Hepatobiliary and pancreatic disease in cystic fibrosis (CF): ERCP findings and outcome of therapy

C. P. Choudari, G. A. Lehman, S. Sherman, E. L. Fogel, W. B. Silverman, M. F. Catalano, J. E. Geenen, K. Block, M. J. Schmalz

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There are limited data on the role of diagnostic/therapeutic ERCP in patients (pts) with CF. We tallied findings and efficacy of ERCP therapy in a multicenter series. Methods: The medical records and radiographs of 19 pts known to have CF who had undergone ERCP were reviewed along with telephone follow-up. All patients were sweat chloride test positive or homozygous by gene testing. Results: Nineteen pts (M:F = 8:11; age range 5-48 yrs) were studied. The average age of onset of hepatobiliary/pancreatic symptoms was 12 (range 1.5-39 yrs) and pulmonary symptoms was 6.5 yrs (range 3 mo - 14 yrs). Four of 19 (21%) patients had no pulmonary symptoms. ERCP indications were abnormal liver tests in 4 and recurrent pancreatitis in 15 pts. Pancreatograms revealed Cambridge grade I chronic pancreatitis (CP) in 5; grade II in 5; grade III in 2; pancreas divisum in 2 (distal stricture with multiple stones in the dorsal duct in one, and mild CP of dorsal duct in the other); normal in 2 and not attempted in 3, In total, three had pancreatic duct (PD) strictures and two patients had PD stones. Cholangiograms were normal in 16 and choledocholithiasis was seen in 3 (one with choledochoduodenal fistula). ERCP therapy was performed in 15 pts: pancreatic stent in 7; minor papilla endoscopic sphincterotomy (ES) with stent in 2; biliary ES with stone extraction in 3; biliary ES in 1; pancreatic stent with combined biliary and pancreatic ES in 1 and pancreatic stone removal in one. Repeat ERCP was performed in 12 pts (re-stenting in 6; stent removal in 1; balloon sweep of common bile duct in 3; biliary ES in 1 and pancreatic ES in one patient). Two pts underwent surgery (resection of pancreatic tail and a Puestow procedure). At a mean follow-up of 32 months, 10 pts were asymptomatic, 4 pts had >50% improvement in symptoms, and 4 pts continued to have recurrent attacks of pancreatitis with daily pain and are on narcotics. One patient died of respiratory failure (age 31 yrs). Summary: 1) ERCP is useful in denning ductal lesions amenable to endoscopic therapy in CF pts. Chronic pancreatitis and its complications are common findings at ERCP. 2) These uncontrolled data suggest that endoscopic therapy improved symptoms in the majority of our patients. Conclusions: Hepatobiliary and pancreatic disease in CF is becoming more apparent as the life expectancy of CF patients increases. Outcomes of endoscopic treatment in this group of pts appears similar to non CF pts with similar severity of chronic/recurrent pancreatitis.

Original languageEnglish (US)
Pages (from-to)AB135
JournalGastrointestinal endoscopy
Issue number4
StatePublished - Dec 1 1998

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Gastroenterology

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    Choudari, C. P., Lehman, G. A., Sherman, S., Fogel, E. L., Silverman, W. B., Catalano, M. F., Geenen, J. E., Block, K., & Schmalz, M. J. (1998). Hepatobiliary and pancreatic disease in cystic fibrosis (CF): ERCP findings and outcome of therapy. Gastrointestinal endoscopy, 47(4), AB135.