BACKGROUND/PURPOSE: The aim of this report is to study the short- and long-term consequences of partial splenectomy (PS) in hereditary spherocytosis (HS).
METHODS: The authors reviewed the files of 5 children who underwent PS from 1993 to 1998. The data collected included clinical presentation, age, gender, indication for the operation, radiographic examination, need for blood transfusion and hematologic values, description of surgical procedure, and early- and late-occurring complications.
RESULTS: From 1993 to 1998, 5 children from one year, 9 months to 7 years of age underwent PS. Indications were hypersplenism and severe anemia. The average age at the time of the operation was 3 years, 5 months. The 2 youngest children (one year, 9 months and 2 years old) needed a second operation because of the recurrence of hypersplenism.
CONCLUSIONS: Subtotal splenectomy seems to preserve the immune role of the spleen and can reduce the need for blood transfusion. But PS is associated with a regrowth of the splenic remnant in children suffering from chronic hemolysis with hypersplenism and seems to be effective for a relatively short period only, especially in young children. Thus, a second operation to perform a total splenectomy can be necessary. The decision to perform a PS on young children with HS as an alternative to total splenectomy with appropriate preoperative vaccination and postoperative prophylactic antibiotics therefore should be weighed carefully, keeping in mind, however, the benefit of postponing total splenectomy in these patients.