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Home , Hereditary spherocytosis, Lipoma, Splenectomy

Invited Commentary Indications for STEVEN C. KATZ, M.D., H. LEON PACHTER, M.D. From the Department of , New York University Medical Center and Bellevue Hospital Center, Nezv York, NY

In the new millennium, indications for splenectomy have expanded. Proper patient selection based on an understanding of the biology of each individual's disease is essential for a favorable outcome. We review the most common diseases for which surgeons may be called on to perform splenectomy and while highlighting potential pitfalls and caveats.

HE MEDITATES important immunologic and Development and Anatomy hematologic functions as well as contributing to T The spleen forms as a mesenchymal condensation numerous pathologic processes. When the spleen is of the dorsal mesogastrium during the fifth week of involved in disease, splenectomy may be performed development. Initially, the spleen functions as a he- with tbe intent of either altering tbe clinical course or matopoietic organ and assumes its more mature lym- providing symptomatic relief. In the new millennium, phoid characteristics at 15 to 18 weeks.^ The spleen is indications for splenectomy have expanded, and sple- related to the posterior wall of the stomach and is nectomy may be beneficial to patients with a broad connected to the stomach and kidney by the gastro- spectium of benign and malignant diseases. Proper splenic and splenorena! ligaments. With the exception patient selection based on an understanding of the bi- of the hilum. the spleen is surrounded by peritoneum. ology of each individual's disease is essential for a On average, tbe spleen is 12 cm long and 7 cm wide. favorable outcome. The splenic artery originates from the celiac axis and Splenectomy is not without potential complications, divides into 5 or more terminal branches that enter the the most feared of which being overwhelming post- splenic hilum. Several tributaries join to form the splenectomy (OPSI). Mortality rates of OPSI splenic vein, which joins the superior mesenteric vein may exceed 50 per cent in unvaccinated patients.' to form the portal vein posterior to the neck of the Therefore, removal of the spleen should be performed pancreas. only when the potential benefits to the patient clearly exceed the risks. Recent advances in surgical tech- A dense fibrous capsule surrounds the spleen and niques allow for a laparoscopic approach in many pa- trabeculae form incomplete parenchymal compart- tients who require splenectomy, thereby minimizing ments within the splenic pulp. Stretching of the cap- operative morbidity and patient discomfort associated sule may cause pain in patients witb . On with open splenectomy. However, a minimally inva- cross-section, the spleen contains both red and white sive approach lo splenectomy is not always feasible or pulp. Arteries within the white pulp, central arteries, desirable and therefore familiarity with the traditional are surrounded by a sheath of lymphocytes known as approach remains vital. We review the spectrum of the periarteriolar lymphatic sheatb (PALS).^ Tbe disease processes for which splenectomy may be in- PALS is comprised primarily of T ceils, whereas ad- dicated. Prerequisites for successful therapeutic or pal- jacent lymphoid follicles are abundant in B cells. The liative splenectomy are a thorough understanding of marginal zone lies between tbe white and red pulp splenic anatomy, physiology, and pathophysiology. containing dendritic cells, which capture and present We discuss the most common diseases for which sur- antigen to lymphocytes. Within the red pulp, the sinu- geons may be called on to perform splenectomy and soids are lined by a fenestrated endothelium similar to consider tbe various surgical approaches while high- the lumen of the hepatic sinusoids."^ lighting potential pitfalls and caveats. Normal Splenic Function Although many functions of the spleen are redun- Address correspondence and reprint requests to H. Leon Pachter. M.D.. HCC 6 6C, 550 First Avenue, New York. NY dant or can be assumed by other organs, splenectomy 10016. can lead to adverse consequences. An appreciation of

