Hereditary pancreatitis (HP): Clinical characteristics, ERCP findings, outcome of endoscopic therapy and surgery

C. P. Choudari, N. J. Nickl, E. L. Fogel, S. Sherman, G. A. Lehman

Research output: Contribution to journalArticle

Abstract

There are limited data on the role of diagnostic/therapeutic ERCP and need for surgical intervention in patients (pts) with HP. We tallied clinical, radiography findings, and outcome of endoscopic and surgical therapy in a two-center series. Methods: The medical records and radiographs of 17 pts known to have HP who had undergone ERCP were reviewed along with telephone follow-up. Results: Seventeen pts (M:F 7:10; age range 9-62 yrs) were studied. The diagnosis was made by identifying a mutation in the cationic trypsinogen gene in 7 and strong family history in 10. The age of onset of symptoms was <5 yrs in 2; 6-15 yrs in 8; 16-20 yrs in 4; >20 yrs in 3. The age at diagnosis was <5 yrs in 1; 6-15 yrs in 4; 16-20 yrs in 1; and > 20 yrs in 11. All pts (100%) had abdominal pain and 53% had more than 5 hospitalizations prior to determining the correct hereditary etiology. Pancreatograms revealed Cambridge grade I chronic pancreatitis (CP) in 4; grade II in 4, grade III in 9; pancreatic duct (PD) strictures in 6 (head-4; body/tail 2); PD stone disease in 7 and pseudocyst in two. ERCP therapy was performed in 11 pts: biliary endoscopic sphincterotomy (ES) in 5 (with cystgastrostomy in 1); pancreatic stents in 4 (with stricture dilation in 1, combined biliary and pancreatic ES in 2, pancreatic ES alone in 1); pancreatic ES with PD stone extraction in 2. Four pts underwent repeat therapy. At a mean follow-up of 20 months after endoscopic therapy, 5 pts were pain free, 3 pts had >50% improvement in symptoms, and 3 pts underwent surgery (2 had cancer). Seven pts (3 following endo therapy) underwent surgery (duodenal-sparing pancreatectomy in 4; distal pancreatectomy in 2; gastric bypass in 1). At a mean surgical follow-up of 18 months, 4 pts remain asymptomatic, one has recurrent pain, one pt died (pancreatic cancer), and one is ill (pancreatic cancer). Summary: 1) 82% had their first attack of pancreatitis before their 20th yr, but only 35% were diagnosed as having HP by that age. 2) Chronic pancreatitis and its complications (strictures in 35%; stones in 41% and pseudocyst in 12%) are common findings at ERCP. 3) Forty-one percent (7/17) underwent surgery and 2 (12%) had pancreatic cancer. 4) Following therapy (endoscopic or surgery) around 50% remain asymptomatic. Conclusions: CP in HP tends to run an aggressive course with early age onset. Endoscopic/surgical therapy appear to benefit selected patients. Careful follow-up is necessary as pancreatic cancer may supervene.

Original languageEnglish (US)
Pages (from-to)AB135
JournalGastrointestinal endoscopy
Volume47
Issue number4
StatePublished - Dec 1 1998
Externally publishedYes

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Gastroenterology

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