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Surgery_Schwartz. insoluble red blood cells that cannot release oxygen normally and may precipitate with cell aging. Both underproduction of hemoglobin and excess production of unpaired hemoglobin subunits contribute to thalassemia-associated morbidity and mortality.A diagnosis of thalassemia major (homozygous form) is made by demonstrating hypochromic microcytic anemia asso-ciated with randomly distorted red blood cells and nucleated erythrocytes (target cells) on peripheral blood smear.7 Elevated reticulocyte count and white blood cell count are among the associated findings. Because α chains are needed to form both fetal hemoglobin and adult hemoglobin, α-thalassemia becomes symptomatic in utero or at birth. By contrast, β-thalassemia becomes symptomatic at 4 to 6 months because β chains are involved only in adult hemoglobin synthesis.The clinical spectrum of the thalassemias is wide. Heterozygous carriers of the disease are usually asymptomatic. Homozygous individuals, on the other hand, typically


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Surgery_Schwartz. in adult hemoglobin synthesis.The clinical spectrum of the thalassemias is wide. Heterozygous carriers of the disease are usually asymptomatic. Homozygous individuals, on the other hand, typically present before 2 years of age with pallor, growth retardation, jaundice, and abdominal swelling due to liver and spleen enlargement. Among other characteristics of thalassemia major are intractable leg ulcers, head enlargement, frequent infections, and the need for periodic blood transfusions. Untreated individuals usually die in late infancy or early childhood from severe anemia.7Treatment for thalassemia involves red blood cell transfu-sions to maintain a hemoglobin level of >9 mg/dL, along with Brunicardi_Ch34_p1517-p1548.indd 152523/02/19 2:36 PM 1526SPECIFIC CONSIDERATIONSPART IIintensive parenteral chelation therapy with deferoxamine. Sple-nectomy is indicated for patients with excessive transfusion requirements (>200 mL/kg per year), discomfort due to spleno-megaly, or painful


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Surgery_Schwartz. parenteral chelation therapy with deferoxamine. Sple-nectomy is indicated for patients with excessive transfusion requirements (>200 mL/kg per year), discomfort due to spleno-megaly, or painful splenic infarction. Careful assessment of the risk-benefit ratio is essential. Thalassemia patients are at high risk for pulmonary hypertension after splenectomy; however, the precise pathophysiology of this sequela remains unclear.45,46 The increase in infectious complications is likely to be due to a coexisting immune deficiency, in large part brought about by iron overload, which may be associated both with the thalas-semia itself and with transfusions. The disproportionately high rate of overwhelming postsplenectomy infection in thalassemia patients has led some investigators to consider partial splenec-tomy in children; some success in reducing mortality has been reported.47 However, splenectomy should be delayed until after the age of 4 years unless it is absolutely necessary.Platelet


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Surgery_Schwartz. splenec-tomy in children; some success in reducing mortality has been reported.47 However, splenectomy should be delayed until after the age of 4 years unless it is absolutely necessary.Platelet Disorders Idiopathic Thrombocytopenic Purpura Idiopathic thrombo-cytopenic purpura (ITP), also called immune thrombocytopenic purpura, is an autoimmune disorder characterized by a low platelet count and mucocutaneous and petechial bleeding. The low platelet count stems from premature removal of platelets opsonized by antiplatelet immunoglobulin G autoantibodies produced in the spleen. This clearance occurs through the inter-action of platelet autoantibodies with Fc receptors expressed on tissue macrophages, predominantly in the spleen and liver. The estimated incidence of ITP is 100 persons per million annually, about one-half of whom are children.48 Adult-onset and child-hood-onset ITP are strikingly different in their clinical course and management.Patients with ITP typically present with


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Surgery_Schwartz. million annually, about one-half of whom are children.48 Adult-onset and child-hood-onset ITP are strikingly different in their clinical course and management.Patients with ITP typically present with petechiae or ecchymoses, although some experience major bleeding from the outset. Bleeding may occur from mucosal surfaces in the form of gingival bleeding, epistaxis, menorrhagia, hematuria, or even melena. The severity of bleeding frequently corresponds to the deficiency in platelets: Patients with counts greater than 50,000/mm3 usually present with incidental findings; those with counts between 30,000 and 50,000/mm3 often have easy bruis-ing; those with platelet counts between 10,000 and 30,000/mm3 may develop spontaneous petechiae or ecchymoses; and those with counts less than 10,000/mm3 are at risk for internal bleeding.48 The incidence of major intracranial hemorrhage is approximately 1%, and it usually occurs early in the disease course. The duration of the bleeding helps to


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Surgery_Schwartz. are at risk for internal bleeding.48 The incidence of major intracranial hemorrhage is approximately 1%, and it usually occurs early in the disease course. The duration of the bleeding helps to distinguish acute from chronic forms of ITP. Children often present at a young age (peak age of approximately 5 years) with sudden onset of petechiae or purpura several days to weeks after an infectious illness. In contrast, adults experience a more chronic form of disease with an insidious onset. Splenomegaly is uncommon with ITP in both adults and children, and its occurrence should prompt a search for a separate cause of thrombocytopenia. Up to 10% of children, however, have a palpable spleen tip.Diagnosis of ITP is based on exclusion of other possibili-ties in the presence of a low platelet count and mucocutane-ous bleeding. Other diseases resulting in secondary forms of ITP, such as systemic lupus erythematosus, antiphospholipid syndrome, lymphoproliferative disorders, human


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Surgery_Schwartz. platelet count and mucocutane-ous bleeding. Other diseases resulting in secondary forms of ITP, such as systemic lupus erythematosus, antiphospholipid syndrome, lymphoproliferative disorders, human immunodefi-ciency virus (HIV) infection, and hepatitis C, should be identi-fied and treated when present. In addition, any history of use of a drug known to cause thrombocytopenia, such as certain antimicrobials, anti-inflammatories, antihypertensives, and 5Table 34-2Second-line treatment options for immune thrombocytopeniaSplenectomyAdvantages• Effective, time-honored treatment• Most effective response (overall 88%, partial 22%, complete 66%)Disadvantages• Risk of overwhelming postsplenectomy infection• Increased risk of thrombotic events• Morbidity and mortality associated with an operationRituximabAdvantages• Nonsurgical• Good experience since 1999• Initial immediate response 63%• 31% response at 2 yearsDisadvantages• Severe toxicity in 2%–6%Thrombopoietin Receptor


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Surgery_Schwartz. with an operationRituximabAdvantages• Nonsurgical• Good experience since 1999• Initial immediate response 63%• 31% response at 2 yearsDisadvantages• Severe toxicity in 2%–6%Thrombopoietin Receptor AgonistAdvantages• Nonsurgical• Oral agent self-administered weekly• 80% response rateDisadvantages• Long-term treatment required• Potential toxicity• Long-term data lacking at this timeantidepressants, should be sought. In addition to low platelet count, another laboratory finding characteristic of ITP is the presence of large, immature platelets (megakaryocytes) on peripheral blood smear.The usual first line of therapy is oral prednisone at a dos-age of 1.0 to 1.5 mg/kg per day.49,50 No consensus exists as to the optimal duration of steroid therapy, but most responses occur within the first 3 weeks. Response rates range from 50% to 75%, but relapses are common. IV immunoglobulin, given at 1.0 g/kg per day for 2 to 3 days, is indicated for internal bleeding when platelet counts remain less


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Surgery_Schwartz. weeks. Response rates range from 50% to 75%, but relapses are common. IV immunoglobulin, given at 1.0 g/kg per day for 2 to 3 days, is indicated for internal bleeding when platelet counts remain less than 5000/mm3, when extensive pur-pura exists, or to preoperatively boost platelets. IV immunoglob-ulin is thought to impair clearance of immunoglobulin G–coated platelets by competing for binding to tissue macrophage recep-tors. An immediate response is common, but a sustained remis-sion is not. The medical approach to ITP has been modified with the advent of rituximab and thrombopoietin receptor agonists.51 Therapeutic recommendations are summarized in Table 34-2. Splenectomy is selectively indicated for failure of medical ther-apy, for prolonged use of steroids with undesirable effects, and in selected cases after first relapse.7 Prolonged use of steroids can be defined in various ways, but a persistent need for more than 10 to 20 mg/d for 3 to 6 months to maintain a platelet count of


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Surgery_Schwartz. in selected cases after first relapse.7 Prolonged use of steroids can be defined in various ways, but a persistent need for more than 10 to 20 mg/d for 3 to 6 months to maintain a platelet count of greater than 30,000/mm3 generally prompts referral for sple-nectomy. Splenectomy is an effective option for refractory ITP and provides a permanent response without subsequent need for steroids in 75% to 85% of patients. Two recent reviews and meta-analyses have assessed the global results of splenectomy for ITP, specifically after the use of laparoscopic techniques. Brunicardi_Ch34_p1517-p1548.indd 152623/02/19 2:36 PM 1527THE SPLEENCHAPTER 34In 2004, Kojouri and colleagues reviewed 135 case series from 1966 to 2004, pooling 4955 patients.51 The long-term plate-let count response was assessed, as was the ability to predict response and the incidence of complications. Complete response was achieved in 66% of cases with a follow-up ranging from 1 to 153 months, and complete and partial


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Surgery_Schwartz. as was the ability to predict response and the incidence of complications. Complete response was achieved in 66% of cases with a follow-up ranging from 1 to 153 months, and complete and partial responses occurred in as many as 88% of patients, regardless of the duration of follow-up. They also analyzed 12 preoperative demographic, clinical, and laboratory parameters and found no predictive capability of platelet response in any of them. Mortality was very low (1%), and morbidity was 10%. Limitations of this review included old case series and a low percentage of laparoscopic splenectomies. In a 2009 systemic review involving 1223 patients, Mikhael and colleagues evaluated the shortand long-term outcomes after laparoscopic splenectomy.52 The conversion rate to open sur-gery was 5.6%, and the immediate nonresponder rate was 8.2%; however, eventually a clinical response was achieved in 72% of the patients on long-term follow-up (5 years). The initial con-cerns regarding the potential for


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Surgery_Schwartz. immediate nonresponder rate was 8.2%; however, eventually a clinical response was achieved in 72% of the patients on long-term follow-up (5 years). The initial con-cerns regarding the potential for missing accessory spleens, lon-ger operative times, and increased cost related to laparoscopic versus open splenectomy have been resolved. Laparoscopy is now the approach of choice for elective splenectomy for ITP, with recent studies showing improved short-term results and comparable long-term results to conventional open splenectomy in this condition.53-56In children with ITP, the course is typically self-limited, with durable and complete remission in greater than 70% of patients regardless of therapy. Because of the good prognosis without treatment, the decision to intervene surgically is contro-versial and is largely to obviate intracranial hemorrhage as dis-cussed earlier. Thus, children with typical ITP—and certainly those without hemorrhage—are managed principally by obser-vation,