565 566 THE AMERICAN SURGEON July 2006 Vol. 72

normal spleen function is important for the physician adults often require specific therapeutic intervention. seeking to understand the potential consequences of In the event of persistent or recurrent disease after splenectomy in a given disease process. Under normal treatment with glucocorticoids. cytotoxic agents, or conditions, the spleen contains less than 50 mL of immunoglobulin. patients with ITP may benefit from blood and does not serve as a depot for intravascular splenectomy. Demonstration ot megakaryocytes in the volume, , leukocytes, or erythrocytes. In the and the absence of splenomegaly are setting of splenomegaly or portal hypertension, the essential for establishing the diagnosis of ITP before storage volume of the spleen expands and formed el- splenectomy is undertaken. Musser reported that after ements of Ihe blood are .sequestered."^ As much as one splenectomy for ITP, up to 77 per cent of patients third of the total mass may be stored in the show a complete response. In addition, 14 per cent of spleen and released during inHammatory states/' patients show a partial response, whereas only 9 per Although active hematopoiesis occurs in the fetal cent failed to demonstrate a significant improvement spleen, it does not normally occur in postnatal life. in platelet count.'"^ Unfortunately, preoperative char- However, in certain pathologic states such as myelo- acteristics do not necessarily predict which patients fibrosis, extramedullary hematopoiesis may indeed with ITP will respond to splenectomy.''^ Failure of take place within the spleen. The splenic white pulp is splenectomy to improve the may bo the largest accumulation of lymphoid tissue in the the result of an unappreciated or in body and is a site of lymphocyte production and acti- traabdominal implantation of splenic tissue should vation, from which cells migrate into the red pulp to fragmentation occur during surgery or extraction alter reach the lumen of the splenic sinusoids. Dendritic a iaparo.scopic approach. Particular attention should be cells and macrophages in the marginal zone are in- paid to the latter, especially during the "morseli/ation" volved with antigen trapping, processing, and presen- process used to facilitate removal while limiting iho tation. Splenic macrophages are particularly adept at size of the incision. recognizing and clearing opsonized bacteria.^ Both Similarly, autoimmune hemolytic may re- dendritic cells^ and T lymphocytes" within the spleen sult in the need for splenectomy if the patient fails to appear to have potent immunologic function. respond to medical management, including oral corti- In addition to its role in the immune system, the costeroids.""' Like with ITP, the pathogenesis of atito- spleen is the site of senescent erythrocyte destruction. immune involves antibody-mediated cellu- Macrophages in the splenic cords phagocytose eryth- lar destruction and complement activation within the rocytes and metabolize . Aged erythro- splenic substance. In patients with warm-reacting an- cytes are more sensitive to the relatively acidotic. hyp- tibodies, favorable responses to spienectomy can be oxic, and hypoglycemic milieu of the spleen. The expected in 50 per cent to 80 per cent.'^ heightened sensitivity of senescent erythrocytes to the hostile splenic environment leads to alterations in Felty 's Syndrome membrane carbohydrate moieties.'" thereby facilitat- ing recognition by macrophages and subsequent cull- Felty's syndrome is the occurrence of neutropenia ing or destruction of damaged cells. In addition to and splenomegaly in patients with rheumatoid arthritis culling, pitting leads to removal of intracellular inclu- (RA). These manifestations are present in less than I sions from erythrocytes. The loss of pitting after sple- per cent of patients with RA. Patients with Felty's nectomy accounts for the appearance of particulate syndrome face an increased risk of infection as a result matter such as Howell-Jolly bodies within erythro- of granulocytopenia. which results, in part, from in- cytes postoperatively. Furthermore, limits trasplenic destruction of granulocytes.'^ Granulocyte- bacterial clearance" and production of IgM and other macrophage colony-stimulating factor may ameliorate opsonin proteins.'^ the neutropenia in some cases.''^ Splenectomy is indi- cated only in patients with severe or recurrent neutro- penia or in tbose patients demonstrating recurrent and Hematologic Conditions resistant .-" Splenectomy results in increased Autoimmune Thrombocytopenia and granulocyte levels in 80 per cent of patients, with 55 per cent of patients experiencing no further infec- The pathogenesis of autoimmune thrombocytopenia tions.-' or idiopathic (ITP) in- volves the formation of antibodies to several antigenic determinants, including glycoproteins llb/IIIa and hi/ Thrombocytopenic Purpura Ila.'"* The spleen is both a site of autoantibody pro- Thrombocytopenic purpura (TTP) presents with the duction and platelet destruction. Although ITP tends to pentad of fever, thrombocytopenia, hemolytic anemia, be a self-limiting disorder in the pediatric population, neurologic manifestations, and renal failure. TTP may No. 7 INDICATIONS FOR SPLENECTOMY Katz and Pachter 567 result from an excess of subendothelial collagen, re- effectuate a decrease in the rate of bemolysis and sub- sulting in systemic platelet trapping. Splenectomy is sequent transfusion requirements.-^ indicated only for patients in whom plasmapheresis Glucose-6-phosphate dehydrogenase deficiency fails or who relapse when this therapy is discontinued. (G6PD) is an X-linked disorder that renders the patient Excellent results may be achieved in refractory susceptible to hemolysis after exposure to certain cases.^-^ Over 50 per cent of patients with TTP who drugs or chemicals such as methylene blue and fava undergo splenectomy may respond favorably.--^ beans. Deficiency of glutathione leads to the destabi- lization of membrane skeletons through Erythrocyte Membrane Disorders oxidative mechanisms.-*^ Splenectomy plays little, if any, role in patients with G6PD deficiency and there- Hereditary (HS) is the most common fore should not be undertaken. The cornerstone of erythrocyte membrane disorder for which splenectomy therapy in this unusual disease remains avoidance of is indicated. An autosomal-dominant results inciting drugs or physiological stressors, particularly in a defective erythrocyte membrane resulting from infections. derangements in and , rendering the cells less deformable and thus susceptible to intra- splenic destruction. As a result of erythrocyte destruc- Hemoglobin apathies tion within the spleen, patients with HS present with In , a single mutation in the beta- anemia. , and splenomegaly. The anemia may hemoglobin chain of Hb A results in rigid, nondeform- be relatively mild with jaundice being the only clinical able erythrocytes that are sequestered and destroyed in manifestation. the spleen. Although the spleen may be enlarged early The role of splenectomy in HS is to treat the under- in life, the organ tends to shrink and autoinfarct as a lying anemia and prevent the development of biliary result of recurring ischemic insults. Likewise, thalas- pathology, aplastic crises, and late hemachromato- semia involves a defect in hemoglobin synthesis and is sis."' Being that erythrocytes are destroyed within the most prevalent among Mediterranean populations, spleen in patients with HS, splenectomy represents a major, the homozygous form, presents in highly effective treatment modality.