Surgery and anesthesia in sickle cell disease

Disease observed 3,765 patients with a mean follow-up of 5.3 k 2.0 years. One thousand seventy-nine surgical proce-dures were conducted on 717 patients (77 % sickle cell ane-mia [SS], 14 % sickle hemoglobin C disease [SC], 5.7 % Spo thalassemia, 39'0 Sp+ thalassemia). Sixty-nine percent had a single procedure, 219'0 had two procedures, and the re-maining 11 % had more than two procedures during the study follow-up. The most frequent procedure was abdomi-nal surgery for cholecystectomy or splenectomy (2496 of all surgical procedures, N = 258). Of these, 939'0 received blood transfusion, and there was no association between preoper-ative hemoglobin A level and complication rates (except re-duction in pain crisis). Overall mortality within 30 days of a surgical procedure was 1.1 % (12 deaths after 1,079 surgical procedures). Three deaths were considered to be related to the surgical procedure andlor anesthesia (0.3%). No deaths were reported in patients younger than 14 years of age. ATIENTS WITH sickle cell disease (SCD; which in-cludes sickle cell anemia [SS], sickle hemoglobin C disease [SC], and the sickle 0 thalassemias) who undergo surgery are generally considered to be at greater risk for perioperative complications than otherwise healthy patients without this hematologic disorder.'" ' Favorable outcomes have been reported without transfusion, but perioperative transfusion is commonly used to prepare SCD patients for surgery and to treat complications of sickle cell d i sea~e.4, ~.~~"~ Although the optimal level of sickle hemoglobin (Hb) to be achieved is unknown, most sickle cell centers adhere to some form of transfusion protocol for SCD patients undergoing The Cooperative Study of Sickle Cell Disease (CSSCD) was a natural history study that observed 3,765 patients from 1978 to 1988.26.27 This report analyzes the course and out-come of the 1,079 surgical procedures performed on 717 patients during this time period