Ultrasonographic characteristics and outcome of Type III umbilical-portal-systemic venous shunt.

Abstract

According to a novel in-utero classification termed "umbilical-portal-systemic venous shunt (UPSVS)" recentlyproposed for an abnormal umbilical, portal and ductal venous system, the portal-systemic shunt belongs to type III UPSVS.This study was designed to examine the ultrasonographic characteristics and outcome of type III UPSVS.Material andmethods: All cases of Type III UPSVS diagnosed at our department from April 2016 to December 2020 were retrospectivelystudied. Seventeen patients with type III UPSVS including 12 type IIIa and 5 IIIb cases were identified. Sonographyshowed a shunt between the inferior left portal vein and the left hepatic vein in all type IIIa cases. Three cases of type IIIb hada combination of another shunt (2 with type I and one with type IIIa). Integrate intrahepatic portal vein system was not seenin those 2 cases of type IIIb combined with type I UPSVS, leading to termination of pregnancy (TOP). TOP occurred in 4patients with type IIIa as requested by the parents. Two cases (type IIIa and type IIIb each) underwent surgical procedure forthe closure of the shunt. Spontaneous complete closure in 4 type IIIa cases and partial closure in one type IIIb case occurredduring a period of 3-16 months. The majority of patients had type IIIa UPSVS presenting a good outcome. Thelack of integrate intrahepatic portal vein system was the main reason for TOP in patients with type IIIb UPSVS. These datasuggest the UPSVS classification is a useful tool for a prognosis prediction of type III UPSVS.