Dorsal agenesis of the pancreas

The diagnosis of dorsal agenesis of the pancreas was made based on the imaging studies performed to evaluate the patient’s abdominal pain. CT scanning demonstrated the pancreatic head, with absence of the pancreatic neck, body, and tail ventral to the splenic vein (see Figures 1 and 2). These results were followed up with MRCP, which confirmed the absence of the body and tail of the pancreas as well as the dorsal ductal system, accessory duct, and minor papilla.

The human pancreas forms from the foregut endoderm’s dorsal and ventral buds during embryogenesis. Failure of the dorsal pancreatic bud to develop can potentially result in agenesis of the dorsal pancreas, an extremely rare congenital anomaly.[1] The dorsal bud forms the upper part of the head, body, and tail of the pancreas. These drain through the duct of Santorini into the minor papilla. The ventral bud forms the major part of the head and uncinate process that drains through the duct of Wirsung into the major papilla.[1] The main pancreatic duct drains through both the major and minor papilla. Agenesis of the dorsal pancreatic duct can result in a complete or partial anomaly.

Complete agenesis of the dorsal pancreas has rarely been described in the medical literature. The diagnosis is usually made when a patient with abdominal pain undergoes diagnostic radiologic testing. Dorsal agenesis of the pancreas is suggested when the body and tail of the pancreas are not visualized ventral to the splenic vein. This may be seen on abdominal ultrasonography or CT scanning.[1] The diagnosis should be confirmed by MRCP or endoscopic retrograde cholangiopancreatography (ERCP) to demonstrate absence of the pancreatic dorsal duct system.[2] During either MRCP or ERCP, the diagnostic triad is required to confirm the diagnosis. This triad includes absence of the dorsal ductal system, the accessory duct or duct of Santorini, and the minor papilla.

Clinically, these patients can present with recurrent pancreatitis, jaundice, and/or a medical history of diabetes mellitus. Most patients, however, remain asymptomatic.Therapie: Diabetes mellitus, Enzymsubstitution.

Artikel Medscape: Dorsale Agenesie des Pankreas

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