title: Polysplenia syndrome with congenital agenesis of dorsal pancreas presenting as acute pancreatitis and the role of endoscopic ultrasonography in its diagnosis.
abstract: A 49-year-old female was admitted to our hospital for acute pancreatitis. The abdomen CT scan incidentally showed midline liver with hepatomegaly, centrally located gallbladder, pancreas truncation, right sided small bowel, left sided large bowel, interruption of the inferior vena cava with azygos continuation, preduodenal portal vein, and multiple spleens in the left upper quadrant. In MRCP, the head of pancreas was enlarged and short main pancreatic duct without accessory duct was showed. EUS revealed enlarged ventral pancreas with a main pancreatic duct of normal caliber, absence of the accessory pancreatic duct and the dorsal pancreas. She was diagnosed as polysplenia syndrome with agenesis of dorsal pancreas. It is a rare congenital anomaly frequently associated with various visceral anomalies including multiple spleens, impaired visceral lateralization, congenital heart diseases, gastrointestinal abnormalities and azygos continuation of the inferior vena cava. We report a case of polysplenia syndrome with agenesis of dorsal pancreas presenting acute pancreatitis.
authors: Department of Internal Medicine, Pusan National University School of Medicine, Pusan National University Hospital, Busan, Korea.
authors: Jae Hoon
authors: JH
authors: Jeong
authors: Gwang Ha
authors: GH
authors: Kim
authors: Geun Am
authors: GA
authors: Song
authors: Dong Gun
authors: DG
authors: Lee
authors: Ji Yoon
authors: JY
authors: Moon
authors: Jae Hoon
authors: JH
authors: Cheong
authors: Suk
authors: S
authors: Kim
mesh_terms: D000208:Acute Disease; D000013:Congenital Abnormalities / Q000175:diagnosis* / Q000000981:diagnostic imaging; D019160:Endosonography; D005260:Female; D059446:Heterotaxy Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D010179:Pancreas / Q000002:abnormalities / Q000000981:diagnostic imaging; D010195:Pancreatitis / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed