three main groups: infective, inflammatory, and neoplastic Palpate from behind , one side at a time – start at submental, then submandibular, Infective Viral preauricular, postauricular, along anterior border of sternocleidomastoid, Epstein-Barr virus, cytomegalovirus (infectious mononucleosis); HIV supraclavicular, posterior triangle, lastly occipital. Use pulps of the fingers in a gentle Bacteria rolling movement. Streptococcus, Staphylococcus, Klebsiella (from intraoral pathology e.g. dental abscess, tonsillitis) - Tenderness to palpation Tuberculosis - Consistency – hard, matted nodes are more suspicious for malignancy Parasitic - Fixation to overlying skin or underlying structures Toxoplasma To comp lete the examination: Fungi Actinomycosis - Complete examination of the face and scalp for any primary site of infection or neoplasia Neoplastic Metastatic Head and neck primary Nasopharyngeal carcinoma - Formal ear, nose, throat examination especially looking at the post-nasal space for Oral cavity, oropharynx, larynx, hypopharynx, nasopharyngeal carcinoma (NPC being the most common cancer causing enlarged thyroid, etc. cervical lymph nodes) Other primary sites (4B’s) - Examination of the thyroid gland Bowel (stomach, colon), breast, bronchus (lung), - Examination of lymphoreticular system – other lymph node groups, liver, spleen balls (testicular) - Full respiratory and abdominal examination especially if supraclavicular lymph node Primary - lymphoma found Inflammatory SLE - Breast examination in female patient Kikuchi’s (necrotising lymphadenitis occurring in young females, presenting as painful cervical lymphadenopathy) INVESTIGATIONS: Sarcoidosis - Fine needle aspiration provides most definitive results (though there is still the possibility of false positive and false negative results) HISTORY - Radiological investigation e.g. ultrasound, CT – to assess nature of lump, extent, - The lump itself – onset, duration, rate of growth, any pain, associated symptoms, other enlarged nodes that are not clinically palpable, and can be used to visualise lumps elsewhere primary tumour if present - Constitutional symptoms o Fever, malaise, arthralgia, myalgia (viral prodrome); MANAGEMENT: o Night sweats, low-grade fever (TB, B symptoms of lymphoma); - According to FNAC results o Loss of appetite, loss of weight (chronic infection, malignancy) - Malignant – work up for primary if present (e.g. squamous cell carcinoma – look for - Local symptoms – intra-oral diseases e.g. tooth decay, oral/tongue ulcer, tonsillitis oral cavity, skin, ENT, lung malignancy; adenocarcinoma – look for breast, GI tract - Past medical history – cancer, TB (contact? Diagnosed? treated or untreated?) malignancy) and treat as appropriate - Social history: travel and contact history, sexual history for HIV - Infective/Inflammatory – treat underlying condition SALIVARY GLAND SWELLINGS - Histology: predominantly serous acini, many ducts (other glands have few ducts) ANATOMY: Submandibular gland

- Consists of a large superficial part and a small deep part that are continuous with one another around the free posterior border of the mylohyoid - The deep part of the gland is closely associated with the lingual nerve (with the - Surrounded by tough fibrous capsule – the parotid sheath (thus mumps is painful as attached submandibular ganglion) above it, and the hypoglossal nerve and the gland swells within a tight envelope) submandibular duct below it – surgery may injure these nerves - Sandwiched between the posterior border of the ramus of the mandible and the - Nerve supply: parasympathetic secretomotor supply from lingual nerve carrying mastoid process postganglionic fibres from the submandibular ganglion (preganglionic fibres in - Important structures that pass through the gland in order from lateral to medial: superior salivary nucleus) (i) Facial nerve and its branches (ii) Retromandibular vein (formed as the maxillary veins drain into the superficial - Submandibular duct (of Wharton) arises from the superficial part of the gland, runs temporal vein) forwards deep to mylohyoid and drains into the oral cavity at the sublingual papilla (iii) External carotid artery (branching into its two terminal branches, the just adjacent to the frenulum superficial temporal and maxillary arteries) - Histology: mixed serous and mucous acini, few ducts - Nerve supply: (i) Parasympathetic secretomotor supply from auriculotemporal nerve carrying Sublingual gland postganglionic fibres from the otic ganglion (preganglionic fibres from inferior - A small almond-shaped gland sitting just under the mucosa of the floor of the oral salivary nucleus); cavity (ii) Somatic sensory supply of the gland from auriculotemporal nerve; sensory - Each gland has 15 or so ducts, half of which drain into the submandibular duct, the supply of the capsule from the great auricular nerve. rest draining directly into the oral ca vity - Parotid duct (of Stensen) runs 5cm across the masseter (surface marking: along the - Nerve supply is similar to the submandibular gland line joining the intertragic notch to the midpoint of the philtrum), drains into the - Histology: almost solely mucous acini, few ducts mouth opposite to the upper second molar tooth 75 HISTORY - Palpate the gland openings for stones. - About the lump: onset, duration, progress, associated symptoms e.g. pain - Bimanual palpation of the submandibular gland - If pain is present, is it precipitated by food ingestion? (suggestive of ) - Facial nerve examination - Symptoms of infection e.g. fever, malaise; if considering mumps, ask about testicular pain and swelling (orchitis), abdominal pain (pancreatitis) Suspicious features of malignancy: - Any noticed asymmetry of the face – incomplete closure of the eye on one side, - Hyperaemic hot skin over lump drooping corner of the mouth, drooling - Pain - Fixation to underlying structures or skin - Does the patient have symptoms of xerostomia (e.g. cannot eat a piece of biscuit or - Hard consistency bread without water), xerophthalmia - Irregular surface or ill-defined border - History of connective tissue disease e.g. rheumatoid arthritis, SLE - Facial nerve involvement

PHYSICAL EXAMINATION CAUSES OF SWELLING OF THE PAROTID Inspect Parenchymal Neoplasia Benign e.g. pleomorphic adenoma, Warthin’s - Put yourself at the level of the patient’s face and look from front for any asymmetry swelling Malignant tumours with an obvious mass on one side – parotid mass is located between the angle of the Lymphoma and leukaemia* jaw and the ear, and lifts the earlobe if large; submandibular mass is located just Sialolithasis under the mandible Stones Infection/ Mumps* - Look for scars – parotidectomy scar runs anteriorly to the ear, below the earlobe and Inflammation Acute around posteriorly before looping forward again under the jaw Chronic recurrent sialadenitis - Look for fistula/sinus HIV - Look at the patient’s face for asymmetry (facial nerve palsy) Autoimmune Sjogren’s syndrome* Infiltration Sarcoidosis* Palpate from behind Systemic Alcoholic liver disease - Ask patient about any pain before starting to palpate disease* Diabetes mellitus - Palpate the obviously enlarged gland, and always remember to also palpate the Pancreatitis contralateral gland for any swelling Acromegaly - Check for warmth of overlying skin, tenderness, consistency, surface, margins Malnutrition - Fixation to underlying structures – for parotid, ask patient to clench the teeth to Non- Lymph node contract the masseter, then try to move the gland parenchymal Facial neuroma swelling Temporal artery aneurysm - Fixation to overlying skin Skin and soft tissue swellings e.g. sebaceous cyst, lipoma - Palpate for cervical lymphadenopathy * are conditions in which parotid swelling is bilateral

Other tests - Examination of the duct openings: o Using a torch and a tongue depressor, examine opposite the second upper molar tooth for the opening of the parotid duct, and under the tongue for the opening of the submandibular duct. o Look for red inflamed duct opening, discharge (purulent), or any visible stone. o For the parotid duct, can palpate the duct along the masseter for stone, and look for discharge inside the mouth while palpating. SIALOLITHIASIS - Surgical removal o Transoral removal of stones for submandibular duct stones (50% can be removed Epidemiology thus), less for parotid duct stones - Stones of the salivary gland that may be impacted within the gland itself or in the o If stones cannot be removed via transoral surgery or is intraglandular, partial duct. gland resection can be performed - Usually occurs in males more than females, and between the ages of 30 and 60. - Other options: Lithotripsy, wire basket removal, - 80% of salivary stones occur in the submandibular gland (due to its higher mucus and calcium content with a long duct, and slow flow of the saliva against gravity); 10% occur in the parotid, 7% sublingual. SALIVARY GLAND TUMOURS - Most submandibular gland stones occur in the duct, while 50% of parotid stones Epidemiology: occur in the gland itself. - 80% occur in the parotid, of which 80% are benign (80% of the benign tumours are - 80-95% of submandibular stones are radio-opaque and can be seen on an X-ray of the pleomorphic adenomas) floor of the mouth, and 60% of parotid stones are radio-opaque. - 10% occur in the submandibular, of which 60% are benign (95% pleomorphic adenoma) Presentation - 15% occur in minor salivary glands, of which 50% are benign (all benign tumours are - Complete obstruction pleomorphic adenomas) Acute pain and swelling of the gland involved at meal times, rapid onset within - 0.3% occur in sublingual glands, of which all are malignant minutes of starting to eat, resolves about an hour after the meal. Pathology - Partial obstruction Epithelial Non-epithelial Occasional symptomatic episodes interspersed by asymptomatic periods of days to weeks, chronically enlarged mass in the submandibular region Adenomas (benign) Carcinomas (malignant) Pleomorphic adenoma Adenoid cystic ca Haemangioma - Can result in sialadenitis, and even abscess formation  worsening of symptoms of Warthin’s tumour Pleomorphic adenoca Lymphangioma pain and redness; systemic symptoms such as fever, chills; purulent discharge from Mucoepidermoid ca Neurofibroma duct opening Acinic cell ca Neurilemmoma - Stone may be palpable along the duct or at the opening of the duct Adenoca Lipoma Squamous cell ca Sarcoma Investigations Undifferentiated Malignant lymphoma - Noncontrast CT scan – can pick up almost all stones when fine cuts are requested - Plain X-rays can pick up radio-opaque stones Pleomorphic adenoma - Sialogram (rarely done today as it is invasive and technically demanding, and CT is better. Contraindicated in acute sialadenitis and contrast allergy.) Epidemiology: - Most common benign tumour Management - 85% occur in the parotid gland - General measures: - Equal sex ratio, occurs in younger patients less than 50 years old o Good hydration, soft diet, good oral hygiene Histology: o Massage of the gland, milking the duct, application of moist hot towel Very heterogeneous appearance, containing epithelial cells surrounded by loose stroma o Analgesia – NSAIDs such as ibuprofen with islands of chondromyxoid (mesenchymal components), and interspersed islands of o Antibiotics if patient has sialadenitis – usually antibiotics to cover Staph and myoepithelial cells. The tumour appears to be encapsulated, but histology shows Strept e.g. Augmentin multiple sites of capsular penetration by tumour cells. o Refer specialist treatment if symptoms persist for several days, or sialadenitis persists despite antibiotic therapy 77 Features: Mucoepidermoid ca is the most common malignant tumour in the parotid, while - Slow-growing, painless swelling occurring in the lower pole of the parotid adenoid cystic ca is the most common in the submandibular, sublingual and minor - Irregular and lobulated surface, texture of ca rtilage (slightly harder than Warthin’s) salivary glands - Does not invade or metastasise - Chance of malignant transformation if left for 10-15 years (1-6% risk) Malignant pleomorphic adenoma - If not completely excised, can recur (recurrence rate of 2%) - Usually occurs in pre-existing pleomorphic adenoma, rarely de novo - Worst prognosis of any salivary gland tumour Diagnosis by clinical, FNAC, + MRI - 30-70% recurrence and metastasis rate Treatment – surgical excision - Parotid: Superficial parotidectomy for superficial tumour; if tumour is deep or large, Treatment of salivary gland cancers total parotidectomy with preservation of the facial nerve Parotid: - Submandibular: Total gland excision together with adjacent connective tissue, - Total parotidectomy with sacrifice of facial nerve if tumour has infiltrated it (may be sparing lingual and hypoglossal nerves grafted with great auricular nerve) - Radical neck dissection if neck nodes positive - Postoperative radiotherapy Warthin’s tumour Epidemiology: Submandibular: - Only occurs in the parotid gland (10% of parotid tumours) - Radical excision of gland with lymphatic clearance of submandibular triangle - More common in males than females (4:1) - Radical neck dissection if neck nodes positive - Occurs in older patients (>50 years) - Postoperative radiotherapy - Related to cigarette smoking COMPLICATIONS OF PAROTIDECTOMY Histology: Also called papillary cystadenoma lymphomatosum or just adenolymphoma. Consists Immediate (within 24 hrs) of cleftlike or cystic spaces lined by two-tiered epithelium, containing mucin, 1. Intraoperative facial nerve transection – lower motor neurone palsy (in surgery to surrounded by a stroma of well-developed lymphoid tissue with germinal centres. the submandibular gland, damage to the hypoglossal and/or lingual nerves can occur intraoperatively) Features: 2. Reactionary haemorrhage - Slowly enlarging, soft to firm cystic fluctuant swelling in parotid tail - Invariably benign with no risk of malignant change Early (1 to 30 days) 1. Wound infection Diagnosis by clinical, FNA + MRI 2. Skin flap necrosis Treatment 3. Temporary facial weakness (neuropraxia of facial nerve) - Can be left alone if absolutely certain that the entire mass is composed of only 4. Salivary fistula  Warthin’s tumour cells, since there is no malignant potential 5. Division of great auricular nerve loss of sensation over pinna - Superficial parotidectomy if causing trouble to patient 6. Trismus (inability to open mouth due to spasm of masseter)

Late (more than 30 days) Malignant tumours 1. Wound dimple, cosmetic problems 2. Hyperaesthesia of local skin Most common malignancies are mucoepidermoid (34%) and adenoid cystic carcinomas 3. Frey’s syndrome – increased sweating and redness of facial skin when eating, due (22%) – equal sex ratio, can occur in any salivary gland, in older patients (usually >60 to reinnervation of divided sympathetic nerves to the facial skin by fibres of the yrs) secretomotor branch of the auriculotemporal nerve THE THYROID GLAND About RISK FACTORS - History of autoimmune disease e.g. type I DM, SLE, RA, pernicious anaemia APPROACH TO THYROID PROBLEMS – 2 MAIN TYPES (associations with Graves and Hashimoto’s) 1. Problem with configuration/anatomy - History of cancer elsewhere – metastatic disease to thyroid; lymphoma; papillary (i) Solitary thyroid nodule (most common in exam) cancer is associated with familial polyposis syndromes  ask about GI polyps/ca (ii) Multinodular goitre - History of thyroid disease – long-standing MNG can progress to lymphoma (iii) Diffuse enlargement - Occupational history – any exposure to radiation (papillary cancer risk) 2. Problem with function (usually hyperfunctioning) - Family history of thyroid cancer – ~20% of medullary cancers are familial (MEN2, (i) Graves’ disease AD inheritance), ~5% of papillary cancers (ii) Toxic adenoma (iii) Toxic multinodular goitre About previous TREATMENT for any thyroid disease (iv) Hashimoto’s disease - Medications given e.g. propylthiouracil, carbimazole, propranolol – for how long, efficacy, side effects AIMS OF ASSESSMENT IN THYROID NODULE/ENLARGEMENT: - Exclude cancer! - Radioactive iodine treatment – what was the result? Is the patient receiving - Address issues of thyroid function replacement? - Look for any complications e.g. compression (of airway, oesophagus, rarely nerve) - Surgery – what kind of surgery, any complications? - Cosmesis – is patient bothered by lump? - Follow-up – what investigations done?