^'* Splenectomy childhood with failure to thrive, ulcers, and anL'mia. for HS is generally deferred until the patient is 4 to 6 Fetal hemoglobin (Hb F) persists and a relative paucity years old to minimize the risks of OPSI. Splenectomy of adult hemoglobin (Hb A) can be demonstrated. results in correction of the anemia associated with HS The indications for splenectomy in sickle cell dis- and prevents subsequent hemolytic episodes.-^ Re- ease and thalassemia include hypersplenism, seques- sponse rates as high as 90 per cent have been re- tration crises, splenic , and massive splenic ported.''' Hereditary elliptocytosis is generally a non- infarction.-'' Acute splenic sequestration crises are pathologic trait, but when more than 90 per cent of the life-thi-eatening events for which the transfusion re- erythrocytes are affected, the disease manifestations quirement may be substantial and pain may he the may become significant. Indications for and results of most significant component of the overall presenta- splenectomy are similar to those of HS.^"^ tion."**' The features of acute splenic sequestration cri- ses include severe anemia, splenomegaly, and abdomi- Erythrocyte Enzyme Deficiencies nal pain. The patient's hemodynamic status may deteriorate into frank circulatory collapse. These pa- Pyruvate kinase deficiency (PKD) is an autosomal- tients require aggressive resuscitation with crystalloid recessive disorder associated with hemolysis and re- and blood products before urgent splenectomy. In ad- duced red blood cell deformability. Consequently, dition to relieving the acute sequestration crises, sple- erythrocytes are sequestered and destroyed in the nectomy also alleviates mechanical symptoms result- spleen. The severity of disease ranges from fully com- ing from splenomegaly and may improve protein and pensated hemolysis to potentially fatal anemia and energy metabolism in children with sickle cell dis- jaundice in the neonate. Although splenectomy does ease.^' Postoperatively. although these patients benefit not eliminate hemolysis in patients with PKD. it does from decreased transfusion requirements and in- result in increased hemoglobin levels. As a result. creased circulating erythrocyte mass,^^ they are par- transfusion requirements are greatly decreased or even ticularly at high risk for postsplenectomy infections. eliminated.-'' Patients witb PKD may also develop cholelithiasis and associated biliary tract pathology as a result of chronic hemolysis. Therefore, cholecystec- Primary Hypersplenism tomy may be indicated in patients with PKD. In pa- Hypersplenism refers to splenomegaly associated tients with PKD undergoing , con- with a decrease in one or more of the formed blood comitant splenectomy merits serious consideration to elements. Although hypersplenism is most often a sec- 568 THE AMERICAN SURGEON July 2006 Vol. 72 ondary manifestation of an underlying disorder such a.s Non-Hodgkin 's portal hypertension, primary or idiopathic hypersplen- istn may occur. Primary hypersplenism or hypersplen- Splenectomy may be performed for non-Hodgkin"s ism in the absence of a specific disease process was lymphoma (NHL) with either therapeutic or diagnostic diagnosed more frequently in the past as a result of our intent. Indications for splenectomy include relief of limited inability to detect the presence of lymphoma or symptoms resulting from splenic enlargement, cytope- . The sensitivity of modem diagnostic mo- nias. facilitation of chemotherapy administration, and dalities to detect lymphoma or leukemia has improved to establish a tissue diagnosis. In a single study, sple- to the extent that primary hypersplenism is now an nectomy improved cytopenias in up to 72 per cent of uncommon diagnosis. In instances in which hyper- patients and may contribute to durable remission.'" splenism is symptomatic and truly idiopathic. splenec- However, the surgical mortality and morbidity were tomy is indicated to relieve the mechanical symptoms 2.9 per cent and 37 per cent, respectively. When lym- of splenomegaly, effect improvement of cytopenias, phoma primarily involves the spleen, cytopenias are and eliminate the possibility of an underlying malig- frequent and are reversed by early splenectomy in 82 nancy. Lymphoma has been pathologically detected in per cent of cases."*'* Berman and colleagues found that 40 per cent to 70 per cent of patients undergoing sple- in patients with hematologic , splenec- nectomy for presumed primary hypersplenism.-^^- ^"^ tomy resulted in sustained normalization of platelet The frequency of this occurrence, as noted earlier, may levels and thereby decreased the need for platelet in- be significantly lower as a result of advances in diag- fusions."^' nostic imaging and the liberal use of fine-needle aspi- ration witb . Chronic Myelogenous Leukemia Chronic myelogenous leukemia (CML) is a myelo- Neoplastic Conditions Requiring Splenectomy proliferative disease in which the normal bone marrow elements are progressively replaced by neoplastic my- eloid cells. CML is characterized by the Philadelphia Hodgkin 's Disease chromosome, which involves fusion of fragments from chromosomes 9 and 22."^- CML is typically in- The role of splenectomy in the management of pa- dolent in onset with a progressive phase heralded by tients with Hodgkin's iymphoma has undergone sig- fever and splenomegaly. The progressive phase may nificant modifications. In the past, staging laparotomy, culminate in a blast crisis with splenic sequestration of which included splenectomy. was an important com- blood elements contributing to the anemia, infection, ponent in the evaluation of these patients. Detection of and hemorrhagic complications. disease below the diaphragm has important prognostic The primary therapy for CML includes hydroxy- and therapeutic implications. When splenectomy was urea, interferon-alpha. and chemotherapy with bone performed as part of a staging laparotomy for Hodg- marrow transplantation. There is clearly a role for pal- kin's disease, over one third of removed were liative spleneetomy to relieve compressive symptoms found to be involved with Hodgkin's disease. As a and improve cytopenias (Fig. l)."^-* However, splenec- result, patients were upstaged by information obtained tomy does not improve survival in patients with CML, during staging laparotomy.'"^ However, improvements and thromboembolic and vascular events are more in computed tomography, positron emission tomogra- common in patients with CML after splenectomy phy, and magnetic resonance imaging techniques have when compared with those managed nonoperatively."*"^ limited the need for staging laparotomy.^^ Moreover, Therefore, a heightened suspicion for thrombotic com- given improved toxicity profiles of chemotherapeutic plications is appropriate when splenectomy is per- agents, patients with a high likelihood of stage 111 or formed in patients with CML and this concern is dis- IV disease may undergo systemic therapy without be- cussed in greater detail subsequently. ing subjected to staging laparotomy. Yet. patients with early disease and equivocal radiologie findings may still be referred for operative staging. Surgical staging Chronic Lymphocytic Leukemia should be limited to patients with stage I or II disease Splenectomy is performed for complications of chron- in whom a negative result may permit withholding ic lymphocytic leukemia (CLL). including hypersplen- chemotherapy after radiation therapy.^^ Staging lapa- ism and the mechanical effects of splenomegaly. rotomy is now performed, at most, in 30 per cent of Anemia and thrombocytopenia may be significantly patient's with Hodgkin's disease. When surgery is re- improved in patients with CLL after splenectomy. quired, the laparoscopic approach is used with increas- Moreover, in contrast to CML, in certain subgroups of ing frequency.^^ patients such as those with hemoglobin levels No. 7 INDICATIONS FOR SPLENECTOMY Katz and Pachter 569

