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

Abstract

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.

Keywords

congenital portosystemic shunt - umbilical-portal-systemic venous shunt - fetal venous anomalies

• References

• 1 McLin VA, Franchi Abella S, Debray D. et al; Members of the International Registry of Congenital Porto-Systemic Shunts. Congenital portosystemic shunts: current diagnosis and management. J Pediatr Gastroenterol Nutr 2019; 68 (05) 615-622
• 2 Guérin F, Blanc T, Gauthier F, Abella SF, Branchereau S. Congenital portosystemic vascular malformations. Semin Pediatr Surg 2012; 21 (03) 233-244
• 3 Blanc T, Guerin F, Franchi-Abella S. et al. Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy. Ann Surg 2014; 260 (01) 188-198
• 4 Kanazawa H, Nosaka S, Miyazaki O. et al. The classification based on intrahepatic portal system for congenital portosystemic shunts. J Pediatr Surg 2015; 50 (04) 688-695
• 5 Achiron R, Kivilevitch Z. Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance. Ultrasound Obstet Gynecol 2016; 47 (06) 739-747
• 6 Wu H, Tao G, Cong X. et al. Prenatal sonographic characteristics and postnatal outcomes of umbilical-portal-systemic venous shunts under the new in-utero classification: a retrospective study. Medicine (Baltimore) 2019; 98 (02) e14125
• 7 Strizek B, Zamprakou A, Gottschalk I. et al. Prenatal diagnosis of agenesis of ductus venosus: a retrospective study of anatomic variants, associated anomalies and impact on postnatal outcome. Ultraschall Med 2019; 40 (03) 333-339
• 8 Demirci O, Akay HÖ. Prenatal diagnosis of abnormality of the umbilical portal DV complex: difficulty in universal classification due to various alternative routes in hepatic circulation for placental drainage. J Matern Fetal Neonatal Med 2022; 35 (20) 3872-3884
• 9 Nagy RD, Iliescu DG. Prenatal diagnosis and outcome of umbilical-portal-systemic venous shunts: experience of a tertiary center and proposal for a new complex type. Diagnostics (Basel) 2022; 12 (04) 873
• 10 Baiges A, Turon F, Simón-Talero M. et al; REHEVASC, VALDIG an EASL consortium, Abernethy group. Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology 2020; 71 (02) 658-669
• 11 van Steenbeek FG, Van den Bossche L, Grinwis GC. et al. Aberrant gene expression in dogs with portosystemic shunts. PLoS One 2013; 8 (02) e57662