Hostname: page-component-8448b6f56d-c4f8m Total loading time: 0 Render date: 2024-04-24T15:47:24.477Z Has data issue: false hasContentIssue false
  • English
  • Français

Agenesis of the venous duct: two cases of extrahepatic drainage of the umbilical vein and extrahepatic portosystemic shunt with a review of the literature

Published online by Cambridge University Press:  22 May 2014

Rohit S. Loomba ,
Michele Frommelt ,
David Moe  and
Amanda J. Shillingford
Rohit S. Loomba*
Affiliation:
Department of Pediatric Cardiology, Children’s Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
Michele Frommelt
Affiliation:
Department of Pediatric Cardiology, Children’s Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
David Moe
Affiliation:
Department of Pediatric Cardiology, Children’s Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
Amanda J. Shillingford
Affiliation:
Department of Pediatric Cardiology, Children’s Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
*
Correspondence: R.S. Loomba, Department of Pediatric Cardiology, Children’s Hospital of Wisconsin, 9000 Wisconsin Avenue, Milwaukee 53226, United States of America. Tel: 414-266-2380; Fax: 414-266-2381; E-mail: rloomba@mcw.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

Agenesis of the venous duct is a rare congenital anomaly resulting in abnormal drainage of the umbilical vein into the foetal venous circulation. The clinical presentation and prognosis is variable, and may depend on the specific drainage pathways of the umbilical vein. We present two foetuses with agenesis of the venous duct, both associated with a postnatal portosystemic shunt, but with markedly different postnatal clinical courses. We also review all previously reported cases to better characterise this foetal disorder and the prognosis.

