Pediatric Splenomegaly Treatment & Management

Because splenomegaly is usually the result of a systemic disease, the primary goal is treatment of the underlying process. In certain circumstances, splenectomy may be the therapy of choice for symptoms or complications caused by the enlarged organ. However, because of the risk of overwhelming sepsis in children who are asplenic, the risks and benefits must be carefully weighed when splenectomy is being considered.^{[11, 12]}

• The new PCV7 conjugated pneumococcal vaccine and the older PPV23 polyvalent pneumococcal vaccines and the Haemophilus influenzae vaccine should be administered to all children who are asplenic and to those about to undergo splenectomy. The polyvalent pneumococcal vaccine is effective only if the patient is aged 2 years or older. Meningococcal vaccine, MCV4, is often administered to these patients in this age group as well. To maximize antibody formation, vaccines should be administered at least 10 days before splenectomy.^[25]
• Daily penicillin is recommended to prevent pneumococcal septicemia in asplenic children younger than 5 years. Antibiotic prophylaxis is often administered for several years after splenectomy in patients older than 5 years, but the role of prophylaxis in these children is less well documented than it is in others.
• In patients with homozygous sickle cell anemia or sickle beta-zero thalassemia, oral penicillin prophylaxis should be started when the diagnosis is established. This therapy should be administered until at least age 5 years. The role of penicillin prophylaxis in patients with hemoglobin sickle cell disease is controversial. Patients with sickle beta+ thalassemia do not appear to need penicillin prophylaxis.
• Febrile illnesses in asplenic children should be approached as potentially life-threatening events and evaluated thoroughly, with a low threshold for treatment with intravenous antibiotics that cover Streptococcus pneumoniae and H influenzae. The increasing prevalence of drug-resistant S pneumoniae and the decreasing incidence of H influenzae infections are complicating factors in determining the optimal choice of antibiotics.

Splenic trauma is the most common indication for splenectomy, although attempts at splenic preservation are increasingly important. Nonsurgical management for splenic trauma has success rates of 52-98%, with failure usually occurring in the first 96 hours. Splenic cysts, tumors, and vascular lesions may also require surgical removal. Whenever possible, splenic tissue is preserved to decrease the risk of septicemia, but total splenectomy is occasionally necessary.^{[26, 27]}

• Splenectomy can cure hypersplenism but is not usually indicated because the secondary cytopenias rarely cause serious problems. However, in patients with portal hypertension, vascular shunts may be necessary to prevent esophageal variceal bleeding.
• Splenectomy may be helpful in improving cytopenias in several medical conditions, including congenital anemias (eg, hereditary spherocytosis, elliptocytosis) and autoimmune disorders (eg, immune thrombocytopenic purpura, autoimmune hemolytic anemia, hypersplenism). In thalassemia major, splenectomy may initially decrease the transfusion requirements caused by hypersplenism. However, the benefit must be carefully weighed against the risk of sepsis.
• In Gaucher disease, splenectomy may be necessary when the mechanical strain of the enlarged spleen requires intervention.
• Splenectomy may be indicated in children with sickle cell anemia and a history of splenic sequestration crisis in order to prevent recurrences of the crisis.
• As part of exploratory laparotomy, splenectomy was once an important component of staging of Hodgkin disease. This procedure is rarely used because of improvements in imaging modalities, the high risk of postsplenectomy sepsis, and the increased use of chemotherapy in patients, which allows treatment decisions to be made on the basis of radiologic evaluation alone. Furthermore, data suggest that splenectomy increases the risk of second malignancy in patients treated for Hodgkin disease.

A pediatric hematologist and/or oncologist is the usual consultant when the cause of splenomegaly is not obvious or when a primary hematologic or oncologic disorder is suspected.

• According to the American Academy of Pediatrics and the Council of Sports Medicine and Fitness, children with acutely enlarged spleens should avoid contact, collision, or limited-contact sports.^[25] Viral-related splenomegaly rarely lasts longer than 2 months.
• The duration for which contact restriction should persist after acute infectious mononucleosis remains uncertain. In the absence of trauma the incidence of splenic rupture is approximately 1:1000, usually occurring in the first 3 weeks of illness. No evidence-based guidelines are available. More conservative suggestions recommend that children with infectious mononucleosis and resolution of splenomegaly noted on examination have 3 weeks of rest with a 4-week graded return to activity. However, it has also been suggested that contact sports can resume as soon as 4 weeks after onset of illness.^[28]
• Even with large spleens, disorders associated with chronic splenomegaly have reduced splenic friability with minimal risk of rupture. In children with chronically enlarged spleens, decision to restrict activity should be made on a case-by-case basis.^[29]