Cystic Dystrophy of the Duodenal Wall in Heterotopic Pancreas with Groove Pancreatitis: A Diagnostic and Therapeutic Challenge.

Background: Cystic dystrophy of heterotopic pancreas is a benign, rare disease characterized by development of true cysts into the duodenal wall. Non-specific clinical manifestations and difficult to interpret imaging provide a diagnostic challenge, especially when pancreatic cancer is suspected. Surgical treatment (pancreatoduodenectomy) offer best outcomes. CASE REPORT: A 48 years-old man, chronic alcohol consumer, with a history of recurrent mild acute pancreatitis episodes, was diagnosed in 2010 with segmental chronic pancreatitis complicated by a 4cm pseudocyst in the pancreatic head. He stopped drinking, but acute episodes continued to reoccur, while the pseudocyst disappeared. In 2012 the patient was admitted to our department with painful chronic pancreatitis and a new acute episode. EUS diagnosed a cystic dystrophy of the duodenal wall with groove pancreatitis, and endoscopic opening of the cysts into the duodenum was performed. Laparoscopic cholecystectomy was imposed by small gallbladder stones seen at EUS. After 6 months of silence, pain and acute episodes reappeared, imposing pancreatoduodenectomy. Patient is now well, without symptoms during 5 years of follow-up. Conclusions: Cystic dystrophy of heterotopic pancreas can be thought of in case of recurrent idiopathic acute pancreatitis. EUS is the best diagnosis tool, and can provide opening of the cysts into the duodenum. Surgery - pancreatoduodenectomy - offer best results.