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Surgery_Schwartz. contro-versial and is largely to obviate intracranial hemorrhage as dis-cussed earlier. Thus, children with typical ITP—and certainly those without hemorrhage—are managed principally by obser-vation, with short-term therapy in select cases.57 Urgent sple-nectomy, in conjunction with aggressive medical therapy, may play a role in the rare circumstance of severe, life-threatening bleeding in both children and adults.Thrombotic Thrombocytopenic Purpura Thrombotic throm-bocytopenic purpura (TTP) is a serious disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and neurologic complications. Abnormal platelet clumping occurs in arterioles and capillaries, reducing the lumen of these vessels and predisposing the patient to microvascular thrombotic epi-sodes. The reduced lumen size also causes shearing stresses on erythrocytes, which leads to deformed red blood cells subject to hemolysis. Hemolysis may also be due in part to sequestration and destruction of


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Surgery_Schwartz. reduced lumen size also causes shearing stresses on erythrocytes, which leads to deformed red blood cells subject to hemolysis. Hemolysis may also be due in part to sequestration and destruction of erythrocytes in the spleen. Research has dem-onstrated that the underlying abnormality is likely related to the persistence of unusually large multimers of von Willebrand fac-tor associated with platelet clumping in the patient’s blood.58,59TTP occurs in approximately 3.7 individuals per million, but this rare disorder’s dramatic clinical sequelae and favorable response to early therapy demand an understanding of its clini-cal presentation to ensure an early diagnosis. Clinical features of the disorder include petechiae, fever, neurologic symptoms, renal failure, and, infrequently, cardiac symptoms such as heart failure or arrhythmias. Petechial hemorrhages in the lower extremities are the most common presenting sign. Along with fever, patients may experience flu-like symptoms, malaise, or


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Surgery_Schwartz. such as heart failure or arrhythmias. Petechial hemorrhages in the lower extremities are the most common presenting sign. Along with fever, patients may experience flu-like symptoms, malaise, or fatigue. Neurologic changes range from generalized headaches to altered mental status, seizures, and even coma. Generally, however, the mere presence of petechiae and thrombocytopenia is sufficient to lead to the diagnosis of TTP and consideration of treatment.The diagnosis is confirmed by the peripheral blood smear, which shows schistocytes, nucleated red blood cells, and baso-philic stippling. Although other conditions such as tight aortic stenosis or prosthetic valves may lead to the presence of schis-tocytes, these conditions generally are not accompanied by thrombocytopenia. TTP may be distinguished from autoimmune causes of thrombocytopenia, such as Evans’ syndrome (ITP and autoimmune hemolytic anemia) or systemic lupus erythemato-sus, by a negative result on Coombs’ test.Plasma exchange


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Surgery_Schwartz. from autoimmune causes of thrombocytopenia, such as Evans’ syndrome (ITP and autoimmune hemolytic anemia) or systemic lupus erythemato-sus, by a negative result on Coombs’ test.Plasma exchange is the first-line therapy for TTP. This treatment consists of the daily removal of a single volume of the patient’s plasma and its replacement with fresh-frozen plasma until the thrombocytopenia, anemia, and associated symptoms are corrected. Therapy is then tapered over 1 to 2 weeks.40 Splenectomy should be considered in patients who experience relapse or who require multiple plasma exchanges to control severe symptoms, and generally is well tolerated without sig-nificant morbidity.59,60Malignant ConditionsWhite Blood Cell Disorders. The role of splenectomy in patients with white blood cell disorders varies. As for the myelogenous diseases mentioned previously, splenectomy for white blood cell disorders can be effective therapy for symp-tomatic splenomegaly and hypersplenism, improving some


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Surgery_Schwartz. varies. As for the myelogenous diseases mentioned previously, splenectomy for white blood cell disorders can be effective therapy for symp-tomatic splenomegaly and hypersplenism, improving some clinical parameters but generally not altering the course of the underlying disease or long-term prognosis. Historically, sple-nectomy has played a role during surgical staging for Hodg-kin’s disease, although this practice has become largely obsolete with the advent of imaging technologies (Computed tomogra-phy [CT] scan and 18F-fluorodeoxyglucose positron emission tomography [18F-FDG PET]).61-63 Careful consideration of the intended benefits of splenectomy must be weighed against the significant perioperative and postsplenectomy risks in this often complex patient population.64Hairy Cell Leukemia Hairy cell leukemia (HCL) is an uncom-mon blood disorder, representing only 2% of all adult leukemias. HCL is characterized by splenomegaly, pancytopenia, and large numbers of abnormal lymphocytes in


Surgery_Schwartz

Surgery_Schwartz. cell leukemia (HCL) is an uncom-mon blood disorder, representing only 2% of all adult leukemias. HCL is characterized by splenomegaly, pancytopenia, and large numbers of abnormal lymphocytes in the bone marrow. These lymphocytes contain irregular hair-like cytoplasmic projections identifiable on the peripheral smear. Many HCL patients have few symptoms and require no specific therapy. Splenectomy for HCL was historically performed as a palliative procedure, alleviating the symptoms associated with splenomegaly and normalizing peripheral blood counts in the majority of patients, but not leading to morphological bone marrow remissions. However, with the advent of diverse new drugs (e.g., ritux-imab, pentostatin, cladribine), splenectomy has become rarely performed.65 Splenectomy should be considered after exhaustive systemic therapy or for those with nontraumatic rupture, and it has also been used to treat pregnant women with HCL to delay onset of chemotherapy.66Hodgkin’s


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Surgery_Schwartz. should be considered after exhaustive systemic therapy or for those with nontraumatic rupture, and it has also been used to treat pregnant women with HCL to delay onset of chemotherapy.66Hodgkin’s Lymphoma Hodgkin’s Lymphoma (HL) is a dis-order of the lymphoid system characterized by the presence of Reed-Sternberg cells (which actually form the minority of the Hodgkin’s tumor). More than 90% of patients with HL pres-ent with lymphadenopathy above the diaphragm. Lymph nodes can become particularly bulky in the mediastinum, which may result in shortness of breath, cough, or obstructive pneumonia. Brunicardi_Ch34_p1517-p1548.indd 152723/02/19 2:36 PM 1528SPECIFIC CONSIDERATIONSPART IILymphadenopathy below the diaphragm is rare on presen-tation but can arise with disease progression. The spleen is often an occult site of spread, but massive splenomegaly is not common. In addition, large spleens do not necessarily signify involvement.62Four major histologic types exist: lymphocyte


Surgery_Schwartz

Surgery_Schwartz. The spleen is often an occult site of spread, but massive splenomegaly is not common. In addition, large spleens do not necessarily signify involvement.62Four major histologic types exist: lymphocyte predomi-nance type, nodular sclerosis type, mixed cellularity type, and lymphocyte depletion type. The histologic type, along with location of disease and symptomatology, influence survival for patients with HD. Stage I disease is limited to one anatomic region; stage II disease is defined by the presence of two or more contiguous or noncontiguous regions on the same side of the diaphragm; stage III disease involves disease on both sides of the diaphragm, but limited to lymph nodes, spleen, and Waldeyer’s ring (the ring of lymphoid tissue formed by the lin-gual, palatine, and nasopharyngeal tonsils); and stage IV disease includes involvement of the bone marrow, lung, liver, skin, gas-trointestinal tract, or any organ or tissue other than the lymph nodes or Waldeyer’s ring.62Staging


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Surgery_Schwartz. tonsils); and stage IV disease includes involvement of the bone marrow, lung, liver, skin, gas-trointestinal tract, or any organ or tissue other than the lymph nodes or Waldeyer’s ring.62Staging laparotomy for HL is less commonly performed in the current era of minimally invasive surgery and advanced imaging techniques. More liberal use of diagnostic tools (CT scan and 18F-FDG PET) and chemotherapy for patients with HL has drastically reduced the indications for surgical staging. Current indications for surgical staging include clini-cal suspicion of lymphoma without evidence of peripheral disease or patients requiring restaging for suspicion of failure after chemotherapy.62,67Non-Hodgkin’s Lymphoma Non-Hodgkin’s lymphoma (NHL) encompasses all malignancies derived from the lymphoid sys-tem except classic HL.63 A proliferation of any one of the three predominant lymph cell types—natural killer cells, T cells, or B cells—may be included in the category of NHL. Because of the wide net


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Surgery_Schwartz. except classic HL.63 A proliferation of any one of the three predominant lymph cell types—natural killer cells, T cells, or B cells—may be included in the category of NHL. Because of the wide net cast by NHL, the clinical presentations of the dis-orders under its umbrella vary. The subentities of NHL may be clinically classified into nodal or extranodal, as well as indolent, aggressive, and very aggressive groups. Patients with indolent lymphomas may present with mild or no symptoms and seek medical attention for a swollen lymph node, whereas the aggres-sive and very aggressive lymphomas create easily noticeable symptoms, such as pain, swelling due to obstruction of vessels, fever, and night sweats. Surgical staging is no longer indicated for NHL because the combination of history and physical exam-ination, chest radiograph and abdominal/pelvic CT scan, biopsy of involved lymph nodes (including laparoscopically directed nodal and liver biopsies), and bone marrow biopsy is


Surgery_Schwartz

Surgery_Schwartz. and physical exam-ination, chest radiograph and abdominal/pelvic CT scan, biopsy of involved lymph nodes (including laparoscopically directed nodal and liver biopsies), and bone marrow biopsy is suffi-cient.63 Splenomegaly exists in some, but not all, forms of NHL. Splenectomy does not alter the natural history of the disease, but it is indicated in cases where a diagnosis cannot be established by obtaining peripheral tissue and clinical suspicion remains or for management of symptoms related to an enlarged spleen as well as for improvement of cytopenias.68-72Chronic Lymphocytic Leukemia Chronic lymphocytic leuke-mia (CCL) is currently considered a subtype of NHL. The main characteristic of CLL is a progressive accumulation of old and nonfunctional lymphocytes.63,73 Symptoms of CLL are nonspe-cific and include weakness, fatigue, fever without illness, night sweats, and frequent bacterial and viral infections. The most fre-quent finding is lymphadenopathy. When the spleen is enlarged,


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Surgery_Schwartz. and include weakness, fatigue, fever without illness, night sweats, and frequent bacterial and viral infections. The most fre-quent finding is lymphadenopathy. When the spleen is enlarged, it may be massive or barely palpable below the costal margin. Splenectomy is indicated to improve cytopenias and was shown to be 75% effective in a combined group of patients who had either CLL or nonmalignant HD.29 Splenectomy may facilitate chemotherapy in patients whose cell counts were prohibitively low before spleen removal. Palliative splenectomy also is indi-cated for symptomatic splenomegaly.Bone Marrow Disorders (Myeloproliferative Disorders). The myeloproliferative disorders are characterized by an abnor-mal growth of cell lines in the bone marrow. They include chronic myeloid leukemia, acute myeloid leukemia, chronic myelomono-cytic leukemia, essential thrombocythemia, polycythemia vera, and myelofibrosis, also known as agnogenic myeloid metaplasia (see “Myelofibrosis [Agnogenic Myeloid


