Intrahepatic portal venous systems in children with noncirrhotic prehepatic portal hypertension: anatomy and clinical relevance

Abstract

Background: Children with extrahepatic portal hypertension typically present with cavernomatous transformation of the portal vein and a poorly defined intrahepatic portal vein system on conventional imaging. With the Meso-Rex Bypass becoming the gold-standard intervention for a cure, a precise assessment of the intrahepatic portal vein system provides helpful data for deciding whether a Meso-Rex Bypass is feasible or not.

Methods: All children with extrahepatic portal hypertension were prospectively assessed by wedged hepatic venous portography. Venous anatomy was categorized into five subtypes (A to E), depending on the presence of thrombosis in the Rex recessus, or not, and its extension within the intrahepatic portal venous system.

Results: Eighty-nine children entered the study. Previous umbilical vein catheterization is usually associated with Rex thrombosis, while the Rex recessus and the intrahepatic portal venous system are patent in idiopathic cases, thus allowing for the performance of a Meso-Rex Bypass with a good outcome.

Conclusions: Wedged hepatic venous portography is a very effective tool for detailed preoperative assessment and identification of children being considered for Meso-Rex Bypass surgery. An anatomic-radiological classification is useful in selecting patients for Meso-Rex Bypass with anticipation of a high rate of success.

Keywords: Children; Extrahepatic portal hypertension; Meso-Rex Bypass; Wedged hepatic venous portography.