A. C.

B.

FIG. 1. Spleen iiwoived with chronic myeiogenous leukemia (CML). This case il!u?,lrales [lie marked degree ot splenomegaly that may occur in patients with CML. Sptenecloniy was indicated in this case for relief of compressive sympionis. (A) Alter mobiii/ation of the spleen, it was delivered through a left upper quadrant incision and then the splenectomy was completed (B). A preoperative computed tomography scan demonstr"ates the degree of splenomegaly and how this may lead to compressive symploins (C). less than 10 g/dL or platelet counts less than 50.000/L. tion or immunologic function."*^ Splenic tumors most splenectomy has been shown to improve long-term commonly present with splenomegaly and diffuse pa- survival."*^ renchymal replacement may result in hematologic consequences. Moreover, a pathologic spleen is at risk Hain' Cell Leukemia for either spontaneous rupture or rupture witb the slightest degree of external trauma, resulting in an in- (HCL), formerly known as traabdominal catastrophe. "surgical leukemia," is a rare, chronic lymphoid leu- The most common primary tumors of the spleen are kemia that generally affects the elderly with spleno- vascular in nature.'*^ Benign primary vascular tumors megaly and pancytopenia. Neoplastic mononuclear of the spleen include and lymphangio- cells with distinctive cytologic features are present in mas. Both tumors are typically found incidtMitally. the peripheral blood and bone marrow. Although a Hemangiomas are typically of the cavernous type and minority of patients present with asymptomatic rarely rupture. On occasion, splenic hemangiomas are splenomegaly, most will require therapy to address responsible for a consumptive coagulopathy, which manifestations of anemia, neutropenia, or thrombocy- may necessitate extirpation of the spleen because Ibe topenia. Historically, splenectomy played a definitive process can be fatal.''" Lymphangiomas are endotbe- role in the treatment of patients with HCL."*^ At pres- lial-lined lesions filled with proteinaceous material. ent, splenectomy is infrequently performed for HCL Both hemangiomas and lymphangiomas may be single because interferon-a, and more recently, purine ana- or multiple.^' Littoral cell angiomas may present as logs have proven to be highly effective in the man- single or multiple lesions.-''^ Splenic hamartomas are agement of this type of leukemia.**"^ well-circumscribed, nonencapsulated benign prolifera- tions of nonnal splenic tissue elements."**^ Although Primary and Metastatic Tumors most commonly an incidental finding at autopsy or on The spleen is a relatively infrequent site of metas- abdominal imaging, diffuse splenic hamartomas may tases of nonhematologic malignancies, and this may result in hypersplenism. In addition, splenic hamarto- be related to peculiarities of the splenic microcircula- mas may be the cause of intraabdominal hemorrhage 570 THE AMERICAN SURGEON July 2006 Vol. 72

after rupture.^-^ Rarely, the spleen gives rise to lipo- mas'^'' and .'^-'^ Hemangiosarcomas (Fig. 2) are the most common primary malignant tumor of the spleen and have re- ceived attention disproportionate to their incidence given the association with environmental factors such as vinyl chloride and thorium dioxide.'^'' Splenic he- mangiosarcomas may present with splenomegaly, ane- mia, pleura! effusion, or spontaneous rupture. The prognosis is generally poor irrespective of the type of treatment rendered. Other such as Kaposi's arise within the spleen infrequently. When the spleen is the only site of disease or when splenic involvement by primary or metastatic sarcoma leads to symptoms, splenectomy would appear to be a reason- able option. Various lymphoid processes may involve the spleen FKI. 3. Priniaiy splenic lymphoma. The patient presented willi primarily. Although the spleen is more often a second- symptoms related to splenomegaly and hypersplenism. This ab- ary site of involvement in Hodgkin's and non- dominal computed tomt>graphy scan with intravenous contrast Hodgkin's lymphoma, both may arise from the splenic demonstrated a low-altenuating lesion in the spleen. After sple- white pulp. Primary lymphoma of the spleen (Fig. 3) nectomy and a search for synchronous sites ot" disease, the diag- nosis of primary splenic lymphoma was made. may replace the organ diffusely or present as a mui- tinodular process.^^ The spleen nnay also be affected by histiocytic tumors and angiofollicular lymphoid hy- hypersplenism. Meta.stases to the spleen are ustially an perplasia or Castleman's disease.*^" indicator of .systemic disease and aggressive lumor The spleen may be involved by metastatic disease in biology, rendering splenectomy with curative inteni up to 7 per cent of patients with malignant solid tu- generally inappropriate in these settings (Fig. 4).^" mors.-^^ Among the tumors thai metastasize to the Like with any tumor involving the spleen, rupture or spleen, , adenocarcinoma of the breast, and formation may oceur and spienectomy may be lung are most common. When the spleen is required on these bases. involved with metastatic disease, it is rarely the sole site of disease and therefore the role of spienectomy is generally reserved for palliation of splenomegaly or

Fi(i. 2. Hemangiosarcoma of the spleen. Computed tomogra- phy scan of ihc with intravenous contrast demonstrates a mass within the spleen with areas of hypoattenuation and enhance- FIG. 4. Melanoma meiastatic to the spleen. Several years after ment. The areas of enhancement are typical for vascular tumors the excision of a cutaneous melanoma, this patient was found to such as hemangiosarcoma. The specitnen measured approximately have numerous low-attenuating lesions in the spleen and liver 8 cm in diameter and was completely resected. (arrows). These lesions were consistent with metastatic melanoma. No. 7 INDICATIONS FOR SPLENECTOMY Katz and Pachter 571

