Perioperative complications of surgical procedures are frequently encountered in patients with sickle cell disease. We have reviewed our series of patients with hemoglobinopathies who underwent cholecystectomy from 1978 to 1991 to evaluate their perioperative management and clinical outcome. Twenty-two children with major sickle hemoglobinopathy underwent cholecystectomy for symptomatic cholelithiasis. All 22 were transfused to achieve a hemoglobin (Hgb) level greater than 9 g/dL and hemoglobin S (HbS) less than 37%. Fourteen underwent immediate preoperative automated red cell exchange (ARCE). The median preexchange Hgb of these patients was 8.1 g/dL (range, 6.8 to 10.5). Their median HbS was 84% (range, 53% to 97%). These patients underwent placement of an apheresis catheter under local anesthesia followed by red cell exchange. The median volume of packed red blood cells (PRBC) exchanged was 28.1 mL/kg (range, 13.8 to 58.7). The median HbS after exchange was 21% (range, 16% to 37%) and the median Hgb was 10.6 g/dL (range, 6.5 to 16.7). Eight other patients underwent sequential transfusion (3 after an exchange for an acute pulmonary vasoocclusive crisis). These patients had been prepared over an interval of 2 to 8 weeks preoperatively and had received a median of 26.9 mL PRBC/kg (range, 12.8 to 95). Following sequential transfusion the median Hgb was 11.8 g/dL (range, 9 to 15.7) and the median HbS was 19% (range, 5% to 32%) at the time of surgery. All patients received extended antigen matched blood. Complications of preoperative transfusion were minor and included two febrile-/allergic reactions and one mild superficial catheter-induced phlebitis.(ABSTRACT TRUNCATED AT 250 WORDS)