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J Perinat Med. 2018 Dec 19;47(1):1-11. doi: 10.1515/jpm-2018-0163.

Ductus venosus agenesis and fetal malformations: what can we expect? - a systematic review of the literature.

Author information

1
Faculty of Medicine, University of Porto, Porto, Portugal, Tel.: +351938619352.
2
Hospital S. João, Alameda Professor Hernâni Monteiro, 4200-319 Porto, Portugal.
3
Department of Pathology, Centro Hospitalar de S. João, Porto, Portugal.
4
Department of Obstetrics and Gynaecology, Centro Hospitalar de S. João, Faculty of Medicine, EPIUnit, University of Porto, Porto, Portugal.
5
Department of Obstetrics and Gynaecology, Centro Hospitalar de S. João, Faculty of Medicine, University of Porto, Porto, Portugal.

Abstract

Background The ductus venosus agenesis (DVA) is a rare condition with a variable prognosis that relies partly on the presence of associated conditions. The purpose of our study was to analyze the literature regarding the post-natal outcome of fetuses with DVA associated with fetal malformations, in order to discuss the best management options for couples. Methods We performed a systematic review of the literature of MEDLINE and SCOPUS electronic databases in a 25-year period from 1992 to September 2017. Methods We found 340 cases of DVA associated with fetal abnormalities. The most common chromosomal abnormalities were: monosomy X (12/48, 25%), trisomy 21 (11/48, 22.9%) and trisomy 18 (6/48, 12.5%). From the 340 cases with DVA, in 31 cases the umbilical venous shunt type was not reported. Of the fetuses, 60.8% (188/309) had an extrahepatic umbilical venous drainage while 39.2% (121/309) presented an intrahepatic connection. The DVA was associated in 71 cases (23.0%) with cardiac abnormalities, in 82 cases (26.5%) with extracardiac abnormalities and in 85 cases (27.5%) with both cardiac and extracardiac abnormalities. Conclusion DVA associated with both cardiac and extracardiac malformations may confer a poorer fetal outcome, a clinically relevant fact that should clarify what can be expected from this entity and help prenatal counseling.

KEYWORDS:

agenesis; ductus venosus; fetal malformations; outcome

PMID:
29949516
DOI:
10.1515/jpm-2018-0163

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