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Home , Accessory spleen, Asplenia, Polysplenia, Splenic infarction, Wandering spleen

111 2 3 10 4 5 6 Benign of the 7 8 Refaat B. Kamel 9 1011 1 2 3 4 5 6 7 8 9 2011 1 Aims ● Splenic conservation, various tech- 2 niques. 3 ● Identifying the value and functions of the ● Splenic and management. 4 spleen in health and diseases. 5 ● The role of spleen in haematological dis- 6 orders (sickle cell , thalassaemia, Introduction 7 , idiopathic thrombocy- 8 topenic purpura). The spleen has always been considered a mys- 9 terious and enigmatic organ. Aristotle con- ● Haematological functions of the spleen 3011 cluded that the spleen was not essential for life. (haemopoiesis in myeloproliferative 1 As a result of this, was undertaken disorders, red blood cell maturation, 2 lightly, without a clear understanding of subse- removal of red cell inclusions and 3 quent effects. Although Hippocrates described destruction of senescent or abnormal red 4 the anatomy of the spleen remarkably accu- cells) and immunological functions 5 rately, the exact physiology of the spleen con- (antibody production, removal of partic- 6 tinued to baffle people for more than a 1000 ulate antigens as well as clearance of 7 years after Hippocrates. The spleen was thought immune complex and phagocytosis 8 in ancient times to be the seat of emotions but (source of suppressor T cells, source 9 its real function in immunity and to remove of opsonin that promotes neutrophil 4011 time-expired blood cells and circulating phagocytosis and production of 1 microbes, has only recently been recognised. “tuftsin”). 2 3 ● Effects of splenectomy on haematologi- 4 cal and immunological functions. Anatomy of the Spleen 5 ● Complications and sequelae of splenec- 6 tomy including overwhelming post- The development of the spleen begins in the 7 splenectomy (OPSI). fifth week of intrauterine life. Mesenchymal 8 ● Hyposplenism, and associated cells, between the two mesothelial layers of the 9 manifestations. mesogastrium, aggregate and differentiate as 5011 ● Indications for splenectomy whether the anlage of the spleen. Primitive vessels, 1 therapeutic or diagnostic. during the second month of gestation, vascu- 2 ● Alternatives to total splenectomy. larise these cellular aggregates to form a lobu- 311 lated embryonic spleen. Continued growth and

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10 · UPPER GASTROINTESTINAL SURGERY formation occurs during fusion of the splenic Lymphatic vessels in the red pulp or white 1111 lobules. From the fourth to eighth months, pulp of the human spleen are few. Lymphatic 2 the spleen participates with the liver in haemo- capillaries originating in the capsule and tra- 3 cytopoiesis. After the eighth month and beculae converge on lymph nodes of the hilum 4 throughout postnatal life, the spleen resumes and pancreaticoduodenal lymph nodes. Nodes 5 haemocytopoiesis only when bone marrow is in the splenic hilum are often involved in 6 incapable of meeting the demands of the disease processes such as , when the 7 body (extramedullary haemocytopoiesis), or in spleen is involved. Accessory may be 8 pathological circumstances. confused with these lymph nodes, whose 9 The parenchyma of the spleen appears as appearance is vascular (haemolymph) on gross 1011 greyish-white areas, the white pulp scattered examination. 1 in a spongy deep-reddish-purple substance, 2 the red pulp. The white pulp consists of 0.2– 3 0.8 mm masses of diffuse and nodular lymphatic Physiology 4 tissue surrounding small arteries called central 5 The spleen is the largest mass of lymphoid tissue arteries. The white pulp undergoes involution 6 in the body. Like a , the spleen pro- between the ages of 10 and 14. After the age of 7 vides for the storage of lymphocytes and their 60, the spleen as a whole undergoes involution. 8 production; it removes foreign matter in the The red pulp possesses unique venous sinuses 9 blood by the reticular cells; it prolongs the life supported in a spongy reticular stroma con- 2011 of red cells by providing temporary shelter from taining free erythrocytes, macrophages, reticu- 1 certain ionic changes to which they are exposed lar cells and other cells. 2 in the circulation; it stores blood and can expel 3 the contents into the circulation during haem- 4 orrhage, exercise or at high altitudes. Not only 5 Blood Supply is the spleen involved in many systemic diseases 6 but unsuspected splenic abnormalities may The blood supply to the spleen is provided by 7 produce widespread effects. Unlike the lymph the splenic artery, the largest of the three 8 nodes, which are interposed in chains of lym- branches of the coeliac artery. During its course, 9 phatic vessels to filter lymph, the spleen is situ- it sends branches to (1) the (via the 3011 ated in the course of the blood vascular system left gastroepiploic artery and a short gastric 1 to filter blood. Added to this, the spleen receives artery), (2) the (via the pancreatic 2 a disproportionate amount of the circulating artery) and (3) the spleen (via the end of the 3 blood volume for its relatively small size. Hence, splenic artery). About 3.5 cm from the spleen, 4 it becomes involved secondarily in a wide range the splenic artery divides into superior and infe- 5 of haematological disorders. rior terminal branches, each of which further 6 subdivides into several smaller branches prior 7 to penetrating the hilum of the spleen. Haematological Functions of 8 On the basis of comparative anatomy, the the Spleen 9 spleen has been divided into segments sepa- 4011 rated by fibrous septa [1]. Gupta et al. [2] Because of the peculiar anatomical arrangement 1 inferred segmentation of the spleen on the of its blood vessels, the spleen is ideally suited 2 basis of avascular planes. In one of our studies, as a site for fine quality control of the erythro- 3 we showed the parenchymal distribution of cyte population. It removes fragmented, 4 the splenic artery -and clarified the avascular damaged or senescent red cells from the circu- 5 planes in the human spleen. The mode of lating blood, a process known as “culling”. It 6 termination of the splenic artery was studied also plays a role in remodelling the surface of 7 in 25 cadavers. Observation of the parenchymal the maturing erythrocytes and in preserving the 8 distribution of the artery in 17 cases revealed normal relationship between their membrane 9 avascular planes that divided the spleen into surface area and volume. Target cells, which 5011 lobes, inside which other avascular planes have a relatively high ratio of membrane to 1 separated the lobes into segments [3] (Figure intracellular content, appear in the peripheral 2 10.1). blood soon after splenectomy. 311

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111 2 3 4 5 6 7 8 9 1011 1 2 3 4 5 6 7 8 9 2011 1 2 3 4 5 6 7 8 9 Figure 10.1. Arterial supply of the spleen. Arrows indicate avascular planes coinciding with surface notches. (S, Splenic; ST, superior 3011 terminal, MT, middle terminal; IT, inferior terminal, LP, lower polar; SG, segmented.) 1 2 3 A variety of intra-erythrocyte inclusions are to sequester a significant number of platelets, 4 removed by the spleen (through a process however, and after splenectomy there is nearly 5 known as pitting), after which the red cells are always a transient thrombocytosis so that the 6 returned to the circulation. Among the inclu- need for preoperative platelet transfusion is not 7 sions removed are Howell–Jolly bodies, which important. The increase in platelet count occurs 8 are probably nuclear remnants, siderotic gran- intraoperatively shortly after splenic artery 9 ules, which are haemosiderin aggregates laid ligation during splenectomy. 4011 down during normal erythroid maturation, and 1 Heinz bodies, which are pathological aggregates Splenic Pooling and Hypervolaemia 2 of denatured haemoglobin (normally the per- 3 centage of these abnormal cells and inclusions It has been known for many years that plasma 4 does not exceed 3%). Thus after splenectomy, volume is increased in patients with splen- 5 Howell–Jolly bodies and siderotic granules may omegaly, while the red cell mass is normal or 6 be seen in the peripheral blood and the red cells even increased, despite the venous haematocrit 7 show striking changes in shape and size with the being depressed. Anaemia is to a large extent 8 appearance of acanthocytes, irregularly cre- due to haemodilution. Similar observations 9 nated cells and target cells (their percentage were reported in patients with Gaucher’s disease 5011 may reach up to 20–25%) (Figures 10.2 to 10.6). and massive . In patients with cir- 1 The human spleen unlike that of many rhosis of the liver, expansion of the plasma 2 animals contains relatively little blood and volume is common and is not closely related to 311 hence has no important storage role. It appears splenic enlargement. On the other hand, with

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1111 2 3 4 5 6 7 8 9 1011 1 2 3 4 5 6 7 8 9 2011 1 2 3 4 5 6 7 8 Figure 10.2. Target cells in the blood film. 9 3011 1 2 moderate to massive splenomegaly, the spleen very contractile and serve as a reservoir of blood 3 size does play a role, as there is a decrease in at high haematocrit. In times of “fight or flight”, 4 the plasma volume following splenectomy, splenic contraction, which is produced by 5 although it may remain above normal. myoepithelial cells in the capsule or trabeculae, 6 Some authors have postulated that increased transfers blood from the reservoir into the cir- 7 blood flow through an enlarged spleen acts as culation, and the splenic filtration function is 8 a functional arteriovenous shunt, the increased put “on hold”, since all blood flows via the fast 9 venous return to the heart causing a high pathways in contracted spleens, until the organ 4011 cardiac output together with an increase in relaxes again. In the normal human spleen, this 1 the blood volume. The increase in plasma reservoir function appears to be lacking. In 2 volume has also been suggested to be the patients with splenomegaly, splenic contraction 3 result of expansion of the intravascualr space can increase portal venous pressure (paroxys- 4 consequent upon the development of mal ), which can predispose 5 splenomegaly. to variceal , and this may have seasonal 6 Blood “doping” in athletes is a fairly recent variation. 7 innovation, but in some mammalian species 8 the expulsion of high haematocrit intrasplenic Splenic Contraction 9 blood, in order to raise the oxygen-carrying 5011 capacity of peripheral blood, is an effective It is a common clinical observation that 1 physiological mechanism. Spleen of such during an attack of haematemesis, the spleen 2 species as horse, dog, cat, and diving seal are diminishes in size because of contraction and 311

