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Holzheimer RG, Mannick JA, editors. Surgical Treatment: Evidence-Based and Problem-Oriented. Munich: Zuckschwerdt; 2001.

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Surgical Treatment: Evidence-Based and Problem-Oriented.

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Splenectomy for hematological disorders

, M.D., FACS and , M.D.

Author Information

, M.D., FACS1 and , M.D.2.

1 Karl-Franzens University School of Medicine, Department of Surgery,
2 Division of Hematology, Graz, Austria

Introduction

The spleen is one of those few organs whose morphology and function long remained unstudied and whose real importance was unrecognized. The spleen is one of the largest lymphatic organ and the only one that is directly connected to the blood-circulatory system. The spleen has only efferent lymph vessels, which originate in the white pulp. It makes an important contribution to the cellular and humoral immune response, in addition to its filter, catabolic and reservoir functions. Phagocytosis, antibody synthesis, production of lymphocytes and cellular immunity are the most important immunologic functions. As a filter organ, it strains senescent and/or defective red blood cells out of the circulating blood. Its “pitting” function removes cellular inclusions like Heinz and Howell Jolly bodies without destruction of erythrocytes. The spleen is the largest reservoir and disposal site for blood platelets (Uranüs 1995).

Primary splenic disease is rather rare; the spleen is often involved in hematological disorders. Primary tumors are not often found in the spleen, and tend to be benign cystic lesions or hamartomas.

The operation performed most often on the spleen for hematological disorders is splenectomy. Only in some indications such as Gaucher's disease has partial splenectomy replaced total splenectomy. In rare cases with unclear splenomegaly and when blood work and marrow puncture are inconclusive, a diagnostic partial resection can be indicated. (table 1)

Table I. Indications for total/partial Splenectomy.

Table I

Indications for total/partial Splenectomy.

There are four basic reasons to perform a splenectomy for hematologic indications:

a.

to treat a disease in which blood cells are destroyed in the pulpa;

b.

to prevent splenomegaly and hypersplenism;

c.

to stage Hodgkin's disease;

d.

to clarify unclear splenomegaly with non-distinctive hematologic features.

Splenectomy

Preoperative preparation

All elective splenectomy patients should receive pneumococcus vaccine. The immunization should be given at least 1 week before the scheduled operation; 3 weeks is preferable. If not done beforehand, the immunization should be administered 2 weeks after surgery, presuming that the patient is free of infection. The procedure is the same for children. There is no evidence-based data to support long-term antibiotic prophylaxis and decisions in favor of or against it should be made on an individual basis.

Operative procedure

Open splenectomy

The incision is chosen depending on the surgeon's preference and the disease process. A midline incision is used for staging laparotomies and in cases with highly enlarged spleens. Otherwise the left subcostal approach is suitable. After the abdominal cavity has been carefully explored for potential accessory spleens, the lesser sac is accessed through the gastrocolic ligament, while the gastroepiploic arcade at the greater curvature is spared. The splenic artery is ligated prehilar and as close to the spleen as possible. The vein is snared. In order to maintain venous blood flow during mobilization, it remains open for the time being. This minimizes blood loss, especially with large spleens. Mass ligatures and indiscriminate clamping must be avoided. If the superior polar artery branches off early from the splenic trunk artery or if the splenic artery ramifies early and distant to the hilus, the branches must be ligated separately. A ligature near the coeliac artery should be avoided in order to maintain the integrity of the pancreatic branches of the splenic artery.

After the preliminary ligation of the splenic artery the gastrosplenic ligament and the short gastric vessels are cut between the ligatures. Further mobilization is carried out dorsally from caudal to cranial. With the left hand the spleen is elevated ventrally and medially, so that the lateral attachments are tightened and severed only a few centimeters away from the spleen. Any intraligamentous vessels are coagulated or ligated. Mobilization should be so performed as to avoid injury to the capsule and blood loss. In splenomegalies, bleeding can impede the progress of the operation and further increase the inevitable blood loss from the large splenic reservoir.

After complete mobilization a large pad is placed into the splenic bed. The splenic vein is ligated and the hilus completely severed. After removal of the spleen the splenic bed is carefully searched for possible residual bleeding. Drainage of the splenic bed is only necessary when contamination occurred during the operation or when there are organic lesions. The rapid development of ultrasonography has obviated the need for drains as a safety measure to indicate postoperative bleeding.

