Diagnosis and therapeutic management of cystic dystrophy of the duodenal wall in heterotopic pancreas. A case report and revision of the literature.

Cystic dystrophy in heterotopic pancreas is a rare and serious condition. Diagnosis is difficult because of non-specific clinical manifestations and radiologic and endoscopic imaging are pivotal. Therapeutic management is still under debate. We describe a case of cystic dystrophy of the duodenal wall in heterotopic pancreas complicated with chronic pancreatitis and pancreatic cystadenoma. Computed tomography and magnetic resonance are very useful in demonstrating the presence of cysts in a thickened duodenal wall but, for the most part, endoscopic ultrasonography is the most useful imaging examination. The choice of different therapeutic options is still under debate; although some authors have proposed a medical approach using octreotide or endoscopic treatment for selected patients, a pancreaticoduodenectomy is usually proposed for symptomatic patients. When surgery is needed, a pancreaticoduodenectomy is preferred, reserving by-pass procedures for high risk patients. Because of the non-specific clinical manifestation and the very difficult diagnosis and therapeutic management, these patients should be studied and treated in specialized and dedicated centers.