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Surgery_Schwartz. myeloid leukemia, chronic myelomono-cytic leukemia, essential thrombocythemia, polycythemia vera, and myelofibrosis, also known as agnogenic myeloid metaplasia (see “Myelofibrosis [Agnogenic Myeloid Metaplasia]” later in this chapter). The common underlying problem leading to sple-nectomy in these disorders is symptomatic splenomegaly. Symp-toms due to splenomegaly are due to mass effect and consist of early satiety, poor gastric emptying, heaviness or pain in the left upper quadrant, and even diarrhea. Hypersplenism, when it occurs in these conditions, usually is associated with splenomeg-aly. Splenectomy performed in the setting of the myeloprolifera-tive disorders is generally for treatment of the pain, early satiety, and other symptoms of splenomegaly. Radiation has been used since 1903 to treat symptomatic splenomegaly in myeloprolif-erative disorders, but today it is principally used in situations in which splenectomy is not an option.Chronic Myelogenous Leukemia Chronic


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Surgery_Schwartz. 1903 to treat symptomatic splenomegaly in myeloprolif-erative disorders, but today it is principally used in situations in which splenectomy is not an option.Chronic Myelogenous Leukemia Chronic myelogenous leu-kemia (CML) is a disorder of the primitive pluripotent stem cells in the bone marrow that results in a significant increase in erythroid, megakaryotic, and pluripotent progenitors in the peripheral blood smear. The genetic hallmark is a transposition between the bcr gene on chromosome 9 and the abl gene on chromosome 22. CML accounts for 7% to 15% of all leukemias, with an incidence of 1.5 in 100,000 in the United States.44 It is often asymptomatic, but CML can cause fatigue, anorexia, sweating, and left upper quadrant pain and early satiety sec-ondary to splenomegaly. Enlargement of the spleen is found in roughly one-half of patients with CML. Current therapy includes imatinib or allogeneic stem cell transplantation. Splenectomy is indicated to relieve symptoms of massive


Surgery_Schwartz

Surgery_Schwartz. of the spleen is found in roughly one-half of patients with CML. Current therapy includes imatinib or allogeneic stem cell transplantation. Splenectomy is indicated to relieve symptoms of massive splenomegaly, but it does not prevent blast crisis or alter the disease process.45,70Acute Myeloid Leukemia Like CML, acute myeloid leu-kemia (AML) involves the abnormal growth of stem cells in the bone marrow. Unlike CML, AML has a presentation that is more rapid and dramatic. The proliferation and accumula-tion of hematopoietic stem cells in the bone marrow and blood inhibit the growth and maturation of normal red blood cells, white blood cells, and platelets. Death usually results within weeks to months if AML goes untreated. The incidence of AML is approximately 9200 new cases each year in the United States, and it accounts for 1.2% of all cancer deaths.74 Patients with other myeloproliferative disorders, such as polycythemia vera, primary thrombocytosis, or myeloid metaplasia, are at


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Surgery_Schwartz. the United States, and it accounts for 1.2% of all cancer deaths.74 Patients with other myeloproliferative disorders, such as polycythemia vera, primary thrombocytosis, or myeloid metaplasia, are at increased risk for leukemic transformation to AML. Presenting signs and symptoms of AML include a viral-like illness with fever, malaise, and frequently bone pain due to the expansion of the medullary space. Standard treatment is combined induction therapy with daunorubicin, cytarabine, and stem cell transplan-tation. Splenectomy is indicated in AML only in the uncom-mon circumstance that left upper quadrant pain and early satiety become unbearable. The benefit must be weighed against the heightened risk of postsplenectomy infection in AML patients immunocompromised due to neutropenia and chemotherapy.74Brunicardi_Ch34_p1517-p1548.indd 152823/02/19 2:36 PM 1529THE SPLEENCHAPTER 34Chronic Myelomonocytic Leukemia Like CML and AML, chronic myelomonocytic leukemia (CMML) is characterized


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Surgery_Schwartz. 152823/02/19 2:36 PM 1529THE SPLEENCHAPTER 34Chronic Myelomonocytic Leukemia Like CML and AML, chronic myelomonocytic leukemia (CMML) is characterized by a proliferation of hematopoietic elements in the bone marrow and blood. CMML differs from CML in that it is associated with monocytosis in the peripheral smear (>1 × 103 monocytes/mm3) and in the bone marrow. Splenomegaly occurs in one-half of these patients, and splenectomy can result in symptomatic relief.75Essential Thrombocythemia Essential thrombocythemia (ET) represents abnormal growth of the megakaryocyte cell line, resulting in increased levels of platelets in the bloodstream. The diagnosis is made after the exclusion of other chronic myeloid disorders such as CML, polycythemia vera, and myelofibrosis that may also present with thrombocytosis.76 Clinical manifesta-tions of ET include vasomotor symptoms, thrombohemorrhagic events, recurrent fetal loss, and the transformation to myelofi-brosis with myeloid metaplasia or


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Surgery_Schwartz. with thrombocytosis.76 Clinical manifesta-tions of ET include vasomotor symptoms, thrombohemorrhagic events, recurrent fetal loss, and the transformation to myelofi-brosis with myeloid metaplasia or AML. Hydroxyurea is used to reduce thrombotic events in ET but does not alter transforma-tion to myelofibrosis or leukemia. Splenomegaly occurs in one-third to one-half of patients with ET. Splenectomy is not felt to be helpful in the early stages of ET and is best reserved for the later stages of disease, when myeloid metaplasia has devel-oped.69 Even in these circumstances, candidates should be cho-sen selectively because significant bleeding has been reported to complicate splenectomy in these patients.Polycythemia Vera Polycythemia vera (PV) is a clonal, chronic, progressive myeloproliferative disorder characterized by an increase in red blood cell mass, frequently accompanied by leukocytosis, thrombocytosis, and splenomegaly. Patients with PV typically enjoy longer survival than those


Surgery_Schwartz

Surgery_Schwartz. disorder characterized by an increase in red blood cell mass, frequently accompanied by leukocytosis, thrombocytosis, and splenomegaly. Patients with PV typically enjoy longer survival than those affected by hematologic malignancies but remain at risk for transformation to myelofibrosis or AML. The disease is rare, with an annual incidence of 5 to 17 cases per million population.76,77 Physical findings include ruddy cyanosis, conjunctival plethora, hepato-megaly, splenomegaly, and hypertension. Treatment should be tailored to the risk status of the patient and ranges from phle-botomy and aspirin administration to the use of chemothera-peutic agents. As in ET, splenectomy is not helpful in the early stages of disease and is best reserved for patients with late-stage disease in whom myeloid metaplasia has developed and spleno-megaly-related symptoms are severe.76,77Myelofibrosis (Agnogenic Myeloid Metaplasia) The term myelofibrosis may be used to describe either the generic condi-tion


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Surgery_Schwartz. metaplasia has developed and spleno-megaly-related symptoms are severe.76,77Myelofibrosis (Agnogenic Myeloid Metaplasia) The term myelofibrosis may be used to describe either the generic condi-tion of fibrosis of the bone marrow (which may be associated with a number of benign and malignant disorders) or a specific, chronic, malignant hematologic disease associated with sple-nomegaly, the presence of red blood cell and white blood cell progenitors in the bloodstream, marrow fibrosis, and extramed-ullary hematopoiesis, otherwise known as agnogenic myeloid metaplasia (AMM). AMM also can be referred to as myeloscle-rosis, idiopathic myeloid metaplasia, and osteosclerosis. In this chapter, the term myelofibrosis is synonymous with AMM.In AMM, fibrosis of the bone marrow is believed to be a response to a clonal proliferation of hematopoietic stem cells. Marrow failure is common. The true incidence of AMM is unknown due to the scarcity of epidemiologic data, but one study estimated its U.S.


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Surgery_Schwartz. to a clonal proliferation of hematopoietic stem cells. Marrow failure is common. The true incidence of AMM is unknown due to the scarcity of epidemiologic data, but one study estimated its U.S. incidence at 1.46 per 100,000 popula-tion.78-80 The diagnosis is made by a careful examination of the peripheral blood smear and bone marrow. Nucleated red blood cells and immature myeloid elements in the blood are present in 96% of cases and strongly suggest the diagnosis. Teardrop poikilocytosis is another frequent finding. Care must be taken, however, to exclude a history of a primary neoplasm (such as lymphoma or adenocarcinoma of the stomach, lung, prostate, or breast) or tuberculosis because patients with these conditions may develop secondary myelofibrosis.Treatment depends on symptoms: Asymptomatic patients are closely followed, whereas symptomatic patients undergo therapeutic intervention targeted to their symptoms. The only curative therapy is allogeneic bone marrow transplantation in


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Surgery_Schwartz. patients are closely followed, whereas symptomatic patients undergo therapeutic intervention targeted to their symptoms. The only curative therapy is allogeneic bone marrow transplantation in younger, high-risk patients. Supportive therapy for clinically symptomatic anemia includes steroids, danazol, erythropoietin, or blood transfusion.78,80 Splenomegaly-related symptoms are best treated with splenectomy. Although some chemotherapeu-tic agents (busulfan, hydroxyurea, interferon-α) and low-dose radiation can reduce splenic size, their discontinuation usually results in rapid splenic regrowth.A thorough preoperative workup must precede sple-nectomy in patients with AMM. The candidate must possess acceptable cardiac, pulmonary, hepatic, and renal reserve for the operation. The coagulation system should be examined; testing should include measurement of coagulation factors V and VIII and fibrin split products, platelet count, and bleeding time. Low platelet counts may require


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Surgery_Schwartz. system should be examined; testing should include measurement of coagulation factors V and VIII and fibrin split products, platelet count, and bleeding time. Low platelet counts may require administration of ste-roids and/or platelet transfusion at the time of surgery. Sple-nectomy provides durable, effective palliation for nearly all patients with AMM, although postoperative complications are more common in patients with AMM than in those with other hematologic indications. The Mayo Clinic recently published its 30-year experience with 314 myelofibrosis patients who underwent splenectomy. Nearly half of the operations (49%) were performed to alleviate the mechanical symptoms of sple-nomegaly; the remainder were undertaken to manage anemia, thrombocytopenia, or portal hypertension. Response to splenec-tomy was 76% overall at 1 year; overall complication rate was 28%, including 21 perioperative deaths.80 Thrombosis, hemor-rhage, and infection complications were common, with


