Complete agenesis of dorsal pancreas: a rare cause of diabetes mellitus

Abstract

Agenesis of dorsal pancreas is a rare congenital anomaly that results from defective development of pancreas with less than 100 cases described in the literature. Patients may present with epigastric pain, acute or chronic pancreatitis and various disorders of glucose metabolism, can even be asymptomatic. About half of affected individuals develop diabetes mellitus, resulting from reduced islet cell mass. Being very rare, it is generally not kept in mind while dealing these cases and not suspected until imaging investigations are done. In our case study, 14-years-old twin brothers presented with polyuria and polydipsia for seven days. During evaluation diabetes mellitus with dorsal pancreatic agenesis was detected in both brothers.

BIRDEM Med J 2026; 16(2): 109-112