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111 2 3 4 5 6 7 8 9 1011 1 2 3 4 5 6 7 8 9 2011 1 2 3 4 5 6 7 8 Figure 10.3. Howell–Jolly bodies in blood film. 9 3011 1 the spleen may become impalpable or just through the spleen exceeds the combined cell 2 palpable. traffic through all the lymph nodes of the body, 3 In the differential diagnosis of causes of hae- with a daily exchange rate of about 5 × 1011 lym- 4 matemesis, the size of the spleen is usually put phocytes. 5 as a differentiating point between cases of portal The role of the spleen is relative to the liver 6 hypertension and variceal haemorrhage and in the clearance of particulate and is increased 7 other causes such as bleeding peptic ulcer, but in the absence of opsonins. The spleen has a 8 because of contraction of the spleen it is not special role in the elimination of polysaccha- 9 helpful in the differential diagnosis of the cause ride-encapsulated bacteria species. It is also an 4011 of hematemesis. important source of antibody synthesis, partic- 1 In our experience, injection of vasoactive ularly of the IgM class, and in the development 2 material into the splenic artery during surgery of effector T lymphocytes. The population of 3 produces contraction of the spleen and can act lymphocytes in the spleen is in constant motion, 4 as a form of autotransfasion. a substantial proportion recirculating between 5 lymph nodes and the spleen by way of the tho- 6 Immunological Functions racic duct and bloodstream. In the case of the 7 spleen about half of the small lymphocytes 8 The anatomical location of the spleen in the cir- recirculate fairly rapidly. 9 culatory system, and its structural organisation, The spleen is one of the principal sites of 5011 provides a critical opportunity for contact with clearance of damaged and effete cells from the 1 bloodborne antigens and for participation in the blood. It is also involved in the removal of cir- 2 system of circulating lymphocytes. It has been culating antibody-coated cells generated during 311 calculated that the traffic of lymphoid cells autoimmune responses, which may give it a crit-

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1111 2 3 4 5 6 7 8 9 1011 1 2 3 4 5 6 7 8 9 2011 1 2 3 4 5 6 7 8 9 Figure 10.4. Acanthocytes in the blood film. 3011 1 2 3 4 ical role in autoimmune haemolytic disease. The Congenital Anomalies of 5 spleen, together with the liver, is an important 6 site of the fixed macrophages, which remove the Spleen 7 particulate antigens from the blood. 8 9 Tuftsin • Accessory spleens • Splenic band 4011 1 The spleen is the normal site of one step in the • Asplenia 2 production of the immunomodulatory molecule • tuftsin. Among the numerous immunomodula- 3 tory molecules that have been identified, tuftsin • that may predispose 4 is uniquely related to the spleen: tuftsin activity to torsion 5 is not found in asplenic individuals. Among the • Splenic-gonadal fusion 6 biological activities attributed to tuftsin is the 7 stimulation of phagocytosis. Tuftsin can help in Accessory spleens 8 management of overwhelming post-splenec- 9 torny infection (OPS1). Accessory spleens are found in about 10–18% of 5011 The main functions of the spleen are listed in the general population. The most common loca- 1 Table 10.1. tion is within the splenic hilum, along the course 2 311

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111 2 3 4 5 6 7 8 9 1011 1 2 3 4 5 6 7 8 9 2011 1 2 3 4 5 6 7 8 9 3011 Figure 10.5. Pitted red blood cells. 1 2 3 4 5 6 Table 10.1. Main Functions of Spleen 7 Immunological 8 1. Antibody production and cell-mediated responses 9 2. Removal of particulate antigens and clearance of immune complex 4011 3. Phagocytosis: 1 • Maturation of lymphoid cells 2 • Significant lympopoiesis 3 • Source of suppressor T cells 4 • Source of opsonins that promote neutrophil phagocytosis 5 • Production of immunomodulatory molecule “tuftsin” 6 Haematological 7 • Haemopoiesis during intrauterine life, and compensatory haemopoiesis later in life, as in myeloproliferative disorders 8 • Red blood cell remodelling and maturation 9 • Filtration of particles from blood: non-specific or antibody coated 5011 • Removal of red cell inclusions 1 • Destruction of senescent or abnormal red cells 2 • Storage of platelets, iron and factor VIII 311

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1111 2 3 4 5 6 7 8 9 1011 1 2 3 4 5 6 7 8 9 2011 1 2 3 4 Figure 10.6. Normoblasts in blood film. 5 6 7 of the splenic artery, or within the tail of the Asplenia 8 pancreas. Other common locations include the 9 omentum, the gastrosplenic and splenocolic lig- The absence of the spleen (asplenia) occurs after 3011 aments, and the of the small bowel. surgical removal (iatrogenic), or it is congeni- 1 Occasionally an is found in tal. Trauma is the most common reason for 2 presacral, pelvic or paratesticular locations. The removing the spleen in children and sickle cell 3 accessory spleen is generally involved through disease is the most common cause of functional 4 the same pathological process as the primary asplenia in children. Congenital absence of 5 spleen. the spleen is usually associated with serious 6 When total splenectomy is performed for malformations, primarily cardiovascular and 7 disorders such as hereditary spherocytosis, abdominal heterotaxia. 8 hereditary elliptocytosis or idiopathic thrombo- The embryological control of splenogenesis 9 cytopenic purpura, a careful search should be resides in the homeobox gene, HOXDII. In 4011 made and any accessory spleens present should humans, the spleen is the site of early 1 also be removed. After splenectomy, an acces- haematopoietic development, particularly of 2 sory spleen may enlarge and cause recurrence erythrocytes, for the first four months of gesta- 3 of the symptoms for which the original surgery tion. After birth, the spleen has several impor- 4 was performed. Howell–Jolly bodies normally tant functions, importantly the provision of 5 appear within the erythrocytes after splenec- primary immunological defensive responses. 6 tomy; when these are absent, an accessory spleen The spleen has an active role in phagocytosis, 7 should be suspected. The combination of CT production of IgM antibodies, and complement; 8 scan and radionuclide scan provides satisfactory it also plays a significant role in the functional 9 diagnostic accuracy in identifying the location of maturation of antibodies. It is a significant 5011 the accessory spleen. The treatment of choice is reservoir for T lymphocytes. The percentages 1 surgical removal, if an accessory spleen causes of total T cells (CD3), T-helper cells (CD4) and 2 recurrence of a haematological disorder. the lymphoproliferative responses to mitogens 311

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111 (concanavalin A, phytohaemagglutinin and viewed as bilateral right-sidedness of which 2 pokeweed mitogen) may decrease in asplenic Ivemark’s syndrome is an example. 3 patients. However, these T-cell changes reflect Polysplenia is usually associated with other 4 the loss of the spleen as a reservoir rather than congenital anomalies of the cardiovascular or 5 a direct T-cell abnormality. gastrointestinal systems (polysplenia syn- 6 The impaired clearance of opsonized parti- drome) (Figure 10.7). Polysplenia syndrome is 7 cles, decreased IgM levels, and poor antibody more predominant in females, whereas asplenia 8 production (especially to polysaccharide anti- is more common in males. 9 gens) contribute to the increased susceptibility Accessory spleens should be distinguished 1011 of these patients to serious and often fatal infec- from polysplenia. In polysplenia, there is 1 tions. Apart from this filtering and immuno- absence of a normal spleen along with multi- 2 logical function, the spleen is an important system involvement. Accessory spleens are 3 scavenger: it participates in the destruction of usually located in the hilum of the spleen or 4 all three-blood elements – the erythrocytes, the tail of the pancreas in addition to the pres- 5 white cells and the platelets. It plays and impor- ence of a normal spleen. Normally the accessory 6 tant role in selective removal of abnormal cells splenules are very small and clinically insignif- 7 (spherocytes, poikilocytes) and intracellular icant but may hypertrophy under certain situa- 8 inclusions (Heinz bodies, Howell–Jolly bodies). tions. 9 These functions, known as culling and pitting 2011 respectively, and are the basis for the hemato- Splenic Torsion (Ectopic Spleen) 1 logical abnormalities seen in patients with 2 absent splenic function. Elongation of the splenic pedicle and increase in 3 its mass predispose the organ to torsion and 4 Causes of Hyposplenism or Asplenism subsequent . This rare condition 5 occurs in mutiparous women and in children. 6 Common Surgical Causes An elongated pedicle without torsion results 7 1. Splenectomy in an ectopic or “wandering spleen”. This is 8 2. Partial splenectomy or segmental thought to be congenital and due to a persistent 9 splenectomy (a certain volume of the dorsal mesogastrium in children, but may be 3011 spleen should be left a little larger than acquired in multiparous women. 1 normal to maintain a state of eusplenism; Diagnosis of acute splenic torsion requires 2 cf. euthyroidism) some clinical awareness, and is often inade only 3 at laparotomy. Splenectomy is indicated if the 4 Common Medical Causes spleen remains non-viable after untwisting 5 1. , cirrhosis, and the pedicle, and may be necessary if the mobile 6 systemic lupus erythematosus organ cannot be safely fixed in the left upper 7 quadrant. Splenic preservation has been 8 Other Causes of Hyposplenism achieved by plication of the elongated pedicle 9 1. Cyanotic heart disease with fixation of the spleen to the abdominal wall 4011 or left hemidiaphragm (splenopexy). 1 2. Ulcerative colitis 2 3. Haemoglobinopathy Splenogonadal Fusion 3 4. Splenic arterial or venous occlusion 4 5. HIV infection Splenogonadal fusion is a rare congenital 5 6. High dose corticosteroid anomaly whereby the left gonad is typically 6 fused to a segment of splenic tissue. A single 7 Polysplenia case of splenogonadal fusion occurring on the 8 right side has been reported. The clinical pre- 9 Congenital anomalies of the spleen may be iso- sentation of splenogonadal fusion is usually as 5011 lated, but most cases of asplenia/polysplenia a scrotal mass, left inguinal hernia, hydrocele 1 result from interference of establishment of and undescended testis. It may be associated 2 normal right–left asymmetry during embryoge- with pain in the region of the testicles during 311 nesis (laterality sequences). Asplenia may be running (due to splenic contraction).