Benign Conditions Requiring Splenectomy Splenic Cysts

Gaucher's Disease Cystic lesions of the spleen may be divided into primary or true cysts and secondary or false cysts. Gaucher's disease is an autosomal-recessive disor- True splenic cysts are parasitic, congenital, or neoplas- der in which glucocerebrosidase deficiency results in tic and typically have an epithelial lining. In contrast, the systemic accumulation of glucocerebrosides, false cysts lack a true epithelial lining and are most which may be accompanied by hepatosplenomegaly, anemia, thrombocytopenia, atid skeletal manifesta- commonly a consequence of trauma. Splenic cysts tions. The spleen may be enlarged 60 to 70 times its may cause , become secondarily in- normal size and 10 to 20 times its normal weight, and fected, or rupture. The appropriate management of the degree of splenomegaly may correlate with disease splenic cysts depends on an understanding of their progression.*'" Enzyme replacement therapy may re- derivation and natural history. sult in hematologic improvement and reduction in or- Primary splenic cysts may originate as embryologic ganomegaly and bone pain for as long as 5 years.*^' mesothelial inclusions*'-^ or represent a neoplastic pro- Before enzyme replacement therapy, most patients cess in which the squamous epithelial lining may be with Gaucher's disease required splenectomy. Sple- keratinized/'^ However, on a global basis, parasitic nectotny is now reserved for persistetit pancytopenias cysts are far more common than nonparasitic cysts of or pain, which may result from massive splenic ex- the spleen."^^ Although splenectotny may be necessary pansion with associated infarcts. However, total sple- in the setting of excessively large cysts, splenic pres- nectomy may promote accumulation of glucocerebro- ervation is often possible. Cyst enucleation, partial sides in other reticuloendothelial sites. Therefore, cystectomy, and partial splenectomy have proven to be partial spienectomy is a reasonable option to limit the technically feasible. Splenic preservation may be pos- effects of hypersplenism and splenomegaly while sible in over 40 per cent of patients with echinococcal minimizing the infectious and tnetabolic conse- disease with recurrence tates comparable to those hav- quences 62 ing undergone splenectomy.^^' False cysts or pseudocysts of the spleen most com- monly arise after trauma and account for the majority Wiskott-Aldrich Syndrome of nonparasitic splenic cysts.^"^ Interestingly, only 30 Wiskott-Aldrich syndrome (WAS) is an X-linked per cent of such patients can recall a traumatic event. associated with autoimmune and Splenic pseudocysts likely result from ihc formation of inflammatory sequelae, including hemolytic anemia, an inflammatory fibrous capsule surrounding the he- thrombocytopenia. neutropenia, arthritis, va.sculitis. matoma, which ultimately resorbs. A calcified rim is and eczema.''' Deranged natural killer T cell (NKT) often present around the cyst (Fig. 5j and, in the ab- function, which is known to possess immunoregulato- ry properties, may in part account for the autoimmune manifestations.'^ Bone marrow transplantation is the only definitive therapy for WAS but in some centers is reserved only for those with life-threatening compli- cations.*^ Although the hematologic manifestations of WAS may respond to treatment with corticosteroids. immunosuppressive agents, or intravenous immuno- globulin. splenectomy is indicated for persistent thrombocytopenia.^"* f Chediak-Higashi Syndrome Chediak-Higashi syndrome (CHS) is an autosomal- recessive immunodeficiency disorder involving defec- tive leukocyte chemotaxis and phagocytosis. CHS may degenerate into an accelerated phase involving FiG. 5. Splenic pseudocyst. Several months after blunt ab- pancytopenia and cellular infiltration of the spleen.'^'^^ dominal trauma, this patient presented with left upper quadrant Splenectomy may effectively address manifestations pain. A cotiiputcd tomography scan of the abdomen demonstrating a large, well-defined cystic lesion of the spleen with a calcified rim of hypersptenism when they are unresponsive to other (arrow). These imaging features are typical for a splenic pseudo- modalities, including corticosteroids. cyst. 572 THE AMERICAN SURGEON July 2006 Vol. 72

sence of documented parasitic disease, is almost al- risk of secondarily infecting the lesion is not insignifi- ways indicative of a trauma. Small pseudocysts (<4 cant. cm) tend to regress and should be observed in the absence of specific symptoms. Symptomatic pseudo- cysts or those greater than 5 cm should be treated Splenic Abscesses surgically. If possible, preservation of a portion of the Perhaps owing to the potent immunologic capacity spleen shouid be attempted. In this regard, surgical of resident .splenic leukocytes.''- ^^ abscesses are rarely options include cystectomy without splenectomy, par- encountered in the spleen. The predominant manifes- tial splenectomy, or "decapsulization" of the cyst with tations are systemic such as ieukocytosis and fever. or without omentopexy. Any of the aforementioned Few patients present with localized symptoms. Splenic options may be approached traditionally or laparo- abscesses are unilocular in 65 per cent, multilocular in scopically. It has been our experience that splenic 8 per cent, and multiple in 27 per cent of cases/"** The pseudocysts >6 cm, especially those involving the me- overall mortality rate is 13 per cent."^" Like with other dial portion of the spleen, have a tendency to recur splenic lesions, abscesses may rupture with the poten- when unroofed laparoscopically despite excising a tial for hemorrhage and disseminated peritonitis. Im- large portion of the cyst wall. In our opinion, these munodeficiency is a common underlying factor and pseudocysts are best managed by either partial splenic predisposes to fungal abscesses (Fig. 8). Other poten- resection (Fig. 6). Occasionally, total splenectomy tial causes include endocarditis, intravenous drug may be required {Fig. 7). Although percutaneous abu.se, pyogenic infections at distant sites, sickle cell drainage of splenic pseudocysts has been described, anemia, and secondary infection of a traumatic pseu- the effectiveness of this approach is uncertain and the docyst.''

B.

D,

Fic. 6. Excision of a splenic pseudocyst. (A) Computed tomography scan of the abdomen demonstrating ;t large, well-delined cystic lesion of the spleen, which was found several weeks after blunt trauma. (B) The border between the pseudocyst and normal splenic parenchyma was identified (arrow). A cystectomy was pertbrmed. and the (C) intact pseudocyst and (D) resid'ual spleen iu:c demon- strated. Note that the greaier omentum Is generally conveniently situated for use in eliminating the cavity in the spleen after cyst excision. No. 7 INDICATIONS FOR SPLENECTOMY Katz and Pachter 573

FiG. 7. Total splenectomy performed for pseudocyst. At times, splenic preservation is not possible given the si/e or location of a pseudocyst. In this case, splenectomy was performed for the treatment of this symptomatic pseudocyst. Note that the normal splenic parenchyma was markedly compressed (arrows) as a consequence of the pseudocyst (A and B). _

multilocular abscess is encountered. Under these con- ditions, splenectomy would appear to be the most ef- fective definitive therapy.

