Agenesis of Dorsal Pancreas
Brooke R. Jeffrey, MD
Michael P. Federle, MD, FACR
Key Facts
Terminology
Complete or partial pancreatic agenesis, aplasia, or hypoplasia
Imaging
Absence of pancreatic tissue in the expected location of neck, body, and tail segments
ERCP: Filling of ventral duct without identifiable dorsal ducts or minor papilla in agenesis of entire dorsal pancreas
CT: Short pancreas, normal pancreatic head, absence of tail with complete or partial absence of body
MRCP: In complete agenesis of dorsal pancreas, accessory and dorsal duct system are not observed
Top Differential Diagnoses
Pancreatic ductal carcinoma
Abrupt, irregular obstruction of pancreatic duct seen on ERCP
Pancreatic duct dilated upstream from mass
Pancreas divisum
Contrast injection into minor papilla fills dorsal duct
Chronic pancreatitis
Parenchyma in body/tail often atrophic
Pathology
Associated conditions
Complete pancreatic agenesis (very rare); correlated with stillbirth or early neonatal death
Polysplenia syndrome (7 cases reported)
Bowel malrotation