Patients with cystic fibrosis have a high incidence of cholelithiasis.
However, few studies have addressed the operative therapy for choleli
thiasis in this group of patients with poor pulmonary function. We rev
iewed six patients with cystic fibrosis who were treated for symptomat
ic biliary stones. Five patients underwent cholecystectomy for chronic
cholecystitis. One patient with extremely poor pulmonary status prese
nted with choledocholithiasis and cholangitis, which was successfully
treated with endoscopic sphincterotomy followed by ursodeoxycholic aci
d therapy. Five of these six patients had significant relief of their
symptoms. One patient never recovered completely from the operation an
d eventually died from continued pulmonary deterioration. We conclude
that in patients with cystic fibrosis and symptomatic biliary stones,
careful attention to pulmonary care can afford safe, elective cholecys
tectomy. More conservative treatment is indicated in patients with mar
ginal pulmonary reserve.