Objective: The objective of this study was to assess the imaging characteristics and classify congenital short pancreas on the basis of morphologic features on multidetector computed tomography (MDCT) and to determine the associated diseases and congenital anomalies of each type.

Methods: We conducted a retrospective search from 2006 to 2012 using the keywords “short pancreas,” “agenesis or hypoplasia of the dorsal pancreas,” or “hypoplasia of the ventral pancreas.” Clinical data and images were analyzed; finally, 24 patients with congenital short pancreas were included in this study. Imaging features of the 3 types of congenital short pancreas and their associated anomalies on MDCT were evaluated.

Results: Congenital short pancreas was classified into type 1 (agenesis or hypoplasia of the dorsal pancreas): no congenital anomaly but presence of diabetes mellitus (45%); type 2 (agenesis or hypoplasia of the pancreatic uncinate process): intestinal malrotation (100%); and type 3 (combined hypoplasia or agenesis of the uncinate process and dorsal pancreas): a spectrum of various congenital anomalies, including abdominal heterotaxy and abnormal spleen (100%).

Conclusions: Recognizing the spectrum of agenesis or hypoplasia of the pancreas and morphologic classification of congenital short pancreas on MDCT may help radiologists detect and understand disease associated with congenital short pancreas.