Abstract
Twenty-seven children aged 18 years and under with homozygous sickle-cell disease had open cholecystectomy for symptomatic gallstones over the 12-year period 1985-1997. Emergency procedures (done during period of acute exacerbation of symptoms) were performed on 16 patients. Four with haemoglobin levels greater than 1 g/dl below their steady state received a simple blood transfusion preoperatively designed to raise haemoglobin levels to 10 g/dl. All had acute or acute on chronic cholecystitis based on histological examination of gallbladder specimens. Twelve had common bile duct stones. In two patients calculi were missed intraoperatively but these subsequently passed into the duodenum after a period saline irrigation via an in-situ t-tube. Six developed the acute chest syndrome (aetiology not determined) and this progressed to multi system failure and death in one. This high level of postoperative mobility and mortality may in part be due to the high proportion of emergency procedures.
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