Cholecystectomy in children with sickle-cell disease

Cholelithiasis is a common complication of sickle-cell disease (SCD); its frequency is variable, ranging from 4% to 55%. Twenty-two children with SCD (Hb SS) underwent cholecystectomy for cholelithiasis. All were managed with a preoperative transfusion regimen to achieve a hemoglobin concentration of 10–12 g/dl and a hematocrit of 30%–40%. On the evening before surgery, each patient was hydrated with IV fluids (5% dextrose in 1/2 or 1/4 normal saline) at 1.5 times the maintenance rate and postoperatively they received IV morphine infusions for analgesia. Intraoperative cholangiograms revealed common bile duct (CBD) stones in 7 patients (30%), only 4 of which were detected preoperatively by ultrasound (US). Two patients also had a splenectomy together with the cholecystectomy, 1 for a splenic abscess and the other for recurrent sequestration crises. There was no mortality, but 2 patients developed acute chest syndrome, 1 developed a minor wound infection, and 1 had postoperative bleeding from the splenic bed. Careful perioperative management permits safe cholecystectomy in children with SCD. Routine intraoperative cholangiography is recommended in these cases, as while 7 of our patients had CBD stones, only 4 of them were detected preoperatively by US.