Polysplenia Syndrome with Congenital Agenesis of Dorsal Pancreas Presenting as Acute Pancreatitis and the Role of Endoscopic Ultrasonography in Its Diagnosis.
- Author:
Jae Hoon JEONG
1
;
Gwang Ha KIM
;
Geun Am SONG
;
Dong Gun LEE
;
Ji Yoon MOON
;
Jae Hoon CHEONG
;
Suk KIM
Author Information
- Publication Type:Case Reports
- Keywords: Polysplenia; Heterotaxy syndrome; Pancreatitis; Preduodenal portal vein; Agenesis of the dorsal pancreas
- MeSH: Acute Disease; Congenital Abnormalities/*diagnosis/ultrasonography; Endosonography; Female; Heterotaxy Syndrome/*diagnosis/ultrasonography; Humans; Magnetic Resonance Imaging; Middle Aged; Pancreas/abnormalities/ultrasonography; Pancreatitis/*diagnosis; Tomography, X-Ray Computed
- From:The Korean Journal of Gastroenterology 2012;60(1):47-51
- CountryRepublic of Korea
- Language:English
- 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.
