Background/purpose: Hereditary spherocytosis is an autosomal dominant disorder associated with an intrinsic defect in the red blood cell membrane often necessitating splenectomy to prevent sequestration of spherocytes. When cholelithiasis is present, these patients undergo cholecystectomy at the same surgical setting as splenectomy. After splenectomy alone, it is uncertain whether the amount of hemolysis is adequately decreased to prevent subsequent gallstone formation. This study set out to evaluate the frequency in which symptomatic cholelithiasis subsequently develops in children treated by splenectomy alone.
Methods: All patients less than 18 years old with hereditary spherocytosis who underwent splenectomy without cholecystectomy at our institution during the past 27 years were included in this study. A retrospective chart review and telephonic patient follow-up was performed. Gallstones were excluded in these patients either by preoperative ultrasound scan, or by intraoperative palpation of the gallbladder. The main study outcomes of this group included documented cases of cholelithiasis, subsequent need for cholecystectomy secondary to cholelithiasis, and questionnaire to determine the incidence of "subclinical" cholelithiasis (not reported to a physician).
Results: Twenty-three subjects were identified who met the inclusion criteria. Complete follow-up data were obtained for 17 of these patients (74%). The mean age at splenectomy was 6.6 +/- 0.69 years, and the mean follow-up was 15.65 +/- 2.03 years (median, 18 years). None of the patients in this series subsequently have undergone cholecystectomy, nor have any had either clinical or subclinical evidence of cholelithiasis since splenectomy.
Conclusion: Prophylactic cholecystectomy at the time of splenectomy is not indicated in patients with hereditary spherocytosis who do not have gallstones.