|
KMID : 0614620120600010047
|
|
Korean Journal of Gastroenterology
2012 Volume.60 No. 1 p.47 ~ p.51
|
|
|
Polysplenia Syndrome with Congenital Agenesis of Dorsal Pancreas Presenting as Acute Pancreatitis and the Role of Endoscopic Ultrasonography in Its Diagnosis
|
|
Cheong Jae-Hoon
Kim Gwang-Ha
Song Geun-Am
Lee Dong-Gun
Moon Ji-Yoon
Kim Suk
|
|
|
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. (Korean J Gastroenterol 2012;60:47-51)
|
|
|
KEYWORD
|
|
|
Polysplenia, Heterotaxy syndrome, Pancreatitis, Preduodenal portal vein, Agenesis of the dorsal pancreas
|
|
|
FullTexts / Linksout information
|
|
|
|
|
Listed journal information
|
|
   
|
|