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B 1111 2 3 4 5 A 6 7 8 9 1011 1 D C 2 3 4 5 6 E 7 8 9 2011 1 2 3 Figure 10.7. Commonly associated anatomic findings in patients who have and billary atresia. A, polysplenia; B, 4 interrupted inferior vena cava with azygous discontinuation; C, aberrant arterial supply with left hepatic artery from either left gastric 5 artery or from superior mesenteric artery; D, preduodenal portal vein; E, gut malrotation. 6 7 8 9 haemolytic anaemia, the myeloproliferative 3011 Hypersplenism states, Felty’s syndrome and some 1 in which there may be a significant degree of 2 Hypersplenism can be considered as a state in splenomegaly and hypersplenism, but it is not 3 which the spleen is more harmful than benefi- clear to what degree the spleen is contributing 4 cial and splenectomy will improve the patient’s to the clinical disability. 5 state. Hypersplenism is usually associated with In , alterations in the white blood 6 anaemia, neutropenia or thrombocytopema. picture are rather marked and stable and 7 The neutropenia may be so severe as to cause hence are used by clinicians as one of the dif- 8 recurrent and the ferential diagnostic symptoms. These changes 9 may be associated with purpura. It is vitally are largely characterized by leucopenia, neu- 4011 important to obtain a good bone marrow tropenia with a shift in the direction of staff 1 sample as a part of the routine work-up of neutrophils and eosinopenia with relative lym- 2 patients with suspected hypersplenism. Table phocytosis [4]. 10.2 lists conditions that may be associated with 3 hypersplenism. 4 In assessing whether the removal of an Focal Disease of the Spleen 5 enlarged spleen will benefit the patient there are 6 two main factors to be considered: the cause of Cysts 7 splenomegaly and whether there is a significant 8 degree of hypersplenism. Cystic lesions of the spleen have a varied aetiol- 9 There are some conditions in which splenec- ogy, and include the “true” cyst containing an 5011 tomy is almost always associated with an epithelial lining and believed to be congenital 1 improvement in the patient’s clinical condition. in origin, the “false” cyst, which is probably 2 However, there are many disorders including post-traumatic, and other less common lesions 311

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111 Table 10.2. Conditions associated with hypersplenism 2 Syndrome Specific diseases of clinical importance 3 Primary Idiopathic non-tropical splenomegaly Dacie’s syndrome 4 Cysts and tumours Haemangioma 5 Haemangiosarcoma 6 7 Secondary Acute and chronic infections Tuberculosis Subacute bacterial 8 Kala-azar 9 Tropical splenomegaly Malaria 1011 Hodgkin’s disease 1 Non-Hodgkin’s lymphoma 2 Infiltrative disorder 3 (a) Hereditary Gaucher’s disease 4 (b) Acquired Sarcoidosis 5 Arnyloidosis 6 Autoinimune disorders Felty’s syndrome 7 Systemic lupus erythematosus Haematological “big spleen” syndromes Primary myeloid metaplasia 8 Chronic leukaemia 9 2011 Portal hypertension Presinusoidal Schistosomiasis 1 Sinusoidal Cirrhosis 2 Postsinusoidal Budd–Chiari syndrome Other presinusoidal: 3 Portal vein occlusion 4 Cavernomatous transformation as in cases of neonatal umbilical 5 Splenic vein obstruction 6 (“Left-sided portal hypertension”) 7 8 9 3011 1 such as cystic and parasitic cysts Splenic 2 (hydatid cyst). 3 On radiographic examination, a large, usually This condition is most often secondary to 4 solitary splenic mass is identified in the left disease elsewhere. Many cases of left-sided sub- 5 upper quadrant of the abdomen. While these are phrenic abscess originate as of the 6 most commonly found in patients in the second spleen, and as a rule result from contiguous or 7 or third decade, they may also be discovered in from metastatic infection; the latter is more fre- 8 children. quent and begins with clotting or from emboli. 9 On 99mtechnetium-sulphur colloid scintigra- Contiguous infection spreads from the lung 4011 phy, a splenic cyst will be seen as a large pho- sometimes after ; from the colon; rarely 1 topenic zone representing the mass, often from the stomach. The appendix and fallopian 2 partially surrounded by a crescent of normal tube are distant sources of infection, and splenic 3 but compressed spleen. abscess can follow typhoid fever, dysentery, 4 On CT a splenic cyst appears as a large, well- relapsing fever and influenza. The abscess fol- 5 defined, non-enhancing, near-water density lowing typhoid fever is said to have the best 6 lesion having a smooth wall. It is usually found prognosis. Chronic tuberculosis may occur. 7 in subscapular locations, but approximately Splenic abscess may be multiple in 8 one-third are located deep within the spleen. haematogenous spread (75%) or solitary due to 9 Although approximately 80% are solitary and infarction, haematoma and direct extension. 5011 unilocular, 20% are multiple or multilocular. Complications of splenic abscess include fistula 1 Ultrasound is also useful for identifying the formation, rupture (43% mortality) and sepsis. 2 splenic origin of the cyst, as well as in distin- Ultrasonography reveals the appearance of 311 guishing cystic from solid complex lesions. splenic abscess as focal or multifocal lesions,

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10 · UPPER GASTROINTESTINAL SURGERY simple cysts or complex mass and bright echoes, Haemangioma 1111 if there is a gas. CT scan reveals a hypodense 2 area, fluid ± gas density or portal venous gas Although rare, the haemangioma is the most 3 (rare). common benign tumour of the spleen, it is 4 usually asymptomatic and frequently presents 5 as splenomegaly or as a mass in the left upper 6 Candidal Abscess quadrant 7 8 • Occurs most frequently in immunocom- Radiology 9 promised patients. 1011 • Hasa microabscess pattern. • Plain film: splenomegaly, rare calcifica- 1 • May mimic Pneumocystis carinii, MAC, tion. 2 Bordetella, Gaucher’s disease, sarcoid. • Anglography: hypovascular or predomi- 3 nantly hypervascular. 4 Ultrasonography reveals: • Ultrasonography: echogenic or complex. 5 6 • wheel within wheel • CT: calcification, homogeneous solid or multicystic and rim enhancement; may 7 • bulls-eye fill in. 8 • hypoechoic 9 • echogenic, shadowing. Lymphangioma 2011 1 • Usually diffuse but may be focal. 2 Hamartoma • May be part of systemic angiomatosis 3 4 Splenic hamartomas are usually solitary but (lung, liver, kidney, bone, etc.). 5 may be multiple. White pulp or red pulp tissue, • Patients complain of pain, rarely coagu- 6 or a mixture thereof, may predominate. Plain laopathy; there may be other symptoms 7 films are either normal or show some degree of and signs if other organs are involved. 8 splenomegaly. • Simple (capillary), cavernous, and cystic 9 Calcifications found in splenic hamartomas types. 3011 range from punctate to stellate in configuration, • Plain films: splenomegaly- calcification 1 and have been found in the fibrotic portions of rare. 2 the lesions rather than within the vascular chan- • Angiography: “Swiss cheese” pattern. 3 nels. The solid lesion may appear nearly iso- 4 dense with the spleen on contrast-enhanced CT. • Ultrasonography/CT/MRI: multilocular 5 Cystic hamartomas demonstrate both solid and cysts or multiple cysts – rarely, one 6 cystic components on CT and ultrasound. (abdominal) cyst. • Internal echoes or higher density if 7 blood, debris or proteinaceous fluid 8 Lymphoma present – MR characteristics of lymph. 9 4011 Lymphomatous involvement of the spleen Treatment 1 as a manifestation of systemic lymphonia is 2 common. whereas primary splenic lymphoma is Treatment of proven splenic abscesses has pre- 3 relatively uncommon. Lymphoma in the spleen viously been splenectomy and . 4 characteristically involves the white pulp, fre- However, over the last 20 years there has been 5 quently appearing as nodules. In advanced a definite trend towards splenic conservation 6 disease, the entire spleen may be replaced by in relation to trauma, with clear evidence of 7 tumour. Splenomegaly in splenic lymphoma is predisposition to fatal sepsis and haematologi- 8 not a reliable sign. cal disturbance after splenectomy. This can be 9 On ultrasonography, lymphomatous involve- achieved by percutaneous drainage of splenic 5011 ment of the spleen is usually seen as single or abscesses (ultrasound-guided drainage) if there 1 multiple inhomogeneous, hypoechoic, nodular is no improvement in the condition after antibi- 2 lesions of variable size. otic chemotherapy. 311

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111 splenic vein obstruction – splenic artery 2 Diffuse Disease aneurysm – hepatic schistosomiasis – conges- 3 tive heart failure – hepatic echinococcosis – 4 Splenomegaly portal hypertension (due to any of the previous 5 causes): Bantl’s disease. Splenomegaly is probably the most common 6 finding in the presence of diffuse splenic 7 disease. The differential diagnosis of spleno- Infiltration of the Spleen (Intracellular or 8 megaly and associated findings may help in dif- Extracellular) 9 ferentiating the various causes. However, many 1011 Amyloidosis – Gaucher’s disease – Niemann– diseases causing splenomegaly without specific 1 Pick disease – Tangier disease – Hurler’s syn- characteristics can be diagnosed on the basis of 2 drome and other mucopolysaccharidoses – clinical or laboratory data. 3 hyperlipidaemias. 4 5 Splenomegaly due to Increased Demand Benign and Malignant Cellular 6 for Splenic Function Infiltrations 7 l. Reticuloendothelial system hyperplasia 8 Letikaemias (acute, chronic, lymphoid, (for removal of defective erythrocytes). 9 myeloid, monocytic) – – Hodgkin’s Spherocytosis – early sickle cell anaemia 2011 disease – myeloproliferative syndromes (e.g. – ovalocytosis – thalassaemia major – 1 polycythamia vera) – angiosarconias – metasta- haemoglobinopathies – paroxysmal noc- 2 tic tumours (melanoma is most coininon) – turnal haemoglobinuria – nutritional 3 eosinophilic granuloina – histiocytosis X – anaemia. 4 hamartomas – haemangiomas, fibromas, lym- 5 2. Immune hyperplasia: (response to infec- phangiomas – splenic cysts. 6 tion). – AIDS 7 – viral hepatitis – – SBE Unknown Aetiology – bacterial septicaemia – congenital 8 Idiopathic splenomegaly – berylliosis – iron syphilis – splenic abscess – tuberculosis 9 deficiency anaemia. 3011 – histoplasmosis – malaria – leishmani- 1 asis – trypanosomiasis. 2 3. Disordered immuno regulation. 3 Rheumatoid arthritis (Felty’s syndrome) Spleen and Haematological 4 – systemic lupus erythematosus – colla- 5 gen vascular diseases – serum sickness – Diseases 6 immune haemolytic anaemias – immune 7 thrombocytopenia – immune neutrope- The Spleen and Sickle Cell 8 nias – drug reactions – angioim- Disease 9 munoblastic – 4011 sarcoldosis – thyrotoxicosis – (benign is due to homozygous inher- 1 lymphoid hypertrophy). itance of the HbS variant. It is one of the 2 4. Extramedullary haematopoiesis. Myelo- commonly inherited haemoglobinopathies 3 fibrosis – marrow damage by toxins, worldwide, with a variable spectrum of severity. 4 radiation, strontium – marrow infiltra- In the eastern province of Saudi Arabia, sickle 5 tion by tumours, leukaemias, Gaucher’s cell disease (SCD) is common and has been 6 disease. reported to be more benign than in other parts 7 of the world. This has been attributed to high 8 Splenomegaly due to Abnormal Pplenic levels of HbF and the frequently associated 9 or Portal Blood Flow alpha thalassaemia. 5011 The standard treatment of SCD has remained 1 Cirrhosis – hepatic vein obstruction – portal largely unchanged. It involves general preven- 2 vein obstruction (intrahepatic or extrahepatic) tive measures, as well as therapy for specific 311 – cavernous transformation of portal vein – complications. One of the main organs to be