ISTORY TAKING H - PHYSICAL EXAMINATION About the LUMP - Onset (gradual or sudden), duration A. THYROID GLAND - Size (Diffuse or one side predominant? Any sudden increase in size? – malignant GREET PATIENT, ASK FOR PERMISSION to examine (and listen to the voice – is it hoarse?) growth; ddx includes haemorrhage into necrotic nodule or cyst, subacute thyroiditis) POSITION PATIENT – on a chair with space behind the chair for you to stand. - Any pain – bleeding into cyst can result in sudden increase in size and pain; rarely pain can occur in anaplastic carcinoma and thyroiditis I NSPECT FROM THE FRONT - Compressive symptoms: difficulty swallowing, difficulty breathing, hoarseness of 1. Any swelling? Where is it? voice (benign pathologies almost never compress the recurrent laryngeal nerve) 2. Any scars (thyroidectomy scar may be difficult to spot as it is often hidden in a - Cosmetic effects skin crease)? Sinuses? 3. Any skin changes over the mass? Abo ut THYROID FUNCTION Hyperthyroid Hypothyroid 4. Check if mass moves on swallowing by asking patient to take a sip of water – Weight loss despite increased appetite Decreased appetite, weight gain, lethargy “Please take a sip of water and hold it in your mouth, do not swallow until I tell you Heat intolerance Cold intolerance to.” Increased sweating Dry skin, loss of outer third of eyebrows 5. Check if mass moves on protruding the tongue – “Please open your jaw slightly. Proximal myopathy (Graves’) Muscle fatigue Now, without moving your jaw, please stick your tongue out and back in again.” Diarrhoea, frequent bowel movement Constipation NB. A thyroid swelling moves only on swallowing; a thyroglossal cyst will move Tachycardia, atrial fibrillation Bradycardia on both swallowing and protrusion of the tongue. Oligomenorrhoea, amenorrhoea Menorrhagia Nervousness; easily irritable; emotional Slow thought, speech and action; 6. Check for plethora of face, distended neck veins – may be due to compressive lability; insomnia depression; dementia nature of mass (but rarely seen). Fine tremor Carpal tunnel syndrome symptoms 79 PALPATE FROM BEHIND – one side at a time, the opposite hand stabilises the gland. PROBLEMS WITH GLAND CONFIGURATION Ask for pain before palpating! 1. Characteristics of lump: site (anterior triangle), size (discrete nodule or PART I: R ELEVANT ANATOMY multinodular enlargement or diffuse enlargement?), consistency (soft, cystic, hard, multinodular?), mobility (fixed to skin? Fixed to underlying structures?), tenderness. Structure: 2 lateral lobes joined by an isthmus that lies in front of the 2 nd, 3rd and 4th tracheal rings. 2. Check swallowing while palpating to confirm mass moves on swallowing. Strap muscles of the neck lie superficial to the thyroid gland. 3. Check tongue protrusion. 4. Palpate lymph nodes Nerves and vessels:  Superior thyroid artery (from external c arotid) PALPATE TRACHEA from in front for tracheal deviation.  Inferior thyroid artery (from thyrocervical trunk, a branch of the first part of the subclavian artery). PERCUSS – any retrosternal extension?  External laryngeal nerve – supplies the cricothyroid muscle which controls pitch of AUSCULTATE – bruit in Graves’ voice; runs close to superior thyroid artery. OFFER to do Pemberton’s sign to check for thoracic inlet obstruction; check thyroid  Recurrently laryngeal nerve – supplies all the other intrinsic muscles of the larynx status; ask patient about compressive symptoms. (except for cricothyroid) and runs close to the branches of the inferior thyroid. The nerve runs behind the pretracheal fascia and so will not be damaged if the fascia is not breached during operation. Important to visualise nerve and avoid damaging it! B. THYROID STATUS

HANDS (get patient to stretch arms out in front of him, palms down) Embryonic origin: 1. Feel palms – warm sweaty palms Thyroglossal tract from foramen caecum of the tongue (in the midline, at the junction 2. Nails – thyroid acropachy, onycholysis (both seen in Graves’) between anterior two-thirds and posterior one-third of the tongue) descends close to the hyoid bone  expansion of the caudal end of the tract forms the thyroid gland. 3. Feel pulse – tachycardia, atrial fibrillation (AF more in toxic MNG than Graves’) 4. Fine postural tremor – accentuate by placing a sheet of paper on the hands Parathyroid glands: 2 superior and 2 inferior glands that lie behind the lateral lobes. 5. Palms up – palmar erythema Level VI lymph nodes – first nodes that a thyroid malignancy spreads to; they lie in FACE the tracheo-oesophageal groove and are not palpable. 1. Expression – staring, unblinking (hyperthyroid); lethargic, apathetic (hypothyroid) 2. Complexion – dry, ‘peaches-and-cream’ complexion, loss of outer third of eyebrows (hypothyroid) 3. Eyes PART II: APPROACH TO THE SOLITARY THYROID NODULE - Lid retraction (can see sclera between upper limbus of iris and upper eyelid) Prevalence: - Exophthalmos (sclera between lower limbus and lower eyelid) About 4-8% of population in US have palpable thyroid nodules; prevalence in - Chemosis (oedema and erythema of conjunctiva) Singapore not known. - Ophthalmoplegia (restriction of eye movements; ask about diplopia!) - Lid lag (eyelid lags behind eye when patient follows your finger downwards) History and ph ysical examination – as above - Proptosis (look from above patient’s head – eye visible over supraorbital ridge) Differential diagno ses: NEUROMUSCULAR 1. Cancer (only 10-20% of nodules is malignant, but need to exclude!) 1. Proximal myopathy (Graves’) 2. Follicular adenoma 2. Reflexes – slow to relax in hypothyroidism 3. Cyst (simple, colloid, or haemorrhagic) 3. Legs for pretibial non-pitting oedema (Graves’ or hypothyroid) 4. Dominant nodule of a multinodular goitre Clinical features suspicious of malignancy: - Suspicious sonographic features: 1. Male gender (thyroid nodules less common in male but more likely to be malignant) (i) Microcalcifications (in psammoma bodies  papillary cancer) 2. Age <15yrs or >60yrs (majority of nodules occurs in 3rd to 6th decades – likely (ii) Indistinct margins benign) (iii) Sonolucent halo around lesion 3. History of head and neck radiation or thyroiditis (iv) Hypoechoeic or anechoeic lesion – carcinoma is almost never 4. Family history of thyroid cancer (or MEN2, Gardner’s syndrome, FAP) hyperechoeic (v) Increased intranodular vascularity 5. Rapidly enlarging nodule 6. Hard, single nodule and/or nodules fixed to surrounding structures - Ultrasound still does not provide as good diagnostic value as FNAC 7. Hoarseness (i.e. recurrent laryngeal nerve invasion) 3. THYROID FUNCTION TEST 8. Cervical lymphadenopathy - Easy to perform, establish baseline, detect any abnormal function 9. Other symptoms of invasion e.g. haemoptysis, stridor, dysphagia - No real diagnostic value

Investigations: 4. R ADIO-ISOTOPE SCAN 1. FINE NEEDLE ASPIRATION CYTOLOGY - Hot nodule: only 1% malignant; but cold nodule: 10-20% malignant - The most important investigation modality! - But not very useful diagnostically - 90-95% sensitivity and specificity 5. BASELINE TUMOUR MARKERS (IF SUSPECTED OR CONFIRMED MALIGNANCY) - 4 possible results: - For differentiated thyroid cancer: thyroglobulin (i) Benign (thyroiditis, dominant nodule of MNG) - For medullary thyroid cancer: calcitonin, carcinoembryonic antigen (CEA) (ii) Malignant (papillary, medullary, anaplastic, mets) (iii) Suspicious (follicular, Hurthle cell change in follicular lesion) 6. CT SCAN OR MRI (iv) Inadequate  repeat FNAC - Not routine in thyroid nodular study - Can be both therapeutic and diagnostic for cyst – chocolate-brown fluid - Uses: aspirated; feel lump after aspiration to check for resolution (i) Evaluating invasion of surrounding structures - Cannot differentiate follicular adenoma from follicular carcinoma as the mark of (ii) Retrosternal extension malignant disease is capsular invasion – can only tell from a histological (iii) Lymph node involvement specimen of the nodule - Care to be taken with CT as contrast contains iodine and will affect post-op - Procedure: inject local anaesthetic in area, insert 20-22G needle and apply radioactive iodine body scan once given suction while fanning needle in region of nodule, release suction before pulling - MRI has same functions as CT but higher cost out needle, expel contents onto slide, then fix - Best to have experienced cytologist on hand to view slides and re-do FNAC if 7. ENT EXAMINATION OF VOCAL CORDS the sample is inadequate - In the rare occasion that there is pre-existing vocal cord palsy on one side  take extra care not to injure opposite recurrent laryngeal nerve as that can cause 2. ULTRASOUND OF THYROID bilateral vocal cord palsy - Advantages: (i) Objective measurement of nodule Management of benign nodule: (ii) Detection of subclinical nodule/screening – of value in papillary - Soft, small, round nodule with benign FNAC results, non-functional, not causing carcinoma since multicentric disease occurs in 15% any symptoms  can follow-up and monitor any increase in size (iii) Detection of lymph node enlargement (especially level VI nodes) - A lump >4cm has a greater risk for malignancy (iv) Can define consistency of nodule – solid, cystic, or complex 81

PART III: THYROID CANCERS Differentiated thyroid carcinom a Medullary carcinoma Anaplastic carcinoma Lymphom a Papillary carcinom a Follicular carcinom a Proportion 75% 10% 7% 3% 5% Age 25-40 years 40-50 years >50 years for sporadic type; 20-30 years for familial 60-70 years >50 years F:M ratio 3:1 3:1 1:1 3:2 2:1 Risk factors - Radiation exposure - Follicular adenoma is NOT a risk - Significant family history in the familial type – - Longstanding goitre - History of lymphoma or - Polyposis syndromes (FAP, factor MEN2 (AD, complete penetrance, associated with - History of previous MALT elsewhere Gardner’s, etc) - Iodine deficiency may be parathyroid adenoma and phaeochro mocytoma – see differentiated thyroid ca - Hashimoto’s thyroiditis - Positive family history in 5% associated notes below) (30% of anaplastic ca) (60X increased risk) Pathological - Characteristic Orphan Annie - Follicular structures similar to - Arise from parafollicular C cells (which produce - Small blue round cells - FNAC may suggest features nuclei, nuclear pseudoinclusions normal thyroid calcitonin) that are highly lymphoma but definitive - Papillary architecture with - Diagnosis of cancer made on - Distinctive deposits of acellular amyloid material – anaplastic – may diagnosis requires trucut psammoma bodies evidence of capsular or vascular altered calcitonin collections resemble lymphoma or excision biopsy - Tall cell variant (nuclear features invasion by tumour cells (vs - Multicentric C-cell hyperplasia may be seen in - Almost always non- of papillary ca within follicular follicular adenoma) familial cases Hodgkin’s of B-cell lesion) behaves like papillary ca, - Hurthle cell variant – worse type has worse prognosis prognosis Clinical - Slow-growing tumour - Solitary - Sporadic cases usually solitary, worse prognosis - Large bulky mass - Usually presents as features - Spread by lymphatics - Haematologic spread to bone, - Familial cases all multicentric, better prognosis involving neck rapidly enlarging goitre - 30-50% multicentric lung, liver, brain - Aggressive growth; spread via local, lymphatic, structures – locally with compressive - LN involvement in 80% of disease - LN involvement in 10% (rare) haematological routes advanced symptoms at diagnosis (level VI first) - 95% produce calcitonin, 80% produce CEA - Aggressive growth - 60-80% aggressive and - Unilat LN involved in 60-80%, contralat LN in 40% 30% more indolent - Very good prognosis - Multiple metastases - Always exclude MEN2 – serum calcium, 24hr probably present at - Poor prognostic factors (AMES): Age>40, presence of metastases, extra- urinary catecholamines presentation thyroid invasion, size>4cm (more details on risk stratification below) Treatment Surgical resection Surgical resection Palliative therapy for Chemotherapy and/or - Hemithyroidectomy for selected low-risk patients see( below) - Aggressive resection – total thyroidectomy with compressive effects radiotherapy depending - Total thyroidectomy for the majority level VI node clearance - Chemotherapy to shrink on type of lymphoma - LN clearance: tracheo-oesophageal nodes cleared, and neck dissection if - Sampling of cervical and mediastinal nodes and tumour neck nodes are positive modified dissection where positive - Surgical debulking - For suspicious lesion – hemithyroidectomy with histology, KIV TT - Tracheostomy No good adjuvant therapy Adju vant therapy Follow-up - Radioactive iodine at ablative levels to ablate remnant thyroid and any - Thyroxine replacement (not for TSH suppression but cancer tissue (only for total thyroidectomy) to maintain euthyroid state) - External radiotherapy (only shown to have good results in pts with locally - Serum calcitonin and CEA six mths after surgery (if advanced follicular ca) normal, considered cured – 5% 5yr recurrence) TSH suppr ession – give L-thyroxine to suppress TSH levels to <0.005U/L - High calcitonin – screen for residual or metastatic disease, treat surgically, with RT or chemo as Follow-up appropriate - Check TSH levels - Thyroglobulin as a tumour marker of recurrence - Radioactive iodine scan to detect recurrence, followed by ablation 5yr survival 95% in low-risk pts, 88% in intermediate-risk pts, 50% in high-risk pts 60-70% Median survival <6mths Dependent on histo, stage, Slightly worse for follicular ca treatment, etc. Differentiated thyroid cancer Disadvantages of TT: - Papillary and follicular cancers are considered differentiated thyroid cancer (as - Risk of bilateral recurrent laryngeal nerve injury and permanent hypoparathyroidism opposed to anaplastic – undifferentiated – thyroid cancer) - Very low incidence of cancer recurrence in residual thyroid – microfoci probably not - Prognosis is excellent clinically significant - Limited thyroidectomy may spare patient from having to be on lifelong thyroid R ISK STRATIFICATION: hormone replacement - Risk factors can be divided into patient factors and disease factors Thus, risk stratification helps to guide the extent of surgical resection in differentiated - Patient factors: A ge – >45 years old is high risk; Gender – male is high risk thyroid cancer according to the patient’s disease. - Tumour factors: Size – nodule >4cm has higher risk Lymph node clearance Histology – tall cell variant of papillary ca and Hurthle cell variant of follicular ca - Tracheo-oesophageal groove (level VI) node clearance usually done are considered unfavourable - Radical neck dissection or modified radical neck if: Extrathyroidal extension into surrounding structures – worse (i) Tracheo-oesophageal groove nodes histologically positive for cancer Lymph node or distant metastases – worse (ii) Clinically positive nodes in the neck – palpable or enlarged on ultrasound - Various score systems have been formulated to stratify risk: AMES – Age, Metastases, Extent, Size Radical neck dissection AGES – Age, Grade (Histological), Extent, Size) – rarely used as histological - The removal, en-bloc, of the entire ipsilateral lymphatic structures of the neck, from grading is not commonly performed the mandible superiorly to the clavicle inferiorly, from the infrahyoid muscles MACIS – Metastasis, Age, Completeness of resection, Invasion, Size medially to the anterior border of the trapezius laterally - Patients can be divided into three groups: - Classic radical neck dissection (Crile’s) – internal jugular vein, sternocleido- (i) Low risk – low risk patient and low risk disease (i.e. no high risk features) mastoid muscle, and accessory nerve are resected. Structures not resected: carotid (ii) Intermediate risk – low risk patient with high risk disease, or high risk patient arteries, vagus nerve, hypoglossal nerve, brachial plexus, phrenic nerve with low risk disease - Modified radical neck (iii) High risk – high risk patient and high risk disease (i) Type I: one of the three structures not removed, usually accessory nerve - Risk helps to guide treatment – low risk patients can undergo hemithyroidectomy (ii) Type II: two of the structures not removed – accessory and IJV without ablative radioiodine therapy post-op, while high risk patients undergo total (iii) Type III: all of the three structures not removed thyroidectomy with post-op ablative RAI treatment; treatment in intermediate risk (iv) Extended radical neck dissection: resection of lymph nodes and/or structures patients is tailored to the disease, but usually is similar to that in high risk patients not included in the classic neck dissection - 5 year survival is also prognosticated by the risk: low risk patients have a survival - Complications of radical neck dissection: of 95-98%, intermediate risk patients 88%, and high risk patients 50% (i) Injury to nerves – vagus (vocal cord paralysis), cervical sympathetic chain (Horner’s), mandibular branch of facial (lower lip weakness) TOTAL THYROIDECTOMY VERSUS HEMITHYROIDECTOMY (ii) Haematoma  bring back to OT to find source of bleeding and stop it Advantages of TT: (iii) Salivary fistula (usually when pt has received RT to the neck, and if the upper - Evidence for microfoci of disease and multicentricity of cancer – removal of the GI tract was opened during the surgery) – infection can result entire thyroid decreases risk of recurrence (iv) Wound infection – risk factors: previous irradiation, if upper aerodigestive tract - Ability to use adjuvant radioiodine to ablate any residual cancer tissue after surgery is opened during surgery with salivary contamination, salivary fistula - Ability to use radioiodine to detect recurrent disease (normal thyroid picks up iodine (v) Carotid blowout – risk factors: infection, irradiation  resus, apply constant better than cancer cells, thus the presence of the thyroid gland will decrease the pressure all the way to the OT! ability of RAI to pick up recurrent cancer) and as treatment for recurrence (vi) Poor healing – usually in irradiated skin; weakest point is the junction of the - Ability to use serum thyroglobulin as a cancer marker for recurrence trifurcate incision 83