Wandering Spleen and Splenic Torsion Failure of the dorsal mesogastrium to fuse lo the posterior abdominal wall may result in the absence of the spleen's normal peritoneal attachments. The lack of normal attachments such as the splenorenal and gastrosplenic ligaments predisposes the spleen to tor- sion and ischemia. Acquired factors such as the hor- monal milieu of pregnancy may play a role as well.^- Although most patients are asymptomatic, splenic tor- sion may manifest as recurrent bouts of abdominal pain. Persistent pain with a palpable, mobile mass sug- gests ischemia and possible infarction. The diagnosis may be reached by noting absence of intravenous con- trast uptake within the spleen on computed tomogra- phy scanning. In the event of torsion with infarction, . , . , , splenectomy is FIG. 8. Fungal abscesses ot the spleen. Multiple low- attenuating lesions of the spleen representing abscesses resulting from Caiiduhi alhicans. In this case, drainage was not possible as Trctuma a result of the number of abscesses. The patient was treated with systemic antifungal agents and splenectomy was avoided. Although splenectotny is often necessary after trau- matic injury, nonoperative management of blunt Intervention, in some form, is mandatory for splenic in the stable patient is currently the stan- abscesses because the mortality rate for untreated dard of care.'''* Spontaneous healing results in ana- cases approaches 100 per cent. Initial management totnic and functional restoration of the spleen.' Suc- should be nonoperative, and image-guided percutane- cessful nonoperative management is also possible in ous is particularly effective when the abscess is soli- patients with intrinsic splenic pathology. Eleven pa- tary and thick-walled.^' If appropriate antibiotics have tients with HIV. acute leukemia, infectious mono- been administered in conjunction with percutaneous nucleosis, or sickle cell anemia were successfully drainage in patients with unilocular abscesses and the managed without surgical intervention after splenic clinical picture fails to improve within 48 hours, sple- injury, demonstrating their ability to heal parenchymal nectomy should be undertaken without delay. Percu- disruption (Fig. 9).'^^ However, in the setting of ongo- taneous drainage is less likely to be effective when a ing hemorrhage, hemodynamic instability, or con- 574 THE AMERICAN SURGEON July 2006 Vol. 72

t-Ki. y. SplcniL- rupitnc in (he setting of HIV infeciion. The spleen undergoes pathologic changes in the setting o\' HIV infection. In this HIV-positive paiient. a signihcant splenic injury was inctirred after a motor vehicle accident. The splenic injury (A) was managed nonoperatively and a computed tomography scan I month after the injury reveals nearly complete healing. comitant intraabdominal injury, splenectomy should injuries caused during left hemicolectomies. antireflux be undertaken without unnecessary delay.^^ To procedures, and left nephrectomies.^^ With techniqties achieve splenic preservation, a multidisciplinary ap- described in this article, the overwhelming majority of proach may be necessary. Adjunctive splenic emboli- these injuries can be managed without resorting sple- zation may increase the chances for successful nonop- nectomy. Although preservation of splenic paren- erative management in appropriate patients.^-" When chyma should be attempted, splenectomy is the pro- operative intervention is required, splenorrhaphy cedure of choice in the setting of excessive blood loss. should be considered depending in the stability of the patient (Fig. 10). Surgical Therapy latrogenic Injuries Medical therapy may be inadequate or, at times, inapptopriate in some patients with diseases involving Of note, up to 40 per cent of all splenectomies per- the spleen. Patients may experience persistent abdomi- fomied in the United States are a result of iatrogenic nal pain, left shoulder pain, or early satiety. Massively