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Surgery_Schwartz. Response to splenec-tomy was 76% overall at 1 year; overall complication rate was 28%, including 21 perioperative deaths.80 Thrombosis, hemor-rhage, and infection complications were common, with preop-erative thrombocytopenia an independent predictor of mortality risk. These data underscore the severity of this malignancy and emphasize the need for careful patient selection when consider-ing splenectomy in AMM.79Tumors and Metastasis. Primary tumors of the spleen are typ-ically benign or malignant variants of vascular neoplasms. The most common primary tumors of the spleen are sarcomas, many of which have been linked to various environmental and occu-pational exposures such as vinyl chloride or thorium dioxide. Isolated splenic metastases are extremely unusual but may occur in the setting of a concomitant carcinoma.81 Lung carcinoma is the tumor that most commonly spreads to the spleen, although, colorectal, ovarian, and melanoma may also metastasize to the spleen.82,83 If after a


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Surgery_Schwartz. of a concomitant carcinoma.81 Lung carcinoma is the tumor that most commonly spreads to the spleen, although, colorectal, ovarian, and melanoma may also metastasize to the spleen.82,83 If after a thorough examination, an isolated splenic metastasis is confirmed, a laparoscopic splenectomy with intact spleen retrieval may be considered.81-83Miscellaneous Disorders and LesionsInfections and Abscesses. Primary infections of the spleen although uncommon in immunocompetent adults, are particu-larly reported in the immunocompromised population or those with a history of intravenous recreational drug abuse.84,85 The potential effects of certain systemic infections on the spleen merit close attention, mostly because of the dreaded risk of spontaneous splenic rupture. Infectious mononucleosis due to Brunicardi_Ch34_p1517-p1548.indd 152923/02/19 2:36 PM 1530SPECIFIC CONSIDERATIONSPART IIeither Epstein-Barr virus or cytomegalovirus infection imparts a small but often-discussed risk of


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Surgery_Schwartz. due to Brunicardi_Ch34_p1517-p1548.indd 152923/02/19 2:36 PM 1530SPECIFIC CONSIDERATIONSPART IIeither Epstein-Barr virus or cytomegalovirus infection imparts a small but often-discussed risk of spontaneous splenic rup-ture in both adults and children. The true incidence may be underreported, however. Recent case reports abound in the lit-erature regarding spontaneous splenic rupture due to a variety of infectious causes (malaria, Listeria infection, fungal infec-tions, dengue, and Q fever, to name a few) as well as a vari-ety of neoplastic and other noninfectious causes (lymphoma, angiosarcoma, amyloidosis, pregnancy). The presumed patho-physiologic mechanism is infiltration of the splenic paren-chyma with inflammatory cells, which distorts the architecture and fibrous support system of the spleen and thins the splenic capsule.86 In this setting, splenic rupture can occur spontane-ously or after a seemingly minor external trauma or even a Valsalva maneuver.Abscesses of the spleen


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Surgery_Schwartz. of the spleen and thins the splenic capsule.86 In this setting, splenic rupture can occur spontane-ously or after a seemingly minor external trauma or even a Valsalva maneuver.Abscesses of the spleen are uncommon, with an incidence reported to be 0.14% to 0.7%.87-89 They occur more frequently in tropical locations, where they are associated with throm-bosed splenic vessels and infarction in patients with sickle cell anemia. Five distinct mechanisms of splenic abscess formation have been described: (a) hematogenous infection; (b) contigu-ous infection; (c) hemoglobinopathy; (d) immunosuppression, including HIV infection and chemotherapy; and (e) trauma. The presentation of splenic abscess frequently is delayed, with most patients enduring symptoms for 16 to 22 days before diagno-sis. Clinical manifestations include fever, left upper quadrant pain, leukocytosis, and splenomegaly in about one-third of patients. The diagnosis is confirmed by ultrasound or CT scan, which has a 95%


Surgery_Schwartz

Surgery_Schwartz. Clinical manifestations include fever, left upper quadrant pain, leukocytosis, and splenomegaly in about one-third of patients. The diagnosis is confirmed by ultrasound or CT scan, which has a 95% sensitivity and specificity. Common organ-isms are aerobic microbes (streptococci and Escherichia coli), but other microorganisms have also been isolated (Mycobac-terium tuberculosis and Salmonella typhi). Upon discovery of a splenic abscess, broad-spectrum antibiotics should be initi-ated, with adjustment to more specific therapy based on culture results and continuation of treatment for a minimum of 14 days. Splenectomy is the operation of choice, but percutaneous and open drainage are options for patients who either cannot toler-ate splenectomy or where the clinical scenario warrants splenic preservation, particularly in children.90 Percutaneous drainage is often successful for patients with unilocular disease and may result in avoidance of splenectomy. Patients with multilocular disease


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Surgery_Schwartz. preservation, particularly in children.90 Percutaneous drainage is often successful for patients with unilocular disease and may result in avoidance of splenectomy. Patients with multilocular disease will often require multiple drains and therefore often benefit from total splenectomy.Cysts. Splenic cysts (Fig. 34-5) can be categorized according to a number of criteria; one clinically relevant scheme is to char-acterize true splenic cysts as either parasitic or nonparasitic.86Parasitic Infections. Parasitic infection is the most common cause of splenic cysts worldwide, and the majority are due to Echinococcus species. These cysts are more commonly found in areas where the pathogen is endemic. Symptoms, when present, generally are related to the presence of a mass lesion in the left upper quadrant or a lesion that impinges on the stomach. Ultra-sound can establish the presence of a cystic lesion and occasion-ally incidentally detect asymptomatic lesions as well. Serologic testing for


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Surgery_Schwartz. quadrant or a lesion that impinges on the stomach. Ultra-sound can establish the presence of a cystic lesion and occasion-ally incidentally detect asymptomatic lesions as well. Serologic testing for echinococcal antibodies can confirm or exclude the cystic lesion as parasitic, an important piece of informa-tion when planning operative therapy. Symptomatic parasitic cysts are best treated with splenectomy. Avoidance of spillage of parasitic cyst contents into the peritoneal cavity to avoid the possibility of anaphylactic shock is an important principle in surgical management despite its rare occurrence.91Cysts resulting from trauma are termed pseudocysts due to their lack of an epithelial cell lining. Less common examples of nonparasitic cysts are dermoid, epidermoid, and epithelial cysts.92 The treatment of nonparasitic cysts depends on whether or not they produce symptoms. Asymptomatic nonparasitic cysts may be observed with close follow-up by ultrasound to exclude significant growth


Surgery_Schwartz

Surgery_Schwartz. The treatment of nonparasitic cysts depends on whether or not they produce symptoms. Asymptomatic nonparasitic cysts may be observed with close follow-up by ultrasound to exclude significant growth or expansion. Patients should be advised of the risk of cyst rupture with even minor abdominal trauma if they elect nonoperative management for large cysts. Small symptomatic nonparasitic cysts may be excised with splenic preservation, and large symptomatic nonparasitic cysts may be unroofed. Both of these operations may be performed laparoscopically.86Storage Diseases and Infiltrative Disorders Gaucher’s Disease Gaucher’s disease is an inherited lipid stor-age disorder characterized by the deposition of glucocerebroside in cells of the macrophage-monocyte system. The underlying abnormality is a deficiency in the activity of a lysosomal hydro-lase. Abnormal glycolipid storage results in organomegaly, particularly hepatomegaly and splenomegaly.93,94 Patients with Gaucher’s disease frequently


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Surgery_Schwartz. deficiency in the activity of a lysosomal hydro-lase. Abnormal glycolipid storage results in organomegaly, particularly hepatomegaly and splenomegaly.93,94 Patients with Gaucher’s disease frequently experience symptoms related to splenomegaly, including early satiety and abdominal discom-fort, and to hypersplenism, including thrombocytopenia, normo-cytic anemia, and mild leukopenia. These latter findings occur as a result of excessive sequestration of formed blood elements in the spleen. Other symptoms in patients with Gaucher’s dis-ease include bone pain, pathologic fractures, and jaundice. Splenectomy may alleviate hematologic abnormalities in these patients with hypersplenism, but it does not correct the underly-ing disease process.95Niemann-Pick Disease Niemann-Pick disease is an inherited disorder of abnormal lysosomal storage of sphingomyelin and cholesterol in cells of the macrophage-monocyte system. Four types of the disease (A, B, C, and D) exist, with unique clinical


Surgery_Schwartz

Surgery_Schwartz. an inherited disorder of abnormal lysosomal storage of sphingomyelin and cholesterol in cells of the macrophage-monocyte system. Four types of the disease (A, B, C, and D) exist, with unique clinical presentations. Types A and B result from a deficiency in lyso-somal hydrolase and are the forms most likely to demonstrate splenomegaly with its concomitant symptoms. Symptoms of splenomegaly are relieved by splenectomy.Amyloidosis Amyloidosis is a disorder of abnormal extra-cellular protein deposition and protein conformation disorder associated with a clonal plasma cell dyscrasia. There are mul-tiple forms of amyloidosis, each with its own individual clini-cal presentation, and the severity of disease may range from asymptomatic to multiorgan failure. Patients with primary amy-loidosis, associated with plasma cell dyscrasia, have splenic involvement in approximately 5% of cases. Secondary amy-loidosis, associated with chronic inflammatory conditions, also may present with an enlarged


Surgery_Schwartz

Surgery_Schwartz. with plasma cell dyscrasia, have splenic involvement in approximately 5% of cases. Secondary amy-loidosis, associated with chronic inflammatory conditions, also may present with an enlarged spleen and even spontaneous rup-ture.96 Symptoms of splenomegaly are relieved by splenectomy.Sarcoidosis Sarcoidosis is an inflammatory disease of young adults characterized by noncaseating granulomas in affected tissues. Signs and symptoms of the disease range in severity and typically are nonspecific, such as fatigue and malaise. Any organ system may be involved. The most commonly involved organ is the lung, followed by the spleen. Splenomegaly occurs in approximately 25% of patients. Massive splenomegaly (>1 kg) is rare with a reported incidence of 3%.97,98 Other affected tissues include the lymph nodes, eyes, joints, liver, spleen, and heart. When splenomegaly occurs and causes symp-toms related to size or hypersplenism, splenectomy effectively relieves symptoms and corrects hematologic


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Surgery_Schwartz. lymph nodes, eyes, joints, liver, spleen, and heart. When splenomegaly occurs and causes symp-toms related to size or hypersplenism, splenectomy effectively relieves symptoms and corrects hematologic abnormalities such Brunicardi_Ch34_p1517-p1548.indd 153023/02/19 2:36 PM 1531THE SPLEENCHAPTER 34as anemia and thrombocytopenia. Spontaneous splenic rupture has been reported in sarcoidosis.94Splenic Artery Aneurysm Although splenic artery aneurysm is rare, it is the most common visceral artery aneurysm. Women are four times more likely to be affected than men. The aneu-rysm usually arises in the middle to distal portion of the splenic artery.99,100 Indications for treatment include presence of symp-toms, pregnancy, intention to become pregnant, and presence of pseudoaneurysms associated with inflammatory processes. Aneurysm resection or ligation alone is acceptable for amenable lesions in the mid-splenic artery, but distal lesions in close prox-imity to the splenic hilum should be