Multiple endocrine n eoplasia PART IV: SURGERY IN BENIGN THYROID DISEASE

A group of inherited diseases resulting in proliferative lesions (hyperplasia, adenomas, Indications for surgery: carcinomas) of multiple endocrine organs. 1. Cannot be treated medically - failed medical therapy or unsuitable for medical tx 2. Cancer FEATURES: 3. Compression on neighbouring structures - Tumours occur at younger age than sporadic cancers 4. Cosmesis - Multiple endocrine organs involved, either synchronously or metachronously 5. Compliance/cost problems – with long-term medical therapy (but patient may still - Multifocal tumours in each organ involved require long-term therapy after op if he/she becomes hypothyroid or is still - Tumour usually preceded by asymptomatic stage of endocrine hyperplasia hyperthyroid) - More aggressive and higher chance of recurrence compared to sporadic type of 6. Child-bearing (not a very strong indication since medical therapy can still be given, tumours in the same organs but not RAI) MEN 1 - Autosomal dominant inheritance Types of surgery available: - Gene involved is the tumour suppressor gene MEN1 located on chromosome 11q13 1. Hemithyroidectomy – removal of one lobe of the gland, including the isthmus and where mutations cause loss of function of the gene the pyramidal lobe; usually for suspicious thyroid nodules - Three P’s: 2. Total thyroidectomy – entire gland removed completely; usually done in MNG Parathyroid (95%) – hyperparathyroidism from hyperplasia of parathyroid glands 3. Subtotal thyroidectomy Pancreas (>40%) – aggressive metastatic tumours (e.g. gastrinoma, insulinoma), - Conventional subtotal thyroidectomy – leave a thumb-sized amount (about 4-6g) leading cause of death in MEN 1 patients of remaining thyroid tissue on both sides Pituitary (>30%) – most commonly prolactin-secreting macroadenoma; some have - Harley-Dunhill subtotal thyroidectomy – leave a thumb-sized amount only on growth hormone-secreting tumours one side with removal of the rest of the gland

MEN 2 Total versus subto tal thyroidectomy (for hyperfunctioning thyroid disease) - Autosomal dominant inheritance - Result of total thyroidectomy is always hypothyroidism, thus the patient will require - Gene involved is RET protooncogene at 10q11.2 where activating mutations occur life-long thyroid replacement and follow-up  problems with compliance, cost, - Two distinct groups of disorders: inconvenience 1. MEN 2a (Sipple syndrome) Medullary carcinoma of the thyroid (almost all) - Results of subtotal thyroidectomy (at 5 years): Phaeochromocytoma (50%, of which less than 10% are malignant) o 60-70% euthyroid (do not require medication but still have to be followed up Parathyroid hyperplasia and hyperparathyroidism (30%) closely) o 16-20% hypothyroid (usually becomes evident within 1 year of surgery) 2. MEN 2b (William syndrome) o 8-10% hyperthyroid (percentage increases proportionately with time  failure of Thyroid and adrenal involvement like MEN 2a, but no hyperparathyroidism surgical therapy) Neurocutaneous manifestations: ganglioneuromas on oral mucosa, lips eyelids  Other features: Marfanoid habitus, SCFE, delayed puberty Difficulty in managing post-operatively and in the long term as patients need close monitoring (better off to just replace everyone after TT?), but weigh this against the benefits of not requiring any medication (for which there is a good chance) Complications of thyroid surgery: (Mostly H’s, one I and one T) LLATEATE ((MORE THAN 3030 DAYS)) 1.1. Hypothyroidism IIMMEDIATE (<24HRSHRS)) 1.1. Haemorrhage with haematoma formation 2.2. Hyperthyroidism (failed treatment) -- Haematoma forms in the paratracheal region, mostly below the strap muscles  3.3. Permanent hypoparathyroidism can result in compression of airway if not released (patient can die!) 4.4. Hypertrophic scarring or keloid formation – – ask patient if he/she has keloids -- Cut the subcuticular stitches and also the stitches holding the strap muscles opposed to let the blood drain out

2.2. Hoarseness or airway compromise from recurrent laryngeal nerve injury -- Risk of nerve injury is <1% -- Unilateral nerve injury for hemithyroidectomy, bilateral nerve injury for total or subtotal thyroidectomy -- If bilateral nerve palsy resulting in compromised airway, will require tracheostomy 3.3. Hyperthyroidism -- Resection of gland can release large amounts of stored thyroid hormone into bloodstream -- May result in thyroid storm ( see Management of thyroid stormrm))

4.4. Tracheomalacia -- Floppiness of trachea resulting from chronic compression e.g. by large goitre -- Requires intubation to secure airway

II NTERMEDIATE (1(1 DAY TO 11 MTH)) 1.1. Infection 2.2. Hypoparathyroidism leading to hypocalcaemia -- Risk of permanent hypoparathyroidism is 1-4% (only in total or subtotal thyroidectomies); 10-20% of patients may have te mporary hypocalcaemia -- Important to check the serum calcium levels post-operatively – – POD 1,3,5 -- Ask patient for any symptoms and look for signs of hypocalcaemia – – paraesthesia around the mouth, difficulty breathing, carpopedal spasm,, Chvostek’s sign (spasm of the facial muscles on tapping the facial nerve), Trousseau’s sign (carpopedal spasm on inflating blood pressure cuff over arm) -- Dangerous as it can cause laryngeal spasm and airway compromise -- Check serum calcium together with albumin to get corrected calcium!!

MM easeasurur ed ssed erum calciu m + 0.02 (40 – – Albumin)

-- Replacement: 5mmol/6h if symptoms mild, 10ml of 10% calcium gluconate over 30 minutes if severe -- Hypocalcaemia may also occur due to “hungry bone syndrome” after thyroidectomy in long-standing thyrotoxicosis 8585

PERIPHERAL ARTERIAL DISEASE -- The anterior tibial crosses into the anterior compartment of the leg and supplies the muscles there, and then continues as the dorsalis pedis in the foot (surface landmark: AANATOMY OF THE LOWER LIMB ARTERIES one third of the way down a line joining the midpoint of the two malleoli to the cleft between the first and second toes)) -- The posterior tibial supplies the posterior compartment of the leg and passes posterior to the medial malleolus (surface landmark: one third of the way down a line joining the medial malleolus to the heel) before dividing into medial and lateral plantar arteries to supply the sole of the foot -- Refer to diagram – – important to know the arrangement of the anterior tibial, posterior tibial and peroneal vessels at the trifurcation as you may be asked to read an angiogram of these vessels. -- From lateral to medial: Anterior tibial, Peroneal, Posterior tibial

FFORMS OF PERIPHERAL ARTERIAL DISEASE

Peripheral arterial disease

Acute Chronic

Critical Non-critical.

-- External iliac artery continues as the femoral artery after crossing the inguinal Asymptomatic Claudicants. ligament (surface landmark: the mid-inguinal point i.e. midway between the pubic symphysis and the anterior superior iliac spine) -- The femoral artery then divides into the superficial femoral and the profunda femoris (or deep femoral) arteries about 4cm below the inguinal ligament AACUTE LIMB ISCHAEMIA -- The profunda femoris supplies the compartments of the thigh via two main branches, Acute limb ischemia is defined as a sudden decrease in limb perfusion that causes a the medial and lateral circumflex femoral arteries potential threat to limb viability (manifested by ischemic rest pain, ischemic ulcers, -- The superficial femoral runs distally and passes through the adductor hiatus to reach and/or gangrene) in patients who present within two weeks of the acute event (if >2 the popliteal fossa, where it changes its name to become the popliteal artery weeks, it is considered chronic ischaemia). -- The popliteal artery divides into the anterior tibial artery and the posterior tibial The decrease in perfusion is usually due to sudden occlusion of a feeding arterial vessel, (also called tibioperoneal trunk by some), and the posterior tibial will give off the and this may be in a setting of already narrowed vessel lumen (acute on chronic peroneal artery ischaemia) or in a normal lumen. CAUSES: 4. 4. Dissecting aortic aneurysm 1 . 1. Arterial embolism -- As the blood dissects between the intima and media of the aorta, it can cause -- Most common cause of acute limb ischaemia (60-80% of the time) occlusion of the aortic branches at their origins -- The most likely source of embolus is the heart (80%), of which 70% is due to atrial fibril lation, 20% to AMI with left ventricular mural thrombus, and a small PATHOPHYSIOLOGY proportion toto prosthetic heart valves In order of sensitivity to ischaemia, the tissues affected are nerves (most sensitive), -- Non-cardiac emboli arise from other arteries where there are atherosclerotic muscle, skin, and bone (least sensitive); thus early signs of ischaemia involve pain and plaques or an aneurysm (the embolic material may be thrombus or part of aa numbness, and muscle paralysis as well as skin changes occur later. The lower limb can plaque, but atheroemboli are less likely to cause complete arterial occlusion)) survive about 6 to 8 hours in an ischaemic state before injury becomes irreversible. -- Most common sites where emboli lodge:  Bifurcation of the femoral artery (most common site) PRESENTATION  Trifurcation of the popliteal artery (next most common site in the lower limb) The classic 66 P’sP’s of of acute limb ischaemia: Pain, Paraesthesia, Pallor, Pulselessness,  Aortic bifurcation Paralysis, Perishingly cold  External and internal iliacs  Arm (about 20% of emboli) Pain -- Emboli usually cause lower limb ischaemia mostly -- Develops acutely -- After emboli obstructs the vessel, thrombus can propagate distally (due to stasis -- Starts off in a distal part of the extremity and then progresses proximally, increasing of blood) and proximally (due to turbulence of incoming blood hitting embolus) in severity with time by derangements in the Virchow’s triad -- Further progress leads to decrease in pain as the nerves die off from ischaemia -- Important to ask for any previous claudication painn (10% of claudicants can develop 2 . 2. Acute thrombos isis acute ischaemia due to thrombosis of the stenosed vessel) -- Thrombosis of a previously stenotic but patent artery ( atherosclerotic vessel)) -- Less common cause of acute limb ischaemia Paraesthesia -- When thrombotic occlusion of a vessel does occur, the resulting ischaemia is -- Starts off with paraesthesia (develops relatively early in the course of ischaemia) and usually less severe than in an embolic occlusion, because collaterals have had develops to complete loss of sensation time to form around the chronically stenosed vessel Pallor -- Other less common causes of acute thrombosis include the arteritides (usually -- Assess skin colour, temperature, and capillary refill affecting medium-sized arteries), ergotism, and hypercoagulable states (notably antiphospholipid syndrome). -- The limb may still be slightly pink though pale, but in severe ischaemia it can be marble-white (especially in embolus where there are no collaterals) 3 . 3. Arterial traum aa -- Other colours: -- Increasing incidence of acute arterial occlusion due to endovascular diagnostic  Mottling/Marbling (patches of blue on white): deoxygenation of stagnated blood; or interventional procedures surrounding areas of pallor are due to vasoconstriction -- Trauma can cause development of an arteriovenous fistula that shunts blood  Duskiness: due to deoxygenation of stagnated blood; if there is fixed staining (i.e. away from the limb does not blanch on pressure) then the limb is non-viable -- Fracture or dislocations can stretch an artery and cause an intimal tear while the  Black: gangrene media and adventitia layers are intact (because they contain elastin and can -- The disclouration usually affects a large part of the distal limb e.g. the toes, foot; stretch)  a thrombus forms at the site of the tear where underlying rarely does it only affect one toe (more in chronic ischaemia) thrombogenic collagen is exposed -- The site of arterial occlusion is usually one joint above the line of demarcation -- Compartment syndrome can result from trauma as well between normal and ischaemic tissue 87 Pulselessness EARLY ANTICOAGULATION - If able to feel one good pulse (PT or DP), quite unlikely that the limb is ischaemic, - Important to start anticoagulation with heparin if the suspicion of acute limb but still possible ischaemia is high - If unable to feel, assess with a handheld Doppler the arterial and venous flow in the - Give IV heparin bolus 3000-5000 units limb – there can still be flow without a palpable pulse - Follow with IV heparin infusion at 1000 units/hour - Also feel the pulses on the other limbs – gives a clue as to whether the cause is - Ideal PTT is 2 to 2.5 times normal embolic or thrombotic (see below) INVESTIGATIONS Paralysis - Pre-operative investigations - Total paralysis occurs late and usually indicates that the limb is non-viable - FBC, U/E/Cr, PT/PTT, GXM - Can assess viability of muscle by making a cut – viable muscle will be shiny and twitches in response to flicking, while dead muscle will be dull and will not twitch - CXR and ECG if patient is older than 40 yrs old - Dangerous to save dead muscle as reperfusion can cause circulation of toxic - If suspecting an AMI with mural thrombus, do cardiac enzymes metabolites in the muscle - Angiogram can be done in patients with viable limb, but in patients with threatened limb there is no time for angiogram  may do on-table angiography CLASSIFICATION OF SEVERITY (SVS/ISCVS) [Angiography is useful in confirming an occlusion, the cause – thrombotic or Three categories: viable, threatened and non-viable embolic – and also pinpointing the level of occlusion and the anatomy ] (i) Viable: No immediate threat of tissue loss (ii) Threatened: Salvageable if revascularised promptly DEFINITIVE TREATMENT OPTIONS (iii) Non-viable: Limb cannot be salvaged and has to be amputated, no emergency to Surgical Endovascular operate. Patient may require revascularisation to allow lower amputation or help - Embolectomy - Thrombolysis the amputation to heal - Endarterectomy - Angioplasty Viable Threatened Non-viable - Bypass grafting - Stenting Pain Mild Severe Variable - Fasciotomy Capillary refill Intact Delayed Absent (fixed stain) - Primary amputation Motor deficit None Partial Complete Sensory deficit None Partial Complete In general, embolectomy is done for embolic occlusion, while thrombolysis is done for Arterial Doppler Audible Inaudible Inaudible thrombotic occlusion. Venous Doppler Audible Audible Inaudible Treatment Urgent work-up Emergency surgery Amputation Embolectomy - Can be done under LA but still require anaesthetist to monitor patient as he may be quite sick (e.g. AMI), and hyperkalaemia with cardiac arrhythmia can occur after DIFFERENTIATING BETWEEN EMBOLIC AND THROMBOTIC CAUSE reperfusion Embolic Thrombotic - Involves clamping of the involved artery and making an arterotomy Identifiable sou rce Present – AF, recent AMI Less common Claudication hx Negative Positive - A Fogarty balloon catheter is inserted into the artery until distal to the clot, then the Physical findings Contralat pulses present Contralat pulses diminished balloon is inflated to trawl the clot out of the artery White limb (no blood) Dusky limb (collaterals still - Check for forward-bleeding and back-bleeding of the vessel (i.e. free spontaneous supplying limb) flow from proximal and distal ends of the artery when unclamped) Angiography Minimal atherosclerosis, Diffuse atherosclerosis, - Flush with heparinised saline sharp cut-off, few collaterals irregular cut-off, well- developed collaterals - Check foot – warm foot with good pulse indicates reperfusion - Important to monitor ECG for any arrhythmias! CHRONIC LIMB ISCHAEMIA - Closure of arterotomy with meticulous haemostasis as patient is on heparin Chronic limb ischaemia can be divided into critical and non-critical limb ischaemia, - Post-op: patient monitored in high-dependency; look out for reperfusion injury and non-critical ischaemia further subdivided into that which causes symptoms (usually  The reperfused muscles become oedematous, increasing pressure in the claudication) and that which is asymptomatic. compartments of the leg, like compartment syndrome Most common cause is atherosclerosis with gradually developing diffuse stenosis of  Patient complains of calf pain the peripheral arteries resulting in diminished blood supply to the lower limb  Unable to dorsiflex ankle as the anterior compartment is affected first (imbalance between supply and demand). Multiple collaterals form to bypass the  Requires three compartment fasciotomy to release pressure obstructed vessels as a compensatory mechanism - Need to convert to full warfarin anticoagulation, uptitrating dose until INR 2-2.5 before stopping heparin (patient at risk of further embolic events) CRITICAL LIMB ISCHAEMIA - Discharge patient to anticoagulation clinic for follow-up with warfarin advice Critical limb ischaemia is defined as decrease in limb perfusion that causes a potential threat to limb viability (manifested by ischemic rest pain, ischemic ulcers, Thrombolysis and/or gangrene) in patients who present more than two weeks after the acute event - Angiogram done before thrombolysis to locate occlusion (the converse of the definition of acute limb ischaemia). - Thrombolysis catheter inserted into the clot, and the thrombolytic agent is infused FEATURES: - Patient will be in high-dependency with thrombolytic infusion for 6 hours (~1000- 1. Rest pain requiring regular opioid analgesia (e.g. codeine) lasting >2 weeks 4000 units per minute) AND/OR - After 6 hours, redo angiogram to check for residual clot; if some clot remains, adjust 2. Gangrene or ulcers over the toes or feet catheter into the clot and infuse for 6 more hours AND - After complete lysis of the clot, can do angioplasty 3. Objective indication of poor vascular supply to the lower limbs - Takes much longer than embolectomy (a) Ankle brachial pressure index <0.5 - Thrombolysis may be preferred for embolism in a diseased artery, since it may be (b) Toe pressure <30 mmHg difficult to trawl out the clot in a diffusely stenosed vessel – the clot may get caught I. Rest p ain on a proximal stenosed segment - Severe pain in the distal portion of the lower limb (usually toes, foot but may involve more proximal areas if disease is severe) occurring at rest Results: - Pain is aggravated or precipitated by lifting the limb, relieved by dependency of - Embolectomy has a 20% mortality, almost full success rate the limb – many patients sleep with the leg hanging over the side of the bed to - Thrombolysis has a 10% mortality, only 35% successful relieve the pain - So severe as to disturb sleep at night - Not easily controllable with analgesia – requires opioids to control pain

