Biliary stone disease in adults with cystic fibrosis

A 27-year-old woman with cystic fibrosis had a 6-month history of fatty food intolerance and biliary colic. Ultrasonographic studies confirmed the presence of gallstones. A cholecystectomy and appendectomy were performed. An intraoperative cholangiogram showed anomalous drainage of the cystic duct into an accessory right hepatic duct. Patients with cystic fibrosis now commonly survive into adulthood and are at high risk for the development of cholelithiasis. The diagnosis may be obscured by other common gastrointestinal complications of cystic fibrosis. Optimal surgical management includes meticulous preoperative and postoperative pulmonary care. The surgeon must also have a thorough knowledge of the biliary tract anomalies that have been described in patients with cystic fibrosis.