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10 · UPPER GASTROINTESTINAL SURGERY affected early in SCD is the spleen, which is a patient with sickle cell anaemia and must 1111 commonly enlarged during the first decade of be promptly treated. Treatment of the acute 2 life, but then undergoes progressive atrophy, splenic sequestration is directed toward the 3 due to repeated attacks of vaso-occlusion and prompt correction of hypovolaemia with 4 infarction, leading to siderofibrotic nodules plasma expanders followed by red cell transfu- 5 (). sion. A dramatic regression of splenomegaly 6 and rise in haemoglobin level can occur in a 7 Indications for Splenectomy in SCD short time after transfusion. 8 9 As a general rule, splenectomy should be 1011 avoided in patients with SCD, as these patients Spleen and Idiopathic 1 are already susceptible to infection, especially 2 during the first 3 years of life. This is attributed Thrombocytopenic Purpura (ITP) 3 to several factors, which include early splenic Idiopathic thrombocytopenic purpura mainly 4 dysfunction. Other contributory factors include affects females between the ages of 15 and 50 5 defective opsonisation due to abnormality of years. It presents with bruising, usually after 6 the alternative pathway for complement activa- trauma or pressure, and examination reveals 7 tion, neutrophil dysfunction, deficiency in variable numbers of petechial haemorrhages 8 heat-labile serum opsonising activity and lack of in the skin. The clinical course is often inter- 9 circulating specific antibodies characteristic mittent and chronic. The platelets of affected 2011 of early infancy. patients become sensitised by antiplatelet IgG 1 To obviate the risks of splenectomy, and autoantibodies and are then removed from the 2 especially of overwhelming post-splenectomy circulation. Many patients need no treatment if 3 infection (OPSI), splenic preservation is being their platelet count remains over 50 × 109/l and 4 increasingly advocated. The main indications no spontaneous bleeding occurs. If the count 5 for splenectomy in SCD are (1) acute splenic falls below 20 × 109/l, the risk of bleeding 6 sequestration crisis, (2) hypersplenism, and (3) increases. Splenectomy is indicated, the spleen 7 splenic abscess. being the initial and major site of antibody pro- 8 Splenic Sequestration duction and also the major site of platelet 9 destruction. Good results are usually obtained, 3011 Much of the severe morbidity and mortality of with approximately 80% of patients having a 1 sickle cell anaemia in the first few years of life satisfactory outcome, no longer requiring 2 is a consequence of the so-called acute splenic steroids to maintain an adequate number of 3 sequestration crisis. These catastrophic events platelets. However, in more heavily sensitised 4 are characterised by severe anaemia, spleno- patients, platelets can continue to be destroyed 5 megaly, hypovolaemic shock and sudden death, elsewhere in the reticuloendothelial system, 6 and it was suggested that patients had literally including the liver, and after an initial good 7 “bled into their spleen”. Infants and young response a relapse may also occur secondarily 8 children with sickle cell anaemia whose spleens to an acute bacterial or viral illness. A good 9 have not yet undergone multiple response is most likely in patients under 45 4011 and subsequent fibrosis, and those individuals years, in those in whom the thrombocytopenia 1 with other forms of sickle cell disease, whose is less severe, and in those who have shown at 2 spleens remain enlarged into adult life, can sud- least an initial response to steroid therapy. At 3 denly have intrasplenic pooling of vast amount operation, the spleen is usually of normal size 4 of blood. and no special technique is needed for its 5 During severe splenic sequestration, the removal, although a careful search must be 6 spleen becomes enormous, filling the abdomen made to ensure that no splenunculi remain. 7 and even reaching into the . The usual Although the preoperative platelet count may be 8 clinical manifestations of this complication are very low, platelet transfusion is not begun until 9 sudden weakness, pallor of the lips and mucous the splenic vessels have been ligated, when 6 to 5011 membranes, tachycardia, tachypnoea, and 10 units of platelet may be transfused. In addi- 1 abdominal fullness. Splenic sequestration is tion, some patients may benefit from high doses 2 one of the most dangerous events in the life of of intravenous gammaglobulin preoperatively 311

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111 to raise the platelet count to an acceptable level Treatment 2 (greater than 20 × 109/l). 3 1. Medical care. Simple blood transfusion 4 Spleen and Spherocytosis for anaemia or exchange transfusion 5 for severe hyperbilirubinaemia may be 6 The disorders of the red cell membrane are a needed for symptomatic newborns. 7 very heterogeneous group. They fall into two 2. Surgical care. For surgical care, consider 8 main categories – inherited and acquired. splenectomy and partial splenectomy. 9 Inherited red cell membrane disorders are Preoperative (Pneumovax) 1011 mainly caused by a decrease in the expression of and preoperative antibiotics should be 1 one or more specific proteins on the membrane given to avoid OPSI. 2 of the cell. This causes the membrane to become 3 unstable by splenic pitting, causing spherocyto- Splenectomy 4 sis of the cell. Spherocytes, being less deformable • This surgical procedure is frequently 5 than discocytes, become trapped in the micro- performed to eliminate or minimise the 6 circulation of the spleen and are destroyed by need for blood transfusion. 7 the spleen and the reticuloendothelial (RE) • Splenectomy is not curative but may 8 system, resulting in haemolytic anaemia. eliminate or decrease the need for blood 9 The most common of the hereditary mem- transfusions in transfusion-dependent 2011 brane defects is hereditary spherocytosis (HS). patients. 1 This is a very clinically heterogeneous disorder 2 in which the whole spectrum of gravity may be • In an attempt at splenic conservation, 3 covered, from no clinical expression to death in partial splenectomy has been tried but 4 utero. Most patients experience haemolytic results are not satisfactory and total 5 anaemia, with or without the need for regular splenectomy may be resorted to. 6 blood transfusion, but the disease may be com- 7 plicated by aplastic or haemolytic crises and Gallstones 8 biliary tract disease. Pigment stones are a common complication of 9 The diagnosis of HS has always been based on congenital spherocytosis and are found most 3011 clinical symptoms and laboratory data, the most often in young adults in the third decade of life. 1 common clinical signs being jaundice, anaemia, The presence of stones is usually considered an 2 splenomegaly and biliary stones together with indication for cholecystectomy at the same time 3 the presence of spherocytes in the peripheral as splenectomy. If not done simultaneously, we 4 blood film. The traditional methods of diagnos- prefer splenectomy first. Bile duct stones should 5 ing spherocytosis are the osmotic fragility test, be classically treated. 6 autohaemolysis, acid glycerol lysis, Pink test 7 and cryohaemolysis test. These tests are insen- Spleen and Thalassaemia 8 sitive because the results are subjective as they 9 rely on human judgement to decide whether the The pathogenesis of the anaemia of thalas- 4011 test is positive or negative. A new technique has saemia is multifactorial. Inclusion bodies are 1 been developed, the dye-binding flow cytomet- rarely found in the peripheral blood of thalas- 2 ric method for detection of red cell membrane saemic patients with a normally functioning 3 abnormalities. spleen. However, splenectomised patients with 4 Pyruvate kinase deficient reticulocytes are thalassaemia major show inclusion bodies 5 able to circumvent their defect by using the within their red cells, indicating that the spleen 6 oxidative phosphorylation pathway to produce is important for the clearance of globin chain 7 ATP. This ability is diminished when the retic- precipitates. 51Cr-labelled red cell studies show 8 ulocytes are exposed to hypoxia or when they increased uptake of the thalassaemic cells by the 9 mature to adult red cells. This explains why spleen. 5011 most of the haemolysis occurs when the reticu- Light microscopic examination of splenic 1 locytes are trapped in the hypoxic environment tissue from patients with thalassaemia major 2 of the spleen and why a paradoxical rise in retic- shows an accumulation of red cells within 311 ulocyte number occurs after splenectomy. the splenic cords. The interendothelial slits are

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10 · UPPER GASTROINTESTINAL SURGERY the most important barrier to red cell passage: aly, marked elevations in levels of serum IgM 1111 electron microscopic studies demonstrate and malaria antibody. Hepatic sinusoidal lym- 2 marked distortion of the thalassaemic cells as phocytosis is also seen. In about 10% of African 3 they pass through these apertures, and red cells patients, it may be associated with peripheral 4 containing inclusion bodies are disrupted lymphocytosis (B cells). 5 during their passage through them. The peripheral smear shows manifestations 6 Splenomegaly is a common sequela of thalas- of hypersplenism. Malarial parasites are not 7 saemia major. This can result in hypersplenism found in the peripheral blood. There is an 8 and increased red cell transfusion requirements. increase ill the serum levels of polyclonal IgM 9 Hypertransfusion programmes designed to with cryoglobulinaemla. 1011 maintain the patient’s haemoglobin within the 1 normal range have decreased the spleen size Differential Diagnosis 2 in some patients, which may be due to dimin- 3 The condition should be differentiated from ished red cell pooling, or possibly a reduction 4 other causes of splenomegaly in the tropics, i.e. in extramedullary haematopoiesis within the 5 kala-azar, schistosomasis, post-necrotic cirrho- spleen. 6 sis, thalassaemia, leukaemia, lymphoma, myelo- Some thalassaemia patients benefit from 7 fibrosis, non-tropical idiopathic splenomegaly splenectomy performed to alleviate the hyper- 8 and Felty’s syndrome. splenism and decrease red cell transfusion 9 requirements. Several studies have attempted to 2011 define factors capable of predicting the optimal Surgery in Hyper-reactive Malarial 1 time for splenectomy in these patients. Some Splenomegaly 2 would recommend splenectomy when transfu- In patients with uncomplicated hyper-reactive 3 sion requirements progressively rise above their malarial splenomegaly, splenectomy seems to 4 previous baseline. produce initial symptomatic improvement, with 5 correction of anaemia, IgM and hypersplenism, 6 and with an acceptable operative mortality. 7 Spleen and Tropical However, subsequent mortality rates, appar- 8 ently due to overwhelming sepsis or malaria. 9 Diseases Splenectomy seems indicated in the minority of 3011 patients who do not respond to prolonged anti- 1 malarial medication. 2 Tropical Splenomegaly 3 Syndrome (Hyper-reactive 4 Malarial Splenomegaly, Spleen and Kala-azar (Visceral 5 Leishmaniasis) 6 Big Spleen Disease) 7 Leishmania donovani parasites invade the retic- 8 Definition uloendothelial cells of the spleen (liver and 9 Tropical splenomegaly syndrome or big spleen lymph nodes) and multiply. Patients with pro- 4011 disease is massive enlargement of the spleen gressive disease develop marked splenomegaly 1 resulting from an abnormal immune response due to hyperplasia of reticuloendothelial cells 2 to repeated attacks of malaria. It is seen among that are filled with parasites, and splenic infarcts 3 residents of areas for endemic malaria and it is are common. In acute cases, the spleen is 4 not species specific. It occurs mainly in tropical smooth and friable, but it is firm in the more 5 Africa, but also in parts of Vietnam, New usual chronic cases. 6 Guinea, India, Sri Lanka, Thailand, Indonesia, Splenectomy is occasionally employed in 7 South America and the Middle East. It must be patients with visceral leishmaniasis who are 8 differentiated from splenomegaly associated resistant to other forms of treatment. There 9 with acquisition of immunity in endemic and is usually a rise in haemoglobin levels and white 5011 hyperendemic areas. blood cell and platelet counts after splenec- 1 Tropical splenomegaly syndrome is charac- tomy, but additional chemotherapy is neces- 2 terized by massive splenomegaly, hepatomeg- sary, because cure is unlikely otherwise. 311

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111 Spleen and Schistosomiasis (b) With history of haematemesis, either 2 with normal or critical portal vein pres- 3 Splenomegaly and hypersplenism result from sure or with high portal vein pressure. 4 chronic Schistosoma mansoni infection and 5 are frequent indications for splenectomy in Group 3. Bilharzial splenomegaly with ascites 6 endemic areas of the world. Partial splenectomy but no varices, either controllable by medical 7 (segmental splenectomy) has been described treatment or not. 8 for this disorder [5,6]. Fifty-one patients who 9 underwent segmental splenectomy and 44 Group 4. Bilhazial splenomegaly combined with 1011 patients treated by total splenectomy for schis- varices and ascites. 1 tosomiasis were followed: the percentage of 2 T lymphocytes increased after segmental Complications of Splenectomy in 3 splenectomy, and there was also an increased 4 T-helper:T-suppressor cell ratio after segmental the Tropics 5 splenectomy. Only two patients required con- 6 version from partial to total splenectomy for Although complications of splenectomy are the same as elsewhere, some tropical diseases can 7 technical reasons, and no increase in size of the have an impact on the spleen. In addition, acute 8 splenic remnant was noted during the subse- malaria is common immediately after splenec- 9 quent 2 to 4 years. To our knowledge, this is the tomy, especially when blood transfusion is 2011 largest series of randomized elective partial used. Infestation of donated blood is common 1 (segmental) splenectomies reported and pro- in endemic areas and the infective dose of par- 2 vides encouraging data in favour of the concept asites is small. The risk of acute and chronic 3 of partial splenectomy for hypersplenism. malaria after splenectomy in patients with 4 Massive splenomegaly may suggest the pres- hyper-reactive malarial splenomegaly is proba- 5 ence of a splenic follicular lymphoma. This rare bly increased by the genetic defect these patients 6 tumour occurred in 1% of patients with hepatic have in their immune response to the parasite. 7 schistosomiasis who underwent splenectomy. It A therapeutic course of an antimalarial such as 8 is the only whose incidence is defi- 9 chloroquine is indicated perioperatively in all nitely increased in hepatic schistosomiasis. patients undergoing splenectomy; prophylaxis 3011 Although no definite relation between the size 1 is then necessary for life in malarious regions of the spleen and other data could be detected, to reduce the risk of fatal malaria. Asplenic 2 it can be said that a shrunken liver, varices, hae- 3 patients from malaria-free countries should be matemesis and ascites were fairly common with warned against travel to malarious areas, even 4 huge enlargement of the spleen. Ascites, in par- 5 with appropriate chemoprophylaxis. ticular, was commoner with huge enlargement Overwhelming has been reported 6 of the spleen than otherwise. 7 as a rare long-term complication of splenec- 8 tomy. The incidence of overwhelming bacterial Classification of portal hypertension as sug- sepsis after splenectomy may be greater in the 9 gested by [7] is outlined below. 4011 tropics than temperate zones, and the mortality 1 rate also seems higher, perhaps due to delays Group 1. Bilharzial splenomegaly without other 2 and deficiencies in treatment. Pneumococcal clinical evidence of portal obstruction or ascites. 3 and other antibacterial vaccines may offer pro- 4 (a) With normal or critical portal vein tection, but are no substitute for preservation of 5 pressure. at least a portion of normally perfused spleen whenever technically possible. 6 (b) With high portal vein pressure. 7 8 Group 2. Bilharzial splenomegaly with varices 9 but no ascites. Spleen and Brucellosis 5011 1 (a) With no history of haematemesis, either Human brucellosis is a multisystem disease with 2 with normal or critical portal vein pres- a broad spectrum of clinical manifestations. 311 sure or with high portal vein pressure. After haematogenous dissemination, circulat-

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10 · UPPER GASTROINTESTINAL SURGERY ing Brucella organisms are removed by reticu- 1111 loendothelial cells in the liver, spleen and bone 2 marrow. Suppurative disease of the liver and/or 3 spleen is a rare and serious complication of 4 human brucellosis. In the English language lit- 5 erature, only nine cases have been reported, all 6 involving adults with chronic infection. [8] 7 reported the case of a young child in whom 8 abscesses of the liver and spleen developed 9 during acute brucellosis. Brucella melitenesis 1011 was cultured from an aspirate of the liver and 1 from bone marrow. After percutaneous 2 drainage of the liver abscess, the patient 3 responded to a 56-day course of antimicrobial 4 therapy. 5 With the advent of non-invasive imaging 6 techniques such as ultrasonography and CT, 7 diagnosis and localisation of abscesses are 8 now relatively easy, which allows for the timely 9 initiation of appropriate therapy. Clinicians 2011 should be aware of this unusual complication of 1 brucellosis in children and should include 2 Brucella on the list of pathogens causing pyo- 3 genic liver and splenic abscesses. 4 5 6 Tuberculous Splenomegaly 7 8 Splenic tuberculosis is rare and may present as 9 a splenic abscess or with hypersplenism. The 3011 presence of multiple hypoechoic lesions on 1 ultrasonography of the spleen in an HIV-posi- 2 tive patient is highly suggestive of disseminated 3 tuberculosis. The diagnosis is usually made fol- 4 lowing surgical resection of the diseased spleen. 5 The size of the spleen in tuberculosis varies Diagram showing tuberculosis of the spleen. 6 from normal to very large. In acute miliary The cut surface is studded with caseous foci 7 1 tuberculosis, the spleen is commonly involved varying in size from about 1 ⁄2 cm downwards 8 and the cut surface shows numerous tubercles 1 and in parts coalescing into larger areas. 9 to 2 mm in diameter. The miliary tubercles are 4011 similar in size to the lymphoid follicles, but are 1 more numerous. They are pale yellow, unlike Spleen and HIV Infection 2 the lymph. follicles, which are grey. In the less 3 acute forms the tubercles are large and fuse The important role of the spleen in maintaining 4 together. In chronic non-miliary tuberculosis immune competence is clear, prompting 5 involvement of the spleen is much less frequent numerous researchers and clinicians to ask 6 and the number of tubercles may be small. whether removing the spleen might place indi- 7 Tuberculous splenomegaly may complicate viduals with compromised immune status 8 tuberculous . A therapeutic test with (people with HIV or AIDS), and those receiving 9 antituberculous drugs brings about some chemotherapy, at an increased risk for sepsis or 5011 improvement, and there is less danger of dis- opportunistic infections. 1 semination of the tubercle bacilli if splenectomy Lymphoreticular tissues such as lymph nodes 2 is undertaken. and spleen are thought to be major sites for HIV 311

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111 viral replication throughout the natural history 2 of HIV infection and AIDS, The spleen, while Niemann–Pick Disease 3 integral to the maintenance of immune compe- This is caused by specific genetic mutations; the 4 tence, also represents a “reservoir” where HIV four forms of Niemann–Pick disease are all 5 virions pool, accumulate and replicate during characterised by an accumulation of sphin- 6 the disease’s asymptomatic phase. Up to 20% of gomyelin and cholesterol in cells, particularly in 7 individuals with asymptomatic HIV disease the cells of major organs, such as the liver and 8 experience ITP (idiopathic thrombocytopenic the spleen. The three most commonly recog- 9 purpura), which is often successfully treated nised forms of the disease are types A, B and C. 1011 via splenectomy, yielding long-lasting platelet Types A and B Niemann–Pick are both caused 1 increases in more than 80% of patients. by the deficiency of a specific enzyme activity, 2 Splenectomy has been associated with a relative acid sphingomyelinase (ASM). If ASM is absent 3 leucocytosis and lymphocytosis. Increased lym- or not functioning properly, sphingomyelin 4 phocyte counts, as well as changes in the cannot be metabolised properly and is accumu- 5 cytokine environment after splenectomy, may lated within the cell, eventually causing cell 6 also play a role in moderating the course of HIV death and the malfunction of major organ 7 infection, but it is not known whether (or how) systems. The mutations for types A and B have 8 such immunomodulatory changes relate to the been extensively studied, particularly among 9 surgical removal of the HIV reservoir, the the Ashkenazi Jewish population, and DNA tests 2011 spleen. Randomised clinical trials are required for these forms of Niemann–Pick are available. 1 to evaluate more fully the role of splenectomy 2 in HIV and to elucidate the complex role of 3 immunomodulatory agents in this clinical Amyloidosis of the Spleen 4 setting. 5 Sago spleen is a morbid condition of the spleen, 6 produced by amyloid degeneration of the organ, 7 Gaucher’s Disease (GD) in which a cross-section shows scattered grey 8 translucent bodies looking like grains of sago 9 GD is the most prevalent lysosomal sphin- (dry granulated starch, much used for making 3011 golipid storage disorder. It is an autosomal puddings and also, as starch, for stiffening 1 recessive disorder that results from a deficiency textile fabrics). 2 in ␤-glucocerebrosidase with accumulation of Sago spleen has two different anatomical 3 the substrate glucosylceramide in monocytes patterns. Most commonly, the amyloid deposi- 4 and macrophages. Type 1 GD usually starts tion is limited to the splenic follicles, resulting 5 in childhood with most patients presenting in the gross appearance of a moderately 6 before the age of 10. GD causes growth retarda- enlarged spleen dotted with grey nodules (so- called “sago” spleen). Alternatively, the amyloid 7 tion, hepatosplenomegaly, hypersplenism, bone deposits may spare the follicles and mainly infil- 8 involvement (avascular necrosis) and central trate the red pulp sinuses, producing a large, 9 nervous system involvement. Intravenous firm spleen mottled with waxy discolorations 4011 enzyme replacement is the therapy of choice (“lardaceotis “spleen). 1 and has changed life expectancy, reduced 2 organomegaly and improved haematological 3 disorder. Partial splenectomy (removal of Myelofibrosis 4 85–90%) may improve severe hypersplenism 5 and mechanical problems, but the remaining Myelofibrosis is a disorder in which fibrous 6 spleen enlarges and the preoperative condition tissue may replace the precursor cells that 7 recurs in most patients. Total splenectomy is produce normal blood cells in the bone marrow, 8 postponed as far as possible in life due to the resulting in abnormally shaped red blood cells, 9 risk of post-splenectomy sepsis, accelerated anaemia, and an enlarged spleen. Eventually, 5011 hepatic and bone lipid deposition with earlier fibrous tissue replaces so much of the bone 1 appearance of osteolytic changes (painful marrow that production of all blood cells is 2 crisis). reduced. When this happens, the anaemia 311

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10 · UPPER GASTROINTESTINAL SURGERY becomes severe, the reduced number of white Treatment 1111 blood cells cannot fight infections, and the 2 reduced number of platelets cannot prevent Splenectomy is an easier approach to the man- 3 bleeding. The body produces blood cells outside agement of splenic artery aneurysm in cases of 4 the bone marrow, mainly in the liver and spleen, emergency management of ruptured aneurysm. 5 which may enlarge; this condition is called idio- Other options are either operative repair or 6 pathic myeloid metaplasia. percutaneous transcatheter embolisation. The 7 Often, myelofibrosis produces no symptoms latter is performed via selective arterial 8 for years. Eventually, the anaemia makes people catheterisation with initial deployment of a coil 9 weak and tired, they feel unwell and lose weight. in the neck extending proximally. 1011 The enlarged spleen and liver may cause pain in 1 the abdomen. Splenic Vein 2 3 Treatment The pathophysiology of splenic vein thrombo- 4 sis includes compression, encasement and 5 No available treatment can effectively reverse or inflammation around the splenic vein. In most 6 permanently slow the progression of this disor- cases thrombosis is related to pancreatic carci- 7 der, although the anticancer drug hydroxyurea noma or chronic pancreatitis; however, the list 8 may decrease the size of the liver or spleen. of causes is long and includes trauma and large 9 Rarely, the spleen becomes extremely large and masses causing compression. 2011 painful and may have to be removed. Infections Radiological findings may demonstrate evi- 1 are treated with antibiotics. dence of the underlying cause of the splenic vein 2 thrombosis; for example, pancreatic calcifica- 3 tions on plain films in a patient with chronic 4 Vascular Diseases pancreatitis, or a large pancreatic mass seen on 5 CT. 6 Splenic Artery Aneurysm 7 Splenic Infarct 8 Splenic artery aneurysms and pseudoa- 9 neurysms are rare, but clinically important vas- Splenic infarct is a rare form of pathology. The 3011 cular lesions with a high risk of rupture. Splenic infarct may be segmental or global, involving the 1 artery aneurysms are the most frequent visceral entire organ. It is the result of arterial or venous 2 arterial aneurysms, accounting for 60% of all compromise, and is associated with a heteroge- 3 splanchnic artery aneurysms. neous group of diseases. Surgery is indicated 4 The most common aetiologies include medial only in the presence of complications such as 5 degeneration with superimposed atherosclero- haemorrhage, rupture, abscess or . 6 sis, congenital, mycotic, portal hypertension, 7 fibromuscular dysplasia, and pseudoaneurysms Frequency 8 from trauma and pancreatitis. Pseudoaneur- Splenic infarct is most commonly associated 9 ysms of the splenic artery occur secondary to with haematological disorders. The propensity 4011 injury from released pancreatitis enzymes. for in sickle haemoglobin- 1 Splenic artery aneurysm and pseudoa- opathies is well known. 2 neurysm are diagnosed by CT scan, the com- The mechanism of splenic infarction in sickle 3 mon finding is high central density surrounded cell disease is attributed to crystallisation of 4 by a peripancreatic collection (clot within a the abnormal haemoglobin during periods of 5 pseudoaneurysm), although it is common to hypoxia or acidosis. The rigid erythrocyte leads 6 mistake a pancreatic pseudocyst for a splenic to rouleaux formation and occlusion of the 7 pseudoaneurysm and vice versa. splenic circulation. 8 Dissecting aneurysm of the splenic artery Systemic embolisation can also result in 9 may occasionally occur, producing acute severe splenic infarct. It occurs most commonly in the 5011 pain in the left hypochondrium and loin. It is setting of a left atrial or ventricular mural 1 diagnosed by CT, MRI and subtraction digital formed as the result of acute myocar- 2 angiography. dial infarction. While autopsy series report a 9% 311

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111 incidence of splenic infarction in early deaths Splenic Arteriovenous Fistula 2 following an acute , clini- 3 cal series report a much lower incidence of Blunt or penetrating injury to the abdomen 4 splenic embolisation, probably reflecting the leading to splenic arteriovenous fistula (AVF) is 5 silent clinical course of many splenic infarcts. well known and described in the literature. The 6 Iatrogenic splenic embolisation as in the man- other notable causes of splenic AVF are high 7 agement of hypersplenism and portal hyper- intra-abdominal pressure such as in 8 tension may produce localised infarction or and childbirth, post-splenectomy and following 9 massive infarction following splenic artery liga- rupture of a splenic artery aneurysm into the 1011 tion in the treatment of the portal hypertension. splenic vein. The development of portal hyper- 1 Splenic infarct can be diagnosed by imaging tension in patients with AVF could be related to 2 procedures. an increase in intravenous pressure resulting 3 from increased blood flow or arterialisation of 4 Treatment the portal venous system. This is a potentially 5 treatable entity and splenectomy with resec- 6 Medical Therapy tion of fistula resulted in resolution of signs 7 Surgery is indicated only in the presence of and symptoms in all reported cases. The AVF 8 complications. Otherwise, the infarcted spleen evolved over a long time after initial trauma 9 can be left in situ and the patient observed. The or surgery. Increased venous pressure of the 2011 principal mainstay of medical therapy is anal- splenic vein and portal vein may lead to forma- 1 gesia with either narcotics or non-steroidal anti- tion of gastric varices and dilation of the infe- 2 inflammatory agents. rior inesenteric vein, which drains into the 3 splenic vein. 4 It was also believed that normal liver function 5 Surgical Therapy or biopsy in patients with portal hypertension 6 • Splenectomy is indicated in splenic should be followed up by Doppler sonography 7 abscess when aspiration or catheter and angiography to visualise any splanchnic 8 drainage falls to treat the patients. In AVE. A history of abdominal trauma, splenec- 9 view of the small but real risk of fatal tomy, multiple and presence of 3011 overwhelming post-splenectomy sepsis, splenic artery aneurysm are strong indicators. 1 splenic preservation is preferable when- Suggestive clinical features include a pulsatile or 2 ever possible. soft mass in the upper abdomen with palpable 3 thrill and machinery murmur on auscultation. 4 • In cases of torsion of a wandering spleen, The ideal treatment in selected patients would 5 splenopexy with splenic salvage is the be to embolise the AVF. 6 procedure of choice in the well-perfused, refers to autotransplantation of 7 non-infarcted spleen. individual fragments of splenic tissue left 8 • Complications such as bleeding or behind after either operative or traumatic 9 pseudocyst formation may also be removal of the spleen. Although rarely sympto- 4011 amenable to splenic salvage using tech- matic, splenosis may cause intestinal obstruc- 1 niques of conservative splenectomy. tion since the splenules link adjacent loops of 2 • If there is perisplenic inflammation and bowels to each other, kinking and obstructing 3 dense adhesions that make splenectomy them. Tomographic selective splenic scintigra- 4 difficult, preoperative splenic artery phy with sulphur colloid and heat-damaged red 5 embolisation, which purposely infarcts cells is the most sensitive method to detect 6 the remaining spleen, may minimise splenosis. Patients who undergo emergency 7 blood loss, which can otherwise be quite splenectomy for trauma are at a much higher 8 profuse in these difficult dissections. risk of developing splenosis than those splenec- 9 Intraoperative ligation of the splenic tomised due to haematological conditions. 5011 artery at the superior margin of the pan- Spilled splenic tissue seeds the peritoneum, 1 creas in the lesser sac is another alterna- takes root and grows into vascularised 2 tive to minimise blood loss if the spleen splenules. Experimental evidence (Folkman) 311 is enlarged. suggests that the presence of a large amount of

