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Clinical and Experimental Obstetrics & Gynecology  2020, Vol. 47 Issue (1): 12-15    DOI: 10.31083/j.ceog.2020.01.4923
Original Research Previous articles | Next articles
Prenatal diagnosis of ductus venosus agenesis: a retrospective study of associated cytogenetic/congenital anomalies
M. Özsürmeli1, *(), M. Sucu1, E. Arslan1
1Department of Obstetrics and Gynecology, Perinatology Unit, School of Medicine, University of Çukurova, Adana, Turkey
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Introduction: Absence of the ductus venosus (ADV) is a rare vascular anomaly often associated with fetal cardiac/extracardiac anomalies, aneuploidies, and hydrops. This study assesses the abnormal venous circulation, associated malformations, and chromosomal anomalies of ADV. Materials and Methods: The authors performed a retrospective study of 14 cases with ADV diagnosed by the prenatal ultrasound in tertiary referral center from 2009 to 2017. Results: The authors detected 14 patients of ADV. Karyotyping was offered to all cases, and four patients accepted the procedure. Normal karyotype was found in three out of four cases, while one case had 45, X0. Five cases underwent termination of pregnancy, six cases died at neonatal period due to accompanying cardiac anomalies, and heart failure. Three cases survived (the oldest baby is 4-years-old now). Umbilical vein (UV) was connected to portal venous system (PVS) in two cases (intrahepatic drainage, one of them is surviving), while UV was connected to systemic venous circulation in 12 cases (extrahepatic drainage, two of them are surviving). In five cases there was no other reason that might explain cardiomegaly and hydrops except ADV (two of them is surviving). Three cases were diagnosed as cystic hygroma in first trimester additionally found ADV. All of surviving fetuses delivered after 35th weeks of gestation. Conclusion: The presence fetal hydrops, accompanying congenital anomalies, and prematurity are associated with poor prognosis in fetuses with ADV. In isolated cases, the prognosis is generally good.

Key words:  Prenatal diagnoses      Ductus venosus      Umbilical vein      Congenital abnormality     
Published:  15 February 2020     
*Corresponding Author(s):  M. Özsürmeli     E-mail:

Cite this article: 

M. Özsürmeli, M. Sucu, E. Arslan. Prenatal diagnosis of ductus venosus agenesis: a retrospective study of associated cytogenetic/congenital anomalies. Clinical and Experimental Obstetrics & Gynecology, 2020, 47(1): 12-15.

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Table 1  — Associated anomalies, UV connection type, and outcomes in 14 cases with agenesis of the ductus venosus.
GA at diagnosis (weeks) GA at delivery (weeks) Associated anomalies UV
Karyotype Outcomes
1 28 35 Cardiac: VSD, cardiomegaly, tricuspid insufficiency
Extra cardiac: agenesis of corpus callosum
RA Not performed NL
2 35 39 Ebstein anomaly, pulmonary stenosis PVS Not performed NL
3 24 24 Cardiac: AVSD
Extra cardiac: Dandy Walker anomaly, micrognathia


Not performed

4 30 30 Cardiomegaly, hydrops RA Not performed NL
5 35 35 Cardiomegaly RA Not performed 4 years of life, survivor
6 13 13 Cystic hygroma, hydrops RA Not performed TOP
7 30 39 Cardiac: cardiomegaly, VSD
Extra cardiac: ventriculomegaly, pelvic kidney
RA Not performed 3 years of life, survivor
8 13 13 Cystic hygroma RA Not performed TOP
9 12 12 Cystic hygroma, AVSD RA 45, X0 TOP
10 25 37 Cardiac: truncus arteriosus
Extra cardiac: cerebellum hypoplasia
RA Normal NL
11 22 22 Cardiac: heterotaxia
Extra cardiac: ventriculomegaly
VCI Normal TOP
12 34 34 Cardiomegaly, hydrops VCI Not performed NL
13 23 36 Cardiomegaly PVS Normal 8 months of life, survivor
14 32 32 Hydrops, cardiomegaly RA Not performed NL
Figure 1.  — Ultrasonographic imaging of ductus venosus agenesis.

