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