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Complete Agenesis of Dorsal Pancreas and Malformed Vertebrae: Review of Literature and Case Report

Anam Bashir, April Lawson and Pratik Kumar Patel

Context Complete agenesis of dorsal pancreas is an extremely rare entity. The first case was described in 1911. It results from congenital underdevelopment of pancreas. Non-specific abdominal pain leading to imaging usually leads to the diagnosis. Only 100 cases were reported till 2016. Here we report a case of complete agenesis of dorsal pancreas with associated skeletal anomaly because of its rarity. To our knowledge, ours is the first case that has been described to be associated with skeletal anomalies. Case report 16-year-old nondiabetic male with unremarkable past history presented to the Emergency Department with right upper quadrant pain for the last 3 days. Abdominal pain was aggravated by food with no relieving factors. Physical examination was unremarkable. Lab work including blood glucose levels, liver function and kidney function tests were within normal levels. Computed tomography (CT) abdomen pelvis revealed complete absence of body and tail of pancreas. Head and uncinate process were present. Incomplete segmentation of multiple thoracolumbar vertebrae, with a left hemivertebra at the thoracolumbar junction and a right hemivertebra more superiorly, was also found. MRCP was done which confirmed absence of body and tail of pancreas suggestive of dorsal agenesis of pancreas. Patient remained asymptomatic after initial presentation. Conclusion Dorsal agenesis of pancreas can be an incidental finding, but it may require longitudinal observation as well as additional work up to know associated anomalies. It will also help physicians give anticipatory guidance regarding potential complications such as non-alcoholic pancreatitis, neoplasia, and diabetes.

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