Clinical and morphological features of duodenal cystic dystrophy in heterotopic pancreas

Abstract

Background and aims: Cystic dystrophy in heterotopic pancreas (CDHP) is an uncommon complication of pancreatic heterotopia, only described in surgical series, whose natural history is not known. The aim of this study was to determine clinical and morphological features of CDHP in a medical-surgical series of patients and to ascertain the relationship of CDHP with chronic pancreatitis (CP) in the pancreas proper.

Methods: All patients who had duodenal CDHP diagnosed radiologically both with CT scan and endoscopic ultrasonography between 1995 and 2004 were included. The diagnosis was confirmed by surgical specimens when available.

Results: One hundred five patients were included (91% men, 86% chronic alcoholic) with a median follow-up of 15 months. The median age at first symptoms was 46 yr. CDHP was associated with CP in the pancreas proper in 71% of patients. Presenting symptoms were pancreatic pain (91%), severe weight loss (73%), acute pancreatitis (45%), vomiting (30%), steatorrhea (23%), diabetes mellitus (20%), jaundice (13%), and upper gastrointestinal hemorrhage (5%). Cysts were multiple in 75% (median 3). The median diameter of the largest cyst was 10 mm. Endoscopy was normal in 36% of patients and showed duodenal stenosis in 52% (complete 6%, incomplete 46%). Surgical treatment was necessary in only 27% of patients (Whipple procedure 16%).

Conclusions: CDHP may arise in patients with or without CP and with or without chronic alcoholism. Symptoms may be severe but warrant surgery in less than one-third of patients.