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10 · UPPER GASTROINTESTINAL SURGERY vascularised spleen inhibits the growth of other Splenic Index 1111 splenic tissue in mice, providing a theory why 2 patients who undergo partial splenectomy, Bleeding from oesophageal varices with its 3 splenography or are merely observed after high mortality rate is a known complication in 4 splenic rupture have almost no splenosis. patients with cirrhosis. The clinical course 5 Management of splenosis is expectant. in these cirrhotic patients shows progressive 6 splenomegaly. A study by Watanabe et al [9] 7 tried to determine if the splenic size in cirrhotic 8 Investigations of the Cause patients correlated with the severity of 9 oesophageal varices, gastric varices and liver 1011 of Splenomegaly function. 1 The splenic index is also used in malaria 2 Clinical examination must include a very careful surveys to estimate the size of the spleen among 3 study of the superficial lymph nodes and the people in areas endemic for malaria during 4 tonsillar and adenoid tissues, a search for epidemiological studies. 5 stigmas of liver disease, a full examination of the 6 skin for evidence of purpura or bruising, and a 7 complete survey of all the systems. 8 A few simple laboratory investigations will Rupture of the Spleen: 9 provide the cause for most cases of Aetiology 2011 splenomegaly. Almost all the splenomegalies 1 associated with haematological disorders can be Traumatic Rupture 2 diagnosed by a complete blood examination 3 together with a bone marrow aspiration. Liver A can be closed, open or iatro- 4 disease is often associated with abnormal liver genic. Minor trauma such as a fall from a 5 function tests or oesophageal varices on barium bicycle, donkey or a camel may lead to rupture 6 swallow examination. Liver biopsy may be nec- of the spleen in childhood as the ribs are more 7 essary to determine the type of liver pathology. flexible and the spleen is relatively large and 8 The diagnosis of lymphoma or other reticu- less protected. At any age, splenic enlargement 9 loendothelial malignancies may be accom- produced by malaria, vascular malformation, 3011 plished by biopsy of a suitable node, the liver or haematological causes, infectious mononueleo- 1 another involved organ, lymphangiography or sis and splenic fixity by perisplenic adhesion 2 bone marrow biopsy, in some cases diagnostic may predispose to easy rupture after minor 3 splenectomy may be necessary. injuries and that is why corporal punishment is 4 The various infections that produce prohibited in areas endemic for malaria. 5 splenomegaly may be identified from appropri- The importance of preserving splenic func- 6 ate haematological or serological studies. Infec- tion for both children and adults is now well 7 tious mononucleosis is diagnosed by the finding recognised. 8 of atypical lymphocytes in the blood, a positive The early complications of the non-operative 9 Paul–Bunnell test (or related screening proce- management of a ruptured spleen include 4011 dures) and a rising anti-Epstein–Barr virus titre. failure to detect continuing splenic haemor- 1 In those who have travelled to the tropics, rhage and associated injury requiring laparo- 2 malarial parasites should be looked for and tomy. In the longer term, delayed rupture of a 3 the marrow examined for Leishman–Donovan subcapsular haematoma or post-traumatic cysts 4 bodies in cases of leishmaniasis (kala-azar). may occasionally follow splenic preservation 5 Special investigations in splenic disorders and should be managed by capsular repair, 6 include radiography, ultrasonography, com- partial splenectomy, or unroofing and drainage 7 puted tomography (CT), magnetic resonance of cysts, whenever possible. Intraperitoneal 8 imaging (MRI), angiography, splenopor- implantation of splenic fragments (splenosis) 9 tography, measurement of portal venous does not seem to provide asplenic patients 5011 pressure (percutaneous intrasplenic pressure with protection from severe infection and is no 1 and wedged hepatic venous pressure) and substitute for appropriate lifetime prophylaxis. 2 scintigraphy. 311

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111 Non-traumatic Rupture 1. Trend now toward saving the spleen 2 (splenorrhaphy) with the use of intraop- 3 Non-traumatic rupture of an entirely normal erative packing, pledged sutures, polyg- 4 spleen arguably does not occur in the absence lycolic mesh wrap, hemisplenectomy, 5 of a coagulopathy, and even the diseased spleen omental wrap. 6 rarely ruptures spontaneously. Subcapsular 2. Splenectomy (total or partial) for grade 7 haemorrhage in an acutely inflamed, enlarged 4–5 injuries. 8 and congested spleen is probably the most 3. Conservative therapy should be critically 9 common mechanism of pathological rupture. evaluated the patient has multiple 1011 Trivial trauma may have been forgotten or dis- injuries (i.e. head injuries). 1 regarded. 4. Operative trauma results in injury to the 2 Acute malaria is the most common cause of spleen in roughly 2% of operations and 3 non-traumatic splenic rupture in the tropics. most of these can be repaired if the 4 The incidence is highest during initial attacks of injury is noted at the time. 5 malaria, but even then it is rare. The chronically 6 enlarged spleen may undergo a degree of cap- 5. Other concerns: autotransplantation of 7 sular fibrosis, which protects it somewhat from splenic mass does not protect against 8 rupture. infection. 9 Infectious causes of spontaneous rupture, 2011 such as Salmonella, Epstein–Barr virus and 1 hepatitis virus, probably occur more commonly 2 in the tropics, while haematological and neo- 3 plastic causes have a similar incidence in tropi- 4 cal and temperate areas. 5 6 7 Staging of Splenic Trauma 8 Staging classification: 9 3011 Grade 1: capsule tear or minor parenchymal 1 laceration. 2 Grade 2: capsular avulsion. 3 Grade 3: major parenchymal fracture or 4 laceration or penetrating gunshot or stab 5 wound. 6 Grade 4: severe parenchymal stellate frac- 7 ture, crush, bisection or hilar injury. 8 9 Grade 5: shattered spleen (or multiply 4011 injured patient). 1 Diagram showing that when the spleen has been 2 Management severely damaged, but the hilar vessesls are 3 intact, a synthetic absorbable mesh wrap may be 4 Non-operative management: used to provide haemostasis by gentle com- 5 1. Selectively stable patients with grade 2 or pression. 6 less injuries (controversial) 7 2. In children higher levels of splenic injury 8 tend to be accepted for observation Splenectomy 9 (follow with CT, serial haematocrits, 5011 close observation). Indications 1 2 Operative management (after failure of non- The accepted indications for splenectomy have 311 operative management): changed dramatically over time. Splenic trauma

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10 · UPPER GASTROINTESTINAL SURGERY is the most common surgical indication for and distortion with premature culling by the 1111 splenectomy, although attempts at splenic spleen), thalassaemia and acquired immune 2 preservation are increasingly important. Splenic haemolytic anaemia. 3 cysts, tumours, and vascular lesions may also 4 require surgical removal. Whenever possible, 5 splenic tissue is preserved to decrease the risk Surgical Indications 6 of septicaemia, but total splenectomy occasion- 1. Rupture of spleen. 7 ally is necessary. 2. Removal of tumours, cysts or vascular 8 Early reports of splenectomy for trauma anomalies. 9 exist, but the first documented splenectomy 1011 occurred in 1849 for splenomegaly. By the 3. Need for adequate exposure of left upper 1 1950s, splenomegaly with hypersplenism was quadrant (LUQ). 2 the most common indication for splenectomy, 4. Certain shunting procedures. 3 whereas staging of Hodgkin’s disease predomi- 5. For staging of Hodgkin’s disease and 4 nated in the 1970s. other lymphoreticular malignancies. 5 Indications for splenectomy were determined 6 on the basis of the patient’s primary diagnosis. Complications of Splenectomy 7 Trauma included all splenectomies performed 8 for blunt or penetrating injury to the spleen. Overwheiming Post-splenectomy 9 Incidental splenectomies included all spleens Infection (OPSI) 2011 removed as a planned portion of another pro- 1 cedure (e.g., distal pancreatectomy). Iatrogenic There is overwhelming sepsis, fatal within 2 cases were defined as removal of the spleen sec- 12–24 hours. Sixty per cent of cases are due 3 ondary to intraoperative injury. Any splenec- to pneumococci, the rest to meningococcal, 4 tomy performed for treatment or staging of Haemophilus influenzae, staphylococci, etc. 5 leukaemias or Hodgkin’s or non-Hodgkin’s • Risk of sepsis is 200 greater than in 6 lymphomas was included under haematological normal children. 7 malignancies. Cytopenia included anaemia, 8 leukopenia, or thrombocytopenia not sec- • Risk of overwhelming sepsis is low 9 ondary to a haematological malignancy and (0.5–1%) when splenectomy is done for 3011 was subcategorised into thrombocytopenia rupture of spleen, spherocytosis or ITP. 1 (Idiopathic and thrombotic thrombocytopenic • Risk is higher for alpha-thalassaemia 2 purpura (ITP and TTP)) and haemolytic (24.8%), histiocytosis or lipidosis. 3 anaemia. All splenectomies for causes or com- • Risk is highest in conditions that predis- 4 plications of left-sided portal hypertension or pose to infection such as Wiskott– 5 during decompressive shunt procedures were Aldrich syndrome. 6 included in the category of portal disorders. 7 Diagnostic splenectomies were performed to Pneumococcus vaccine and vaccine against H. 8 clarify preoperative uncertainty, including cases influenzae should be given as a prophylaxis; 9 of splenic tumours, splenomegaly of unclear these are best given 10 days before elective 4011 aetiology, and fevers of unknown origin. All splenectomy and preoperatively in anticipation 1 miscellaneous conditions not assignable to of splenic trauma. 2 the above categories were included I “other”, Booster doses of Pneumovax 23 are not rec- 3 including pyruvate kinase deficiency, infarc- ommended due to possible reactions, except 4 tions secondary to septic emboli, and Felty’s maybe for those who are vaccinated before 2 5 syndrome. years of age. Surgery should be deferred until 6 5–6 years of age due to the higher risk of sepsis 7 Medical Indication for infants and young children and also the poor 8 response to vaccines before 18–24 months of 9 Some causes of haemolytic anaemia are suc- age. Accessory spleen (present in 10–18%) 5011 cessfully treated by splenectomy, including con- should be looked for in surgery. Penicillin pro- 1 genital spherocytosis, elliptocytosis, pyruvate phylaxis (for life?) or intermittent penicillin 2 deficiency (membrane damage of the red cells with illness should be given. Immediate medical 311