II. Ischaemic ulc ers - Usually arise from minor traumatic wounds with poor healing - Often painful - Most often occur on the tips of the toes, bunion area, over the metatarsal heads (ball of the foot), lateral malleolus (as opposed to venous ulcers that occur over the medial malleolus) 89 - Usually deep, dry, punctate (unlike venous ulcers that tend to be superficial, moist, NEUROGENIC CLAUDICATION diffuse) - Vascular intermittent claudication needs to be differentiated from neurogenic - May become infected resulting in cellulitis, even abscess formation, and spread to claudication which can also present as pain in the lower limb on exertion involve the underlying bone and joints  osteomyelitis, septic arthritis - The characteristic of neurogenic claudication is “park bench to park bench” where the patient has to sit down and flex the spine to relieve the pain (pain results from III. Gangr ene compression of the cord and spinal nerves in spinal stenosis; extension of the spine - Cyanotic, anaesthetic tissue associated with or progressing to necrosis further narrows the spinal canal while flexion widens it) - Occurs when arterial blood supply falls below that which is necessary to meet - “Claudication distance” of neurogenic claudication is more variable minimal metabolic requirements - Either dry or wet: - Pulses will be absent/diminished in vascular but not in neurogenic claudication DRY – hard, dry texture. Often has a clear demarcation between viable and necrotic tissue. Occurs in patients with atherosclerotic disease. Safe and can be TAKING A HISTORY OF CHRONIC LIMB ISCHAEMIA allowed to autoamputate after demarcation with precautions against infection. 1. Claudication WET – moist, swollen, frequently blistered. Often occurs in diabetics with - Which part of the lower limb does the pain occur in decreased sensation and unrecognised trauma. An emergency requiring surgical - Nature of the pain debridement or amputation. - Radiation - Severity - Aggravating factors – exertion NON-CRITICAL LIMB ISCHAEMIA WITH CLAUDICATION - Relieving factors – rest (just standing is sufficient) Intermittent claudication is defined as a reproducible discomfort of a defined group - Associated symptoms e.g. impotence in LeRiche’s of muscles that is induced by exercise and relieved with rest. Usually described as the - When did pain first start patient as a cramping, aching pain in the muscle group on exertion such as walking, and - Progress since first noticed until currently (worsening pain, increasing areas of alleviated on stopping (patient does not have to sit down for pain to go away) – “shop lower limb affected, pain on less exertion, development of rest pain) window to shop window”. - Current claudication distance - Usually affects the superficial femoral near to the adductor hiatus, or the popliteal - How has symptoms affected lifestyle e.g. impaired mobility artery  calf pain 2. Any rest pain - Foot claudication results from involvement of the tibial and peroneal arteries, but - Site, nature, severity rarely do patients with claudication due to atherosclerosis get foot pain alone (more - Aggravating factors – raising the limb common in Buerger’s) - Relieving factors – putting limb in a dependent position - Thigh claudication results in common femoral artery or aortoiliac disease - Able to relieve with normal analgesics? Or require opioid analgesia? - LeRiche’s syndrome arises from occlusion of the aortoiliacs, and is composed of a - How long has rest pain lasted for requiring opioid analgesia (if more than 2 classical tetrad of buttock claudication, impotence in men, absent femoral pulses (and weeks, considered a feature of critical limb ischaemia) distal pulses), and occasionally presence of aortoiliac bruits. 3. Any ulcer or gangrene in the lower limb? - Important to determine the “claudication distance” – within a short period of time the distance is usually fairly constant, but can shorten as the disease progresses - Ask about onset of ulcer/gangrene - Progress (stable, or increasing in size, getting worse) CAUSES OF VASCULAR CLAUDICATION - If ulcer, any preceding trauma? Ill-fitting shoes? Altered sensation in the foot? Does patient take care to protect foot ? Pain? Redness/swelling/warmth in - Most commonly atherosclerotic disease surrounding skin? Purulent/foul-smelling discharge from the ulcer? - Other less common causes: ergot toxicity, Takayasu’s arteritis, Buerger’s disease (thromboangiitis obliterans), vasospasm - If gangrene, is it wet or dry? Redness/swelling/warmth in surrounding skin? Any  Neuropathic ulcers form at areas such as the ball of the foot and the heel feeling in the toe involved? Any sensory changes in the other normal toes, foot, - Size, shape limb? - Edges (punched out – arterial; sloping – venous) - Any systemic signs of infection – fever, chills, rigors, malaise - Base 4. Risk factors (“Arteropath”)  Depth of the ulcer (can see underlying tendon? Down to bone?) - Diabetes mellitus – take a full diabetic history including other complications  Appearance of the base – Necrotic? Granulating (beefy-red)? Sloughy? - Hyperlipidaemia  Any discharge – pus, blood? - Heart disease - Surrounding skin - Stroke  Erythema (cellulitis) – there may be an underlying abscess (confirm on - Smoking palpation) - Family history  Blistering, purplish colour (possibility of necrotising fasciitis) 5. Drug history 5. Presence of gangrene - Aspirin intake - Wet (infected) or dry (not infected) - Any allergies to contrast (for angiography) - Extent of gangrene (level of demarcation) - Ergots 6. (If the patient has diabetes, may see deformities – Charcot’s joint) 6. Social history - Premorbid function and current function Feel - Social support and home condition (need to climb stairs?) 1. Warmth of the skin - Use the dorsum of the fingers of both hands to simultaneously run up the PHYSICAL EXAMINATION patient’s feet to the shins and thighs bilaterally - Compare the temperature on both sides (note if one side is cooler) Examine the patient’s lower limbs in a warm room, with the patient exposed optimally - If one limb feels cool, feel for the level where the skin becomes warm (from the thighs to the feet, wearing underwear). Patient is supine with the bed flat. 2. Capillary refill Look - Press hard on a toe for a few seconds, then release 1. Colour of the lower limb - Normal capillary refill should be 2 seconds or less - White (pallor); pink (normal); blue/dusky (cyanosed); mottled - If a toe is blue, check for blanchability (fixed staining = dead toe) 2. Trophic changes 3. Palpating the ulcer if present - Loss of hair - Any surrounding tenderness (infection) - Dry, shiny skin - Bogginess of surrounding tissue (may have abscess formation) - Nail changes - See if any discharge from the ulcer when palpating - Wasting 4. Pulses 3. Presence of any diabetic dermopathy - Feel the distal pulses and work your way proximally 4. Presence of ulcer - Posterior tibial pulse: one-third of the way down a line joining the medial - Look carefully at the entire lower limb, including the heels (ask patient to flex at malleolus to the heel the knee so you can look at the heel) and between the toes - Dorsalis pedis pulse: one third of the way down a line joining the midpoint of - Site of the ulcer the two malleoli to the cleft between the first and second toes  Venous ulcers form at the medial malleolus - Popliteal pulse: Ask patient to bend the knee ~60-90 degrees, then palpate  Arterial ulcers are more distal, usually between the toes, and at pressure deeply in the popliteal fossa with the fingers of one hand pressing the fingers of points such as the lateral malleolus; the other [If the pulse is very well felt, suspect a popliteal aneurysm] 91  - Femoral pulse: Midpoint of the line joining the pubic symphysis to the anterior This ankle pressure is then divided by the brachial pressure (the higher of the superior iliac spine (mid-inguinal point), just below the inguinal ligament two brachial pressures for both upper limbs) to get the ankle-brachial pressure index - Grading of pulses: 2+ is normal; 1+ is diminished (but may be normal for popliteal); negative if not felt  label on a stick-figure diagram - Interpreting the values  Normal ABPI is greater than 0.9 (can be more than 1.0 as ankle pressures Move tend to be higher than brachial; if >1.3, suggests non-compressible calcified 1. Buerger’s test vessel) - Do one side at a time  ABPI between 0.5 to 0.9 – occlusion, often associated with claudication - Holding the heel of the foot, with the patient’s lower limb straightened, slowly  ABPI <0.5: Critical ischaemia lift the entire lower limb, looking at the colour of the toes - Accuracy of the index - Stop when the toes become pale (white)  ABPI below 0.9 has 95% sensitivity and 100% specificity for detecting - Estimate the angle the lower limb makes with the horizontal – this is the angiogram-positive peripheral arterial disease and is associated with >50% Buerger’s angle stenosis in one or more major vessels  Normal lower limb can be raised to 90 degrees without turning white; if the - Exercise treadmill testing Buerger’s angle is less than 20 degrees, this indicates severe ischaemia  Measure ABPI before and after patient exercises on a treadmill - There may be venous guttering of the lower limb at this angle as well  If the ABPI falls by >0.2  claudication - If the patient is lying near the side of the bed, tell the patient that you’re going to put his leg over the edge of the bed before gently abducting the hip and then 2. Duplex ultrasound letting the leg drop over the edge of the bed - Non-invasive test, good alternative to angiogram - Look at the leg for reactive hyperaemia (the leg turns purple-red) - Duplex (means two modalities) = 2D ultrasound (like the normal kind) plus Doppler ultrasound (measures flow and waveforms) Complete the exam - Normal arterial flow waveform should be triphasic; biphasic and monophasic - Examine the rest of the pulses waves are abnormal - Offer to auscultate over the femoral and popliteal arteries for bruits - Can define anatomy of occlusions and also look for relatively good arteries - Examine the abdomen for any abdominal aortic aneurysm distally for “landing zone” of bypass graft - Measure the ankle-brachial pressure index (ABPI) 3. Angiogram (arteriogram) - Invasive and associated with risks of bleeding from arterial puncture, INVESTIGATIONS dissection/damage to artery with worsening ischaemia 1. Ank le-brachial pressu re index - Usually only done if planning intervention e.g. angioplasty, stenting - How the ankle-brachial pressure index is done - Preparing for angiogram:   Brachial pressure is measured with a blood pressure cuff around the arm and Take informed consent from patient  a Doppler probe at the brachial artery – cuff is inflated until the arterial Ask about contrast allergy, asthma, renal disease, metformin  signal is obliterated, then slowly deflated until the signal just starts being Investigations: FBC (platelets impt), PT/PTT, creatinine detected, at which the pressure is recorded - Angiogram with digital subtraction – the images of the underlying bone are removed so as to better visualise the arteries (if the bones are visible, then it is a  Ankle pressures are measured in a similar manner, with the cuff around the normal angiogram, without digital subtraction) calf and the Doppler at the dorsalis pedis and posterior tibial arteries – one reading for each artery  The ankle pressure to be used for each leg is the higher of the two taken ASSESSMENT OF SEVERITY 1. Angioplasty  The three L’s of peripheral arterial disease: Stenting usually not done for lower limbs except in aortoiliacs (since stent (i) Life – does disease threaten life (e.g. sepsis; other complications of atherosclerosis needs to be placed in a vessel which is relatively fixed and won’t be e.g. stroke, AMI;) or will intervention cause risks kinked/bent by movement) (ii) Limb – will patient lose the limb  Angioplasty only effective for focal stenotic lesions and better for large (iii) Lifestyle – is the lifestyle of the patient severely handicapped, does it require vessels intervention  Problem with angioplasty is that it is not long-lasting – restenosis can occur  New method: subintimal angioplasty – if lumen is so occluded that guide Fontaine system wire cannot pass through, the guidewire is threaded into the subintimal space Stage I: Asymptomatic to create a dissection around the occluded segment, and this space is then Stage IIa: Mild claudication angioplastied to create a channel parallel to the actual lumen for blood to Stage IIb: Moderate to severe claudication flow through Stage III: Ischaemic rest pain Stage IV: Ulceration or gangrene 2. Bypass grafting  Consider bypass when lesions cannot be treated by angioplasty i.e. lesion TREATMENT OF CLAUDICATION extends for long distance through the vessel and/or no lumen for guide wire Conservative to pass through (complete occlusion) - Smoking cessation  Needs a good “landing zone” for graft distally – if vessel is diffusely - Exercise training diseased, difficult to perform bypass  Exercise at least half to one hour every day  Walk until pain comes, rest 2-3 minutes, walk again TREATMENT OF CRITICAL LIMB ISCHAEMIA  Keep a walk diary recording daily claudication distance in paces Need to revascularise – see interventions above  Will stimulate collateral formation  symptoms get better - Podiatrist to teach foot care - Assessment of cardiovascular risk factors and treatment to optimise control – AMPUTATION cardiologist Indications (3 D’s) - Teach patient about symptoms of critical ischaemia, to return to ED if such 1. Dead symptoms arise  Necrotic tissue - Antiplatelets e.g. aspirin 2. Dangerous   Gangrene, ascending sepsis - ?Use of Vasteral (methoxyphylline) 3. Damn nuisance - Monitor regularly with measurement of ABPI  Non-functional limb; bad smell; pain; constant need to dress wound

Intervention (endovascular or surgical) - Level of amputation depends on vascularity of the limb and the indication (e.g. if - At least 6 months of conservative treatment first infected, need to amputate above level of infection) - Monitor claudication distance and ABPI – intervene if deteriorating despite - As far as possible try to preserve function of the lower limb conservative management - May require revascularisation interventions before amputation to ensure good healing, - If parameters improve but then plateau, discuss with patient about whether he can or to enable lower amputation accept the level of symptoms, and the risks of intervention  weigh risks against - Do not simply amputate without ensuring good vascular supply to the surgical site, benefits otherwise the wound will not heal - Usually do angioplasty rather than bypass as it is less invasive, though may not be as effective in treating the symptoms 93 ABDOMINAL AORTIC ANEURYSM PHYSICAL EXAMINATION EPIDEMIOLOGY - Ensure vitals stable More common in men than in women (4:1 ratio) - Visible pulsation over abdomen Predominantly in older patients (>60 years old) - Pulsations and mass in epigastric region felt on deep palpation Other risk factors: smoking, hypertension, strong family history (Marfan, Ehler-Danlos) - Mass is expansile – when fingers of both hands are placed at the edges on either side of the mass, the fingers are pushed upwards and outwards PATHOLOGY - Auscultate for bruit over the mass - An aneurysm is a localised abnormal dilatation of a blood vessel wall or the heart - Check the other arteries – femoral, popliteal – for any aneurysm, and listen for bruits - True aneurysms are bound by all layers of the blood vessel wall, while a false - Look at the lower limbs for any gangrene, infection, etc aneurysm is a breach in the blood vessel wall leading to an extravascular haematoma that freely communicates with the intravascular space INVESTIGATIONS - Atherosclerosis is the most common aetiological factor – plaque formation results in Mostly imaging to delineate aneurysm  CT Scan destruction of the tunica media (and the elastin fibres in it)  arterial wall thinning  and loss of elastic recoil dilatation MANAGEMENT - Other causes: cystic medial degeneration (in Marfan), trauma, infection (mycotic) Dependent upon clinical context – is patient asymptomatic, symptomatic but not yet - Location: usually infrarenal (95% of cases), may extend to involve common iliac ruptured, or ruptured? arteries, rarely beyond Ruptured AAA - Size: 3 to 15 cm (normal aorta is 2cm in diameter) - Very high mortality – nearly 100% if frank rupture (will not get to ED in time) - Shape: Usually fusiform – long dilated segment (versus saccular which is spherical) - Most of the patients who reach the ED (about 50% reach ED alive) have a leaking - Often contains mural thrombus due to turbulence and stasis AAA with a tamponade effect by the retroperitoneal structures - High suspicion in unstable hypotensive patient complaining of severe sharp pain RISK OF RUPTURE radiating to the back; may feel a pulsatile mass in the a bdomen - Small aneurysms <5cm have a 2-3% chance of rupture per year, while aneurysm 1. Stabilise patient – resuscitation with fluid and blood products larger than 5.5 cm will have a 10% risk of rupture per year 2. Call for vascular surgeon - 75% of aneurysms 7cm or larger will rupture in 5 years 3. Do not intubate as neuromuscular blocking agents will reduce tamponade effect, worsening haemorrhage PRESENTATION 4. Bring to operating theatre for open repair – surgeon’s main task is to quickly - Most commonly asymptomatic, found incidentally during imaging isolate the aorta and clamp it proximally (can be clamped for about 30 minutes - Most feared presentation is that of rupture – patient complains of intense abdominal without significant visceral ischaemia) pain radiating to the back, becomes rapidly hypotensive and goes into shock 5. After clamping the aorta, the AAA is incised, the surrounding haematoma and - Thromboembolism distally – gangrene of feet (trash feet) mural thrombus within the AAA are cleared out - Local compression on neighbouring structures e.g. ureter 6. Synthetic graft (Dacron – polytetrafluoroethylene) is placed within the aorta and - Obstruction of branches from aorta e.g. iliac, renal, mesenteric, vertebral the vessel wall closed up over the graft i.e. the graft forms the lumen of the aorta 7. Most common complication postoperatively is renal insufficiency – can be reduced by giving frusemide or mannitol pre-operatively before anaesthesia induction - Mortality rate of repair operation in this setting is about 50% Non-ruptured AAA - Time available for investigation of size of AAA and related anatomy - Indications for surgery: (a) Aneurysm > 5.5 cm in largest diameter (b) Increase in diameter of more than 1cm per year (c) Symptomatic aneurysm – back pain, tenderness on palpation, distal embolism, ruptured/leaking aneurysm - Patient’s fitness for surgery needs to be properly assessed because it is a major operation – need to optimise cardiovascular function - Operation is the same except that it is done under elective setting - Mortality is <5% in the elective setting, serious morbidity ~10%