_ FIG. 10. Spicnorrhaphy splenic preservation alter iniiimatic injury lA) to the s|>kvn is o!k-n possible and is desirable to ;noiil ilic risk of overwhelming postspleneciomy infection with its associated high monality rate. In this patient, suture splenorrhaphy was performed with the edges of the injured splenic parenchyma reapproximated, whereas compression of the intervening tissue achieved hemostasis (B). No. 7 INDICATIONS FOR SPLENECTOMY Katz and Pachter 575 enlarged spleens may also cause an alteration of bowel itiiportant not only to reduce the size of the spleen habits or urinary frequency resulting from bladder when it is enlarged, but it also allows for the safe compression. Nonoperative interventions may also fail transfusion of platelets without the fear that they will to control anemia, thrombocytopenia. or neutropenia. be rapidly consumed by the spleen. As the spleen de- leaving the patient susceptible to a variety of second- compresses and shrinks in size, further manipulation is ary complications. In some instances, splenectomy facilitated and the risk of iatrogenic is significantly tnay also be necessary to reach a diagnosis such as in reduced. Moreover, maintaining the integrity of the cases of idiopathic hypersplenisrn. As noted earlier, splenic capsule is advisable for both oncologic and rnany of these patients harbor occult and hemostatic reasons to minimize the chance of tumor therefore pathologic analysis of the splenic paren- dissemination or bleeding from the parenchyma. chytna is important when a cause for splenomegaly is not apparent. The importance of the aforementioned statement cannot be overemphasized, especially if a Minimally Invasive Splenectomy tiiinimally invasive approach is used. Under these cir- Laparoscopic splenectomy (LS) was initially de- cumstances, the extraction site should be large enough scribed in 1991.''' Because of a constellation of fac- so that adequate splenic parenchymal tissue is pre- tors, including the advent of ultrasonic dissectors, im- served for pathologic analysis. proved optical technology, and use of endoscopic stapling devices, laparoscopic splenectomy is cur- Open Splenectomy rently the preferred approach for benign splenic dis- eases.^' The conversion rate for laparoscopic splenec- After the decision to perform splenectomy on a pa- tomy performed for has been tient, the surgeon must ensure that the patient has re- reported to be 5 per cent.^' Although LS is associated ceived the appropriate immunizations and that hema- with increased operative titnes. the duration of hospital tologic deficiencies have been sufficiently corrected. stays are shorter and splenectomy-related morbidity is Patients should be immunized against pneumococcus. decreased.'^^ Specifically, when splenectomy was per- Haemoplnliis infhienzae type B. and group C menin- formed for hematologic disease, the laparoscopic ap- gococci.''^^ vaccination may be considered as proach resulted in earlier initiation of oral feeding, well. In addition, all patients should be prepared in earlier discharge, fewer blood transfusions, and a de- anticipation of open splenectomy given the chance of creased analgesia requirement.^^ conversion even if a laparoscopic approach is initially Patients with ttialignant disease requiring splenec- undertaken. totny may have longer operative times and more blood When performing open splenectomy (OS), adher- loss during LS.**' This is most likely related to the ence to sound principles of splenic surgery will maxi- higher splenic volume typically presetit in patients mize the chance of a successful outcome. Moreover, with malignant disease in contrast to the benign pro- most of the concepts used in performing open sple- cesses such as ITP. Despite the increased operative nectomy are applicable to the laparoscopic approach. time and blood loss, when LS is performed in the The major pitfalls of splenectomy include hemor- setting of splenomegaly, there were no statistically dif- rhage, injury to the greater curvature of the stomach, ferences in conversion rates, titne to discharge, or tiior- pancreatic injury, failure to detect accessory spleens, bidity.**"* Splenomegaly cannot be considered an abso- and iatrogenic rupture of the spleen with subsequent lute contraindication to a minimally invasive approach implantation of splenic tissue within the peritoneal but warrants caretul patient selection and preoperative cavity. Ensuring complete hemostasis in the bed of the planning.'^^ Hand-assisted may be of ad- spleen at the conclusion of the operation is crucial. ditional benefit when performing splenectomy in pa- Areas deserving careful attention include the tail of the tients with splenomegaly and allow for removal of the pancreas, the left adrenal gland, the posterior abdomi- intact organ when a histologic diagnosis is required.*^^ nal wall, and the left diaphragmatic surface. Diffuse One criticism of the laparoscopic approach to sple- oozing may require the administration of blood prod- nectomy has been the lack of tactile feedback and ucts, depending on the patient's underlying pathologic perhaps an impaired ability to identify accessory condition. To avoid pancreatic injury, the surgeon spleens. The incidence of accessory spleens is ap- should clearly identify the pancreatic tail when man- proximately 15 per cent.^^ Failure to detect and ablate aging the vasculature at the splenic hilum. In addition, accessory splenic tissue tnay lead to treatment failure the short gastric vessels tnust be defined and ligated in cases in which the spleen is responsible for the with precision to avoid injury to the stomach and a destruction of platelets, erythtocytes. or leukocytes. potential gastric fistula.^"' These concerns, however, have not proven to be a Early control and ligation of the arterial inflow is significant itnpediment. Like in all of laparoscopic 576 THE AMERICAN SURGEON July 2006 Vol. 72 surgery, lack of tactile sense is compensated for by risk for subsequent infectious complications.'-* In ad- experience. Furthermore, laparoscopy may instead fa- dition to commonly recognized postoperative compli- cilitate detection of accessory splenic tissue because cations such as wound infection, , and sep- visualization is usually better than with an open ap- sis, patients may develop a pancreatic or gastric proach. In a large meta-analysis. the rate of accessory fistula. When splenectomy is performed for hemato- spleen identification was similar among patients un- logic disease, particular attention must be paid to the dergoing LS and OS.**^ blood and platelet coutits in the perioperative period. Splenectomy tnay also be associated with an el- Splenic-Preserving Techniques evated risk for adverse cardiovascular events. Patients who undergo splenectomy for Partial splenectomy may be appropriate for treat- may experience a higher incidence of tnyocardial in- ment for certain benign conditions in which residual farction, stroke.*^' and pultnonary hypertension.'*- In splenic tissue will not have undue hematologic or on- addition, splenectomy places patients at risk for other cologic consequences. In most situations, preservation thromboembolic cotnplications^'' such as splenic or of sufficient normal splenic tissue lowers the risks of portal vein thrombosis. Thrombotic prophylaxis with infectious complications. Between 30 per cent and 50 heparin products"^ and screening Doppler sonogra- per cent of splenic tissue should be preserved along phy"*"*^ should be considered in high-risk patients. with an identifiable blood supply to preserve mean- When screened with ultrasonography. poiial or splenic ingful splenic function.' Subtotal splenectomy per- vein throtnbosis occurred in 55 per cent of patients formed in children for hereditary spherocytosis can after LS and 19 per cent after OS.'^'' Furthermore, pa- result in a prolonged decrease in hemolysis while of- tietits with fever or abdotninal pain after splenectomy fering the benefit of retained reticuloendothelial func- should be suspected of having portal and splenic vein tion in the splenic remnant.^** As discussed earlier, thrombosis should be treated with anticoagulation splenic cysts have been treated by partial excision of therapy if this condition is documented.**** the cyst wall or with "unroofing" procedures, and pa- tients with Gaucher's disease may benefit from partial An additional clinical manifestation of splenectomy splenectomy. Partial cystectomy with omentopexy has may be thrombocytosis. Theoretically, thrombocytosis been used in the management of hydatid disease**'^ and after splenectomy may increase blood viscosity and traumatic pseudocysts have been effectively treated create a more thrombogenic milieu. However, the re- with subtotal splenectomy.''^ Approaches to splenic lationship between postsplenectomy thrombocytosis preservation in the setting of trauma include the use of and venous thrombosis is unclear.'**' The use of aspirin suture splenorrhaphy. mesh splenorrhaphy. fibrin glue, to treat postsplenectotny thrombocytosis must be con- and argon beam laser coagulation.' sidered on an individual basis taking into account the overall thrombotic diathesis, particularly the presence of an underlying myeloproliferative process. Most sur- Splenic Bed Drainage geons initiate antiplatelet or antithrombotic therapy at Drainage of the splenic bed remains a controversial a platelet count greater than 750.00/tnL.'" Despite tho issue. The theoretic rationale for drainage of the potential for these complications, splenectomy can be splenic bed after splenectomy includes the removal of performed safely in the vast majority of patients with accumulated fluid, which could become a nidus for benign and tnalignant disease who may benefit from infection. Yet, it is uncertain if drainage of the splenic removal of the spleen. bed is of any significant benefit. Associated gastroin- testinal injury and duration of drainage seem to be of LiUe Morbidity greater significance in terms of postoperative morbid- ity than the presence or absence of drains."" If drains The immunologic effects of spienectomy include are to be used, then the closed suction variety such as impaired responsiveness to new antigens, itnpaired a Jackson-Pratt should be used. To minimize infec- phagocytic clearance of opsonized or unopsonized tious complication, drains should be removed as bacteria, and impaired production of the opsonin pro- quickly as possible. teins tuftsin and properdin.'**^ Therefore, patietits hav- ing undergone splenectomy are at lifelong risk from Postsplenectomy Complications bacterial . Encapsulated bacteria such as Streptococcus pneu- Early Morbidity moniae are the most common pathogens involved in After splenectomy for hematologic conditions, the postsplenectomy sepsis.'*'^ The precise incidence of postoperative complication rate may reach 24 per cent. OPSI is not known because published reports vary Patients having received corticosteroids are at highest considerably, which is in part the result of variations in No. 7 INDICATIONS FOR SPLENECTOMY Katz and Pachter 577 the study populations and definitions of OPSL The should receive immediate broad-spectrum intravenous annual incidence of OPSI has been reported to be as antibiotic coverage subsequent to the withdrawal of high as 0.23 per cent to 0.42 per cent'"" per year with blood samples for culture. Bacteremia can be demon- a lifetime risk being 5 per cent.^** In contrast. CuUing- strated in nearly all cases of OPSI and bacteria are ford repotted the incidence of OPSI to be 0.04 per 100 often present on a peripheral smear of the patient's person-years.'"' Ahhough it is difficult to determine blood.^'^ Although intravenous immunoglobulin is the precise incidence for OPSI, it is clear that its true theoretically appealing, evidence for its routine use is occurrence has been often overstated. lacking.

