Recurrent idiopathic pancreatitis in familial adenomatous polyposis: report of a case-series and review of the literature

Familial adenomatous polyposis (FAP) is characterized by the development of multiple adenomatous polyps predominantly in the colon but also in the duodenum. Scattered case reports indicate that there is a risk for pancreatitis in FAP. The most likely cause of pancreatitis in FAP is obstructing ampul...

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Bibliographic Details
Published in:Familial cancer 2007-01, Vol.6 (3), p.275-280
Main Authors: van Esch, Aura A J, Drenth, Joost P H, te Morsche, Rene H M, Jansen, Jan B M J, Nagengast, Fokko M
Format: Article
Language:English
Subjects:
Adenomatous Polyposis Coli - complications
Adenomatous Polyposis Coli - genetics
Adolescent
Adult
Carrier Proteins - genetics
Duodenal Neoplasms - complications
Duodenal Neoplasms - diagnosis
Duodenal Neoplasms - surgery
Female
Humans
Male
Middle Aged
Pancreatitis, Chronic - complications
Pancreatitis, Chronic - diagnosis
Pancreatitis, Chronic - therapy
Polyps - complications
Polyps - diagnosis
Polyps - surgery
Recurrence
Risk Factors
Trypsin Inhibitor, Kazal Pancreatic
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Summary:Familial adenomatous polyposis (FAP) is characterized by the development of multiple adenomatous polyps predominantly in the colon but also in the duodenum. Scattered case reports indicate that there is a risk for pancreatitis in FAP. The most likely cause of pancreatitis in FAP is obstructing ampullary adenomas. We describe 7 FAP patients who experienced one or more episodes of pancreatitis. Two patients experienced pancreatitis after endoscopic treatment of ampullary adenoma. The cause of the pancreatitis in 5 of 7 patients could not be determined, as none of the patients had obstruction of the ampulla. Furthermore, other risk factors for pancreatitis such as pancreatic serine protease inhibitor Kazal type I (SPINK1) gene mutations were ruled out. A review of literature identified 20 FAP patients who developed the first episode of pancreatitis at a mean age of 45 years (range 23-72 years). Some 55% had recurrent episodes of pancreatitis. Eight patients had (peri) ampullary adenomas or carcinomas. In most cases, the course of pancreatitis was mild with an uneventful outcome, but one patient died after an episode of acute pancreatitis.
ISSN:1389-9600
1573-7292
DOI:10.1007/s10689-007-9118-z