Asplenia is often noted with complex congenital heart disease as well as bilateral “right-sidedness” such as bilateral three-lobed lungs, a right-sided stomach, and a central liver (Ivemark syndrome). Intestinal malrotation has also been observed with asplenia. These infants are at risk for severe infection and sepsis and should receive antibiotics for prophylaxis.

Polysplenia usually consists of a cluster of small splenic masses and is often found with biliary atresia. Other associated conditions include a preduodenal portal vein, situs inversus, malrotation, and cardiac defects. In the instance of heterotaxy, the splenic mass(es) are always on one side of the body and along the greater curvature of the stomach. These children have adequate splenic immune function.

Wandering spleen is characterized by a lack of ligamentous attachments to the diaphragm, colon, and retroperitoneum, resulting in a mobile spleen. This is likely due to failure of development of the splenic ligaments from the dorsal mesentery.

This rare condition can present with a variety of symptoms mimicking other abdominal pathologies, which can make diagnosis difficult. Children can present with an abdominal mass and episodic pain, but they can also present acutely with torsion and infarction. ^, Two cases of portal/mesenteric varices due to a wandering spleen have been reported in adolescent patients, both of which resolved with splenectomy. Pancreatitis has also been noted as a presenting sign. Splenopexy is the preferred method of treatment and can be performed with placement of the spleen into a mesh basket, suture splenopexy, colonic displacement with gastropexy, positioning in an omental basket, or placement in an extraperitoneal pocket. The laparoscopic approach is the preferred technique, and the use of an absorbable or nonabsorbable mesh with fixation in the left upper quadrant is shown in Fig. 46.1 . ^, Placement of the spleen in an extraperitoneal pocket is shown in Fig. 46.2 . Cases of torsion with infarction require splenectomy. Chronic torsion has also been reported with massive splenomegaly that may necessitate splenectomy.

Laparoscopic splenopexy with placement of the spleen between two sheets of absorbable mesh with fixation in the left upper quadrant.

© IUSM Visual Media.

(A) The upper pole of the spleen was placed in the retroperitoneal pouch, and the upper aspect of the pouch ( dotted arrow ) was closed with interrupted sutures. Note the splenic vessels ( solid arrow ) coursing into the spleen. A generous opening was left in the pouch for these vessels so that the vessels would not be compressed by closure of the pouch. (B) One of the interrupted silk sutures is being placed to approximate the peritoneal flaps over the spleen. At this point, most of the spleen has been placed into the extraperitoneal pouch.

From Upadhyaya P, St. Peter SD, Holcomb GW III. Laparoscopic splenopexy and cystectomy for an enlarged wandering spleen and splenic cyst. J Pediatr Surg . 2007;42:E23–E27. Reprinted with permission.

Splenic gonadal fusion occurs when the left gonad and the spleen are attached and is a result of early fusion between the two structures prior to descent of the testis. The remnant can be a continuous band or can be discontinuous with splenic tissue attached to the gonad. A splenic remnant has also been noted in the left scrotum as an accessory splenic remnant type of abnormality.

Splenic cysts are categorized into true cysts and pseudocysts. True cysts are lined with epithelium and may be congenital or acquired as a result from a parasitic infection ( Fig. 46.3 ). Splenic pseudocysts have no cellular lining and may arise as a result of trauma, hemorrhage, or inflammation. Patients may present with symptoms related to the size of the cyst, with gastric compression or pain, an abdominal mass, rupture, or infection with an abscess. ^, Simple cysts less than 5 cm can be observed, but cysts that are enlarging, symptomatic, or larger than 5 cm usually require treatment. Most symptomatic cysts are larger than 8 cm. Percutaneous aspiration and sclerosis utilizing alcohol or other agents have been reported with variable success. ^, One study demonstrated sclerosis with alcohol caused disappearance in 20% of pediatric patients.

(A) A large epithelial splenic cyst ( arrow ) is seen on the CT scan. (B) At laparoscopy, the large cyst (seen in [A]) is seen to occupy most of the spleen.

Marsupialization can be performed but has been associated with a high recurrence rate if an adequate amount of cyst wall is not removed ( Fig. 46.4 ). Some have suggested marsupialization followed by lining the cyst with Surgicel (Ethicon, Inc., Somerville, NJ) and performing an omentopexy to reduce risk of recurrence. High recurrence rates with laparoscopic partial excision have also been observed. ^, However, others have reported good success with this technique. Some authors recommend partial splenectomy.

(A) The wall of the large epithelial splenic cyst seen in Fig. 47.4 is being excised. (B) The cyst was marsupialized, and the remnant lining of the cyst was ablated with the argon beam coagulator.

Hereditary hemolytic anemias are a group of disorders including hereditary spherocytosis, sickle cell disease, thalassemia, and pyruvate kinase deficiency and are characterized by damaged red blood cells via a variety of mechanisms. Damaged red blood cells are removed by splenic macrophages, making splenectomy a potential treatment strategy in the management of severely affected patients. The benefit of splenectomy is well-documented in patients with hereditary spherocytosis but is not as clear for other anemias in this group. A summary of splenectomy recommendations for these hemolytic disorders is included in Table 46.1 .