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111 evaluation for any febrile illness should always may appear in unusual forms. There is increased 2 be undertaken. risk of cerebral malaria, and of febrile illness in 3 Pneumovax 23 only covers 85% of serotypes which the classical intermittent pattern is not 4 and meningococcal vaccine only cover present and the parasites are demonstrated only 5 serotypes A, C, Y and W-135. Conjugated pneu- with difficulty in the blood, but which responds 6 mococcal vaccine (seven serotypes) for those < promptly to antimalarial drugs. 7 2 years of age is currently being tested. 8 Although post-splenectomy sepsis is uncom- Other Complications of Splenectomy 9 mon in the immediate postoperative period, 1011 patients who undergo incidental splenectomy • Haemorrhage (postoperative haemor- 1 as part of another procedure or secondary to rhage) 2 an iatrogenic injury do appear to have an • Pulmonary atelectasis 3 increased rate of morbidity and mortality • Subphrenic abscess 4 compared with their counterparts without • Pancreatic fistula 5 splenectomy. Traditionally, splenectomy was a • Gastric fistula 6 common part of operations for cancer in the left 7 upper quadrant. Recent studies have failed to • Thrombocytosis 8 show a survival advantage for splenectomy as a 9 routine part of resection for gastric carcinomas 2011 unless the distal pancreas is directly involved. Alternatives to Splenectomy 1 Furthermore, an increased mortality has been 2 shown for the additional unnecessary proce- Partial Splenectomy 3 dure. Animal studies have also shown an 4 increased risk for hepatic metastases for colon With the recognition of post-splenectomy sepsis 5 cancer in splenectomized mice, possibly related and infections and the availability of improved 6 to the impact on the host immune system. radiographic imaging, efforts have increased 7 Because of the increased morbidity and mortal- over the past two decades to avoid unnecessary 8 ity from splenectomy, more attempts are being splenectomy. The initial efforts were made 9 made at splenic conservation after iatrogenic within the field of trauma, clearly showing non- 3011 injury. operative splenic salvage as a viable alternative 1 The rise in diagnostic splenectomies is possi- in appropriately selected patients. 2 bly a result of improvement in radiographic Although the risk of post-splenectomy sepsis 3 techniques. Asymptomatic splenic masses was originally thought to be restricted to the 4 accounted for a large portion of the splenec- first 2 to 3 years following splenectomy, it is now 5 tomies for diagnostic purposes in the second clear that the asplenic adult may be vulnerable 6 5-year period after CT scanning developed its for many years after the event and perhaps 7 current prevalence. These lesions may have indefinitely. The critical task is to achieve a 8 gone undetected and unnoticed had a CT scan balance between ablation and conservation of 9 not been performed on these patients for other the spleen. A possible solution was suggested by 4011 indications. Maddison [10] who first used the technique of 1 There has been a decrease in overall incidence embolisation of the spleen with autologus blood 2 of splenectomy in cytopenic patients over the clots. Unfortunately, there was a high risk of 3 past 20 years, with that decrease secondary to complications, and in particular abscess forma- 4 the thrombocytopenic population. Patients with tion, which precluded this from being used 5 haemolytic anaemia had a clear increase in as an alternative to surgical splenectomy. 6 incidence of splenectomy. The reason for the Subsequently Witte et al [11] showed that 7 decline in the thrombocytopenic group is hypersplenism could be treated by partial abla- 8 unclear. Several studies have shown an ongoing tion of the splenic mass by segmental embolisa- 9 role for splenectomy in the treatment of patients tion of parenchyinal arteries with gel foam. 5011 with ITP and TTP, particularly patients refrac- Although this method was also associated with 1 tory to plasmaphoresis and steroids. complications including pleural effusion, sepsis 2 Parasitic infections are liable to recur after and splenic abscess, the preservation of a degree 311 splenectomy in patients from endemic areas and of prograde arterial flow does appear to protect

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10 · UPPER GASTROINTESTINAL SURGERY against the serious and frequent complication of patients who required splenectomy to relieve 1111 total splenic infarction. Partial embolisation has the symptoms of schistosomal splenomegaly, 2 also been used successfully to treat myelofibro- and their course was compared with that of 44 3 sis, spherocytosis and thalassaemia, while patients who underwent total splenectomy in an 4 others have used it to treat hypersplenism in unrandormised study (Figures 10.8 and 10.9). 5 portal hypertension. 6 Surgical Technique [5] 7 8 Segmental Splenectomy Segmental splenectomy involves removal of 9 a part of the spleen, based on its segmental 1011 Increasing recognition of unique and significant blood supply. This procedure is a compromise 1 functions of the spleen has stimulated interest between the need to remove the great bulk of 2 in the development of therapeutic alternatives splenic tissue responsible for hypersplenism 3 to total splenectomy. Techniques for partial and discomfort, and the desirability of preserv- 4 splenic resection and splenic salvage, initially ing normal splenic function. The opinion of the 5 developed for the management of splenic authors is to leave splenic tissue nearly equal to 6 trauma, are being applied to an increasing the weight of normal spleen, i.e. 150 g of spleen. 7 number of conditions in which reduction, The authors applied segmental splenectomy on 8 rather than total ablation, of splenic tissue is five patients with hepatosplenic schistosomiasis 9 desired. In a study by Kamel and Dunn [5] seg- and they concluded that this procedure is safe 2011 mental splenectomy was evaluated in 51 and well tolerated in such patients. 1 2 3 4 5 6 7 8 9 3011 1 2 3 4 5 6 7 8 9 4011 1 2 3 4 5 6 7 8 9 5011 1 Figure 10.8. Haemostatic compression of the spleen using manual pressure by the thumbs and forefingers of the second assistant. 2 The cut on the diaphragmatic surface of the spleen is shown as done in segmental splenectomy. 311

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111 Splenorrhaphy blood-clotting agents applied to the bleeding 2 areas, mesh wrapped around the spleen to hold 3 Indications it together or removal of small fragments of 4 the spleen. 5 Spleens that have been injured and are bleeding 6 require splenorrhaphy (repair of the spleen) or 7 if this is not successful a splenectomy is indi- Laparoscopic Splenectomy 8 cated. Most commonly these injuries are from car or bike accidents, falls, kicks, etc. 9 Laparoscopic splenectomy (LS) was first 1011 Preoperative Evaluation described by Delaitre et al [12], but unlike 1 laparoscopic cholecystectomy, did not gain 2 All patients require blood tests to verify that immediate widespread adoption. As laparo- 3 they are bleeding internally despite blood trans- scopic techniques have expanded, the frequency 4 fusions and medications. Frequently a CT scan of LS has increased rapidly since 1995. 5 is used to locate the spleen as a cause of inter- The location and size of the spleen make its 6 nal bleeding. exposure relatively difficult. The spleen’s prox- 7 imity to the pancreatic tail and its ample blood 8 Procedure supply require tedious operative dissection 9 The cracks in the spleen, which cause the bleed- and advanced laparoscopic skills. ITP is the 2011 ing, are repaired using sutures. Frequently this most common non-traumatic indication for 1 splenectomy, and the small size of the spleen 2 does not completely stop the bleeding and in this disease renders it amenable to the 3 additional techniques must be used including 4 5 6 7 8 9 3011 1 2 3 4 5 6 7 8 9 4011 1 2 3 4 5 6 7 8 9 5011 1 2 311 Figure 10.9. The splenic remnant with an omental patch in place. The final appearance after segmental splenectomy.

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10 · UPPER GASTROINTESTINAL SURGERY laparoscopic approach. Although controversial, 1111 a maximum splenic size of approximately 20 cm Questions 2 in diameter has been recommended. However, 1. List congenital abnormalities of the 3 with increasing experience, LS is being applied spleen. 4 successfully to patients with larger spleens. 2. What are the indications for splenec- 5 Indications such as hereditary spherocytosis, tomy? 6 haemolytic anaemia, Hodgkin’s disease, lym- 7 3. What are the complications of splenec- phoma, idiopathic neutropenia, sarcoidosis and 8 tomy? thrombotic thrombocytopenic purpura are now 9 4. List tropical diseases affecting the spleen. considered for LS. 1011 LS is feasible, safe and effective. With increas- 1 ing surgical experience, shorter operative time, 2 the postoperative advantages of laparoscopic References 3 versus open surgery, and lower costs, LS is 1. Kyber E. Über die Mitz des Menchen und effiliger 4 emerging as the procedure of choice and Säugetiere. Arch mikroskop Anat Entwicklungsme- 5 soon may be the standard method of elective chanik 1870;6:540–70. 6 2. Gupta CD, Gupta SC, Arora AK, Heya Singh P. Vascular 7 splenectomy. segments in the human spleen. J Anat 1976;121: 613–6. 8 3. Mikhail Y, Kamel R, Nawar NNY, Rafla MFM. Observa- 9 Hepatisation of the Spleen tions on the mode of termination and parenchymal 2011 distribution of the splenic artery with evidence of 1 Allogeneic human hepatocyte transplantation splenic lobation and segmentation. J Anat 1979;128(2): 2 253–8. 3 has been used in both acute and chronic liver 4. Kassirsky, IA, Demiana NA, Lysenko AY. Malaria. In: A failure. Both transplantation into the liver via Handbook on tropical diseases. Moscow, 1974; 38 (111 4 the portal vein and ectopic transplantation into Russian). 5 the spleen have been also used. In pilot studies, 5. Kamel R, Dunn MA. Segmental splenectomy in schisto- 6 liver and splenic hepatocyte transplantation has somiasis. Br J Surg 1982;69:311–13. 7 6. Kamel R, Dunn MA,; Skelly RR et al. Clinical and 8 been shown to be both safe and potentially immunological results of segmental splenectomy in effective in humans as a bridge to orthotopic schistosomiasis. Br J Surg 1986;73:544–7. 9 transplantation. 7. Kamel R et al. 1966 3011 Immunosuppression is required in all 8. Vallejo JG, Stevens AM, Dutton RV, Kapla SL. 1 patients receiving allogeneic human hepatocyte Hepatosplenic abscesses due to Brucella melitensis: 2 transplantation. report of a case involving a child and review of the lit- 3 erature. Clin Infect Dis 1996;22:485–9. The spleen appears to be the preferred site 9. Watanabe S, Hosomi N, Kitade Y et al. Assessment of 4 due to fibrosis and loss of blood supply to the presence and severity of oesophagogastric varices by 5 the liver. In animal models, hepatisation of the splenic index in patients with liver cirrhosis. J. Comput 6 spleen is a well-described phenomenon and Assist Tomogr 2001; 24:788–94. 7 results in replacement of the splenic pulp with 10. Maddison, FE. Embolic therapy of hypersplenism. 8 Invest Radiol 1973;8:280–1. cords of functioning hepatocytes that perform 11. Witte CL, Van Wyck DB, Witte MH et al. and 9 hepatic functions including synthesis of partial resection for control of splenic hyperfunction. 4011 albumin and clotting factors, detoxification of Br J Surg (1982);69:531–5. 1 ammonia and oxidative metabolism. 12. Delaitre B, Maignien B. Splenectomy by the laparo- 2 scopic approach. A report of a case (letter). Press Med 3 1991;20(44):2263. 4 Acknowledgement 5 6 I would like to acknowledge Professor Manar El- Further Reading 7 Tonsy (Professor of Parasitology, Faculty of Bowdler A,J. A Textbook on: The spleen, structure, function 8 Medicine, Ain-Shams University) for her assis- and clinical significance. London: Chapman and Hall 9 tance during preparation of this chapter. Medical, (1990). 5011 1 2 311

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