Endovascular stenting - An alternative to open repair which is less invasive, can be done under GA - Mortality ~1%, but serious morbidity rate is similar to open repair: 10% - Involves deployment of a non-porous stent within the aneurysm to form the lumen of the aorta – requires adequate “neck” proximally and good landing site distally - Not as good results as open surgery; need to do an angiogram every 6 months to check position of the stent (ensure that stent has not migrated)

COMPLICATIONS OF SURGERY Intraoperative/early 1. Acute myocardial infarction – most patients already have atherosclerotic disease of coronary vessels and are at risk of AMI (responsible for 50-60% of mortality) 2. Stroke (due to hypotension or embolism) 3. Renal insufficiency 4. Colon ischaemia – occurs in 2-6% 5. Trash foot – embolism of thrombus from the aneurysm 6. Infection of graft 7. Spinal cord ischaemia (quite uncommon)

Late 1. Aortoenteric fisula – frank PR bleeding, torrential 2. Late infection of prosthetic graft material 3. Sexual dysfunction 95 PERIPHERAL VENOUS DISEASE - Course of the great saphenous vein:  Arises from the medial end of the dorsal venous arch of the foot  Passes anterior to the medial malleolus ANATOMY OF THE VENOUS SYSTEM OF THE LOWER LIMB  Runs up the leg posteriorly to pass behind the medial surface of the knee  Then runs anteriorly and laterally up the thigh  Pierces the cribriform fascia at the saphenofemoral junction to drain into the femoral vein

- Course of the small saphenous vein:  Arises from the lateral end of the dorsal venous arch of the foot  Passes posterior to the lateral malleolus  Runs up the midline of the calf  Pierces the deep fascia over the popliteal fossa to drain into the popliteal vein

- The superficial system and the deep system communicate through communicating veins that contain valves which allow only one-way flow of blood from the superficial vein into the deep vein

- Locations of the communicating veins:  Saphenofemoral junction (great saphenous drains into femoral vein): located 2.5 cm below and lateral to the pubic tubercle  Hunterian perforator: mid-thigh  Dodd’s perforator: distal thigh  Boyd’s perforator: knee  Calf perforators: at 5, 10, and 15 cm above the medial malleolus

- Physiology of venous d rainage:  Main mechanism is the calf muscle pump  Contraction of the calf muscles compresses large venous sinuses in the muscles, squeezing the blood into the popliteal vein and back to the heart  The deep veins have many valves to prevent backflow, so blood only flows towards the heart  During calf muscle relaxation, the intramuscular veins open and suck blood in from the superficial system through the communicating veins, thus draining the - The venous drainage of the lower limb is divided into a deep venous system and a superficial veins superficial venous system separated by the deep fascia of the lower limb - The deep venous system is composed of veins corresponding to the arterial supply e.g. anterior and posterior tibial veins, popliteal vein, femoral vein - The superficial venous system is composed of two major veins, the great saphenous vein and the small saphenous vein CHRONIC VENOUS INSUFFICIENCY 4. Venous ulcer formation - Typical location is over the medial malleolus Chronic venous insufficiency develops when there is venous hypertension, which can - Shallow, flat ulcer with sloping edges; base may be sloughy or granulating, result from: usually quite moist-looking 1. Obstruction to venous flow e.g. tumour compression in the pelvis, pregnancy, deep vein thrombosis - Surrounding skin will show signs of CVI 2. Dysfunction of venous valves e.g. varicose veins - In long-standing ulcer SCC can develop (Marjolin’s ulcer) – If ulcer enlarges, becomes painful and malodorous, edge becomes thickened or raised, if inguinal 3. Failure of the “venous pump” – dependent on adequate muscle contraction lymph nodes are enlarged (stroke, muscular weakness can cause failure) as well as c ompetent venous valves These manifestations can be asymptomatic or a ssociated with symptoms of leg fullness, MANIFESTATIONS OF CHRONIC VENOUS INSUFFICIENCY: aching discomfort, heaviness, nocturnal leg cramps, or bursting pain upon standing.

1. Venous dilatations (a) Telangiectasias (spider veins or venous stars – intradermal veins) CEAP CLASSIFICATION OF CHRONIC VENOUS INSUFFICIENCY (b) Reticular veins (slightly larger intermediate veins) (c) Varicosities (visible, dilated tortuous superficial veins; formed by main tributaries of the saphenous veins because these do not have a strong coat of smooth muscle in their walls, unlike the saphenous veins; they are more superficial and not bound down to the deep fascia) (d) Corona phlebectactica (a network of small dilated venules beneath the lateral and/or medial malleolus with severe venous hypertension)

2. Oedema – pitting : The hallmark of CVI; present in all but the earliest stages Unilateral oedema worsened by dependency (worse at the end of the day) and better with recumbency

3. Skin changes (a) Hyperpigmentation of the skin over medial lower third of the leg (gaiter area) – due to extravasation with haemosiderin deposits (b) Atrophie blanche – hypopigmented scars of healed venous ulcers (avascular and fibrotic skin) (c) Venous stasis eczema – pruritic, weeping, scaling, with erosions and crusting (d) Lipodermatosclerosis – a fibrosing panniculitis of the subcutaneous tissue that results in a firm area of tender, indurated, hyperpigmented skin that is fixed to VARICOSE VEINS subcutaneous tissue. Varicose veins are dilated, tortuous veins. They can be divided into primary varicose  Results from severe venous hypertension veins, where the cause is unknown (may be related to posture and components and  Starts in the gaiter area and extends circumferentially to surround the leg structure of the vein wall), and secondary varicose veins, which result from proximal  If severe can result in an “inverted champagne bottle” appearance of the venous obstruction, destruction of the valves by thrombosis or an increase in flow and leg with brawny oedema above and below the area of lipodermatosclerosis pressure caused by an arteriovenous fistula. (e) Cellulitis 97 PATHOPHYSIOLOGY Special tests - Inherent weakness in the vein wall, leading to dilation and separation of valve cusps TOURNIQUET TEST so they become incompetent - This may be aggravated by obstruction to venous return (as above) - Lie the patient down and empty the varicosities - Tie a tourniquet just below the SFJ RISK FACTORS - Ask the patient to stand up - Age - Look for filling up of the varicosities above and below the tourniquet - Parity - If the veins dilate above but not below the tourniquet, this indicates that the - Occupation – requiring long periods of standing perforators below the level of the tourniquet are not incompetent and that the SFJ is - Weight incompetent  confirm this by releasing the tourniquet and watching the veins dilate - Posture – crossing legs all the time - If the veins below the tourniquet are dilated when the patient stands up, then the - Increased abdominal pressure – constipation, chronic cough, etc incompetent perforator is below the level of the tourniquet - Pelvic tumour or other lesion compressing on the deep veins - Repeat the test, placing the tourniquet at different sites: (i) Mid thigh (just below the Hunterian perforator HISTORY (ii) Below the knee Usually varicose veins do not cause symptoms and problems unless they are related to (iii) Mid-calf chronic venous insufficiency - The incompetent perforator is located between just above the level where the tourniquet prevents dilation of the veins in the limb on standing EXAMINATION: [The alternative is the triple tourniquet test, where three tourniquets are tied with the Examine patient standing with adequate exposure of the lower limbs patient lying down and then released from the bottom up to locate the site of Inspection (look all around the limb!) insufficiency] 1. Presence of signs of chronic venous insufficiency (as above) - Oedema TRENDELENBURG TEST - Skin changes - The SFJ is occluded (landmark is 2.5 cm below and lateral to the pubic tubercle) with - Venous ulcers from pressure necrosis from insude the patient lying down 2. Look at course of great saphenous vein and short saphenous vein for varicosities - Get the patient to stand while holding the SFJ occluded 3. Look at the inguinal region for any saphena varix - If varicosities do not fill up, the SFJ is the site of incompetence; if they fill up, there are other sites of incompetence (the SFJ may or may not be incompetent) Palpate 1. Feel any dilated varicosities PERTHES’ TEST 2. Palpate along the course of the saphenous veins and their tributaries to feel any - Tie a tourniquet around the calf or thigh and ask patient repeatedly stand on tiptoe varicosities present (may be more palpable than visible especially in fat legs) and then relax - In a person with normal deep venous drainage and competent venous valves in the 3. Palpate the inguinal region for a saphena varix (compressible lump that refills communicating veins the superficial veins should drain into the deep veins when released) - If the patient’s varicosities remain enlarged then he or she has obstructed deep 4. Do the cough test to feel for reflux at the saphenofemoral junction (2.5 cm below venous drainage or incompetent valves in the communicating veins and lateral to the pubic tubercle) 5. Percussion (tap test) – place two hands some distance apart. First percuss the Completing the examination distal veins to feel the wave of blood flowing orthogradely  normal. Then percuss - Auscultate over the varicosities for any bruit (indicate arteriovenous malformation) the proximal veins – if the distal hand can feel the wave of blood flowing - Examine the abdomen for any mass that may be causing the varicosities retrogradely then there is valvular incompetence (not a very valuable test) Use of a handheld Doppler probe to detect incom petence VENOUS ULCERS - Doppler probe is placed in the popliteal fossa between the two heads of the gastrocnemius – over small saphenous vein CAUSE – ANY CAUSE OF CHRONIC VENOUS INSUFFICIENCY - Squeeze the calf to empty the veins – should hear a whoosh as blood flows through 1. Obstruction to venous flow – thrombosis the small saphenous vein 2. Incompetent valves – varicose veins, deep vein reflux (post-DVT) - When the calf is relaxed there should not be any sound – a second whoosh indicates 3. Muscle pump failure – stroke, neuromuscular disease reflux of blood i.e. there is valvular incompetence

INVESTIGATIONS INVESTIGATIONS Venous duplex ultrasound 1. Exclude infection of the ulcer and other complications - Indications: - FBC for raised total white count  Recurrent varicose veins - Swabs of the ulcer for Gram stain and cultures  History of superficial thromobophlebitis - X-ray of the area to exclude underlying gas, bone involvement  History of DVT 2. Venous duplex to map out venous system  Venous eczema 3. Check for peripheral arterial disease by doing ABPI  Haemosiderin staining 4. Biopsy if cannot exclude malignant transformation (Marjolin’s ulcer)  Lipodermatosclerosis  Venous ulceration - Can delineate deep and superficial venous systems and locate sites of incompetence MANAGEMENT: - Exclude presence of deep vein thrombosis – stripping is contraindicated Conservative 1. 4 layer compression stockings (change once per week) MANAGEMENT (a) Non-adherent wound dressing over ulcer (e.g. Menolin) followed by wool bandage Conservative (b) Crepe bandage 1. Lifestyle changes (c) Blue-line bandage (Elset) - Decrease amount of time spent standing (d) Adhesive bandage (Coban) - If due to job, change job or ask for change to position that involves less standing and walking 2. Analgesia 2. Graduated compression stockings 3. Antibiotics if infected 3. Medications e.g. Daflon 4. Warn patient to avoid trauma to affected area 5. Encourage rest and elevate leg Surgical 6. Once healed, compression stockings should be fitted and continued for life Indications: 1. Cosmesis – large unsightly varicosities Surgical 2. Symptoms – pain, discomfort - If ulcer fails to heal 3. Complications – signs of chronic venous insufficiency, venous ulceration - First, exclude malignancy or other causes of ulcer (biopsy) Available modalities: - Split skin graft can be considered with excision of dead skin and graft attached to 1. Most commonly done: High tie with great saphenous vein stripping, and stab healthy granulation tissue avulsion of varicosities - Venous surgery for the underlying pathology 2. Ultrasound-guided foam sclerotherapy 3. Endovenous laser therapy (burns vein from within) 99 UROLOGICAL DISEASES HISTORY Post-renal Causes APPROACH TO HAEMATURIA 1. Which part of urine stream is blood stained? DEFINITION: - Beginning – urethra distal to UG diaphragm - End – bladder neck or prostate - >3 RBC / hpf. - Throughout – upper urinary tract or upper bladder - DDx: haemoglobinuria, myoglobinuria, pseudohaematuria (menstruating women), medications causing discoloration of urine (eg rifampicin, phenytoin) 2. Painful vs painless haematuria Painful Painless CAUSES - Tumour - Malignancy – RCC, TCC, Prostate Pre- Drugs  Analgesics (NSAIDs) - Hydronephrosis - Drugs Renal  Anticoagulants - Renal cysts - GN  Cytotoxic/immunosuppressive agents (eg cyclophosphamide) - Ureteric stone / clot - Bleeding diathesis  OCP - Pyelonephritis - ITP / HSP  Penicillin - UTI - Infections – malaria, schistosomiasis  Quinine - Bladder outflow obstruction (e.g. - Exercise  Warfarin BPH, strictures) Systemic  Bleeding diathesis  Sickle cell disease 3. Frequency + dysuria + haematuria Metabolic  Hypercalciuria - DDx: nephrolithiasis (colicky), malignancy, UTI (women & children), bladder  Hyperuricosuriia outflow obstruction (men e.g. BPH) Vascular  AV malformations 4. Other urological symptoms  Renal artery disease – thromboembolism, dissecting aneurysms, - Storage problem – frequency, urgency, nocturia, incontinence malignant hypertension - Voiding problem – strangury, hesitancy, dribbling, incomplete emptying etc  Renal vein thrombosis - Others – polyuria, oliguria, urethral discharge Renal Vasculitis  HSP  PAN Pre-renal & Renal Causes  Wegener granulomatosis 5. Associated fever – pyelonephritis, malaria Glomerular  Post-strep GN 6. Screen for pre-renal causes  Post-infectious GN LOW / bone pain / sickness Malignancies, TB, systemic illnesses  IgA nephropathy  Lupus nephritis Rash, arthritis, arthralgia, Autoimmune causes, vasculitis  Other GNs myalgia, fever, oedema Sore throat, skin infxns, URTI Post-strep / post-infective GN Tubulo-  Polycystic kidney disease Ongoing URTI or GE IgA nephropathy interstitial  Nephrolithiasis Iatrogenic Drug causes, radiotherapy dz  Malignancy – RCC, metastatic  Pyelonephritis Travel history Schistosomiasis, malaria  Renal cysts PMHx Renal disease, HPT, diabetic nephropathy, bleeding diathesis, sickle cell dz Post-  Infxns of ureter, bladder, prostate, urethra – eg schistosomiasis, TB etc Family history PKD, sickle cell disease, renal dzes (eg renal  Cancers of ureter, bladder (TCC), prostate, urethra Alport syndrome – ask for deafness), hypt,  Nephrolithiasis urolithiasis Other necessary history 8. Plain KUB 1. Infection - Fever, travel and contact history - Stones, size of kidney 2. Sorethroat - Post-strep/infective GN, IgA nephropathy 9. Ultrasound of the kidneys 3. Autoimmune - Fever, rash, joint pain, oedema - Renal size 4. Malignancy - LOW, bone pain, neuro deficits, SOB, liver function - Presence of any hydronephrosis 5. PMHx - Renal dz, - Renal stones - systemic dz (DM HPT Bleeding sickle cell) 10. Intravenous ur ogram (IVU) – see below for m ore details 6. Drug history / Hx of radiation - Distortion of renal outline and pelvic calyces by RCC, may have specks of 7. Family history – PKD, renal dz, Sickle cell, HPT calcification - Stones (filling defect, proximal dilatation, decreased distal passage of contrast) + PHYSICAL EXAMINATION hydroureter and/or hydronephrosis 1. Check patient’s vitals- stable? - Filling defect in bladder due to TCC 2. Conjunctival pallor - Increased residual volume in bladder after micturition due to BPH 3. Abdomen – renal mass, palpable bladder/bladder mass 11. Cystoscopy 4. Scrotum – varicocoele on the left (may have RCC of the left kidney with extension - Detection of bladder tumour (IVU may not pick up small tumours <1cm) of tumour into renal vein, blocking the testicular vein where it drains into the left - Biopsy can be taken at the same time renal vein) 5. Digital – prostate enlargement (BPH versus cancer) KUB FILM - Margins: Superiorly needs to be above the upper pole of the right kidney (T12), inferiorly needs to show the pubic symphysis INVESTIGATIONS

