Neonate with Congenital Intrahepatic Portosystemic Shunt

Abstract

A 6-week-old infant presented to the emergency department with decreased oral intake, listlessness, diarrhea, and emaciation. The initial laboratory values revealed hypoglycemia, lactic acidosis, hyperammonemia, and azotemia. A diagnostic evaluation was initiated for inborn errors of metabolism. She developed bacteremia and disseminated intravascular coagulation. Hypoalbuminemia, conjugated hyperbilirubinemia, hypotriglyceridemia, and persistent hyperammonemia were noted. Doppler ultrasonography discovered multiple high-flow congenital intrahepatic portosystemic shunts (IHPSS, Figure).

An Interesting Case of Congenital Intrahepatic Porto-hepatic Shunt as a Cause of Unexplained Encephalopathy

“…If radiological intervention is unsuccessful then surgical ligation should be considered. Liver resection is typically reserved for patients with large multifocal intrahepatic shunts or when none of the above therapies work [1,[7][8]. Finally, liver transplantation is a last resort and is almost always carried out on those patients with type 1 extrahepatic shunt after they have failed the occlusion trial or those with multifocal shunt who have developed liver tumor [3].…”

“…Congenital portosystemic shunts can be divided into two types: intrahepatic shunts in which there is an abnormal connection between the branches of the portal vein and either the inferior vena cava or the hepatic veins and less commonly the extrahepatic type in which the portal system is connected by one of the branches of the mesenteric veins. Congenital intrahepatic portosystemic shunts or Abernethy malformations are rare anatomical abnormalities characterized by an abnormal connection between the portal vein and the hepatic vein [1,2]. Intrahepatic shunt anomalies occur predominantly in males and have been reported to affect only 0.0235% of the population [3,4].…”