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Ductus venosus agenesis with intrahepatic drainage

MedGen UID:
1052531
•Concept ID:
CN376965
•
Finding
HPO: HP:6000369

Definition

A form of ductus venosus agenesis in which the umbilical vein connects via the portal sinus to the portal venous system (PVS) without giving rise to the ductus venosus. In some cases an intrahepatic shunt from a portal to a hepatic vein can be detected prenatally. This variant has been reported to have a favorable prognosis. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDuctus venosus agenesis with intrahepatic drainage

Professional guidelines

PubMed

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.
Demirci O, Akay HÖ
J Matern Fetal Neonatal Med 2022 Oct;35(20):3872-3884. Epub 2020 Nov 2 doi: 10.1080/14767058.2020.1842870. PMID: 33138669
Prenatal Diagnosis of Agenesis of Ductus Venosus: A Retrospective Study of Anatomic Variants, Associated Anomalies and Impact on Postnatal Outcome.
Strizek B, Zamprakou A, Gottschalk I, Roethlisberger M, Hellmund A, Müller A, Gembruch U, Geipel A, Berg C
Ultraschall Med 2019 Jun;40(3):333-339. Epub 2017 Sep 21 doi: 10.1055/s-0043-115109. PMID: 28934814

Recent clinical studies

Etiology

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.
Demirci O, Akay HÖ
J Matern Fetal Neonatal Med 2022 Oct;35(20):3872-3884. Epub 2020 Nov 2 doi: 10.1080/14767058.2020.1842870. PMID: 33138669
Prenatal Diagnosis of Agenesis of Ductus Venosus: A Retrospective Study of Anatomic Variants, Associated Anomalies and Impact on Postnatal Outcome.
Strizek B, Zamprakou A, Gottschalk I, Roethlisberger M, Hellmund A, Müller A, Gembruch U, Geipel A, Berg C
Ultraschall Med 2019 Jun;40(3):333-339. Epub 2017 Sep 21 doi: 10.1055/s-0043-115109. PMID: 28934814
A retrospective single centre review of the incidence and prognostic significance of persistent foetal right umbilical vein.
Kumar SV, Chandra V, Balakrishnan B, Batra M, Kuriakose R, Kannoly G
J Obstet Gynaecol 2016 Nov;36(8):1050-1055. Epub 2016 Sep 6 doi: 10.1080/01443615.2016.1196171. PMID: 27599270
Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance.
Achiron R, Kivilevitch Z
Ultrasound Obstet Gynecol 2016 Jun;47(6):739-47. doi: 10.1002/uog.14906. PMID: 25988346
Shunt diameter in agenesis of the ductus venosus with extrahepatic portosystemic shunt impacts on prognosis.
Shen O, Valsky DV, Messing B, Cohen SM, Lipschuetz M, Yagel S
Ultrasound Obstet Gynecol 2011 Feb;37(2):184-90. Epub 2010 Jun 2 doi: 10.1002/uog.7702. PMID: 20521238

Diagnosis

Absence of Ductus Venosus: A Comparison of 2 Distinctive Fetal Autopsy Cases and Embryologic Perspectives.
Chan ES, Suchet I, Yu W, Somerset D, Soliman N, Kuret V, Chadha R
Pediatr Dev Pathol 2024 Mar-Apr;27(2):139-147. Epub 2023 Dec 14 doi: 10.1177/10935266231211760. PMID: 38098271Free PMC Article
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.
Demirci O, Akay HÖ
J Matern Fetal Neonatal Med 2022 Oct;35(20):3872-3884. Epub 2020 Nov 2 doi: 10.1080/14767058.2020.1842870. PMID: 33138669
Prenatal Diagnosis of Agenesis of Ductus Venosus: A Retrospective Study of Anatomic Variants, Associated Anomalies and Impact on Postnatal Outcome.
Strizek B, Zamprakou A, Gottschalk I, Roethlisberger M, Hellmund A, Müller A, Gembruch U, Geipel A, Berg C
Ultraschall Med 2019 Jun;40(3):333-339. Epub 2017 Sep 21 doi: 10.1055/s-0043-115109. PMID: 28934814
Ductus venosus agenesis and fetal malformations: what can we expect? - a systematic review of the literature.
Pacheco D, Brandão O, Montenegro N, Matias A
J Perinat Med 2018 Dec 19;47(1):1-11. doi: 10.1515/jpm-2018-0163. PMID: 29949516
In utero ultrasonographic diagnosis of an aberrant umbilical vein associated with fetal hepatic hyperechogenicity.
Cohen SB, Lipitz S, Mashiach S, Hegesh J, Achiron R
Prenat Diagn 1997 Oct;17(10):978-82. PMID: 9358580

Prognosis

Prenatal Diagnosis of Agenesis of Ductus Venosus: A Retrospective Study of Anatomic Variants, Associated Anomalies and Impact on Postnatal Outcome.
Strizek B, Zamprakou A, Gottschalk I, Roethlisberger M, Hellmund A, Müller A, Gembruch U, Geipel A, Berg C
Ultraschall Med 2019 Jun;40(3):333-339. Epub 2017 Sep 21 doi: 10.1055/s-0043-115109. PMID: 28934814
Ductus venosus agenesis and fetal malformations: what can we expect? - a systematic review of the literature.
Pacheco D, Brandão O, Montenegro N, Matias A
J Perinat Med 2018 Dec 19;47(1):1-11. doi: 10.1515/jpm-2018-0163. PMID: 29949516
A retrospective single centre review of the incidence and prognostic significance of persistent foetal right umbilical vein.
Kumar SV, Chandra V, Balakrishnan B, Batra M, Kuriakose R, Kannoly G
J Obstet Gynaecol 2016 Nov;36(8):1050-1055. Epub 2016 Sep 6 doi: 10.1080/01443615.2016.1196171. PMID: 27599270
Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance.
Achiron R, Kivilevitch Z
Ultrasound Obstet Gynecol 2016 Jun;47(6):739-47. doi: 10.1002/uog.14906. PMID: 25988346
Shunt diameter in agenesis of the ductus venosus with extrahepatic portosystemic shunt impacts on prognosis.
Shen O, Valsky DV, Messing B, Cohen SM, Lipschuetz M, Yagel S
Ultrasound Obstet Gynecol 2011 Feb;37(2):184-90. Epub 2010 Jun 2 doi: 10.1002/uog.7702. PMID: 20521238

Clinical prediction guides

A retrospective single centre review of the incidence and prognostic significance of persistent foetal right umbilical vein.
Kumar SV, Chandra V, Balakrishnan B, Batra M, Kuriakose R, Kannoly G
J Obstet Gynaecol 2016 Nov;36(8):1050-1055. Epub 2016 Sep 6 doi: 10.1080/01443615.2016.1196171. PMID: 27599270
Shunt diameter in agenesis of the ductus venosus with extrahepatic portosystemic shunt impacts on prognosis.
Shen O, Valsky DV, Messing B, Cohen SM, Lipschuetz M, Yagel S
Ultrasound Obstet Gynecol 2011 Feb;37(2):184-90. Epub 2010 Jun 2 doi: 10.1002/uog.7702. PMID: 20521238

Recent systematic reviews

Ductus venosus agenesis and fetal malformations: what can we expect? - a systematic review of the literature.
Pacheco D, Brandão O, Montenegro N, Matias A
J Perinat Med 2018 Dec 19;47(1):1-11. doi: 10.1515/jpm-2018-0163. PMID: 29949516

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