1. Urine dips tick I NTRAVENOUS UROGRAM - Causes of false-positive for blood: haemoglobinuria, beetroot, drugs (rifampicin), - Intravenous contrast used to delineate anatomy of the kidneys and urinary system metabolic (alkaptonuria, porphyria) - Various phases: 2. UFEME (i) Control film – plain KUB - Confirm presence of red blood cells (ii) Nephrogram phase (taken 1 minute after contrast given) – contrast fills - Casts  nephritis kidney parenchyma so the kidneys become more visible, can measure size - Elevated WBC (pyuria is >5 WBC per hpf), organisms  infection (iii) Pyelogram phase (3-5 minutes) – contrast fills calyces and pelvis, can 3. Urine cytology for malignant cells detect dilated calyces/pelvis (hydronephrosis), any filling defects (iv) Release film (abdominal binder which was placed to slow the flow of 4. Urine phase cont rast contrast into the bladder is released) – shows ureters, any hydroureter, - RBCs – isomorphic or dysmorphic? Dysmorphic RBCs suggest a tubular source, filling defects; bladder – any filling defects, abnormal appearance of the while isomorphic RBCs suggest post-renal source (ureter, bladder, etc) bladder (fir-tree appearance in neurogenic bladder) 5. Urine culture and sensitivity (v) Post-micturition – any residual urine in bladder after voiding

6. Full blood count - Contraindicated in: - How low is the Hb? (a) Contrast allergy - Elevated TW – infection (b) Renal impairment (Cr >200) (c) Patients on metformin (can cause lactic acidosis; patients need to stop 7. Urea, electrolyt es and creatinine metformin 2 days before and after study) - Any renal impairment and electrolyte abn (renal or pre-renal dz more likely) (d) Patients with asthma (given steroids for 3 days before study) 101

R ENAL CELL CARCINOMA INVESTIGATIONS EPIDEMIOLOGY - 3% of adult malignancy D IAGNOSTIC - Most frequent occurring solid lesion within kidney 1. Imaging – CT and/or ultrasound - 2:1 male predominance - Presumptive diagnosis is made on imaging – a renal parenchymal mass with - Peak incidence 60-70 years thickened irregular walls and enhancement after contrast injection suggests malignancy

PATHOLOGY 2. Pathological diagnosis - Most common primary renal tumour (80-85% of all tumours of the kidney) - Needle biopsy usually not done for resectable lesions due to fears of tumour - Arise from the renal tubular epithelium seeding - Three cell types: clear cell carcinoma (70-80%), papillary renal cell carcinoma (10- - In these resectable lesions, a partial or total nephrectomy is often performed, and 15%), and chromophobe renal cell carcinoma (5%) provides the tissue diagnosis post-operatively - Other renal tumours: TCC of renal pelvis, Wilms’ tumour, lymphoma - In tumours with metastatic disease on presentation, biopsy of the metastatic site may be easier RISK FACTORS S TAGING - Smoking 1. CT scan of the abdomen - Exposure to cadmium - Perinephric invasion, adjacent organ invasion - Family history - Extension into renal vein, IVC  von-Hippel Lindau syndrome due to mutation of the VHL gene on chromosome - Lymph node enlargement 3p25 (associated with CNS haemangioblastomas (usually cerebellar), bilateral - Liver metastases multicentric retinal angiomas, phaeochromocytomas, etc)  clear cell carcinomas 2. CT scan of the chest  Hereditary papillary RCC (HPRCC) due to mutation of the MET proto-oncogene - For lung metastases on chromosome 7q31  multifocal bilateral papillary carcinomas 3. Bone scan - Acquired polycystic kidney disease (secondary to chronic dialysis) - Only done if patient complains of bone pain and/or alkaline phosphatase is raised

PRESENTATION 4. MRI of abdom en and heart - Initially asymptomatic (may be detected incidentally) - Superior to CT for evaluation of IVC and right atrium involvement - Painless gross haematuria is the most common presenting symptom – >50% of cases T1 Tumour <7cm, limited to the kidney - When tumour has grown large enough, dull flank pain and palpable mass may result T1a: tumour <4cm  Classical triad of RCC: flank pain, painless haematuria, palpable renal mass T1b: tumour >4cm but <7cm (indicates late stage disease) - May have fever a/w night sweats, LOA, LOW, malaise T2 Tumour >7cm, limited to the kidney - Polycythaemia occurs in 1-5% (due to increased erythropoietin) T3 Tumour extends into major veins or invades adrenal gland or perinephric - For left renal tumour, extension of tumour into left renal vein can cause a left tissues, but not beyond Gerota’s fascia varicocoele as the left testicular vein becomes occluded T4 Tumour invades beyond Gerota’s fascia - Extension into IVC can cause lower limb oedema, ascites, liver dysfunction, pulmonary embolism - Symptoms of metastases – lungs, liver, bones, brain, lymph nodes - Paraneoplastic syndromes are uncommon – Cushing’s, hypercalcaemia, hypertension TREATMENT BLADDER TRANSITIONAL CELL CARCINOMA

R ESECTABLE TUM OURS EPIDEMIOLOGY Surgery - Ninth most common cancer in Singaporean males - Laparoscopic versus open methods - Increasing incidence with age (80% diagnosed in patient >60 years old) - Retroperitoneal versus transperitoneal approach - 4:1 male predominance 1. Partial nephrectomy - Done in T1a disease – spares part of the kidney that is not involved  nephron- PATHOLOGY saving - TCC is the most common tumour of the bladder (>90%) - Thought to arise due to exposure to carcinogenic substances in the urine  field 2. Total nephrectomy change effect, thus urothelial tumours often occur multifocally - Done in T1b disease – entire kidney removed - Other types of bladder tumours: adenocarcinoma (1%, arises from remnant of the 3. Radical nephrectomy urachus in the dome of the bladder), SCC (<5%, due to chronic irritation e.g. long - Done in T2 disease – entire kidney together with Gerota’s fascia term indwelling catheter or untreated bladder stone) - In T3 disease, aim for radical nephrectomy and removal of structures affected e.g. adrenal gland RISK FACTORS Adjuvant chemotherapy - Industrial chemicals – naphthylamine, aniline-containing dyes, etc - Cigarette smoking Surveillance after resection to d etect relapse early - Occupational (hairdressers – exposure to hair dyes) Patients who cannot undergo resection - Analgesic abuse (phenacetin) - Most small tumours grow slowly and do not become symptomatic or metastasise – - Chronic cystitis reasonable to manage conservatively with periodic re-evaluation - Schistosomiasis - Alternatives: radiofrequency ablation, cryotherapy of lesions - Radiation (pelvic) - Chemotherapy (cyclophosphamide)

ADVANCED TUMOURS Immunotherapy PRESENTATION - High dose interleukin-2 – associated with good results in patients whose tumours - Haematuria is the most common presenting symptom (90%) – typically gross, respond to treatment, as treatment can induce long-term remissions without relapse. painless, intermittent, occurring throughout the stream However, associated with high toxicity and often not tolerable - LUTS – irritative symptoms (frequency, dysuria, urgency) suggestive of carcinoma - Cytoreductive nephrectomy performed prior to starting immunotherapy can improve in-situ, while obstructive symptoms (decreased stream, intermittent voiding, feeling survival of incomplete voiding, strangury) indicate a tumour at the bladder neck or prostatic urethra Molecular targeted therapy - Pain – in locally advanced or metastatic tumour  flank pain due to urinary - Sorafenib – an inhibitor of tyrosine kinase  blocks intracellular domain of the obstruction, suprapubic pain due to local invasion, bone pain due to metastasis vascular endothelial growth factor (VEGF) receptor - Bevacizumab – monoclonal antibody against VEGF - Constitutional symptoms – LOW, LOA, fatigue

Prognosis DIAGNOSIS Stage I (T1N0): >90% 5 year survival 1. Urine cytology for malignant cells Stage II (T2N0): 75-90% 2. Cystoscopy with cell brushings and biopsy Stage III (T3N0/N1): 60-70% 3. IVU or CT urogram to detect synchronous lesions (3% chance of proximal tumour) Metastatic disease: <10% 103

STAGING o Ileal conduit (a segment of ileum with ureters attached, as a stoma; not 1. CT abdo/pelvis for T, N and M staging continent) 2. Transurethral resection of bladder tumour (TURBT) with histopathology o Neobladder construction using ileum (only if urethra not removed; continent, better quality of life) Ta Superficial, does not involve lamina propria o Stoma with pouch construction under abdominal wall (not continent) Tis Carcinoma in-situ: “flat tumour” - Radiotherapy (not as good as surgery) T1 Superficial, involves lamina propria (up to muscularis propria) T2a Superficial involvement of muscularis propria – up to inner half of muscle T2b Deep involvement of muscularis propria – up to outer half of muscle T3a Microscopic extension outside bladder (from TURBT specimen) UROLITHIASIS T3b Macroscopic extension outside bladder STONE COMPOSITION T4a Invasion of prostate, vagina, uterus - Calcium oxalate or calcium phosphate stones – 75% T4b Invasion of lateral pelvic walls, abdominal wall - Magnesium ammonium phosphate (struvite) stones – 15% Generally can be divided into 2 main groups: - Uric acid and cystine stones – 10% (a) Superficial tumour (70-80% of patients) – Ta, Tis, T1 (b) Muscle-invasive tumour (20-30%) – >T2 PATHOLOGY - Can occur at any level in the urinary tract, but most commonly in the kidney - Most important cause of stone formation is increased urine concentration of the MANAGEMENT DEPENDENT ON STAGE stone’s constituents, such that they exceed their solubility  precipitate as stones S UPERFICIAL TUMOUR - E.g. hypercalciuria with or without hypercalcaemia, hyperuricuria - Primary treatment is TURBT of the tumour - Urinary tract infections can also cause stone formation – struvite stones form in Proteus vulgaris infections as this organism splits urea into ammonium, generating - Intravesical therapy indicated in patients with high risk of tumour recurrence or alkaline urine tumour progression (high grade, multiple primary sites, multiple recurrences, tumour - Bacteria can also form nidi for the formation of any kind of stone size >3cm, primary or coexisting carcinoma in-situ, prostatic urethral involvement)  BCG – 1 instillation per week for 6 weeks PRESENTATION DEPENDS ON SITE  Mitomycin C – single instillation within 24hrs of TURBT, or weekly/monthly treatments for up to 2 years Renal ston es - Most often asymptomatic unless the stone gets lodged in the pelviureteric junction - Follow-up: causing hydronephrosis and subsequent infection  pyonephrosis  3-monthly cystoscopy for 1 year - Vague flank pain may occur  6-monthly cystoscopy for next 4 years Urine cytology with every cystoscopy  Yearly cystoscopy thereafter Ureteric s tones - Even small stones can cause severe symptoms as the ureter is narrow  IVU every 2 years - Classically ureteric colic pain – severe, intermittent loin-to-groin pain - Haematuria – gross or microscopic M USCLE - INVASIVE - Irritative symptoms – frequency, urgency - Radical cystectomy - Can cause upper urinary tract infection  fever, pain  Radical cystoprostatectomy with pelvic lymphadenectomy in male  Anterior exenteration with pelvic lymphadenectomy in female Bladder stones - May be asymptomatic  Ways of diverting urine output - Can cause irritative urinary symptoms – frequency, urgency o Cutaneous ureterostomy (use ureters to create stoma, but easily stenosed due - Haematuria to small calibre; not continent) - If infection is present – dysuria, fever, etc PHYSICAL EXAMINATION - High fluid intake - In ureteric colic, symptoms are often out of proportion to signs – no guarding, - Low salt intake rebound, etc - Restriction of red meat, dairy produce, refined sugars - If the patient has pyelonephritis, renal punch may be positive - Increase citrus fruit intake - Otherwise unremarkable examination S URGICAL INTERVENTION INVESTIGATIONS Indications: - Constant pain 1. Urine tests – dip stick, UFEME, urine culture/sensitivity - Does not pass after one month - Haematuria - Too large to pass spontaneously - Pyuria, micro-organisms (UTI) - Obstructs urine flow 2. KUB - Causes urinary tract infection - May be able to see radio-opaque stone (90% of renal stones a re radio-opaque) - Damages renal tissue or causes significant bleeding - Look at kidney size, any renal stones - Increase in size - Trace path of ureter along tips of transverse processes, across sacroiliac joint, Types of treatment available: and medially into bladder, looking for ureteric stones 1. Percutaneous nephrolithotomy (PCNL) - Look for bladder stones - Done for renal stones that are too large for ESWL to disintegrate 3. Intravenous urogram - Contraindicated in uncorrected bleeding diathesis, patients unfit for GA - Can also help to visualise a stone 2. Extracorporeal shock wave lithotripsy (ESWL) - Can show dilated urinary system secondary to stone obstruction – hydroureter - Calcium oxalate, uric acid and struvite stones fragment easily, but calcium and/or hydronephrosis phosphate and cystine do not - Used for stones below 10mm in size 4. Ultrasound of kidney or bladder - Features of stone: echogeneic rim, posterior acoustic shadowing - Used for renal stones and upper ureter stones – not so good for lower system due to difficulty in access 5. MAG-3 renogr am - Contraindicated in pregnancy, untreated UTI, untreated bleeding diathesis, distal - If pyelonephritis present due to stone obstruction, it is valuable to measure the obstruction that cannot be bypassed with a stent renal function using the MAG-3 renogram 3. Ureteroscopy with lithotripsy (usually laser lithotripsy, can also be done by - The renogram gives the differential function of each kidney – in normal pneumatic drill, electrohydraulic means) individuals the function should be approximately 50% on each side (out of 100% - For stones along the ureter for both kidneys combined) - If one kidney has less than 15% of total renal function, it is not worth salvaging 4. Cystolitholapaxy for bladder stone the kidney 5. Open surgery (pyelolithotomy or ureterolithotomy) – rarely done; only if failed other management strategies, altered anatomy, performing open surgery for another TREATMENT reason anyway, non-functioning kidney

C ONSERVATIVE Adjuncts: Stones smaller than 5mm can be treated conservatively as 60% will be passed out; only - Double-J stent (or DJ stent) – inserted to stent the urinary system when worried that treat if they do not pass out after 4 to 6 weeks, and/or cause symptoms stone fragments after ESWL may cause obstruction e.g. when ESWL used for - Treatment of any urinary tract infection treatment of a large stone; or if system is obstructed to begin with, may want to stent - If underlying disease present that causes increased urinary concentration of stone to ensure good drainage after surgery components e.g. hypercalcaemia  treat disease if possible 105 Summary of treatment mo dalities HISTORY Location Size Treatment Symptoms of ARU: Renal < 5mm Conservative management unless symptomatic/persistent - Inability to pass urine 5-10mm ESWL - Suprapubic distension with pain (unlike chronic retention of urine which is painless) 10-20mm Either ESWL or PCNL Precipitating factors: > 20mm PCNL - Symptoms of urinary tract infection: dysuria, frequency, urgency, nocturia, Upper ureter < 5mm Conservative management unless symptomatic/persistent haematuria 5-10mm ESWL - Constipation > 10mm URS with lithotripsy - Drugs e.g. cough mixture, antihistamines - Immobility Middle ureter/ < 5mm Conservative management unless symptomatic/persistent Distal ureter > 5mm URS with lithotripsy History suggestive of aetiology: - Previous history of obstructive symptoms e.g. poor stream, hesitancy, terminal Bladder < 30mm Cystolitholapaxy dribbling etc  BPH > 30mm Open cystolithotomy (also if there are multiple stones) - Previous history of ureteric colic pain or stones - Previous urethral instrumentation or STD  stricture - Gross painless haematuria recently  TCC, bladder stone - Lower limb weakness/paralysis, bowel incontinence, back trauma, history of spinal disease e.g. PID, spinal stenosis  neurogenic bladder APPROACH TO ACUTE RETENTION OF URINE - Constitutional symptoms: LOW, LOA, malaise (any tumour in general)