Hereditary spherocytosis (HS), an autosomal dominant condition, is the most common inherited red cell disorder among Northern European descendants, with approximately 25% of affected children representing new mutations. Defects in red cell proteins ankyrin or spectrin result in poorly deformable spherocytes. Most affected children have anemia, an elevated reticulocyte count, and a mild elevation in bilirubin. The degree of hemolysis can vary, with some only having mild anemia. The diagnosis is confirmed with the findings of spherocytes on peripheral smear along with a positive osmotic fragility test. Affected children can develop an aplastic crisis associated with parvovirus B19 infection with suppression of the bone marrow red cell production and ongoing splenic red cell destruction. Splenomegaly is common in these patients.

Splenectomy is the standard treatment for patients with moderate-to-severe disease. Total splenectomy is very effective in reducing hemolysis and increasing hemoglobin levels. As a significant percentage of children with HS will develop pigmented gallstones in the first decade of life, symptomatic cholelithiasis may also be a reason for proceeding with splenectomy (with cholecystectomy). There is generally no indication for cholecystectomy in patients undergoing splenectomy who do not have evidence of cholelithiasis.

Partial splenectomy has become an increasingly popular alternative to total splenectomy for treatment of HS, especially in younger children due to the concern for postsplenectomy infection. There is increasing evidence that partial splenectomy may preserve some splenic function in addition to the hematological benefits in children with HS. Further study is needed to adequately compare surgical techniques and assess long-term outcomes.

Sickle cell disease (SCD) results from an amino acid substitution in the β-chain of hemoglobin A, which results in hemoglobin S. Children may be homozygous or have less severe heterozygous types such as sickle C or sickle thalassemia. These red blood cells become rigid as they pass through the hypoxic environment of the spleen, leading to splenic sequestration.

An acute splenic sequestration crisis can be a life-threatening complication and typically presents with abdominal pain, splenomegaly, acute worsening of anemia, and a high reticulocyte count. Due to the associated morbidity and mortality, severe or recurrent episodes merit consideration for splenectomy. Hypersplenism is the second major indication for splenectomy in these children and has a more insidious onset characterized by chronic splenomegaly, anemia, and thrombocytopenia. Although splenectomy does not appear to significantly improve hematologic parameters, it has been shown to have a greater effect on clinical symptoms by reducing transfusion dependence and reducing sequestration. ^,

The thalassemias are characterized by abnormal production of the α or β chains of hemoglobin. Thalassemia major (β-thalassemia) is associated with the most severe clinical anemia among this group. Splenomegaly causes further red cell sequestration and the need for transfusion. Total or partial splenectomy is performed to decrease the need for transfusions in children with severe anemia.

Immune thrombocytopenic purpura (ITP) occurs due to antiplatelet autoantibodies, which subsequently are destroyed in the spleen. In most children, it is primary (idiopathic), and in some it may be secondary to lupus, human immunodeficiency virus, malignancy, or hepatitis C infection. Most children (80%) have acute ITP that resolves with observation or medical management. Most treatment plans target a decrease in platelet destruction. Management includes corticosteroids, which may have their effect by inhibiting the reticuloendothelial binding of platelet/antibody complexes; intravenous immunoglobulin (IVIG), which inhibits the Fc receptor binding of platelets by macrophages; or Rho(D) immunoglobulin in Rh-positive children, which binds red cells that then saturate the splenic receptors, allowing the platelets to avoid destruction.

Many cases of ITP resolve spontaneously, but 20% of children will have prolonged thrombocytopenia leading to chronic ITP, and traditionally would require surgery. With the expanding medical therapies for ITP such as rituximab and thrombopoietin receptor agonists, surgery is required less frequently. However, splenectomy remains a safe and effective treatment. Laparoscopic splenectomy has been shown to induce a complete response in up to 74% of patients. Additionally, complications from the long-term use of medical therapies are an indication for splenectomy ( Table 46.1 ).

Gaucher disease is characterized by a deficiency of the enzyme β-glucocerebrosidase, resulting in excessive glucocerebroside in the macrophages of the spleen, liver, bone marrow, and lungs. Splenomegaly may be severe, and both partial and total splenectomy have been utilized to alleviate the symptomatic hypersplenism and decrease the destruction of the red blood cells, leukocytes, and platelets ( Table 46.1 ).

Splenic abscess (SA) is rare in children. Immunocompromised states, systemic disease, and trauma are recognized as predisposing factors for SA. The most common organisms isolated from cultured abscesses staphylococci, streptococci, Salmonella , and Escherichia coli . Fungi and mycobacteria may be more common in immunocompromised patients. ^, Infectious etiologies such as tuberculosis, enteric fever, and parasitic infections can be seen in developing countries. Patients typically present with nonspecific symptoms such as fever and abdominal pain and diagnosis is made by ultrasound or computed tomography (CT) scan. Historically, definitive treatment with splenectomy and antibiotics was standard in adults and is still favored by some. However, over the last two decades various combinations of antibiotic therapy, percutaneous drainage, open drainage, and partial or total splenectomy have been described as treatment modalities for SA. There is no widely accepted consensus on management of SA in the pediatric population. There are several reports of children with SA successfully treated with antibiotic therapy and percutaneous drainage, even in children with multiple SAs. Because of the importance of its immunologic function, spleen-sparing modalities are generally recommended when possible, and splenectomy is reserved for those who fail initial treatment or after rupture of SA ( Table 46.1 ).