Abstract
Objective: To evaluate the clinical significance of ductus venosus agenesis
Material and method: Ductus venosus agenesis (DVA) cases registered to our hospital between January 2021 and September 2021 were retrospectively analysed. Ultrasound findings, prenatal features and postnatal findings were noted.
Results: During the study period 4 cases with DVA were detected. According to the study, the incidence of DVA was 2.6/10000. Two of the cases were of the intrahepatic type and the other two were of the extrahepatic type of DVA. Genetic test of case 3 and 4 were reported as normal, but genetic test of case 1 and 2 could not be determined because they rejected invasive prenatal testing. On prenatal ultrasonography, there was increased nuchal thickness in case 2 and 3. Hydrops was present in cases 2 and 4 and intrauterine death occured during prenatal folluow up. Because of severe preeclampsia, placental abruption and fetal disstres were detected in case 1, caserean section was performed at 27 weeks 3 days, and neonatal death occurred one week after birth. Pregnancy of case 3 reached term, but pregnant woman was not followed up in our hospital.
Conclusion: The rate of structural abnormality was increased in cases with DVA. Therefore, a detailed prenatal ultrasonography should be perfomed in any case when DVA detected. The long term results are not clearly known. Ductus venosus should be considered in the assessment of fetal anatomy along with the examination of nuchal thickness begining of the first trimester.
Keywords
Ductus venosus agenesis intrahepatic drainage extrahepatic drainage umblical vein portal system
References
- Referans1 Kiserud T, Acharya G. The fetal circulation. Prenat Diagn. 2004;24:1049–59.
- Referans2 Born M. The Ductus Venosus. Rofo. 2021 May;193(5):521-526. English, German Referans3 Strizek B, Zamprakou A, Gottschalk I, Roethlisberger M, Hellmund A, Müller A, Gembruch U, Geipel A, Berg C. Prenatal Diagnosis of Agenesis of Ductus Venosus: A Retrospective Study of Anatomic Variants, Associated Anomalies and Impact on Postnatal Outcome. Ultraschall Med. 2019 Jun;40(3):333-339
- Referans4 Edelstone DI, Rudolph AM, Heymann MA. Liver and ductus venosus blood flows in fetal lambs in utero. Circ Res 1978; 42: 426–33
- Referans5 Huisman TW, Stewart PA, Wladimiroff JW. Ductus venosus blood flow velocity waveforms in the human fetus—a Doppler study Ultrasound Med Biol 1992; 18: 33–7
- Referans6 Hecher K, Campbell S. Characteristics of fetal venous blood flow under normal circumstances and during fetal disease. Ultrasound Obstet Gynecol 1996; 7: 68–83
- Referans7 Acherman RJ, Evans WN, Galindo A et al. Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion. J Ultrasound Med 2007; 26: 1077–1082
- Referans8 Berg C, Kamil D, Geipel A et al. Absence of ductus venosus-importance of umbilical venous drainage site. Ultrasound Obstet Gynecol 2006; 28: 275–281
- Referans9 Contratti G, Banzi C, Ghi T et al. Absence of the ductus venosus: report of 10 new cases and review of the literature. Ultrasound Obstet Gynecol 2001; 18: 605–609
- Referans10 Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 1994; 29: 1239–41
- Referans11 Grazioli L, Alberti D, Olivetti L, Rigamonti W, Codazzi F, Matricardi L, Fugazzola C, Chiesa A. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 2000; 10 820–5
- Referans12 Souka AP, Krampl E, Bakalis S, Heath V, Nicolaides KH. Outcome of pregnancy in chromosomally normal fetuses with increased nuchal translucency in the first trimester. Ultrasound Obstet Gynecol 2001; 18: 9–17
- Referans13 Gembruch U, Baschat AA, 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: 185–9
- Referans14 Achiron R, Gindes L, Gilboa Y et al. Umbilical vein anomaly in foetuses with Down syndrome. Ultrasound Obstet Gynecol 2010; 35: 297–301
- Referans15 Weissmann-Brenner A, Zalel Y. Umbilical vein insertion into the inferior vena cava: an ominous sign of chromosomal abnormalities? J Ultrasound Med 2014; 33: 2207–2210
Abstract
Amaç: Duktus venozus agenezisinin klinik önemini değerlendirmek
Gereç ve Yöntem: Ocak 2020 ve Eylül 2021 tarihleri arasında hastanemizde duktus venosuz agenezisi (DVA) tanısı konulan hastaların kayıtları retrospektif olarak incelendi. DVA olgularının ultrason bulguları, antenatal özellikleri ve postnatal sonuçları değerlendirildi.