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Surgery_Schwartz. with inflammatory processes. Aneurysm resection or ligation alone is acceptable for amenable lesions in the mid-splenic artery, but distal lesions in close prox-imity to the splenic hilum should be treated with concomitant splenectomy. An excellent prognosis follows elective treatment. Splenic artery embolization has been used to treat splenic artery aneurysm; however, this may result in painful splenic infarction or abscess.Portal Hypertension Portal hypertension can result from numerous causes but is usually due to liver cirrhosis. Sple-nomegaly and splenic congestion often accompany portal hypertension, which leads to sequestration and destruction of circulating cells in the spleen. Splenectomy is not indicated for hypersplenism per se in patients with portal hypertension as there is no correlation between the degree of pancytopenia and long-term survival in these patients.7 In rare circumstances in which splenectomy is required to reduce bleeding from esopha-geal varices


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Surgery_Schwartz. is no correlation between the degree of pancytopenia and long-term survival in these patients.7 In rare circumstances in which splenectomy is required to reduce bleeding from esopha-geal varices exacerbated by thrombocytopenia, a concomitant splenorenal shunt procedure may be performed to decompress the portal system.Sinistral hypertension secondary to splenic vein thrombo-sis, on the other hand, is potentially curable with splenectomy. Patients that are bleeding from isolated gastric varices who have normal liver function test results, especially those with a pre-vious history of pancreatitis, should be examined for splenic vein thrombosis and treated with splenectomy if findings are positive.Idiopathic portal hypertension (IPH) is extremely rare, and it is characterized by the absence of cirrhosis or other clear etiologies such as hepatic or portal vein thrombosis, cardiac failure, or hematologic disorders.101-104 Hypersplen-ism often develops in these patients due to hyperactivity


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Surgery_Schwartz. cirrhosis or other clear etiologies such as hepatic or portal vein thrombosis, cardiac failure, or hematologic disorders.101-104 Hypersplen-ism often develops in these patients due to hyperactivity of the spleen; defined as a triad of splenomegaly, pancytopenia, 6ACBDFigure 34-5. A. Computed tomography (CT) scan of giant splenic cyst. B. Three-dimensional CT reconstruction of splenic cyst. C and D. Macroscopic aspect of a multicystic spleen lesion.Brunicardi_Ch34_p1517-p1548.indd 153123/02/19 2:37 PM 1532SPECIFIC CONSIDERATIONSPART IIand normocellularity of bone marrow warranting intervention by way of splenic artery embolization or splenectomy. Par-tial splenic embolization (PSE) has recently been utilized with good success in prohibitively high operative risk patients with severe hypersplenism.105Felty’s Syndrome The triad of rheumatoid arthritis, spleno-megaly, and neutropenia is called Felty’s syndrome. It exists in approximately 3% of all patients with rheumatoid arthritis,


Surgery_Schwartz

Surgery_Schwartz. Syndrome The triad of rheumatoid arthritis, spleno-megaly, and neutropenia is called Felty’s syndrome. It exists in approximately 3% of all patients with rheumatoid arthritis, two-thirds of whom are women. Immune complexes coat the surface of white blood cells, which leads to their sequestration and clear-ance in the spleen with subsequent neutropenia. This neutro-penia (<2000 neutrophils/mm3) increases the risk for recurrent infections and often drives the decision for splenectomy. The size of the spleen is variable, from nonpalpable in 5% to 10% of patients to massively enlarged upwards of 4 times heavier than normal in others. Corticosteroids, hematopoietic growth factors, methotrexate, and splenectomy have all been used to treat the neutropenia of Felty’s syndrome. More recently, ritux-imab has been tried as a second line agent in refractory Felty’s syndrome.106,107Overall response to splenectomy is excellent, with >80% of patients showing a durable increase in white blood cell


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Surgery_Schwartz. has been tried as a second line agent in refractory Felty’s syndrome.106,107Overall response to splenectomy is excellent, with >80% of patients showing a durable increase in white blood cell count. More than one-half of patients who had infections before surgery may clear their infections after surgery.108 Besides symptomatic neutropenia, other indications for sple-nectomy include transfusion-dependent anemia and profound thrombocytopenia.Wandering Spleen A very uncommon anatomic abnormality is the “wandering spleen.” In this condition, the spleen “floats” inside the abdominal cavity due to an anomaly during embryo-genesis and may present itself in a variety of ways includ-ing acute abdomen.109 The wandering spleen is not normally attached to adjacent viscera in the splenic fossa. This may lead to splenic torsion and infarction. Splenopexy or splenectomy may be required.110Partial Splenectomy and Splenic Salvage. The increased awareness of asplenia-related life-threatening


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Surgery_Schwartz. This may lead to splenic torsion and infarction. Splenopexy or splenectomy may be required.110Partial Splenectomy and Splenic Salvage. The increased awareness of asplenia-related life-threatening complications such as overwhelming postsplenectomy infection (OPSI) has led to the development of parenchyma sparing splenic resec-tions for select patients and disorders. The first successful partial splenectomy was reported in 1979 by Shapiro and was followed by Uranus who would perform the first laparoscopic partial splenectomy in 1995.111,112 Previously, many surgeons had been reluctant to perform partial splenectomy because of the technical difficulties and bleeding risk. However, with a better understanding of the segmental vascular anatomy of the spleen and the development of improved laparoscopic skills and technologies, laparoscopic partial splenectomy has been used successfully in patients with hematologic diseases such as hereditary spherocytosis in children, who may benefit the


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Surgery_Schwartz. skills and technologies, laparoscopic partial splenectomy has been used successfully in patients with hematologic diseases such as hereditary spherocytosis in children, who may benefit the most from splenic preservation, as well as for benign splenic cysts.113-119 The technique of partial splenectomy will be dis-cussed later in this chapter.IMAGING FOR EVALUATION OF SIZE AND PATHOLOGYThorough assessment of anatomic detail and functional status of the spleen are essential for proper surgical planning. Spe-cial preoperative consideration needs to be given to patients with splenomegaly because minimally invasive methods of resection may be fraught with additional difficulty in patients with very large spleens, even in skilled hands. Other indica-tions for splenic imaging include trauma, investigations of left upper quadrant pain, characterization of splenic lesions such as tumors, cysts, and abscesses, and guidance for percutaneous procedures.165,166Preoperative imaging of the spleen is


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Surgery_Schwartz. of left upper quadrant pain, characterization of splenic lesions such as tumors, cysts, and abscesses, and guidance for percutaneous procedures.165,166Preoperative imaging of the spleen is primarily performed to obtain an accurate assessment of the splenic volume in order to confirm and document splenomegaly as well as to exclude any large splenic lesion that could affect the surgical resection plane. Identification of the presence of accessory spleens in the preoperative setting is also important, although lack of acces-sory tissue on the imaging should not preclude a thorough intra-operative search.The guidelines of the European Association for Endo-scopic Surgery suggest that for all patients in whom splenec-tomy is indicated, preoperative imaging should be obtained.24 Two of the most commonly used imaging modalities include ultrasound and CT, both enabling measurement of splenic size and volume. When desired, the splenic volume may be calcu-lated using a formula for the volume of


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Surgery_Schwartz. commonly used imaging modalities include ultrasound and CT, both enabling measurement of splenic size and volume. When desired, the splenic volume may be calcu-lated using a formula for the volume of a prolate ellipsoid:Volume (cc) = length (cm) × width (cm) × height (cm) × 0.52.64Other imaging modalities, although not as commonly used, include nuclear medicine studies and magnetic resonance imaging (MRI).PREOPERATIVE CONSIDERATIONSAs part of preoperative discussion prior to splenectomy, patients should be consulted on potential complications associated with this procedure, including overwhelming postsplenectomy sepsis, splenic vein thrombosis, bleeding, arterial thrombosis (myocardial infarction, stroke), deep vein thrombosis, and pul-monary hypertension.Vaccination and Patient EducationConsidering that infection is the most common complication, patient education and vaccinations against encapsulated pathogens are the mainstay of preventive therapy.52,120 Although rare, the most


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Surgery_Schwartz. that infection is the most common complication, patient education and vaccinations against encapsulated pathogens are the mainstay of preventive therapy.52,120 Although rare, the most feared and extreme infectious complica-tion is overwhelming postsplenectomy sepsis (OPSI). (See later section, “Overwhelming Postsplenectomy Infection,” for detailed discussion.) Patients undergoing splenectomy for hematologic or malignant indications have the greatest risk, whereas patients who undergo splenectomy for trauma or iatro-genic injury have the lowest risk. OPSI is more common in the pediatric population, with 4.4% of children less than 16 years of age versus 0.9% of adults developing this life-threatening condi-tion. The risk has been observed to be the greatest in the first 2 years after splenectomy; however, asplenic patients remain at lifelong risk.121-123 Considering that the spleen is the site for spe-cial adaptation of macrophages that target encapsulated organ-isms, asplenic patients


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Surgery_Schwartz. however, asplenic patients remain at lifelong risk.121-123 Considering that the spleen is the site for spe-cial adaptation of macrophages that target encapsulated organ-isms, asplenic patients are at higher risk of infection caused by Streptococcus pneumoniae (responsible for >50% of OPSI), H influenzae type b, Neisseria meningitidis, and Capnocytoph-aga canimorsus (transmitted by dog bites).124In the setting of elective splenectomy, patients should be vaccinated two weeks prior to surgery to optimize anti-gen recognition and processing. If splenectomy is performed emergently, vaccinations can be administered postoperatively and consideration should be given to delaying administration 7Brunicardi_Ch34_p1517-p1548.indd 153223/02/19 2:37 PM 1533THE SPLEENCHAPTER 34for 2 weeks to avoid the transient immunosuppression asso-ciated with surgery. International guidelines also recommend annual influenza vaccine for asplenic patients. The influenza vaccination provides protection from


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Surgery_Schwartz. the transient immunosuppression asso-ciated with surgery. International guidelines also recommend annual influenza vaccine for asplenic patients. The influenza vaccination provides protection from influenza syndrome and secondary bacterial infection. This immunization is associated with a 54% reduced risk of death compared with unimmunized asplenic persons.125Preoperative and postoperative patient education regard-ing OPSI is paramount because patients with OPSI may rap-idly progress from a febrile illness to circulatory collapse and death within a matter of hours. In one study, 28% of asplenic patients were unaware of the potential infection risks, and the main reasons for this lack of awareness were that correct advice was not given or that that advice was forgotten.121,126 The use of currently available vaccines against pneumococcus and other encapsulated organisms has led to a drop in the overall inci-dence of OPSI to <1%. The mechanism by which vaccination protects asplenic


