Abstract
Purpose: There is no strategy for removing gallstones in pediatric patients with hereditary spherocytosis (HS). The purpose of this study was to review the results of splenectomy and cholecystectomy for HS and to clarify the treatment strategy, especially for patients with gallstones.
Methods: We reviewed data from January 2000 to December 2023. The following endpoints were examined retrospectively: sex ratio, age at diagnosis, age at surgery, surgical procedures, preoperative complications, and chronological changes in gallstone complications (gallstones and biliary sludge).
Results: Eleven patients (two boys and nine girls) were included. Four patients were diagnosed in the neonatal period, two in the 0–3 age group, and five in the ≥4 age group. Preoperative complications included hemolytic attacks (7; 5 of which required blood transfusion), cholecystitis (1), gallstones (3), and biliary sludge (2). One patient showed a gallstone whose size increased from 3 mm (at age 10 years) to 7 mm (at age 13 years) and 10 mm (at age 14 years). The ages at surgery were 5 years in two patients and 6 years or older in nine patients [median 10 years (6–14 years)]. Laparoscopic splenectomy (6), laparoscopic splenectomy + laparoscopic cholecystectomy (3), laparoscopic splenectomy + open cholecystectomy (1), and laparoscopic splenectomy + open cholecystectomy + common bile duct incision stone removal (1) were conducted.
Conclusion: HS patients with gallstones may have adverse events due to gallstones, and pediatric surgeons should follow up on the indication for cholecystectomy over time using ultrasonography and decide on the timing of splenectomy and cholecystectomy in collaboration with pediatricians.
