Recurrent pancreatitis in Gardner variant familial polyposis - Etiology, diagnostic approach, and interventional results

Hypothesis: Pancreatitis arising from an obstructing ampullary neoplasm in patients with Gardner variant familial polyposis is an infrequently describ ed clinical entity. We reviewed all patients with Gardner variant polyposis presenting with pancreatitis during a 12-year period in our institution, h oping to better define etiology and the appropriate diagnostic and interven tional approach. Methods: A retrospective record review (1986-1998) defined patient demograp hics, presenting features, initial and subsequent endoscopic retrograde cho langiopancreatography (ERCP) findings, subsequent treatments, and both imme diate and long-term outcomes. Particular consideration was given to initial post-ERCP diagnosis and to endoscopic interventions undertaken at that tim e. We also looked at those patients who eventually required surgical interv ention after long-term failure of medical and endoscopic therapy, the indic ations for surgery, final pathological characteristics, and follow-up resul ts. Results: Eight patients (6 women and 2 men), with a mean age of 42 years at initial presentation, were found. Each patient was known to have Gardner v ariant familial polyposis at the time of the initial bout of pancreatitis. All had undergone prior colectomy and 4 of 8 had undergone prior cholecyste ctomy. None were known to be taking medications or ingesting pancreatoxic s ubstances. Five of 8 patients had obstructing focal or diffuse adenomatous disease involving the ampulla. Two of 8 patients had pancreatitis attribute d to other causes (divisum, stones) and a single patient had no clear etiol ogy. Three of 5 patients with ampullary adenomatous disease underwent pancr eaticoduodenectomy for recurrent adenomatous encroachment and ampullary ste nosis, despite repetitive snare resection and papillotomy. All of these pat ients had ampullary and other duodenal adenomas, and none had malignant dis ease. Conclusions: Patients presenting with pancreatitis in the setting of Gardne r variant familial polyposis will frequently have an obstructing ampullary neoplasm, although additional etiologies should be sought. Initial endoscop ic therapy affords transient relief but may not be definitive. The abnormal scarring and fibrosis (keloid formation, desmoid reaction) that characteri ze this disease likely play a large role in endoscopic or subsequent surgic al failure. A significant number of these patients will go on to require su rgical referral and intervention.