Surgical treatment of symptomatic biliary stones in patients with cystic fibrosis

Abstract

Patients with cystic fibrosis have a high incidence of cholelithiasis. However, few studies have addressed the operative therapy for cholelithiasis in this group of patients with poor pulmonary function. We reviewed six patients with cystic fibrosis who were treated for symptomatic biliary stones. Five patients underwent cholecystectomy for chronic cholecystitis. One patient with extremely poor pulmonary status presented with choledocholithiasis and cholangitis, which was successfully treated with endoscopic sphincterotomy followed by ursodeoxycholic acid therapy. Five of these six patients had significant relief of their symptoms. One patient never recovered completely from the operation and 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 cholecystectomy. More conservative treatment is indicated in patients with marginal pulmonary reserve.