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

Abstract

Context: 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.

Case report: We describe a case of cystic dystrophy of the duodenal wall in heterotopic pancreas complicated with chronic pancreatitis and pancreatic cystadenoma.

Discussion: 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.

Conclusion: 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.