[1] Achiron R., Kivilevitch Z.: “Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance”. Ultrasound Obstet. Gynecol., 2016, 47, 739.
[2] Mavrides E., Moscoso G., Carvalho J.S., Campbell S., Thilaganathan B.: “The anatomy of the umbilical, portal and hepatic venous systems in the human fetus at 14-19 weeks of gestation”. Ultrasound Obstet. Gynecol., 2001, 18, 598.
[3] Moaddab A., Tonni G., Grisolia G., Bonasoni M.P., Araujo Júnior E., Rolo L.C., et al.: “Predicting outcome in 259 fetuses with agenesis of ductus venosus, a multicenter experience and systematic review of the literature”. J. Matern. Fetal Neonatal Med., 2016, 29, 3606.
[4] Kiserud T.: “Physiology of the fetal circulation”. Semin. Fetal Neonatal Med., 2005, 10, 493.
[5] Leonidas J.C., Fellows R.A.: “Congenital absence of the ductus venosus: with direct connection between the umbilical vein and the distal inferior vena cava”. AJR Am. J. Roentgenol., 1976, 126, 892.
[6] Corbacioglu A., Aslan H., Dagdeviren H., Ceylan Y.: “Prenatal diagnosis of abnormal course of umbilical vein and ductus venosus agenesis: report of three cases”. J. Clin. Ultrasound., 2012, 40, 590.
[7] Contratti G., Banzi C., Ghi T., Perolo A., Pilu G., Visentin A.: “Absence of the ductus venosus: report of 10 new cases and review of the literature”. Ultrasound Obstet. Gynecol., 2001, 18, 605.
[8] Berg C., Kamil D., Geipel A., Kohl T., Knöpfle G., Hansmann M., et al.: “Absence of ductus venosus-importance of umbilical venous drainage site”. Ultrasound Obstet. Gynecol., 2006, 28, 275.
[9] Jörgensen C., Andolf E.: “Four cases of absent ductus venosus: three in combination with severe hydrops fetalis”. Fetal Diagn. Ther., 1994, 9, 395.
[10] Sothinathan U., Pollina E., Huggon I., Patel S., Greenough A.: “Absence of the ductus venosus”. Acta Paediatr., 2006, 95, 620.
[11] Hajdu J., Marton T., Kozsurek M., Pete B., Csapó Z., Beke A., et al.: “Prenatal diagnosis of abnormal course of umbilical vein and absent ductus venosus—report of three cases”. Fetal Diagn. Ther., 2008, 23, 136.
[12] Hoppen T., Hofstaetter C., Plath H., Kau N., Bartmann P.: “Agenesis of the ductus venosus and its correlation to hydrops fetalis”. J. Perinat. Med., 2000, 28, 69.
[13] Jaeggi E.T., Fouron J.C., Hornberger L.K., Proulx F., Oberhänsli I., Yoo S.J., et al.: “Agenesis of the ductus venosus that is associated with extrahepatic umbilical vein drainage: prenatal features and clinical outcome”. Am. J. Obstet. Gynecol., 2002, 187, 1031.
[14] Perles Z., Nir A., Nadjari M., Ergaz Z., Raas-Rothschild A., Rein A.J.: “Absent ductus venosus in the fetus: review of the literature and first report of direct umbilical venous drainage to the coronary sinus”. Fetal Diagn. Ther., 2003, 18, 247.
[15] Sau A., Sharland G., Simpson J.: “Agenesis of the ductus venosus associated with direct umbilical venous return into the heart case series and review of literature”. Prenat. Diagn., 2004, 24, 418.
[16] Gembruch U., Baschat A.A., Caliebe A., Gortner L.: “Prenatal diagnosis of ductus venosus agenesis: a report of two cases and review of the literature”. Ultrasound Obstet. Gynecol., 1998, 11, 185.
[17] Maruotti G.M., Saccone G., Ciardulli A., Mazzarelli L.L., Berghella V., Martinelli P.: “Absent ductus venosus: case series from two tertiary centres”. J. Matern. Fetal Neonatal Med., 2017, 12, 1.
[18] Staboulidou I., Pereira S., Cruz Jde J., Syngelaki A., Nicolaides K.H. : “Prevalence and outcome of absence of ductus venosus at 11(+0)to 13(+6) weeks”. Fetal Diagn. Ther., 2011, 30, 35.
[19] Thomas J.T., Petersen S., Cincotta R., Lee-Tannock A., Gardener G.: “Absent ductus venosus outcomes and implications from a tertiary centre”. Prenat. Diagn., 2012, 32, 686.
[20] Takita H., Hasegawa J., Arakaki T., Hamada S., Tokunaka M., Nakamura M., et al.: “Outcomes in the absence of the ductus venosus diagnosed in the first trimester”. J. Matern. Fetal Neonatal Med., 2018, 31, 253.
[21] Iliescu D.G., Cara M.L., Tudorache S., Tudorache S., Antsaklis P., Novac L.V., et al.: “Agenesis of ductus venosus in sequential first and second trimester screening”. Prenat. Diagn., 2014, 34, 1099.
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