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Surgery_Schwartz. of currently available vaccines against pneumococcus and other encapsulated organisms has led to a drop in the overall inci-dence of OPSI to <1%. The mechanism by which vaccination protects asplenic patients is not entirely understood. Serum anti-body titers do not necessarily correspond to clinical immunity. Moreover, antibody levels after pneumococcus vaccination decline steadily within 5 to 10 years. Revaccination is reason-ably recommended for these patients, although the efficacy of this measure is not proven.121-124,126Deep Vein Thrombosis ProphylaxisDeep vein thrombosis (DVT) after splenectomy is not infre-quent, especially in cases involving splenomegaly and myelo-proliferative disorders.76 Risk of portal vein thrombosis (PVT) may reach 50% for patients presenting with both splenomegaly and myeloproliferative disorders.127,128Postsplenectomy PVT typically presents with anorexia, abdominal pain, leukocytosis, and thrombocytosis. Effective PVT treatment is possible by


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Surgery_Schwartz. splenomegaly and myeloproliferative disorders.127,128Postsplenectomy PVT typically presents with anorexia, abdominal pain, leukocytosis, and thrombocytosis. Effective PVT treatment is possible by maintaining a high index of sus-picion, achieving early diagnosis with contrast enhanced CT, and starting anticoagulation immediately. DVT prophylaxis, including use of sequential compression devices and subcu-taneous administration of heparin (5000 U), should be initi-ated for patients undergoing splenectomy.16,77 Each patient’s risk factors for DVT should be evaluated, and when elevated risk exists (obesity, history of prior venous thromboembolism, known hypercoagulable state, older age), a postoperative anti-thrombotic regimen of up to two weeks of low molecular weight heparin should be maintained.SPLENECTOMY TECHNIQUESPatient PreparationAssessment of the potential need for transfusion of blood products and optimization of preoperative coagulation status are necessary. It is the authors’


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Surgery_Schwartz. TECHNIQUESPatient PreparationAssessment of the potential need for transfusion of blood products and optimization of preoperative coagulation status are necessary. It is the authors’ practice to order blood typing and antibody screening tests for normosplenic patients under-going elective splenectomy. Anemic patients should be trans-fused before surgery to a hemoglobin level of 10 g/dL. In more complex cases, including patients with splenomegaly, at least 2 to 4 units of cross-matched blood should be available at the time of surgery. Thrombocytopenia may be transiently cor-rected with platelet transfusions. Thrombocytopenic patients preferably should not undergo transfusion before the day of surgery and ideally not before the intraoperative ligation of the splenic artery. Several authors recommended a platelet count of 30 × 109 / L before the surgery; this may require treatment with IV immunoglobulin or oral corticosteroids if the platelets are below this number. Pooled normal human


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Surgery_Schwartz. recommended a platelet count of 30 × 109 / L before the surgery; this may require treatment with IV immunoglobulin or oral corticosteroids if the platelets are below this number. Pooled normal human immunoglobulin is effective in elevating the platelet count in approximately 75% of patients.129-131Patients who have been maintained on corticosteroid therapy preoperatively should receive parenteral corticosteroid therapy perioperatively. Bowel preparation is not routinely performed for patients undergoing elective splenectomy. All splenectomy patients do receive DVT prophylaxis, as discussed previously. After endotracheal intubation, a nasogastric (NG) tube is inserted for stomach decompression.Open SplenectomyAlthough laparoscopic surgery increasingly has achieved acceptance as the standard approach for normosplenic patients requiring splenectomy, open splenectomy (OS) is still widely practiced. The largest published series is a report of the Nationwide Inpatient Sample (NIS), where of


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Surgery_Schwartz. approach for normosplenic patients requiring splenectomy, open splenectomy (OS) is still widely practiced. The largest published series is a report of the Nationwide Inpatient Sample (NIS), where of 37,006 nontrau-matic splenectomies identified during a 6-year study period (2005–2010), 81.4% of those cases were approached by open surgery.132Traumatic rupture of the spleen continues as the most common indication for OS. Several other clinical scenarios favor an OS approach, including massive splenomegaly, ascites, portal hypertension, multiple prior operations, extensive splenic irradiation, and possible splenic abscess.During OS, the patient is placed in the supine position with the surgeon situated at the patient’s right. A left subcostal incision paralleling the left costal margin and lying two finger-breadths below it is preferred for most elective splenectomies. A midline incision is optimal for exposure when the spleen is ruptured or massively enlarged. The spleen is mobilized by


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Surgery_Schwartz. two finger-breadths below it is preferred for most elective splenectomies. A midline incision is optimal for exposure when the spleen is ruptured or massively enlarged. The spleen is mobilized by dividing ligamentous attachments, usually beginning with the splenocolic ligament (Fig. 34-6). In patients with signifi-cant splenomegaly, once lesser sac access has been achieved through either the gastrosplenic or gastrohepatic attachments, ligating the splenic artery in continuity along the superior bor-der of the pancreas may be preferable. This maneuver may serve several purposes: allowing safer manipulation of the Figure 34-6. Splenocolic ligament is divided at the beginning of open splenectomy.Brunicardi_Ch34_p1517-p1548.indd 153323/02/19 2:37 PM 1534SPECIFIC CONSIDERATIONSPART II5-mm cephalad working port5-mm camera port with 30’ scope2-mm accessory port (flank)10/12-mm caudad working portFigure 34-7. Patient positioning and trocar placement for laparoscopic splenectomy.spleen and


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Surgery_Schwartz. working port5-mm camera port with 30’ scope2-mm accessory port (flank)10/12-mm caudad working portFigure 34-7. Patient positioning and trocar placement for laparoscopic splenectomy.spleen and dissection of the splenic hilum, facilitating some shrinkage of the spleen, and providing an autotransfusion of erythrocytes and platelets. Further medial mobilization of the spleen is achieved by incising its lateral peritoneal attachments, most notably the splenophrenic ligament. Then follows indi-vidual ligation and sequential division of the short gastric ves-sels, steps that if carefully executed reduce the risk of these vessels’ retracting and bleeding. Splenic hilar dissection then takes place. Whenever possible, care should be taken to dis-sect and individually ligate the splenic artery and vein (in that order) before dividing them. As noted in the discussion of splenic anatomy, the tail of the pancreas lies within 1 cm of the splenic hilum in 75% of patients; therefore, during hilar


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Surgery_Schwartz. vein (in that order) before dividing them. As noted in the discussion of splenic anatomy, the tail of the pancreas lies within 1 cm of the splenic hilum in 75% of patients; therefore, during hilar dissec-tion, great care must be taken to avoid injuring the pancreas. Once the spleen is excised, hemostasis is secured by irrigating, suctioning, and scrupulously inspecting the bed of dissection. The splenic bed is not routinely drained. A thorough search for accessory spleens must be undertaken when a hematologic disorder has occasioned splenectomy. At the completion of sur-gery, the nasogastric tube is removed.Laparoscopic SplenectomyLaparoscopic splenectomy (LS) has become the procedure of choice over the last two decades, since it has been first described Delaitre and Maignien in 1991.133 In fact, LS is now the gold standard for elective splenectomy in patients with normal-sized spleens. In experienced hands, LS is associated with decreased intraoperative blood loss, shorter hospital


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Surgery_Schwartz. In fact, LS is now the gold standard for elective splenectomy in patients with normal-sized spleens. In experienced hands, LS is associated with decreased intraoperative blood loss, shorter hospital length of stay, and lower morbidity rates when compared to OS.134,135 Since the introduction of the lateral approach, most LS proce-dures are now performed with the patient in the right lateral decubitus position (Fig. 34-7).136 A midway “double-access” technique in which the patient is in a 45° right lateral decubitus position has also been advocated. This positioning permits con-comitant surgery, such as laparoscopic cholecystectomy, more easily than does the lateral approach. The double-access 8technique requires the placement of five or six trocars. The lateral approach routinely involves the use of three or four trocars posi-tioned as shown in Fig. 34-7. Use of an angled (30° or 45°) lapa-roscope (2, 5, or 10 mm) greatly facilitates the procedure. Exposure of the vital anatomy in a


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Surgery_Schwartz. the use of three or four trocars posi-tioned as shown in Fig. 34-7. Use of an angled (30° or 45°) lapa-roscope (2, 5, or 10 mm) greatly facilitates the procedure. Exposure of the vital anatomy in a manner that allows for a more intuitive sequence of dissection, paralleling that of OS, may be considered an additional advantage of the lateral approach.Placement of trocars in the left upper quadrant should be performed under laparoscopic visualization, particularly if any degree of splenomegaly exists, because the latter can sig-nificantly reduce the available operating space. As with OS, the splenocolic ligament and the lateral peritoneal attachments are divided with resultant medial mobilization of the spleen. The short gastric vessels may be divided usually with hemo-static energy sources such as ultrasonic dissection, diathermy, or radiofrequency ablation. With the lower pole of the spleen gently retracted, the splenic hilum is accessible to further appli-cations of clips or an


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Surgery_Schwartz. such as ultrasonic dissection, diathermy, or radiofrequency ablation. With the lower pole of the spleen gently retracted, the splenic hilum is accessible to further appli-cations of clips or an endovascular stapling device. The splenic artery and vein are divided separately when possible. Good long-term outcomes, however, are increasingly being achieved with mass hilar stapling (Fig. 34-8). Using the lateral approach with the spleen thus elevated, the surgeon can easily visualize the tail of the pancreas and avoid injury when placing the endo-vascular stapler within the sack and allows piecemeal extrac-tion; a blunt instrument should be used to disrupt and remove the spleen to avoid the risk of sack rupture, spillage of contents, and subsequent splenosis (Fig. 34-9 and Fig. 34-10).Hand-Assisted SplenectomyWhen LS is performed in patients with splenomegaly, there have been reports of high rates of both complications and con-version to open splenectomy.137 Hand-assisted laparoscopic


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Surgery_Schwartz. SplenectomyWhen LS is performed in patients with splenomegaly, there have been reports of high rates of both complications and con-version to open splenectomy.137 Hand-assisted laparoscopic surgery (HALS) has been described as an alternative to the LS approach.24,138,139 Spleens greater than 22 cm in craniocau-dal length or 19 cm in width may benefit from HALS over a purely laparoscopic approach.139 It has been reported that the Brunicardi_Ch34_p1517-p1548.indd 153423/02/19 2:37 PM 1535THE SPLEENCHAPTER 34Figure 34-8. Splenic hilum can be divided laparoscopically en masse once the spleen has been rotated medially having been mobi-lized from its lateral attachments.ABFigure 34-9. Spleen extraction. A. Spleen is placed into a ripstop nylon bag before morcellation. B. Splenic morcellation.use of this technique has resulted in a marked reduction in aver-age operative time for patients with massive splenomegaly (146 vs. 295 minutes). Although HALS does require a small incision (7–8 cm)