Partial resection of the spleen

A partial resection of the spleen may be necessary as a diagnostic procedure in suspected hematological disorders with inconclusive hematologic findings, or in Gaucher's disease with significant splenomegaly.

After full mobilization and rotation onto the abdominal wall, the vessels of the part to be resected are selectively ligated and severed. The spleen is compressed digitally at the demarcation line and the parenchyma is thereby squeezed toward the part to be removed. A stapler is used to perform the resection (Uranüs 1996, Uranüs 1995). The cut edge is sealed with fibrin glue and collagen fleece.

Laparoscopic splenectomy

Laparoscopic splenectomy is a technically difficult but feasible procedure that may be performed in patients with hematologic diseases with efficacy; morbidity and mortality rates are similar to those of traditional splenectomy for the same indications (Flowers 1995); it is a reliable procedure for patients with spleens less than 20 cm long. An important prerequisite for this technique is the proper positioning of the patient on the operating table. The patient is placed in a semilateral position at an angle of about 30–40°. The left arm is elevated above the head, permitting a better approach to the spleen via the left thoracic aperture. By turning the table at the beginning of the operation with the patient in this position, the patient can be brought into a supine position with slightly lowered pelvis (reverse Trendelenburg position) so that pneumoperitoneum can be established and the trocars inserted.

To prevent air leakage, the skin incisions for the trocars should be kept small. The camera is inserted supraumbilically through an 11/12 mm trocar ; this port can also be used as necessary for other instruments. Usually we use a 30° angle camera. Under direct video control and with transilluminated abdomen, four other trocars are inserted. One of them is placed in the left subcostal area. This port is usually used for an endo-retractor to elevate the spleen gently. The third port with 11/12 mm diameter is inserted at the midpoint between these two trocars and some 3 cm caudally. This is the main working trocar. The fourth trocar is also 11/12 mm and is some 4 cm supraumbilical and to the right of the midline; it is for the forceps or dissector. The fifth trocar, 5–10 mm in diameter, is positioned subxyphoidally on the midline and serves to retract the liver (fig. 1 and 2).

Figure 1. Trocar and instrument positions during laparoscopic splenectomy due to ITP.

Figure 1

Trocar and instrument positions during laparoscopic splenectomy due to ITP.

Figure 2. The same patient as in figure 1 two weeks after discharge.

Figure 2

The same patient as in figure 1 two weeks after discharge.

The operation begins with a gentle blunt dissection of the omentum and its adhesions to the hilum with a harmonic scalpel. After dissection of the lower and upper polar vessels and ligation with hemoclips, the splenic artery and vein are dissected with an endo stapler. The harmonic scalpel can also be used to dissect the short gastric vessels. For further preparation the spleen is retracted ventromedially. The dissection progresses bluntly from the lower pole to the upper on the posterior aspect. The peritoneum is cut with the electrocoagulator and dissected bluntly. The attachments to the left colic flexure are vascularized and should be ligated with hemoclips or dissected by harmonic scalpel. The freed spleen should be removed in a bag. The specimen is extracted by morcellation or intact by enlarging a trocar incision. The spleen can be extracted by enlarging a trocar incision or by morcellation since exact pathology is not required for ITP (idiopathic thrombocytopenic purpura) patients (fig. 3). It is very important not to lose any splenic fragments in the abdominal cavity (Uranüs 1995) to prevent postoperative splenosis.

Figure 3. Moderate splenomegaly with ITP.

Figure 3

Moderate splenomegaly with ITP. Specimen from patient in figures 1 and 2.

Postoperative management

A nasogastric tube should be left in place for up to 24 hours after open surgery. After laparoscopic splenectomy it can be removed upon leaving the operating room. Oral feeding can begin the next day. Oneshot perioperative antibiotic prophylaxis with penicillin is very suitable. If it is necessary to give an ITP patient a platelet transfusion at all, it should only be administered postoperatively. Thrombosis prophylaxis should be routine.

Congenital red cell disorders

Three types of congenital disorders are associated with hemolytic anemia and may warrent spleenectomy:1-disorders of the erythrocyte membranes, 2-hemoglobinopathies, 3-erythrocyte enzyme deficiencies.

Autoimmune hemolytic anemia may be idiopathic or may develop in the course of other disorders, such as malignant lymphomas, chronic lymphatic leukemia, Inpus erythematosus and others. This disease is often associated with mild to moderate splenomegaly. Autoimmune hemolytic anemias are classified as either warm or cold acting agglutinin syndromes. The warm type is the most common (70%). In these cases the spleen mostly serves both in the production of the antibody and as the site of erythrocyte destruction. The majority of cases of autoimmune hemolytic anemia run a self-limiting course and undergo spontaneous remission. Anemia may necessitate splenectomy when steroids anrd other immunsuppressive agents are ineffective (Schwartz 1997).

