Treatment and Prognosis of Symptomatic Gallbladder Disease in Patients with Cystic Fibrosis

• Published in: Journal of pediatric gastroenterology and nutrition Vol. 5; no. 1; pp. 35 - 40
• Main Authors: Stern, Robert C, Rothstein, Fred C, Doershuk, Carl F
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott-Raven Publishers 01.01.1986; Lippincott
• Subjects: Adenoma, Bile Duct - etiology; Adenoma, Bile Duct - surgery; Adolescent; Adult; Biological and medical sciences; Child; Child, Preschool; Cholangitis - etiology; Cholangitis - surgery; Cholecystectomy; Cholelithiasis - etiology; Cholelithiasis - surgery; Cystic Fibrosis - complications; Female; Gallbladder Diseases - etiology; Gallbladder Diseases - surgery; Gallbladder Neoplasms - etiology; Gallbladder Neoplasms - surgery; Gastroenterology. Liver. Pancreas. Abdomen; Humans; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; Medical sciences; Other diseases. Semiology; Prognosis; Human; Biliary tract; Cholecystopathy; Respiratory disease; Hepatobiliary disease; Concomitant disease; Mucoviscidosis; Surgery; Lithiasis; Digestive diseases; Tumor; Gallbladder; Child; Pancreatic disease
• ISSN: 0277-2116; 1536-4801
• DOI: 10.1097/00005176-198601000-00007

SUMMARYTwenty-four (3.6%) of 670 patients with cystic fibrosis seen over a 25-year period developed symptomatic gallbladder disease. Only four patients were less than 16 years old. Four patients presented with unusual problems, including one with acute cholangitis and two with atonic gallbladder, one of whom required cholecystectomy. Another patient was found to have cholangiocarcinoma of the gallbladder when an exploratory laparotomy was performed to investigate biliary obstruction. Twenty patients had cholelithiasis, 15 of whom underwent cholecystectomy. Only one patient had substantial pulmonary difficulties postoperatively. Patients who presented with classic biliary colic had no further symptoms after cholecystectomy. One patient developed intrahepatic stones 6 years later and required a choledochoduodenostomy. As the pulmonary status of most cystic fibrosis patients will eventually deteriorate, we recommend that serious consideration be given to performing a cholecystectomy as soon as practical after the diagnosis of symptomatic cholelithiasis. Our experience indicates that surgery can be performed safely unless pulmonary status is already extremely compromised and the patient is in overt respiratory failure.