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Surgery_Schwartz. of this technique has resulted in a marked reduction in aver-age operative time for patients with massive splenomegaly (146 vs. 295 minutes). Although HALS does require a small incision (7–8 cm) for hand insertion and specimen extraction, no differences in length of stay were observed when comparing these patients to those managed purely laparoscopically.139When performing HALS splenectomy, patient position-ing is similar to that of LS (Fig. 34-11). For patients with mas-sive spleens, lateral positioning is altered slightly, such that the patient is placed supine with the left side elevated at 45°. Depending on the hand dominance of the surgeon, the hand-assist device can be placed in either a midline position for right-hand dominant or a subcostal position for left-hand dominant surgeons. A 7to 8-cm incision should be made 2 to 4 cm caudal to the inferior pole of the enlarged spleen. The hand-assisted technique allows for a tactile feedback and atraumatic manipu-lation of the


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Surgery_Schwartz. A 7to 8-cm incision should be made 2 to 4 cm caudal to the inferior pole of the enlarged spleen. The hand-assisted technique allows for a tactile feedback and atraumatic manipu-lation of the enlarged spleen.The nondominant hand provides medial retraction and rotation of the spleen through a hand-assist port, while the domi-nant hand carries out the dissection using laparoscopic instru-ments. The anterior and posterior attachments of the spleen are taken down as in the laparoscopic approach, and the hilar pedicle is ligated using an endoscopic vascular stapler.Single-Incision Laparoscopic Surgery SplenectomySingle-incision laparoscopic surgery (SILS) splenectomy emphasizes the concept of surgery through one small transab-dominal incision rather than the standard multiple trocar sites, with theoretical benefits of less pain and better cosmetics. The incision can be hidden periumbilically and is used as the specimen extraction site at the end of the case. This approach for solid organs


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Surgery_Schwartz. theoretical benefits of less pain and better cosmetics. The incision can be hidden periumbilically and is used as the specimen extraction site at the end of the case. This approach for solid organs poses several technical challenges. Since all instruments are closely aligned together, “fencing” of instru-ments and the laparoscope and limited degrees of movement are commonly encountered. The spleen, being a solid organ, cannot be grasped, and thus retraction may be more challeng-ing in these cases. Furthermore, it has been reported that peri-umbilical port position may result in technical challenges when dealing with high body mass index or tall patients, precluding Brunicardi_Ch34_p1517-p1548.indd 153523/02/19 2:37 PM 1536SPECIFIC CONSIDERATIONSPART IIFigure 34-10. Morcellation and extraction of the spleen within nylon sac extending through the 10-mm trocar site. (Reproduced with per-mission from Park A, Marcaccio M, Sternbach M, et al: Laparoscopic vs open splenectomy, Arch Surg.


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Surgery_Schwartz. of the spleen within nylon sac extending through the 10-mm trocar site. (Reproduced with per-mission from Park A, Marcaccio M, Sternbach M, et al: Laparoscopic vs open splenectomy, Arch Surg. 1999 Nov;134(11):1263-1269.)the surgeon from adequately reaching the spleen. Other alter-natives to single port placements have been reported, although to date, no proven benefits of SILS splenectomy have been demonstrated.140-142Robotic SplenectomyThe da Vinci surgical robot (Intuitive Surgical, Sunnyvale, CA) was cleared by the U.S. Food and Drug Administration (FDA) for use in humans in the year 2000 and has been applied to clinical practice in a variety of abdominal procedures includ-ing splenectomy since 2002.143,144 The term robotic surgery, referencing the da Vinci device should more accurately be described as computer assisted surgery (CAS), as it requires a surgeon sitting at a console controlling distant end effectors. A truly robotic, automated system has not yet been devised nor


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Surgery_Schwartz. be described as computer assisted surgery (CAS), as it requires a surgeon sitting at a console controlling distant end effectors. A truly robotic, automated system has not yet been devised nor deployed to perform splenectomy. The reported advantages of CAS have inspired many surgeons to investigate its potential and to broaden its application in the minimally invasive surgery armamentarium. Some of these advantages include increased degrees of freedom as compared to standard “straight-stick” lap-aroscopy, improved optics including three-dimensional imag-ing of the operative field, improved instrument stabilization and reduction in hand tremor, and finally purported enhanced ergonomic and comfort factors for the operating surgeon.145-147 However, detractors of this emerging technology cite high capi-tal expenses as well as on-going disposable costs and the loss of haptic feedback as major downsides of CAS such that the benefits of the robotic platform are still not fully clear in the


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Surgery_Schwartz. high capi-tal expenses as well as on-going disposable costs and the loss of haptic feedback as major downsides of CAS such that the benefits of the robotic platform are still not fully clear in the extant literature. Recent studies suggest that ergonomic ben-efits of robotic surgery may not be as pronounced as previously thought, as “robotic surgeons” still manifested chronic pain related to poor ergonomics.148There have been few published works comparing conven-tional laparoscopic splenectomy to CAS splenectomy. In one recent retrospective case-matched analysis, the authors com-pared hospital length of stay, operating room times and cost between these two groups. Although they were able to conclude that the application of CAS was feasible and safe for splenec-tomy, they cited a nearly 30-minute increase in operative time and over $4000 increase in cost of the procedures.149 This is consistent with a prior study supporting the notion that robotic-assisted splenectomy takes longer and


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Surgery_Schwartz. increase in operative time and over $4000 increase in cost of the procedures.149 This is consistent with a prior study supporting the notion that robotic-assisted splenectomy takes longer and is more costly than con-ventional laparoscopic surgery.150Splenectomy has also been reported using a single-incision CAS approach.151 However, this procedure is likely associated with an even steeper learning curve than traditional multiport robotic-assisted splenectomy, which calls into question the util-ity of this technique.Robotic splenectomy has been performed in the pediatric population, also proving feasibility and safety, but much like the adult population, larger series are needed to determine the true benefits of this procedure as compared to conventional laparo-scopic splenectomy in children.152The role of CAS in the patient requiring more complex surgery, for example, partial splenectomy or resection of a mas-sive spleen, is yet to be determined. At present there appears to be no


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Surgery_Schwartz. role of CAS in the patient requiring more complex surgery, for example, partial splenectomy or resection of a mas-sive spleen, is yet to be determined. At present there appears to be no argument supporting the application of CAS over standard laparoscopy for routine splenectomy in terms of clinical or cost advantage. The entry into the market of competing devices is likely to favorably alter these considerations.Partial SplenectomyThe past few decades have witnessed ever-widening endorse-ment for and practice of partial splenectomy. This technique, initially reported in the early 18th century, is particularly indi-cated to minimize the risk of postsplenectomy sepsis in children. Certain lipid storage disorders leading to splenomegaly (e.g., Gaucher’s disease), some forms of traumatic splenic injury (blunt and penetrating), spherocytosis in children, and focal benign splenic lesions are amenable to treatment with partial splenectomy.153 Both the laparoscopic and open approaches for


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Surgery_Schwartz. injury (blunt and penetrating), spherocytosis in children, and focal benign splenic lesions are amenable to treatment with partial splenectomy.153 Both the laparoscopic and open approaches for partial splenectomy have been well described.153 The spleen must be adequately mobilized, and the splenic hilar vessels attached to the targeted segment, ligated, and divided. The devascularized segment of spleen is transected along an obvi-ous line of demarcation.A useful technical tip is to transect the parenchyma 1 cm inside the ischemic demarcation line to minimize blood loss.154 Bleeding from the cut surface of the spleen usually is limited and can be controlled by various methods, including cauter-ization, argon coagulation, or application of direct hemostatic agents such as cellulose gauze and fibrin glue.Inadvertent Intraoperative Splenic InjuryInadvertent intraoperative injury to the spleen is a noted occur-rence in the surgical literature, familiar to and dreaded by the abdominal


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Surgery_Schwartz. and fibrin glue.Inadvertent Intraoperative Splenic InjuryInadvertent intraoperative injury to the spleen is a noted occur-rence in the surgical literature, familiar to and dreaded by the abdominal surgeon. The true incidence is unknown. The gravity of such injury is not to be underestimated. Significant short-term morbidity is associated with injury to the spleen, including increased blood loss, need for transfusion, and pro-longed hospital stay.155Intraoperative injury to the spleen has been linked with numerous operations, such as gastric fundoplication, colectomy, Brunicardi_Ch34_p1517-p1548.indd 153623/02/19 2:37 PM 1537THE SPLEENCHAPTER 34BCAFigure 34-11. A. Patient table placement for hand-assisted laparoscopic splenectomy (HALS) in case of splenomegaly. B and C. Intraopera-tive images of HALS.Brunicardi_Ch34_p1517-p1548.indd 153723/02/19 2:37 PM 1538SPECIFIC CONSIDERATIONSPART IIparaesophageal hernia repair, nephrectomy, and abdominal and pelvic vascular surgery. There


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Surgery_Schwartz. images of HALS.Brunicardi_Ch34_p1517-p1548.indd 153723/02/19 2:37 PM 1538SPECIFIC CONSIDERATIONSPART IIparaesophageal hernia repair, nephrectomy, and abdominal and pelvic vascular surgery. There are also reports of splenic injuries after endoscopic procedures, such as colonoscopy.Improper traction on the spleen against its peritoneal attachments is the most common mechanism of intraopera-tive injury. Capsular tears are the most common type of injury, but parenchymal lacerations and subcapsular hematomas also occur. The lower pole is more commonly injured, owing to its orientation and the greater concentration of peritoneal attach-ments found here.As with all hemorrhage, prompt temporary control of bleeding should be obtained by direct compression of the spleen itself, packing of the left upper quadrant, compression of the vessels at the splenic hilum, or pressure on the splenic artery at the superior pancreatic margin. The spleen should then be mobi-lized from its peritoneal


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Surgery_Schwartz. the left upper quadrant, compression of the vessels at the splenic hilum, or pressure on the splenic artery at the superior pancreatic margin. The spleen should then be mobi-lized from its peritoneal attachments and the nature of the injury assessed. Overall, the patient’s condition is the primary deter-minant of whether splenic salvage can be attempted, although hilar injury is best managed by splenectomy. When dealing with capsular tears (most common injury), strong consideration should be given to splenorrhaphy techniques: application of topical hemostatics, suture plication of disrupted parenchyma with or without omental buttress, and the use of bioabsorbable mesh sheets.The time-honored surgical tenets of liberal exposure and visualization are particularly germane to the avoidance of splenic injury. Incisions and approaches must be tailored to both patient circumstances and surgeon experience. There is some evidence to support the assertion that use of the lapa-roscopic approach


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Surgery_Schwartz. splenic injury. Incisions and approaches must be tailored to both patient circumstances and surgeon experience. There is some evidence to support the assertion that use of the lapa-roscopic approach may reduce the incidence of splenic injury for certain operations. As with all hemorrhage, prompt tempo-rary control of bleeding is required. Direct compression of the spleen itself, packing of the left upper quadrant, compression of the vessels at the splenic hilum, or pressure on the splenic artery at the superior pancreatic margin can slow or stop hem-orrhage and allow more deliberate consideration of manage-ment options.The type of injury plays a role as well; it has been sug-gested that hilar injury is best managed by splenectomy.80 Bar-ring these unfavorable circumstances, however, and recalling that the majority of intraoperative splenic injuries are capsular tears, it is reasonable to expect that splenic preservation can be achieved in many appropriately selected situations.


