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A Case of Congenital Intrahepatic Portosystemic Venous Shunt

Journal of the Korean Pediatric Society 2000;43(2):283-287.
Published online February 15, 2000.
A Case of Congenital Intrahepatic Portosystemic Venous Shunt
Do Yeon Kim1, Dong Jin Lee1, Jung Hyeok Kwon2
1Department of Pediatrics, Ulsan Dong-Kang General Hospital, Ulsan, Korea
2Department of Radiology, Ulsan Dong-Kang General Hospital, Ulsan, Korea
선천성 Intrahepatic Portosystemic Venous Shunt 1례
김도연1, 이동진1, 권중혁2
1울산동강병원 소아과
2울산동강병원 방사선과
Abstract
Intrahepatic portosystemic venous shunt(IPVS) is an uncommon pathologic condition which occurs mostly in adult patients with portal hypertension and cirrhosis of the liver with only scattered reports are on children. However, asymptomatic IPVS have been detected in an increasing number of patients with recent advances in imaging techniques, such as sonography, CT, MR imaging, and color Doppler sonography. The cause of IPVS remains unknown, but two probable origins have been proposed, congenital and acquired origin. In the congenital origin, it has been proposed that the IPVS develops from embryologic vascular remnants. Acquired IPVS can develop into intrahepatic collateral pathway in the presence of portal hypertension and cirrhosis of the liver. We report a case of congenital IPVS with suspicious sepsis in a 6-day-old female patient. An abdominal ultrasonography and color Doppler sonography demonstrated an aneurysmal type of anomalous shunt connecting the right portal vein to the right hepatic vein. The patient continued to be symptomatic after discharge and a follow-up ultrasonography 3 months later revealed the disappearance of the previous IPVS.
Key Words: Congenital intrahepatic portosystemic venous shunt, Ultrasonography


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