Agenesis of the Dorsal Pancreas: A Rare Cause of Diabetes and Recurrent Upper Abdominal Pain

Abstract

Agenesis of the dorsal pancreas is a rare congenital disorder with only a handful of cases described in the literature. It presents a diagnostic dilemma. Cross-sectional imaging is the cornerstone for diagnosis. It could have a syndromic association with polysplenia and cardiac anomalies. Pancreas divisum and chronic pancreatitis may present with similar symptoms and must be ruled out. We present a case of a 55-year-old male with recurrent non-specific abdominal pain and diabetes mellitus. He was managed with insulin and painkillers for symptomatic relief. We also reviewed approximately 68 cases described in the literature to date.

Keywords: agenesis of dorsal pancreas; congenital anomaly; heterotaxy syndrome; pancreas agenesis; pancreatic anomaly; polysplenia; recurrent abdominal pain; type 1 diabetes mellitus.