Correlation of Prenatal and Postnatal Diagnosis in Umbilical-Portal-Systemic Venous Shunts

Eur J Pediatr Surg. 2023 Feb;33(1):90-95. doi: 10.1055/s-0042-1760379. Epub 2023 Jan 7.


Introduction: Umbilical-portal-systemic venous shunts (UPSVS) are rare anomalies in the development of the fetal venous system. There are several postnatal and prenatal classifications of hepatic venous anomalies but the link between them is missing. We aimed to review the prenatal to postnatal diagnosis correlation in UPSVS at our center.

Methods: It is a retrospective study of patients diagnosed with UPSVS between 2019 and 2021 at our institution. Demographic, obstetric, genetic, and neonatal data were reviewed with special focus on prenatal and postnatal ultrasounds.

Results: A total of seven patients were diagnosed with UPSVS at a median of 24 (20-34) weeks of gestational age. All patients were male and 62% were Caucasian. None of the patients had chromosomopathies or cardiac anomalies. One patient had renal ectopia, another one had a single umbilical artery, and a third one suffered from intrauterine growth retardation. An umbilico-systemic shunt (USS) was found in two patients and a ductus venosus-systemic shunt (DVSS) in the rest. Patients with USS were diagnosed postnatally with intrahepatic portosystemic shunts. One of the DVSS patients was transferred to another hospital and the other four had normal postnatal cardiac ultrasounds, with normal abdominal ultrasounds in two patients and lack of postnatally abdominal control in the other two. All babies were found to be doing well at a median follow-up of 1 month (0-24).

Conclusion: There is a knowledge gap in the natural history of UPSVS between fetal and neonatal life. Building bridges between prenatal and postnatal research is mandatory in order to understand these rare anomalies.

MeSH terms

  • Female
  • Fetus
  • Heart Defects, Congenital*
  • Humans
  • Infant, Newborn
  • Male
  • Pregnancy
  • Retrospective Studies
  • Ultrasonography, Prenatal*
  • Umbilical Veins / abnormalities
  • Umbilical Veins / diagnostic imaging