Bulgular: Çalışma süresi içinde 4 olgu DVA tanısı konulmuş olup, DVA insidansı 2,6/10000 olarak saptandı. Olguların ikisi intrahepatik tip, diğer ikisi ekstrahepatik tip DVA idi. İnvaziv testi kabul eden 3 ve 4 nolu olguların karyotipi normal olarak rapor edildi, 1 ve 2 nolu olgu ise invaziv testi reddetti. Hastaların prenatal ultrasonografisinde 2 ve 3 no lu olgularda artmış ense kalınlığı mevcuttu. 2 ve 4 numaralı olgularda hidrops fetalis saptandı ve bu olgular prenatal takipte intrauterin ex oldu. Olgu 1 ise 27 hafta 3 gün iken şiddetli preekalmpsi, plasental dekolman, fetal stres tanılarıyla acil sezeryan oldu ve doğumdan bir hafta sonra neonatal ölüm gerçekleşti. Olgu 3’ün gebeliği miada kadar ulaşmış, fakat gebe hastane takibimizden çıkmıştı.
Sonuç: DVA saptanan olgularda fetal anomali sıklığı artmıştır. Bu yüzden bu hastalara detaylı bir antenatal ultrasonografi yapılmalıdır. Duktus venozus, birinci trimesterden itibaren ense kalınlığı muayenesi ile birlikte fetal anatomiyi değerlendirilirken bakılması gereken bir oluşumdur.
Keywords
Duktus venozus agenezisi intrahepatik drenaj ekstrahepatik drenaj umblikal ven portal sistem
References
- Referans1 Kiserud T, Acharya G. The fetal circulation. Prenat Diagn. 2004;24:1049–59.
- Referans2 Born M. The Ductus Venosus. Rofo. 2021 May;193(5):521-526. English, German Referans3 Strizek B, Zamprakou A, Gottschalk I, Roethlisberger M, Hellmund A, Müller A, Gembruch U, Geipel A, Berg C. Prenatal Diagnosis of Agenesis of Ductus Venosus: A Retrospective Study of Anatomic Variants, Associated Anomalies and Impact on Postnatal Outcome. Ultraschall Med. 2019 Jun;40(3):333-339
- Referans4 Edelstone DI, Rudolph AM, Heymann MA. Liver and ductus venosus blood flows in fetal lambs in utero. Circ Res 1978; 42: 426–33
- Referans5 Huisman TW, Stewart PA, Wladimiroff JW. Ductus venosus blood flow velocity waveforms in the human fetus—a Doppler study Ultrasound Med Biol 1992; 18: 33–7
- Referans6 Hecher K, Campbell S. Characteristics of fetal venous blood flow under normal circumstances and during fetal disease. Ultrasound Obstet Gynecol 1996; 7: 68–83
- Referans7 Acherman RJ, Evans WN, Galindo A et al. Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion. J Ultrasound Med 2007; 26: 1077–1082
- Referans8 Berg C, Kamil D, Geipel A et al. Absence of ductus venosus-importance of umbilical venous drainage site. Ultrasound Obstet Gynecol 2006; 28: 275–281
- Referans9 Contratti G, Banzi C, Ghi T et al. Absence of the ductus venosus: report of 10 new cases and review of the literature. Ultrasound Obstet Gynecol 2001; 18: 605–609
- Referans10 Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 1994; 29: 1239–41
- Referans11 Grazioli L, Alberti D, Olivetti L, Rigamonti W, Codazzi F, Matricardi L, Fugazzola C, Chiesa A. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 2000; 10 820–5
- Referans12 Souka AP, Krampl E, Bakalis S, Heath V, Nicolaides KH. Outcome of pregnancy in chromosomally normal fetuses with increased nuchal translucency in the first trimester. Ultrasound Obstet Gynecol 2001; 18: 9–17
- Referans13 Gembruch U, Baschat AA, 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: 185–9
- Referans14 Achiron R, Gindes L, Gilboa Y et al. Umbilical vein anomaly in foetuses with Down syndrome. Ultrasound Obstet Gynecol 2010; 35: 297–301
- Referans15 Weissmann-Brenner A, Zalel Y. Umbilical vein insertion into the inferior vena cava: an ominous sign of chromosomal abnormalities? J Ultrasound Med 2014; 33: 2207–2210
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Obstetrics and Gynaecology |
| Journal Section | Case Report |
| Authors | |
| Publication Date | June 30, 2022 |
| Acceptance Date | June 14, 2022 |
| Published in Issue | Year 2022 Volume: 4 Issue: 2 |
