Document Type

Case

Publication Date

11-2023

Abstract

Background: The prevalence of prenatally diagnosed umbilical-portal systemic venous shunts (UPSVS) is reportedly rare, ranging from 0.003-0.209% [1]. Disruption in the normal development of the fetal venous system can be caused by primary failure of a system or by secondary occlusion of an already transformed system [2]. While UPSVS’s are rare, the downstream effects are potentially fatal, including intrauterine growth restriction, high output cardiac failure, associated neonatal morbidity and mortality, and thus necessitate early detection with appropriate monitoring.

Introduction: The patient was a 21-year-old G2P1001 who presented to the Maternal Fetal Medicine Ultrasound Genetics (MFMUG) clinic at 36w2d for a scheduled Biophysical Profile (BPP) as a follow up after a non-reactive NST in the office. During the encounter, the sonographer noted hypervascularity within the liver, abdominal circumference

Discussion: This case demonstrates recognition of an UPSVS in an otherwise uncomplicated pregnancy with previously normal anatomy ultrasound at 20 weeks’ gestation. The role in ultrasonography and BPP for fetal wellbeing is a luxury to facilities with MFM specialists readily available. The patient described in this report had decreased fetal movement, a non-reactive NST in the office, and was referred to MFMUG for a formal BPP. Currently, there are approximately one MFM specialist per 14 OBGYNs, and one MFM per 2,277 births [3]. The states with the leading number of MFM specialists include California, New York, Texas, Florida, and Pennsylvania [3]. Identification of this UPSVS may not have been readily identified by the untrained eye, but growth ultrasounds and Biophysical Profiles are key skills of any obstetrician. This case report aims to raise awareness for identification of these abnormalities to help facilitate proper evaluation and management.

References

1. Nagy, R.D., Iliescu, D.G. 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, April; 12(4): 873. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9027129/#:~:text=A%20total%20of%20 31%20cases,examined%20from%202017%20to%202021.

2. Yagel, S., Kivilevitch, Z., Cohen, S.M., et al. The fetal venous system, Part II: ultrasound evaluation of the fetus with congenital venous system malformation or developing circulatory compromise. Ultrasound Obstetrics & Gynecology. 2010; 36: 93-111. Februrary 5, 2010. DOI: 10.1002/uog.7622.

3. Lee, T.S., Steupert, M. Fact Sheet: The Growth of Maternal-Fetal Medicine and Fetal Care Centers in the United States. Charlotte Lozier Institute. July 21, 2022. https://lozierinstitute.org/fact-sheet-the-growth-of-maternal-fetal-medicine-and-fetal- care-centers-in-the-united-states/

4. Yagel, S., Kivilevitch, Z., Cohen, S.M., et al. Part I: normal embryology, anatomy, hemodynamics, ultrasound evaluation and Doppler investigation. Ultrasound Obstetrics & Gynecology. 2010; 35: 741-750. March 4, 2010. DOI: 10.1002/uog.7618.

5. Achiron, R., Kivilevitch, Z. Fetal Umbilical-Portal-Systemic Venous Shunt: in-utero classification and clinical significance. Ultrasound Obstetrics & Gynecology. 2016; 47: 739-747. May 12, 2015. DOI: 10.1002/uog.14906

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