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Surgery_Schwartz. and recalling that the majority of intraoperative splenic injuries are capsular tears, it is reasonable to expect that splenic preservation can be achieved in many appropriately selected situations. Presented with one of these situations, the surgeon has at his or her dis-posal a number of useful and well-described splenorrhaphy techniques: application of topical hemostatics, suture plication of disrupted parenchyma with or without omental buttress, and the use of bioabsorbable mesh sheets.Currently, incidental splenectomies during laparoscopic procedures such as colorectal resections are rare events, but they are associated with worse short-term outcomes.156Preoperative Grading Score to Predict Technical Difficulty in Laparoscopic SplenectomyA splenectomy grading system based on preoperative parame-ters was developed to predict the surgical difficulty and morbid-ity for elective laparoscopic splenectomies.157,158 Preoperative data concerning demographic, clinical, pathological,


Surgery_Schwartz

Surgery_Schwartz. parame-ters was developed to predict the surgical difficulty and morbid-ity for elective laparoscopic splenectomies.157,158 Preoperative data concerning demographic, clinical, pathological, anatomi-cal, laboratory and radiological factors were compared with three surgical outcomes: operative time, intraoperative bleeding, and surgical conversion. Four preoperative parameters (male Table 34-3Difficulty ScoreAge≤40 years040–60 years1≥60 years2GenderFemale0.5Male1Pathology groupITP0.5Other benign1Malignant2Spleen weighta<400 gr1400–1000 gr3>1000 gr5Difficulty gradeLow≤4Medium4–6High≥6Note: Minimum possible score: 2 points; maximum possible score: 10 points.aSpleen weight formula: width (cm) × length (cm) × height (cm) × 0.6 = splenic weight in grams.gender, age, type of pathology, and spleen weight) were found to be associated with a difficult splenectomy (Table 34-3). This grading score is simple to calculate from the physical examina-tion, laboratory tests, and US or CT images and


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Surgery_Schwartz. weight) were found to be associated with a difficult splenectomy (Table 34-3). This grading score is simple to calculate from the physical examina-tion, laboratory tests, and US or CT images and could be highly practical in a daily clinical setting. It could facilitate training and development of skills while simultaneously fostering dissemina-tion of laparoscopic procedures.SPLENECTOMY OUTCOMESChanges in blood composition resulting from splenectomy include the appearance of Howell-Jolly bodies and siderocytes. After splenectomy, leukocytosis and increased platelet counts are common as well. Although platelet counts most often rise within 2 days, they may not peak for several weeks in patients with preoperative thrombocytopenia (see “Hematologic Out-comes” later). Similarly, within 1 day after splenectomy, the white blood cell count typically rises, and such elevation may continue for several months.Overwhelming Postsplenectomy InfectionThe prevalence of asplenia in the United States


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Surgery_Schwartz. after splenectomy, the white blood cell count typically rises, and such elevation may continue for several months.Overwhelming Postsplenectomy InfectionThe prevalence of asplenia in the United States is estimated to be 1 million; which is comparable to the number of patients carrying the human immunodeficiency virus (HIV).185,186 As with other forms of immunodeficiency, asplenic patients bear an increased susceptibility to specific types of infections for the remainder of their lives. Asplenic patients are at highest risk for infection with encapsulated organisms, most commonly Streptococcus pneumoniae, but also Haemophilus influenza Brunicardi_Ch34_p1517-p1548.indd 153823/02/19 2:37 PM 1539THE SPLEENCHAPTER 34(in particular subtype B) and Neisseria meningitides.21,185-192 Although the overwhelming majority of splenectomized patients experience no ill consequence from the absence of their spleen, the potentially catastrophic consequences of overwhelming postsplenectomy infection


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Surgery_Schwartz. overwhelming majority of splenectomized patients experience no ill consequence from the absence of their spleen, the potentially catastrophic consequences of overwhelming postsplenectomy infection (OPSI) demand lifelong vigilance and intimate knowledge of appropriate precautions and preven-tative measures.ComplicationsComplications of splenectomy may be classified as pulmonary, hemorrhagic, infectious, pancreatic, and thromboembolic.12,159 Left lower lobe atelectasis is the most common complication after OS; pleural effusion and pneumonia also can occur. Hem-orrhage can occur intraoperatively or postoperatively, present-ing as subphrenic hematoma. Transfusions have become less common since the advent of LS, although the indication for operation influences the likelihood of transfusion as well. Sub-phrenic abscess and wound infection are among the periopera-tive infectious complications. The placement of a drain in the left upper quadrant may be associated with postoperative


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Surgery_Schwartz. as well. Sub-phrenic abscess and wound infection are among the periopera-tive infectious complications. The placement of a drain in the left upper quadrant may be associated with postoperative sub-phrenic abscess and is not routinely recommended. Pancreati-tis, pseudocyst, and pancreatic fistula are among the pancreatic complications that may result from intraoperative trauma to the pancreas during dissection of the splenic hilum.Hematologic OutcomesThe results of splenectomy may be appraised according to the level of hematologic response (e.g., rise in platelet and hemo-globin levels) in those disorders in which the spleen contributes to the hematologic problem. Hematologic responses may be divided into initial and long-term responses. For thrombocyto-penia, an initial response typically is defined as a rise in plate-let count within several days of splenectomy. Reported series demonstrate the effectiveness of LS in providing a long-term platelet response in approximately 80% of


Surgery_Schwartz

Surgery_Schwartz. is defined as a rise in plate-let count within several days of splenectomy. Reported series demonstrate the effectiveness of LS in providing a long-term platelet response in approximately 80% of individuals with ITP (Table 34-4). These results are consistent with the long-term success rate associated with OS.For chronic hemolytic anemias, a rise in hemoglobin levels to >10 g/dL without the need for transfusion signifies a successful response to splenectomy. By this criterion, splenec-tomy has been reported to be successful for the vast majority of patients with chronic hemolytic anemia. For hemolytic anemia due to spherocytosis, the success rate is usually higher, ranging from 90% to 100%.Splenectomy results also may be examined in terms of surgical and postsurgical characteristics, including operative time, recovery time, and morbidity and mortality rates, all of which tend to vary according to hematologic indication (see Tables 34-4 and 34-5).12Results of few prospective, randomized


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Surgery_Schwartz. operative time, recovery time, and morbidity and mortality rates, all of which tend to vary according to hematologic indication (see Tables 34-4 and 34-5).12Results of few prospective, randomized trials comparing LS and OS have been published. However, several recent meta-analyses of published comparative series including 38 papers with more than 2914 patients indicate that the laparoscopic approach typically results in longer operative times, shorter hos-pital stays, lower morbidity rates, similar blood loss, and similar mortality rates compared with OS.12,120,136 Questions of the cost effectiveness of LS persist, although analysis of this issue is hindered by a lack of universally accepted metrics as well as a paucity of recent objective data. Proponents of LS argue that the generally higher operating room charges are offset by the reduced hospital stay and presumably shorter time of lost productivity.160-162 For those institutions with experienced per-sonnel and technical


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Surgery_Schwartz. higher operating room charges are offset by the reduced hospital stay and presumably shorter time of lost productivity.160-162 For those institutions with experienced per-sonnel and technical capability, the laparoscopic approach has emerged as the standard for elective, nontraumatic splenectomy.CancerA Taiwanese population-based study found that individuals who had splenectomy have higher risks of developing certain types of cancer (adjusted hazard ratios were 2.64 and 1.29 for nontraumatic and traumatic reasons, respectively). Splenectomy patients were found to have significantly higher risks in esopha-gus, stomach, liver, other head and neck, non-Hodgkin’s lym-phoma, and leukemia cancers. Although the exact mechanism for the possible association between splenectomy and cancer remains unclear, a plausible explanation is that the spleen is thought to be involved in immunological defenses and provides active response through humoral and cell-mediated pathways and that splenectomy may


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Surgery_Schwartz. a plausible explanation is that the spleen is thought to be involved in immunological defenses and provides active response through humoral and cell-mediated pathways and that splenectomy may impair immune surveillance in the host.163,164Table 34-4Outcome after splenectomyOUTCOME VARIABLESTUDIES (N)PATIENTS (N)POOLED RESULTS AND CIPMortality382914–0.01 (–0–02, 0)1Complications Pooled382914–0.11 (–0.16, –0.05)<.00001 Minor362914–0.03 (–0.05, –0.01).13 Severe362745–0.07 (–0.11, –0.03)<.00001 Thrombosis352695–0.01 (–0.02, 0.01)1 Organ injury3426390.01 (–0, 0.02)1Acces. spleen2921350.02 (–0.01, 0.05).87Operative time18137057.4 min (43.3, 71.4)<.00001Blood loss10759–41 mL (–87, 4.71)<.00001Length of stay201566–2.48 d (–2.89, –2.07)<.00001Reproduced with permission from Bai YN, Jiang H, Prasoon P. A meta-analysis of perioperative outcomes of laparoscopic splenectomy for hematological diseases, World J Surg. 2012 Oct;36(10):2349-2345.Brunicardi_Ch34_p1517-p1548.indd 153923/02/19 2:37 PM


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Surgery_Schwartz. P. A meta-analysis of perioperative outcomes of laparoscopic splenectomy for hematological diseases, World J Surg. 2012 Oct;36(10):2349-2345.Brunicardi_Ch34_p1517-p1548.indd 153923/02/19 2:37 PM 1540SPECIFIC CONSIDERATIONSPART IIUltrasoundUltrasound is the least invasive mode of splenic imaging. It is rapid, relatively easy to perform, and does not expose the patient to ionizing radiation. It is often the first imaging modality used to evaluate the spleen in a trauma patient, although questions of sensitivity and specificity remain.133,167 In the elective setting, such as for routine diagnostic purposes or for preoperative plan-ning, it is the least costly modality available, and the sensitivity of ultrasound for detecting textural lesions of the spleen can be quite good in experienced hands.168When examining a normal spleen, differentiation between red and white pulp is not possible, and a homogeneous acous-tic echotexture should be visualized. Splenic artery and vein patency may


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Surgery_Schwartz. hands.168When examining a normal spleen, differentiation between red and white pulp is not possible, and a homogeneous acous-tic echotexture should be visualized. Splenic artery and vein patency may be assessed using Doppler imaging. Splenic artery anatomy has been classified commonly into two patterns: dis-tributed and magistral. This variability in vascular distribution visualized by Doppler imaging becomes important when there is consideration of performing a partial splenectomy due to the possibility of segmental blood flow and its effect on area of resection.Percutaneous ultrasound-guided procedures for splenic disease (e.g., cyst aspiration, biopsy), historically avoided due to the risk of hemorrhage and other complications, are becom-ing more common as the safety of these procedures has become increasingly demonstrated.165,166Computed TomographyCT affords a high degree of resolution and detail of the splenic parenchyma, vasculature, and its relationship to neighboring