J Korean Pediatr Soc.  2000 Feb;43(2):283-287.

A Case of Congenital Intrahepatic Portosystemic Venous Shunt

• Kim DY
• Lee DJ
• Kwon JH

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.

Keyword

Congenital intrahepatic portosystemic venous shunt; Ultrasonography

MeSH Terms

Adult
Aneurysm
Child
Female
Fibrosis
Follow-Up Studies
Hepatic Veins
Humans
Hypertension, Portal
Liver
Magnetic Resonance Imaging
Portal Vein
Sepsis
Ultrasonography