선천적 등쪽 췌장 발육 부전증을 동반한 다비장 증후군 환자에서 나타난 급성 췌장염 1예와 내시경 초음파의 역할

서지정보

· 발행기관 : 대한소화기학회
· 수록지 정보 : 대한소화기학회지 / 60권 / 1호 / 47 ~ 51페이지
· 저자명 : 정재훈, 김광하, 송근암, 이동건, 문지윤, 정재훈, 김석

초록

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.

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