CAUSES Complications: - Infection – symptoms of UTI Mechanical Extraluminal Prostate enlargement (benign/malignant) - Stone disease (if in the bladder, usually asymptomatic) Faecal impaction - Renal failure (more likely in chronic retention) – vomiting, lethargy, drowsiness Pelvic tumour Pregnancy UV prolapse PHYSICAL EXAMINATION Intramural Tumour of the bladder neck (TCC) - General condition – sallow appearance, scratch marks, pedal oedema, etc (uraemia) Urethritis (UTI) - Abdomen Urethral stricture from STD, prev instrumentation  Palpable bladder – tender Intraluminal Stones  Other pelvic masses – fibroid, gravid uterus, ovarian cyst Blood clot (clot retention in haematuria)  Faecal loading Foreign body  Bilateral enlarged kidneys (hydronephrosis) Non- Cord disease/ Cord compression - Digital rectal examination mechanical injury Multiple sclerosis  Any saddle anaesthesia Tabes dorsalis  Anal tone Neuropathy Diabetic autonomic neuropathy  Prostate enlargement – firm and smooth? Or hard, craggy, irregular, rectal Drugs Anticholinergics (cough medicine), antihistamines, mucosa not mobile? anti-depressants, alcohol  Stool impaction Others Prolonged immobility - Neurological examination Post-anaesthesia  LMN paralysis of the lower limbs? Pain  Any sensory level present? IMMEDIATE MANAGEMENT – CATHETERISATION  Due to tubular damage from obstruction of drainage of the pelvicalyceal - Try urethral catheterisation first (impt: urethral catheterisation contraindicated if system, resulting in transient impairment of concentrating function  patient has signs suggestive of urethral injury – blood at urethral meatus, high-riding Can result in hypotension and electrolyte abnormalities (hyponatraemia, prostate – more relevant in the trauma setting) hypokalaemia, hypovolaemia)  Requires close monitoring of urine output and fluid/electrolyte status with  If urethral catheterization cannot pass into bladder, there are two possibilities: 1) appropriate replacement and resuscitation enlarged prostate; and 2) urethral stricture (b) Haemorrhage ex-vacuo  For enlarged prostate, try again with a thicker catheter (stiffer, easier to pass  through) Bladder mucosal disruption with sudden emptying of greatly distended bladder  For stricture (when you feel the catheter is stuck quite proximally along the  Usually self-limiting penile urethra, it is more likely to be a stricture), try a smaller gauge catheter  Do not push too hard – may cause false passage creation if the obstruction is due 3. Trial-off catheter to a stricture - Take off catheter and watch patient’s output, as well as perform bladder scan to measure bladder volume - If urethral catheterisation fails, perform suprapubic catheterisation - When patient passes urine, can perform uroflow to investigate severity of outlet  Requires distended bladder which pushes the surrounding bowel loops away so obstruction, and also do bladder scan post-micturition to check residual volume that risk of bowel injury is lower - If patient cannot pass urine and bladder volume >400ml  re-catheterise  Local anaesthetic injected 2 fingerbreadths above pubic symphysis  Small incision made in the skin and fascia, and trocar inserted  When a gush of urine is seen, the suprapubic catheter is inserted and secured BENIGN PROSTATIC HYPERPLASIA (BPH)

INVESTIGATIONS (FOR CAUSES) EPIDEMIOLOGY 1. Full blood count for raised TW (infection) - Very common problem in men 2. Urea, electrolytes and creatinine for raised creatinine (renal impairment secondary - Frequency rises with age after the age of 30, reaching 90% in men older than 80 to obstructive nephropathy) 3. Urine dipstick, UFEME and culture/sensitivity for infection 4. PSA – keeping in mind causes of raised PSA (cancer, BPH [usually <40], prostatitis, PATHOLOGY instrumentation >11days) - Results from proliferation of both the epithelial and stromal components of the 5. KUB for stones, faecal loading prostate with resultant enlargement of the gland 6. Ultrasound of the bladder for stones, tumour, intravesical protrusion of prostate - Commonly occurs in the central zone of the prostate - Major stimulus for hyperplasia appears to be dihydrotestosterone (produced from testosterone by the enzyme 5-alpha reductase) TREATMENT - Age-related increases in oestrogen levels may also contribute to BPH by increasing 1. Treat reversible causes the expression of dihydrotestosterone receptors on prostatic parenchymal cells - Stop drugs that may have precipitated ARU - Relieve constipation with fleet enema, lactulose, senna etc PRESENTATION - Treat any urinary tract infection if present - Main result of BPH is obstruction of the prostatic urethra resulting in lower urinary 2. Anticipate complications tract symptoms (LUTS) which can be divided into irritative and obstructive (a) Post-obstructive diuresis symptoms – obstructive symptoms predominate  Urine output >200ml/hr for 2 hours or more 107 Obstructive Irritative I. Watchfu l waiting Hesitancy Frequency - Suitable for patients with minimal symptoms, no complications and normal invx Straining to pass urine Urgency - Monitor patient’s symptoms and clinical course annually Weak stream Nocturia Prolonged micturition Dysuria II. Medical treatment Terminal dribbling Urge incontinence 1. Alpha blockers Feeling of incomplete voiding - Prazosin, Terazosin, Doxazosin, Alfuzosin Double voiding (pis-en-deux) - Treatment of symptoms of BPH by acting on the alpha-1 adrenergic receptors that are abundant in the bladder neck, prostate and urethra - May progress to the point of acute urinary retention  admitted to hospital - Result in decreased outflow resistance and decreased bladder instability - In the chronic setting, the patient may have chronic urinary retention with high post- - Side effects include postural hypotension, dizziness void residual volume in the bladder  asymptomatic, may have overflow 2. 5-alpha reductase inhibitors incontinence - Finasteride, Dutasteride - Obstruction leads to hypertrophy of the detrusor muscle and trabeculation of the - Treats the disease (not just the symptoms) by inhibiting the conversion of bladder mucosa as the bladder tries to empty against increased resistance testosterone to dihydrotestosterone by 5-alpha reductase  reduced prostate size - Proven to decrease need for surgery and acute retention rates PHYSICAL EXAMINATION - Only effective after 6 months, and in prostates >40g - Palpable tender bladder in ARU (non-tender in chronic retention) - Most common side-effect is sexual dysfunction - Digital rectal examination: smooth enlarged prostate, rubbery, non-tender III. Surg ery – Transurethral resection o f prost ate (TURP) INVESTIGATIONS Indications: - Creatinine level (renal impairment due to chronic obstruction) - Refractory urinary retention - Recurrent urinary tract infection - Urinary investigations for infection (stasis predisposes to UTI) - Obstructive uropathy - KUB for bladder stone - Bladder calculi - Ultrasound of kidney and bladder – hydronephrosis, post-void residual volume - Recurrent gross haematuria >100ml, bladder stone - Uroflow to confirm obstruction to urinary outflow (normal peak flow rate should be Complications of surgery (TURP) more than 15ml/sec) Early 1. Bleeding PROBLEMS 2. TUR syndrome - Acute/chronic urinary retention - Hyponatraemia due to constant irrigation during TURP (glycine used for - Gross haematuria irrigation – cannot use N/S, as ionic solutions make diathermy non-functional) - Bladder stones - Irrigation fluid is hypotonic, thus water enters open vasculature during surgery - Recurrent UTI - Risk increases with prolonged operation and increased pressure of irrigation, - Renal impairment secondary to outflow obstruction thus op is kept to shorter than one hour, and irrigation pressures <60mmHg - Co-existence of prostate cancer - Symptoms: Nausea, vomiting, confusion, hypertension, visual disturbances - Patient usually given spinal anaesthesia during TURP so the surgeon can assess MANAGEMENT the patient’s mental status during the operation - Divided into watchful waiting, medical management, and surgical management 3. Perforation of the urethra or bladder dome - Objectives of treatment: Rapid and sustained relief of symptoms, prevent long-term Late complications, improve patient’s quality of life 1. Retrograde ejaculation PROSTATIC CANCER STAGING 1. Clinical examination (palpable tumour  T2) EPIDEMIOLOGY 2. TRUS biopsy for staging purpose - Prostate Cancer is the 6th commonest cancer among men in Singapore. 3. CT scan of the abdomen and pelvis to assess extent of tumour invasion and nodal - 5th common cancer in Singapore status (regional, non-regional) - Peak incidence between 65 and 75 years of age 4. Bone scan for metastasis PATHOLOGY - Adenocarcinoma TREATMENT - Arise in the outer parts of the prostate 70-80% of the time and are thus palpable on L OCALISED DI SEASE digital rectal examination 1. Radical prostatectomy - Open, laparoscopic or robot-assisted RISK FACTORS - Open – retropubic or perineal approaches - Hormonal – growth of tumour can be inhibited by orchidectomy or administration of 2. Radiotherapy oestrogens - External beam radiotherapy (EBRT) - Genetic – racial variations in onset and prevalence, family history - Brachytherapy - Environmental – industrial chemical exposure, diet containing high animal fat

L OCALLY ADVANCED DI SEASE PRESENTATION 1. Radiotherapy with androgen ablation - Often asymptomatic, may be incidentally picked up on digital rectal examination or - Castration due to elevated prostate-specific antigen (PSA) level  Surgical - Can cause local symptoms such as obstruction of the prostatic urethra (uncommon as  Medical – LHRH agonist most cancers arise in peripheral zones) – LUTS, bladder outlet obstruction - Anti-androgen - Metastatic symptoms – bone pain  Non-steroidal e.g. Flutamide  Steroidal – cyproterone acetate PHYSICAL EXAMINATION - Combined androgen blockade - Digital rectal examination: Asymmetric area of induration, or frank hard irregular - Oestrogen therapy (diethylstilbestrol) nodule - Percuss spine for any bone pain M ETASTATIC DI SEASE 1. Androgen ablation

DIAGNOSIS H ORMONAL REFRACTORY PROSTATIC CANCER 1. PSA level - 2 consecutive PSA rises no less than 2 wks apart and/or documented dz progression - >10ng/ml: biopsy recommended as >50% of patients will have prostate cancer based on clinical/radiological findings in pts with castrate levels of testosterone - 4-10ng/ml: biopsy advised, though only 20% will have prostate cancer - Management: - <4ng/ml: majority will have negative biopsies, and yet there is a significant 1. Secondary hormonal manipulation proportion of men with prostate cancer with PSA <4ng/ml  biopsy if the rate  Glucocorticoids – prednisone, dexamethasone, hydrocortisone of rise of PSA is >0.75ng/ml per year  Progesterone – megestrol acetate  Adrenal suppressives – ketoconazole, aminoglutethimide 2. Transrectal ultraso und (TRUS) with biops y 2. Chemotherapy - Histology of prostate carcinoma is graded by the Gleason score looking at  Docetaxel + Prednisone (gold standard) glandular architecture at low magnification  Mitoxantrone + Prednisone 109

APPROACH TO SCROTAL SWELLINGS

ANSWER 4 QUESTIONS: 1. Can you get above the swelling? 2. Can you identify the testis and the epididymis? 3. Is the swelling transilluminable? 4. Is the swelling tender?

Cannot Cough impulse Hernia get Reducible above Testis palpable swelling Opaque

No cough impulse Infantile hydrocoele Not reducible Testis not palpable Transilluminable

Can get Testis not definable Opaque Non tender Chronic haematocoele above from epididymis Gumma swelling Tumour

Tender Torsion Epididymo-orchitis Acute haematocoele

Transilluminable Hydrocoele

Testis definable Opaque Non-tender Tumour from epididymis swelling of testis

Non-tender TB epididymis swelling of epididymis

Tender Epididymoorchitis

Transilluminable Cyst of epididymis SURGICAL INSTRUMENTS - Used when small to moderate amounts of drainage are expected or when a passive drainage system won't provide adequate drainage DRAINS - active drainage systems have a clear graduated scale by which you can see the type and amount of drainage and determine when to empty the drainage chamber FUNCTIONS OF DRAINS - Tubing of the low-pressure active drainage system is placed through a separate Drains are inserted to: puncture wound or the tube may exit the edge of the surgical wound - Evacuate collections of pus, blood or other fluids (e.g. lymph) - If the tubing isn't sutured in place, it could become dislodged when you change - Drain potential collections dressings or reposition the patient, so be careful. If a portion of the tube is pulled outside his skin, an air leak will cause the collection chamber to rapidly fill with Rationale: air and the system won't drain properly. - Drainage of fluid removes further fluid collections Passive drains - May allow the early detection of anastomotic leaks or haemorrhage - Passive drains have no suction, rely on gravity - Leave a tract for potential collections to drain following removal - Function by the differential pressure between body cavities and the exterior - Used when a moderate to large amount of drainage is expected COMPLICATIONS: 1. Infection CARE AND PREVENTION OF COMPLICATIONS OF TUBES: 2. Bleeding - Prevent Infection- maintain meticulous skin care and aseptic technique around the 3. Tissue damage- by mechanical pressure or suction insertion site 4. Drain failure- blocked/slipped/kinked Prevent blockage of the drain- do not allow bottles to fill up 5. Incisional hernia- occurs when drain inserted through incision wound site- create a - Prevent slippage by securing drain carefully to skin; refix as required separate incision site for drain! - - Never hold a drainage collection device higher than the tube insertion site to prevent the drainage from flowing backward into the patient TYPES OF DRAINS - Note amount of drainage daily - Drains classified as:  Open or closed REMOVAL OF DRAINS  Active or passive A drain is removed as soon as it is no longer required. The following are general - Active drains require suction. Passive drains rely on gravity. guidelines: - Drains are often made from inert silastic material 1. Drains put in to cover perioperative bleeding and haematoma formation, can come - They induce minimal tissue reaction out after 24 — 48 hours. - Red rubber drains induce an intense tissue reaction allowing a tract to form 2. Where a drain has been put in to drain an infected site e.g. abscess, remove it when - In some situations this may be useful (e.g. biliary t-tube) the fever settles or when there is evidence of complete drainage. Open drains - Corrugated drain, Yeates drain, Penrose drain - Drain fluid collects in gauze pad or stoma bag - Easier to drain infected collections Closed drains - Consist of tubes draining into a bag or bottle - They include chest and abdominal drains - The risk of infection is reduced Active drains - Jackson-Pratt Drain, Redivac Drain, T-tube - Have expandable chambers to create low-pressure suction 111

CENTRAL VENOUS PRESSURE LINE INSERTION Technique of IJV cannulation Place the patient in a supine position, at least 15 degrees head-down to distend the neck INDICATIONS veins and to reduce the risk of air embolism. Turn the head away from the venepuncture 1. Vascular access site. Cleanse the skin and drape the area. Sterile gloves and a gown should be worn to 2. Total parenteral nutrition avoid catheter-related sepsis. 3. Infusion of irritant drugs 4. Measurement of central venous pressure Procedure 5. Cardiac catheterization 1. Use local anaesthetic to numb the venepuncture site. 6. Pulmonary artery catheterization 2. Introduce the large calibre needle, attached to an empty 10 ml syringe. 7. Transvenous cardiac pacing. 3. Surface mark the internal jugular vein at the centre of the triangle formed by the two lower heads of the sternocleidomastoid muscle and the clavicle. Palpate the CONTRAINDICATIONS: carotid artery and ensure that the needle enters the skin lateral to the artery. 1. Do not insert into an infected area. 4. Direct the needle caudally, parallel to the sagittal plane, aiming towards the 2. Avoid infraclavicular approach to subclavian vein if patient has apical emphysema ipsilateral nipple. or bullae. 5. While needle is advanced, maintain gentle aspiration. 3. Avoid internal jugular vein if carotid aneurysm present on the same side. 6. When vein is entered, flush of blood appears in the syringe. Now, cannulate the 4. Bleeding diatheses vein via the Seldinger technique as described below. 5. Septicaemia 6. Hypercoagulable states 7. Remove syringe, holding needle firmly in place. Occlude needle to prevent air embolism or bleeding. ROUTES FOR CENTRAL VENOUS CANNULATION INCLUDE: 8. Advance guide wire, J-shaped end first, into the vessel through the needle. 1. Internal jugular vein 9. Hold guide wire in place and remove needle. Maintain a firm grip on the guide wire 2. Subclavian vein at all times. 3. Femoral vein 10. Use a dilator to enlarge the hole in the vein. Remove the dilator. 4. External jugular vein 11. Thread tip of catheter into the vein through the guidewire. Grasp the catheter near the skin and advance it into the vein with a slight twisting motion. CANNULATION OF THE INTERNAL JUGULAR VEIN 12. Advance catheter into final indwelling position. Hold catheter and REMOVE The internal jugular vein (IJV) is accessible, so cannulation of this vein is associated GUIDEWIRE. with a lower complication rate than with other approaches. Hence, it is the vessel of 13. Check lumen placement by aspirating through all the pigtails and flushing with choice for centr al venous cannu lati on. saline next.

Anatomy of the IJV 14. Suture the catheter to the skin to keep it in place. The vein originates at the jugular foramen and runs down the neck, to terminate 15. Apply dressing according to hospital protocol. behind the sternoclavicular joint, where it joins the subclavian vein. It lies alongside 16. The catheter tip should lie in the superior vena cava above the pericardial reflection. the carotid artery and vagus nerve within the carotid sheath. The vein is initially Perform check chest X-ray to confirm position and exclude pneumothorax. posterior to, then lateral and then anterolateral to the carotid artery during its descent in the neck. The vein lies most superficially in the upper part of the neck. Complications 1. Pneumothorax/haemothorax Relations of the IJV 2. Air embolism - ensure head-down position. Anterior: Internal carotid artery and vagus nerve. 3. Arrhythmias – This happens if cathether “irritates” the heart. Avoid passing Posterior: C1, sympathetic chain, dome of the pleura. On the left side, the IJV lies guidewire too far, observe rhythm on cardiac monitor during insertion. anterior to the thoracic duct. Medial: Carotid arteries, cranial nerves IX-XII 4. Carotid artery puncture/cannulation - palpate artery and ensure needle is lateral to it, NASOGASTRIC TUBE or use ultrasound-guided placement, transduce needle before dilating and passing central line into vessel, or remove syringe from needle and ensure blood is venous. INDICATIONS 5. Chylothorax- Avoid cannulating the vein on the left side as the thoracic duct lies 1. Diagnostic there. a) bleeding from the upper gastrointestinal tract, haematemesis 6. Catheter-related sepsis b) pentagastrin studies (rarely done now) 2. Decompresssion CANNULATION OF THE SUBCLAVIAN VEIN a) intestinal obstruction b) pyloric stenosis The subclavian vein (SVC) may be preferred for central venous access if c) haematemesis, particularly in patients at risk of hepatic encephalopathy 1. Patient has a cervical spine injury d) therapeutic and prophylactic decompression after major abdominal surgery 2. Line is for long-term use e.g. dialysis, feeding. This site may be more comfortable e) prevention of further soilage after gastric perforation for the patient. f) prevention of anastomotic rupture after gastric surgery Anatomy of the SCV g) prevention of obstruction of the operative field by air in the stomach The SCV is the continuation of the axillary vein and originates at the lateral border of 3. Nutrition the first rib. The SCV passes over the first r ib anterior to the subclavian artery, to join a) patients with dysphagia with the internal jugular vein at the medial end of the clavicle. The external jugular b) comatose or weak patients vein joins the SCV at the midpoint of the clavicle. 4. Lavage a) poisoning Technique b) gastrointestinal bleeding 1. Place the patient in a supine position, head-down. 2. Turn the head to the contralateral side (if C-spine injury excluded). 3. Adopt full asepsis. CONTRAINDICATIONS 4. Introduce a needle attached to a 10 ml syringe. 1. Base of skull fracture 5. Surface mark the subclavian vein 1 cm below the junction of the middle and medial 2. Oesophageal tear thirds of the clavicle. Direct the needle medially, slightly cephalad, and posteriorly 3. Severe facial injury behind the clavicle toward the suprasternal notch. 6. Slowly advance the needle while gently withdrawing the plunger. The cuffed endotracheal and tracheostomy tubes should be deflated prior to nasogastric 7. When a free flow of blood appears, follow the Seldinger approach, as detailed tube insertion. previously. 8. The catheter tip should lie in the superior vena cava above the pericardial reflection. PRE-PROCEDURE Perform check chest X-ray to confirm position and exclude pneumothorax. 1. Gather equipment. 2. Don non-sterile gloves. Complications 3. Explain the procedure to the patient and show equipment. As listed for internal jugular venous cannulation. The risk of pneumothorax is far 4. If possible, sit patient upright for optimal neck/stomach alignment with the head greater with this technique. Damage to the subclavian artery may occur; direct pressure forward. Otherwise, prop the patient up at 45 degrees. cannot be applied to prevent bleeding. 5. Deflate the endotracheal tube or tracheostomy cuff Ensure that a chest X-ray is ordered, to identify the position of the line and to exclude 6. Determine the size of the nasogastric tube required (usually 14 – 16FG). If pneumothorax. aspirating, use as large a tube as possible to reduce the risk of blocking during use or the formation of a false passage during introduction; if feeding, a smaller tube may be used (eg. 8FG) because it is more comfortable in the long term. 113 PROCEDURE PROBLEMS AND COMPLICATIONS 1. Estimate the length of the tube to be inserted: from the bridge of the nose to the 1. Technical tragus of the ear to the point halfway between the xiphisternum and the navel. Mark a) insertion into the trachea, resulting in choking. the Mark measured length with a marker or note the distance. b) coiling and reentry into the oesophagus (rare). 2. Examine nostrils for deformity/obstructions (eg. choanal stenosis) to determine best c) trauma to the nose and the pharynx. side for insertion. Select the largest nostril for inserting the tube. d) dislodgement e) perforation of the pharynx and oesophagus. 3. Lubricate tube with water. The nose may be lubricated with lignocaine gel. 2. Lung complications 4. Introduce the tube through the nostril, passing the tube along the floor of the nose. a) decreased ventilation Resistance may be felt as tip reaches the nasopharynx, which is the most b) aspiration pneumonia uncomfortable part of the procedure. In the operation theatre, when the patient is under , the McGill’s forceps may be used to guide the tube down. 3. Loss of fluids and electrolytes, especially sodium, potassium, chloride and hydrogen ions. 5. Instruct the patient to swallow (you may offer ice chips/water if not contraindicated) and advance the tube as the patient swallows. Swallowing of small sips of water 4. Dry mouth and parotitis due to fluid loss and mouth breathing. may enhance passage of tube into . If patient is uncooperative, bend his 5. Gastrointestinal head to elicit a swallowing reflex. a) gastric erosions 6. Continue to advance the tube down the oesophagus. There should not be resistance. b) pressure necrosis of the pharynx, oesophagus or the external nares. If resistance is met, rotate the tube slowly with downward advancement towards the c) traumatic haemorrhage of varices. closer ear. Do not force the tube down against resistance as this may form a false d) gastroesophageal reflux due to functional incompetence of the lower passage. oesophageal sphincter. e) erosions of the oesophagus leading to strictures. 7. Withdraw the tube immediately if changes occur in the patient's respiratory status, if the tube coils in the mouth, or if the patient begins to cough or turns pretty colours. 8. Advance the tube until mark is reached (approximately 40cm). Stop. 9. Check for correct placement by attaching a syringe to the free end of the tube and aspirating a sample of gastric contents to test with litmus, auscultating the epigastrium while injecting air through the tube, or obtaining an x-ray to verify placement before instilling any feedings/medications or if you have concerns about the placement of the tube. 10. Secure the tube with adhesive tape. 11. Re-inflate the endotracheal tube or tracheostomy cuff if necessary. 12. If for suction, remove the syringe from the free end of the tube; connect to suction; set machine on type of suction and pressure as prescribed. 13. Document the reason for the tube insertion, type & size of tube, the nature and amount of aspirate, the type of suction and pressure setting if for suction, the nature and amount of drainage, and the effectiveness of the intervention. TRACHEOSTOMY 8.8. Visualise the thyroid isthmus and retract isthmus. 9.9. Retract cricoid cartilage upwards wth cricoid hook. INDICATIONS FOR TRACHEOSTOMY ndnd rdrd 1.1. Maintenance of airway patency. 10.10. Incise the trachea between the 2 and 3 tracheal rings, making an inverted U-flap 2.2. Protection of the airway from aspiration. incision. 3.3. Application of positive pressure to the airway. 11.11. Insert tracheal dilator through the tracheostoma and remove the cricoid hooks. 4.4. Facilitation of secretion clearance. 12.12. Suction of blood and secretions in the lumen. 5.5. Delivery of high oxygen concentrations. 13.13. Insert the tracheostomy tube. 14.14. Remove the obturator and insert the inner cannula. RELATIVE CONTRAINDICATIONS 15.15. Dress wound and secure to the neck using sutures and adhesive tape. 1.1. Evidence of infection in the soft tissues of the neck at the prospective surgical site. 2.2. Medically uncorrectable bleeding diatheses. 3.3. Gross distortion of the neck anatomy due to hematoma, tumour, thyromegaly, high COMPLICATIONS innominate artery or scarring from previous neck surgery. During Procedure 4.4. Documented or clinically suspected tracheomalacia. 1.1. Bleeding if damage to the innominate or inferior thyroid artery. 5.5. Need for positive end-expiratory pressure (PEEP) of more than 15 cm of water. 2.2. Damage to surrounding structures, eg esophagus, recurrent laryngeal nerve, 6.6. Patient obesity with short neck that obscures neck landmarks. brachiocephalic vein. 7.7. Patient age younger than 15 years. 3.3. Pneumothorax. 4.4. Pneumomediastinum.

TYPES OF TRACHEOTOMY Immediate post-op 1.1. Temporary: Portex (cuffed). 1.1. Surgical emphysema. 2.2. Permanent: Consist of inner and outer tubes made of stainless steel. 2.2. Obstruction, eg clot, mucus. 3.3. Bleeding. Tracheostomy is more useful in the elective setting compared to endotracheal intubation 4.4. Dislodgment. because: 5.5. Subcutaneous emphysema. 1.1. Better tolerated. 2.2. Avoids risk of laryngeal stenosis Late post-op 3.3. Avoids risk of endotracheal obstruction.n. 1.1. Infection . 2.2. Obstruction, eg dislodgment of tube, crust formation from secretions. PROCEDURE 3.3. Tracheo-esophageal fistula. 1.1. Position the patient. Place rolled towel under the patient’s neck to hyperextend the 4.4. Tracheal stenosis. neck for better exposure. 5.5. Wound breakdown. 6.6. Scarring. 2.2. Clean and drape. Clean the skin of the neck from the chin to the suprasternal notch 7.7. Tracheomalacia. and laterally to the base of the neck and clavicles. Drape field. 3.3. Identify anatomical landmarks (thyroid cartilage, cricoid cartilage). POST-OP CARE 4.4. Administer local anaethesia. 1.1. Position patient in a propped up position. 5.5. Incise skin. In the emergency setting, make a vertical incision 3cm from cricoid 2.2. Prevent obstruction by suction, saline irrigation, mucolytic agents (mucomyst, cartilage downwards. In the elective setting, make a tranverse incision 4cm wide, guaifenesin) and humidified air. 3cm above the suprasternal notch. 3.3. Change Portex tube every 3 rdrd day and remove the inner tube for cleaning everyday.. 6.6. Dissect through the subcutaneous layers and platysma. 4.4. Unlock the metal tube every night so that the patient can cough it out if it becomes 7.7. Identify the communicating branch of the anterior jugular vein, clamp and ligate thethe obstructed. artery (ignore this in an emergency). 115115 SENGSTAKEN-BLAKEMORETUBE (OR MINNESOTA TUBE) URINARY CATHETERISATION

INDICATIONS INDICATIONS FOR SHORT-TERM CATHETERISATION Oesophageal varices 1.1. Relief of acute retention of urine, e.g. benign prostatic hypertrophy, bladder outflow obstruction. CONTRAINDICATIONS 2.2. Bladder washout, e.g. blood clots causing acute retention of urine. 1.1. Base of skull fracture 2.2. Oesophageal tear 3.3. Cystourethrogram.. 3.3. Severe facial injury 4.4. Administration ofof intra-vesical drugs.. 5.5. As an adjunctive measure pre/post-operativelyely PROCEDURE a)a) Pre-operatively: 1.1. Measure the length of the tube. Test balloons. Test patency of the tube. (i)(i) to drain the bladder so as to improve access to the pelvis in urologic or 2.2. Sit the patient upright or at 45 degrees. pelvic surgery.. 3.3. Apply local anaesthesia (lignocaine nasal spray). (ii)(ii) to allow accurate measurement of urine output in major surgery. 4.4. Lubricate and insert the tube through the nose, asking the patient to swallow or b) b) Post-operatively: drink water to aid in smoother passage of the tube through the pharynx and (i)(i) to relieve acute urinary retention because post – – op pain results in failure oesophagus. of the sphincter to relax. 5.5. Inflate the gastric balloon slowly with 100-150ml saline. 6.6. Urinary output monitoring, e.g. in patient with hypovolaemic shock or the 6.6. Check that the tube is in the stomach by: critically ill. (i)(i) aspirating fluid and testing it with litmus, (ii)(ii) auscultating the epigastrium while injecting air, or INDICATIONS FOR LONG-TERM INDWELLING CATHETERIZATION (iii)(iii) doing an X-ray. 1.1. Refractory bladder outlet obstruction. 7.7. Traction. 2.2. Chronic retention of urine, eg. neurogenic bladder. 8.8. Inflate the oesophageal balloon to 35 – – 45mmHg: use the Y-connector piece with 3.3. Incontinence, e.g. in palliative care of terminally ill or patient’s preference. one arm to the BP set and the other to the syringe to pump in air. 9.9. Aspirate fluid from the oesophagus through the Ryle’s tube, or if usin g the CONTRAINDICATIONS Minnesota tube, use the additional lumen provided (with the additional lumen for 1.1. Presence of urethral injury, as manifested by: aspirating fluid in the oesophagus, the Minnesota tube decreases the likelihood of a)a) blood from the meatus, aspiration pneumonia occurring). b) b) scrotal haematoma, c)c) pelvic fracture, orr 10.10. Check the oesophageal balloon pressure hourly and release 5mins hourly. d)d) high-riding prostate, elicited from a genital and digital rectal examination. 11.11. Release oeophageal balloon after 24hrs. (alternative: suprapubic drainage) 12.12. Release gastric balloon after 48hrs. 2.2. Urinary tract infection, as an indwelling catheter causes difficulty in treatment. 13.13. The tube should not be used for more than 72hrs. PROCEDURE COMPLICATIONS 1.1. Gather equipment. 1.1. Aspiration pneumonia 2.2. Explain procedure to the patient. Maximize patient’s privacy. Have a chaperone if 2.2. Respiratory obstruction performing the procedure on a member of the opposite sex. 3.3. Oesophageal ulceration and rupture 4.4. Rebleeding 3.3. Assist patient into supine position with legs spread and feet together. 5.5. Gastric varices not controlled 4.4. Open the catheterization kit and catheter. 5.5. Prepare sterile field. Don the sterile gloves from the kit. 19.19. Remove gloves. Dispose equipment appropriately. Wash hands. 6.6. Test the balloon at the tip of the catheter. 20.20. Document size of catheter inserted, amount of water in balloon, patient's response 7.7. Generously coat the distal portion (2 - 5cm) of the catheter with lubricant. to procedure and assessment of urine. 8.8. Using the non-dominant hand to come in contact with the patient and the dominant hand to use items from the kit (recall that once your hand comes in contact with the COMPLICATIONS 1.1. Infection, which may lead to stone formation. patient, it is no longer sterile and cannot be used to obtain items from the kit),kit), 2.2. Stricture formation due either to faulty technique or an irritant material used in the cleanse the peri-urethral mucosa with antiseptic-drenched swabs held by forceps. catheter. Cleanse anterior to posterior, inner to outer, one swipe per swab, discard swab away 3.3. Creation of a false passage due to wrong technique of insertion. from sterile field. 4.4. Occasionally, irritation of the bladder may cause severe bladder spasms. a)a) Male: Hold the penis and retract the foreskin. Swab the penis and surroundingng area, making sure to cleanse beneath the foreskin. b) b) Female: Retract the labia majora. Swab the perineum. 9.9. Apply sterile drape. 10.10. Installation of local anaesthesia. CHEST TUBE a)a) Male: Chest tubes are inserted to drain blood, fluid, or air and allow full expansion of the (i)(i) Smear lignocaine gel around the meatus and apply the gel gently into lungs. The tube is placed between the ribs and into the space pleural space. urethra. (ii)(ii) Massage gel carefully down the urethra to sphincter, squeezing the The area where the tube will be inserted is anesthetized locally. The patient may also be meatus shut sedated. The chest tube is inserted through an incision between the ribs into the chest (iii)(iii) Wait for for 5 minutes (alternatively, with less anaesthetic effect, smear and is connected to a bottle or canister that contains sterile water (underwater seal). gel over the catheter tip). Suction is attached to the system to encourage drainage. A suture and adhesive tape is b) b) Female: used to keep the tube in place. (i)(i) Apply lignocaine gel to urethra or catheter tip.tip. The chest tube usually remains in place until the X-rays show that all the blood, fluid, or 11.11. In the male, lift the penis to a position perpendicular to patient's body and apply air has drained from the chest and the lung has fully re-expanded. When the chest tube light upward traction (with non-dominant hand); in the female, expose the external is no longer needed, it can be easily removed, usually without the need for medications urethral orifice. to sedate or numb the patient. Antibiotics may be used to prevent or treat infection. 12.12. Gently insert tip of catheter into the meatus using forceps until 1 to 2 inches beyond where urine is noted to drain into kidney dish. If no urine appears although the INDICATIONS catheter seems to be in the right place, flush with sterile saline as the lumen may be 1.1. Pneumothorax. blocked with gel. If this is still unsuccessful, withdraw and reinsert. 2.2. Hemothorax. 13.13. Inflate balloon, using correct amount of sterile saline (usually 20 – – 30mls but check 3.3. Drainage of pleural effusion. actual balloon size). This process should be painless. If patient feels pain, deflate 4.4. Chylothorax balloon immediately and reposition catheter. 5.5. Drainage of empyema/lung abcesses 6.6. Prophylactic placement of chest tubes in a patient with suspected chest trauma 14.14. Gently pull catheter until inflation balloon is snug against bladder neck. before transport to specialized trauma center 15.15. Connect catheter to drainage system. 16.16. Secure catheter to abdomen or thigh, without tension on tubing. CONTRAINDICATIONS 17.17. Place drainage bag below level of bladder. 1.1. Infection over insertion site 2.2. Uncontrolled bleeding diathesis/coagulopathy 18.18. Evaluate catheter function and amount, color, odour and quality of urine.