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

Abstract

Aims: According to a novel in-utero classification termed “umbilical-portal-systemic venous shunt (UPSVS)” recently proposed 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 and methods: All cases of Type III UPSVS diagnosed at our department from April 2016 to December 2020 were retrospectively studied.

Results: Seventeen patients with type III UPSVS including 12 type IIIa and 5 IIIb cases were identified. Sonography showed a shunt between the inferior left portal vein and the left hepatic vein in all type IIIa cases. Three cases of type IIIb had a combination of another shunt (2 with type I and one with type IIIa). Integrate intrahepatic portal vein system was not seen in those 2 cases of type IIIb combined with type I UPSVS, leading to termination of pregnancy (TOP). TOP occurred in 4 patients with type IIIa as requested by the parents. Two cases (type IIIa and type IIIb each) underwent surgical procedure for the closure of the shunt. Spontaneous complete closure in 4 type IIIa cases and partial closure in one type IIIb case occurred during a period of 3-16 months.

Conclusions: The majority of patients had type IIIa UPSVS presenting a good outcome. The lack of integrate intrahepatic portal vein system was the main reason for TOP in patients with type IIIb UPSVS. These data suggest the UPSVS classification is a useful tool for a prognosis prediction of type III UPSVS.