Percutaneous Embolization of Congenital Portosystemic Venous Fistula in an Infant with Down Syndrome

Ryan M. McAdams,Terrence Chun,Pattaraporn Tanya Chun,Nghia Vo,Matthew Files

doi:10.1155/2013/127023

Abstract

Congenital intrahepatic portosystemic venous shunts are rare vascular malformations often associated with severe complications. We describe a term male infant with Down syndrome with high output heart failure secondary to a congenital arterial to portal venous fistula that was diagnosed by Doppler ultrasound. Percutaneous embolizations of the left hepatic vein, portal vein, and communicating fistulas were performed without complications, resulting in clinical improvement. A subsequent hepatic ultrasound demonstrated resolution of the pathologic fistulous communication and shunting effects.

Highlights

Congenital portosystemic shunts (CPSS) have been classified into extrahepatic and intrahepatic based on the location of the shunts [1]
Complications of CPSS may manifest in early infancy or later in life, including neonatal hyperammonemia, cholestasis, hypergalactosemia, liver tumors, portosystemic encephalopathy, pulmonary arterial hypertension, right heart failure, and hepatopulmonary syndrome [2]
We report the case of an infant with Down syndrome presenting with high output cardiac failure discovered to be due to intrahepatic CPSS who was successfully treated with a percutaneous embolization procedure

Summary

Introduction

Congenital portosystemic shunts (CPSS) have been classified into extrahepatic and intrahepatic based on the location of the shunts [1]. These vascular malformations can be detected by ultrasonography (US), computed tomography (CT) scan, and magnetic resonance (MR) angiography. Complications of CPSS may manifest in early infancy or later in life, including neonatal hyperammonemia, cholestasis, hypergalactosemia, liver tumors (benign or malignant), portosystemic encephalopathy, pulmonary arterial hypertension, right heart failure, and hepatopulmonary syndrome [2]. We report the case of an infant with Down syndrome presenting with high output cardiac failure discovered to be due to intrahepatic CPSS who was successfully treated with a percutaneous embolization procedure

Case Report

Discussion

Findings

Conclusion

Conflict of Interests