Hereditary spherocytosis

Hereditary spherocytosis is the prototype of congenital disorders of the erythrocyte cell membrane. It is characterized by prominent splenomegaly and anemia. Hereditary spherocytosis is transmitted as an autosomal dominant trait. Spherocytes are less deformable than normal erythrocytes, owing to their structural abnormality, rendering them incapable of transversing the splenic sinus wall. The anemia is rarely associated with thrombocytopenia or leukopenia. The majority of patients with hereditary spherocytosis have a benign course, with hemolysis compensated by erythroid hyperplasia of the bone marrow, although there may be acute aplastic crises, usually associated with infection (Jandl 1983, Wolf 1989). Splenectomy is indicated when conservative treatment is ineffective. Late relapse after splenectomy can occur in up to 20% (Schwartz 1997).

Sickle cell anemia

Sickle cell disease is the most common hemoglobinopathy. It is characterized by the substitution of valine for glutamic acid in the hemoglobin beta chain. It is a hereditary disease which is seen more commonly in blacks. Sickling is initially a reversible phenomenon, but with repeated episodes membrane damage results in irreversibly sickled cells (Wolf 1989). Splenomegaly is rare due to progressive atrophy from repeated infarctions. The chronic anemia and associated jaundice are often accompanied by acute symptoms such as acute abdominal pains. Splenic abscess can occasionally follow infarction of the spleen. Splenectomy usually does not stop the sickling process, but it may improve the anemia.

Thalassemia

Thalassemia is a heterogenous group of disorders and occurs in two major degrees of severity: Thalassemia major is the homozygous type with severe symptoms; thalassemia minor is the heterozygous type with insignificant symptoms. The disease is characterized by the diminished production of structurally normal globin chains resulting in an excess of one type of chain. Beta thalassemia is characterized by deficient synthesis of the beta chain, whereas alpha thalassemia results from deficient synthesis of the alpha chain. Patients with thalassemia major require transfusions at regular intervals; the disease usually results in early death. Splenectomy is reserved for symptomatic splenomegaly and recurrent splenic infarction. The postsplenectomy infection rate in these patients is rather high.

Platelet disorders

Idiopathic Thrombocytopenic Purpura

tIdiopathic thrombocytopenic purpura is the most common hematologic indication for splenectomy (Schwarz 1996). ITP may occur as an isolated condition or in association with such other disorders as chronic lymphatic leukemia or systemic lupus erythematosus, in which it may be the presenting manifestation (Karpatkin 1976). The spleen is the major site where the plateledIgG antiplatelet antibody complex is removed by the RHS. The clinical manifestations include ecchymosis, purpura, and sometimes excessive bleeding from the gums or vagina. Gastrointestinal bleeding and hematuria are also common. The platelet count is characteristically less than 50,000/mm3. The spleen is usually only mildly enlarged. The first choice treatments are steroids or IgG and plasmapheresis (Schwartz 1996). Splenectomy is usually performed only in cases in which corticosteroid therapy fails to achieve remission. A very important part of the operative procedure is the careful search for accessory spleens. Remaining accessory spleens or accidentally reimplanted splenic tissue may cause recurrent thrombocytopenia after splenectomy.

Thrombotic Thrombocytopenic Purpura (TTP)

This rare disorder is characterized by microthrombi in arterioles, capillaries and venules of many organs and causes fever, transient neurologic deficits and renal failure, accompanied by hematologic changes. The etiology is unclear, although it has been postulated that the disease is autoimmune in origin. The first line therapy is plasmapheresis, which is usually effective. When plasmapheresis is ineffective, splenectomy is indicated.

Hypersplenism

Hypersplenism is characterized by enlargement of the spleen, decrease in the number of peripheral cellular blood elements and increasing hemocytopoiesis in the bone marrow. According to Crosby (1972), a further characteristic sign of this disease is the improvement or complete normalization of the peripheral blood picture after splenectomy. Hemocytopoiesis does not depend on the genesis of splenomegaly, but only on the compensatory functional capacity of the bone marrow. Its intensity varies and can affect one or more corpuscular elements of the blood. Peripheral cytopenia can even lead to pancytopenia.

