Distúrbios Pancreáticos e Terapia
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Abstrato

Agenesia completa do pâncreas dorsal - uma anomalia congénita rara: apresentação de um caso com achados imagiológicos e revisão da literatura

Ravinder Kumar, Kapil Vyas, Neha Singh Agrahari and Jyoti Kundu

Complete agenesis of the dorsal pancreas is a rare congenital anomaly due to low frequency of anatomic variations in pancreas. Due to this exceedingly rare occurrence, less than 100 cases have been reported in world literature. We report here a case presentation of thirty five year old male diagnosed with Agenesis of Dorsal Pancreas (ADP) presenting with additional features of diabetes mellitus and acute pancreatitis from heavy alcohol abuse. Biochemical evaluation showed raised serum amylase and serum pancreatic lipase (516 U/L and 912U/L; normal values 0-200 and 0-190, respectively). Ultrasound abdomen exhibited absence of body and tail of pancreas. CT abdomen and MRCP revealed absence of neck, body, and tail of the pancreas along with duct of Santorini, and the minor duodenal papilla. This diagnostic triad confirmed the diagnosis of ADP. This case report is concerned with the description of radiological appearances of ADP, associated symptoms and management in pertinent light of world literature.