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J Med Ultrasound
J Med Ultrasound
JMU
J Med Ultrasound
Journal of Medical Ultrasound
0929-6441
2212-1552
Wolters Kluwer - Medknow India

JMU-32-277
10.4103/jmu.jmu_38_23
Imaging for Residents – Answer
Prenatal Sonographic Diagnosis of Rare Fetal Anomaly
Dhawan Vaishali 1*
Rathod Ashwini 2
Choudhary Shilpa Raut 2
1 Department of Radiodiagnosis, Jawaharlal Nehru Medical College, Wardha, Maharashtra, India
2 Department of Radiodiagnosis, Datta Meghe Institute of Medical Sciences, Nagpur, Maharashtra, India
Address for correspondence: Dr. Vaishali Dhawan, Department of Radiodiagnosis, Jawaharlal Nehru Medical College, Sawangi (Meghe), Wardha - 442 001, Maharashtra, India. E-mail: vaishalibdhawan@rediffmail.com
Jul-Sep 2024
01 9 2023
32 3 277278
07 4 2023
29 4 2023
08 5 2023
Copyright: © 2023 Journal of Medical Ultrasound
2023
https://creativecommons.org/licenses/by-nc-sa/4.0/ This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
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pmcSECTION 2 – ANSWER

Case report

A 24-year-old primi-gravida female came for routine prenatal ultrasound at 26 weeks of gestation. Ultrasonography showed polyhydramnios (Amniotic fluid index: 26 cm) and a cardiac anomaly as evaluated on gray scale [Figures 1 and 2] and color Doppler images [Figure 3]. There was no other structural anomaly seen. What is your diagnosis?

Figure 1 Gray scale image of fetal heart shows mild dilated right atrium

Figure 2 Gray scale image showing dilated fetal umbilical vein (UV), crossing the diaphragm and directly draining into the right atrium (RA)

Figure 3 Colour Doppler image showing dilated fetal umbilical vein (UV), crossing the diaphragm and directly draining into the right atrium (RA)

INTERPRETATION

Ultrasonography showed dilated fetal umbilical vein (UV), crossing the diaphragm and directly draining into the right atrium (RA). The RA was dilated [Figures 1-3]. There was no other structural anomaly seen in this case; hence, a diagnosis of isolated absent ductus venosus (DV) was made.

DISCUSSION

DV agenesis is a rare anomaly which is significantly associated with cardiac, extracardiac, and chromosomal anomalies.[12] Associated anomalies ranging from isolated cardiac anomalies like ventricular septal defect to complex anomalies like Dandy-Walker syndrome, corpus callosum agenesis, and chromosomal anomalies like trisomy 18 and 21 have been documented.[34] With or without associated anomalies, risk of developing fetal hydrops and intrauterine cardiac failure remains high.[34] DV is an important fetal shunt connecting intra-abdominal UV to fetal inferior vena-cava near its entry to heart.[35] In case of agenesis of DV, the blood from UV flows through alternative vasculature that may either be extrahepatic or intrahepatic system (via the portal venous system). Multiple variable connections have been seen in extrahepatic shunts. Bypassing the liver, the blood from UV is directly shunted to the heart via one of the multiple channels viz., inferior vena cava, iliac vein, renal vein, or to the RA and rarely to left atrium or coronary sinus. In intrahepatic shunts, the UV shows usual connection through portal sinus to hepatic sinusoids but without giving rise to the DV.[13456] Prenatal ultrasound may be helpful in early diagnosis.[2] In our case, the route for umbilical venous return was extrahepatic umbilical venous drainage, where the UV was seen shunting blood directly into the RA. Although no other structural anomaly was seen, associate findings of polyhydramnios increased the risk of poor prognosis. Review of literature of absent DV suggests poor intrauterine as well as perinatal prognosis. The would-be parents in our case were thoroughly counseled about poor prognosis and they opted for termination of pregnancy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.
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REFERENCES

1 Dhingra B Makam A Agenesis of ductus venosus: A case series J Fetal Med 2020 7 149 54
2 Choudhary R Choudhary SB Isolated absent ductus venosus with intrahepatic shunt: Case report and review of literature J Fetal Med 2014 1 103 6
3 Berg C Kamil D Geipel A Kohl T Knöpfle G Hansmann M Absence of ductus venosus-importance of umbilical venous drainage site Ultrasound Obstet Gynecol 2006 28 275 81 16826563
4 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 9 11844198
5 Strizek B Zamprakou A Gottschalk I Roethlisberger M Hellmund A Müller A Prenatal diagnosis of agenesis of ductus venosus: A retrospective study of anatomic variants, associated anomalies and impact on postnatal outcome Ultraschall Med 2019 40 333 9 28934814
6 Yagel S Kivilevitch Z Cohen SM Valsky DV Messing B Shen O The fetal venous system, Part II: Ultrasound evaluation of the fetus with congenital venous system malformation or developing circulatory compromise Ultrasound Obstet Gynecol 2010 36 93 111 20205158
