Short Pancreas in Children With Fanconi Anemia: A Report of Three Cases.

A short pancreas (SP), characterized by the absence of the pancreatic tail, is a rare anomaly resulting from abnormal development of the dorsal pancreas and has been documented to cause endocrine pancreatic insufficiency. Fanconi anemia (FA) is associated with multiple congenital malformations and abnormalities in glucose-insulin metabolism; however, dorsal pancreatic anomalies have not been recognized as part of its phenotype. We report three children with FA in whom computed tomography (CT) demonstrated an SP anomaly. SP may be an underrecognized feature of FA, with potential implications for imaging assessment, endocrine surveillance, and future genotype-phenotype studies.