The clinician must assess the individual patient's I I tisk based on factors including the time interval from , Summary splenectomy. age, and underlyitig medical conditions. The risk of infection is highest in the first 2 years after The spleen is an important component of the im- splenectomy.'"- although it is unclear whether the risk mune and hematologic systems. When the spleen is of frank OPSI declines over time. '"^ The risk of OPSI responsible for or involved with pathologic processes, is elevated in children, in those with underlying he- or when splenomegaly produces significant symp- matologic or malignant processes, and those otherwise toms, operative therapy may be indicated. Given the immunosuppressed.'"-^ Moreover, in patients with potenfial complications of splenectomy, including thaiassemia major and sickle cell anemia, the inci- OPSI, preservation of a critical mass of splenic paren- dence of infection and associated mortality is notably chyma should be attempted when possible. In in- higher.'"^ Less controversial is the lethal nature or stances in which splenectomy is indicated, the laparo- OPSI. After the occurrence of OPSI, the mortality scopic approach may offer significant benefits. A rates reported to range from 38 per cent to 69 per sound understanding of the underlying pathophysiol- cent.''" Therefore, the primary fear with OPSI may lie ogy of the process necessitating splenectomy and an- in its lethality rather than the frequency with which ticipation of the potential postsplenectomy complica- this complication occurs. tions will optimize the likelihood of successful clinical outcomes. Several strategies to reduce the risk of OPSI have been used. Preservation of splenic tissue after trauma or utilization of splenic-sparing surgical techniques REFERENCES when possible should prevent OPSI when an adequate 1. Pachter HL, Grau J. The current status of splenic preserva- volume of parenchyma remains. Ideally, to maintain tion. Adv Surg 2000;34:L^7-74. splenic immunoiogic function, at least 30 per cent of 2. Larsen W. Htiman Embryology. New York: Churchill Liv- splenic parenchyma attached to an identifiable blood ingstone; 1993. source should be preserved. Although autotrans- 3. Groom AC. The Microcirculatory Society Eugene M. Landis planted splenic tissue has been shown to be both viable award lecture. Microcirculation of the spleen: new concepts, new and functional, the true clinical impact of this proce- challenges. Microvasc Res 1987:34:269-89. dure is uncertain^^ because the response to a specific 4. Katz SC. Piilarisetty VG. Bleier Jl. et al. Liver sinusoidal bacterial antigenic challenge in humans is presently endothelial cells are insufficient to activate T cells. J Immunol 2OO4;173:23O-5. unknown. However, given its conceptual appeal and 5. Jandl JH. Aster RH. Increased splenic pooling and the patho- low risk of complications, autotransplantation of genesis of hypersplenism. Am J Med Sci l967;253:383-98. splenic tissue, if feasible, should be attempted. 6. Aster RH. Pooling of platelets in the spleen: rule in the If the entire spleen must be removed, the patient pathogenesis of 'hypersplenic' thrombocytopenia. J Clin Invest should be immunized with the Pneumovax vaccine 1966:45:645-57. against S. pneumoniae. Ideally, vaccinations should be 7. Jandl JH. Greenberg MS. Yonemoto RH. Castle WB. Clini- given before splenectomy because the humoral re- cal determination of the sites of red cell sequestration in hemolytic sponse may be better with the spleen intact. Booster . J Clin Invest 1956;35:842-67. immunization should be given at 3- to 5-year inter- 8. Piilarisetty VG. Shah AB. Miller G, et al. Liver dendritic vals.'^ Vaccination against Haemophilus influenzae cells are less immunogenic than spleen dendritic ceils because of type b. Neisseha meningitidis type C. and influenza is differences in subtype composition. J Immunol 2004;I72: generally recointnended as well. Ahhough prophylac- 1009-17. tic antibiotics should be given in the event of any 9. Katz SC, Piilarisetty VG, Bleier JL et al. Conventional liver CD4 T cells are functionally distinct and suppressed by environ- dental or invasive medical procedure, routine antibi- mental factors. Hepatology 2005:42:293-300. otic administration should be limited to 5 years in 10. Lux SE, John KM. Isolation and partial characterization of children and 2 years in adults after splenectomy.'"^ a high molecular weight red cell membrane protein complex nor- Any sign of infection in an asplenic individual must mally removed by the spleen. Blood 1977;50:625-4L be considered a true medical emergency. The patient 11. Brown El. Hosea SW, Frank MM. The role of the spleen in 578 THE AMERICAN SURGEON July 2006 Vol. 72

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