Congestive splenomegaly is a particularly frequent form of hypersplenism. It is caused by portal hypertension. The increase in pressure in the portal circulation is mostly due to hepatic cirrhosis, hepatic vein thrombosis, pyelothrombosis or thrombophlebitic splenomegaly (Martin 1982).

In addition, cardial congestion can sometimes cause hypersplenism which, however, usually remains minimal.

The treatment of choice in hypersplenism due to defective blood cells is splenectomy (Calavrezos 1981, Mozes 1979). Neverless, it should be performed in patients with liver cirrhosis.

Involvement of the spleen in lymphoproliferative disorders

Hodgkin's disease (lymphogranulomatosis) and non–Hodgkin's lymphomas are grouped together under the heading “malignant lymphomas”. They develop primarily in lymphatic tissue, mostly in lymph nodes. Only in less than 1% of all cases do they occur primarily in the spleen (Remmele 1984).

Hodgkin's disease (lymphogranulomatosis)

The etiology of this disease is still unknown. The elementary histological sign of Hodgkin's disease is Sternberg's giant cell, which develops from the mononuclear Hodgkin cell.

The disease usually begins with painless enlargement of the lymph nodes. In addition, the so-called B-category of symptoms can occur, i.e. systemic symptoms such as intermittent attacks of fever, weight loss, and night sweats.

In all cases, the tentative clinical diagnosis must be confirmed histologically by lymph node biopsy. The disease, which begins primarily in the lymphatic tissue of lymph nodes (in most cases) or in tonsils (rarely), can secondarily involve almost any organs, including the spleen in 40% to 74% (Höllwarth 1978,Powell 1982).

While other staging operations can have a certain therapeutic effect through excision or reduction of a tumor, the goal of the Hodgkin's staging operation is to prove or exclude infradiaphragmatic tumor dissemination.

On the other hand, nobody denies the importance of staging laparotomy with consecutive splenectomy as a diagnostic and prophylactic procedure in certain cases.

Non–Hodgkin's lymphomas (fig. 4, 5)

In non–Hodgkin's lymphomas, the spleen plays only a secondary role (Christo 1962). This is mainly because the tumors become generalized throughout the entire lymphatic system early, and mainly elderly people are affected whose disease is usually advanced once the diagnosis is made (Farthmann 1987).

Figure 4. Elevation of the abdominal wall by a highly enlarged spleen in non–Hodgkin's lymphoma.

Figure 4

Elevation of the abdominal wall by a highly enlarged spleen in non–Hodgkin's lymphoma.

Figure 5. Intraoperative view of the patient in figure 4.

Figure 5

Intraoperative view of the patient in figure 4.

Non–Hodgkin's lymphoma does not spread systematically from one neighboring lymph group to the next. The generally poor condition of the patient, the increased susceptibility to infection and the lack of a positive therapeutic effect are the main reasons that exploratory laparotomy or splenectomy are performed less frequently in these cases (Ochsner 1991). The most common indication for splenectomy with simultaneous abdominal exploration is severe, peripheral cytopenia together with marked splenomegaly (Johnston 1973).

Involvement of the spleen in myeloproliferative disorders

Myeloproliferative diseases form a heterogeneous spectrum with pathological changes in all hemopoietic cells. Acute and chronic leukemias, polycythemia vera, osteomyelofibrosis and essential thrombocythemia belong to this group. Also, myelodysplastic syndromes as well as aplastic and sideroblastic anemias can be categorized in this group if all three hemopoietic cell groups are affected (Scher 1983).

Treatment generally consists of transfusions and chemotherapy. Splenectomy may be indicated for symptomatic splenomegaly and when medical therapy fails to reduce the transfusion requirements (Schwartz 1996).

Miscellaneous Conditions

Felty's Syndrome

This is a rare syndrome of advanced rheumatoid arthritis with splenomegaly and neutropenia with increased risk of infections. Splenectomy is often effective in reversing a profound neutropenia and thereby reducing susceptibility to infections. The arthritis is not affected by splenectomy.

Gaucher's Disease

Gaucher's disease is characterized by the accumulation of glucocerebroside due to the deficiency of the enzyme glucocerebrosidase A. The adult form is often accompanied by significant splenomegaly and enlargement of the lymph nodes but is compatible with long life, in contrast to the acute infantile neuronopathic variety, which is rapidly fatal. Splenectomy can correct the signs of hypersplenism.

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Copyright © 2001, W. Zuckschwerdt Verlag GmbH.
Bookshelf ID: NBK6913

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