Keywords

Venous ductabsenceagenesisumbilical veinAbernethy malformationportosystemic shuntductus venosus
Type
Review Articles
Information
Cardiology in the Young , Volume 25 , Issue 2 , February 2015 , pp. 208 - 217
Copyright
© Cambridge University Press 2014 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Kiserud, T, Rasmussen, S, Skulstad, S. Blood flow and the degree of shunting through the ductus venosus in the human fetus. Am J Obstet Gynecol 2000; 182: 147–153.Google Scholar
2. Edelstone, DI, Rudolph, AM. Preferential streaming of ductus venosus blood to the brain and heart in fetal lambs. Am J Physiol 1979; 237: H724–H729.Google Scholar
3. 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.Google Scholar
4. Paltauf, R. ein Fall vin Mangel des ductus venosus Arrantii. Wien Klin Wochenschr 1888; 9: 127–131.Google Scholar
5. Sadler, TW, Langman, J. Langman’s Medical Embryology. Wolters Kluwer Health/Lippincott Williams & Wilkins, Philadelphia, 2012.Google Scholar
6. Moss, AJ, Allen, HD. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. Wolters Kluwer Health/Lippincott Williams & Wilkins, Philadelphia, 2008.Google Scholar
7. Rychik, JTZ. Fetal Cardiovascular Imaging. Elselvier, Philadelpha, PA, USA, 2011.Google Scholar
8. Thomas, JT, Petersen, S, Cincotta, R, Lee-Tannock, A, Gardener, G. Absent ductus venosus – outcomes and implications from a tertiary centre. Prenat Diagn 2012; 32: 686–691.Google Scholar
9. Hendson, L, Emery, DJ, Phillipos, EZ, Bhargava, R, Olley, PM, Lemke, RP. Persistent pulmonary hypertension of the newborn presenting as the primary manifestation of intracranial arteriovenous malformation of the Vein of Galen. Am J Perinatol 2000; 17: 405–410.CrossRefGoogle ScholarPubMed
10. Ohno, T, Muneuchi, J, Ihara, K, et al. Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics 2008; 121: e892–e899.Google Scholar
11. 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.CrossRefGoogle Scholar
12. Cohen, SB, Lipitz, S, Mashiach, S, Hegesh, J, Achiron, R. In utero ultrasonographic diagnosis of an aberrant umbilical vein associated with fetal hepatic hyperechogenicity. Prenat Diagn 1997; 17: 978–982.Google Scholar
13. Hofstaetter, C, Plath, H, Hansmann, M. Prenatal diagnosis of abnormalities of the fetal venous system. Ultrasound Obstet Gynecol 2000; 15: 231–241.CrossRefGoogle ScholarPubMed
14. 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: 185–189.Google Scholar
15. Kinare, AS, Ambardekar, ST, Bhattacharya, D, Pande, SA. Prenatal diagnosis with ultrasound of anomalous course of the umbilical vein and its relationship to fetal outcome. J Clin Ultrasound 1996; 24: 333–338.Google Scholar
16. Moore, L, Toi, A, Chitayat, D. Abnormalities of the intra-abdominal fetal umbilical vein: reports of four cases and a review of the literature. Ultrasound Obstetrics Gynecol 1996; 7: 21–25.Google Scholar
17. Avni, EF, Ghysels, M, Donner, C, Damis, E. In utero diagnosis of congenital absence of the ductus venosus. J Clin Ultrasound 1997; 25: 456–458.Google Scholar
18. 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: 418–423.Google Scholar
19. Perles, Z, Nir, A, Nadjari, M, Ergaz, Z, Raas-Rothschild, A, Rein, AJ. 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: 247–251.Google Scholar
20. 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: 69–73.CrossRefGoogle ScholarPubMed
21. Tutar, E, Fitoz, S. Absent ductus venosus associated with persistent truncus arteriosus: prenatal diagnosis. Cardiol Young 2010; 20: 345–348.CrossRefGoogle ScholarPubMed
22. Sothinathan, U, Pollina, E, Huggon, I, Patel, S, Greenough, A. Absence of the ductus venosus. Acta Paediatr 2006; 95: 620–621.Google Scholar
23. Staboulidou, I, Pereira, S, Cruz Jde, J, Syngelaki, A, Nicolaides, KH. Prevalence and outcome of absence of ductus venosus at 11(+0) to 13(+6) weeks. Fetal Diagn Ther 2011; 30: 35–40.Google Scholar
24. Clerici, G, Rosati, A, Di Renzo, GC. Absent ductus venosus associated with skeletal anomalies of the ulna and radius. Prenat Diagn 2010; 30: 83–85.Google Scholar
25. Jowett, V, Paramasivam, G, Seale, A, Choudhry, M, Yates, R, Gardiner, H. Diaphragmatic hernia: a postnatal complication of anomalous drainage of the umbilical vein. Ultrasound Obstet Gynecol 2013; 41: 589–591.CrossRefGoogle ScholarPubMed
26. Tanaka, A, Kondo, Y, Hidaka, N, et al. Prenatal features of omphalocele and absent ductus venosus: case with a favorable postnatal outcome. J Ultrasound Med 2010; 29: 307–311.Google Scholar
27. Hajdu, J, Marton, T, Kozsurek, M, et al. Prenatal diagnosis of abnormal course of umbilical vein and absent ductus venosus – report of three cases. Fetal Diagn Ther 2008; 23: 136–139.CrossRefGoogle ScholarPubMed
28. Achiron, R, Hegesh, J, Yagel, S, Lipitz, S, Cohen, SB, Rotstein, Z. Abnormalities of the fetal central veins and umbilico-portal system: prenatal ultrasonographic diagnosis and proposed classification. Ultrasound Obstet Gynecol 2000; 16: 539–548.Google Scholar
29. Jaeggi, ET, Fouron, JC, Hornberger, LK, 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: 1031–1037.Google Scholar
30. Volpe, P, Marasini, M, Caruso, G, et al. Prenatal diagnosis of ductus venosus agenesis and its association with cytogenetic/congenital anomalies. Prenat Diagn 2002; 22: 995–1000.CrossRefGoogle ScholarPubMed
31. 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: 605–609.Google Scholar
32. Loomba, RS, Telega, GW, Gudausky, TM. Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula. J Pediatr Surg 2012; 47: E25–E31.Google Scholar
33. Jouk, PS, Champetier, J. Abnormal direct entry of the umbilical vein into the right atrium: antenatal detection, embryologic aspects. Surg Radiol Anat 1991; 13: 59–62.Google Scholar
34. Greiss, HB, McGahan, JP. Umbilical vein entering the right atrium: significance of in utero diagnosis. J Ultrasound Med 1992; 11: 111–113.Google Scholar
35. Hartung, J, Chaoui, R, Kalache, K, Tennstedt, C, Bollmann, R. Prenatal diagnosis of intrahepatic communications of the umbilical vein with atypical arteries (A-V fistulae) in two cases of trisomy 21 using color Doppler ultrasound. Ultrasound Obstet Gynecol 2000; 16: 271–274.CrossRefGoogle Scholar
36. Jorgensen, C, Andolf, E. Four cases of absent ductus venosus: three in combination with severe hydrops fetalis. Fetal Diagn Ther 1994; 9: 395–397.Google Scholar
37. Taddei, F, Signorelli, M, Prefumo, F, Franceschetti, L, Marasini, M, Groli, C. Prenatal imaging of ductus venosus agenesis using 4D ultrasound with a matrix array transducer. Ultrasound Obstet Gynecol 2008; 31: 477–479.CrossRefGoogle ScholarPubMed
38. Shih, JC, Shyu, MK, Hsieh, MH, et al. Agenesis of the ductus venosus in a case of monochorionic twins which mimics twin-twin transfusion syndrome. Prenat Diagn 1996; 16: 243–246.Google Scholar
39. Langman, G, Wainwright, H, Matthews, L. Absence of the ductus venosus with direct connection between the umbilical vein and right atrium. Pediatr Dev Pathol 2001; 4: 298–303.Google Scholar
40. Cayol, V, Braig, S, Noto, S, et al. Agenesia of the canal of Arantius. A case report. J Gynecol Obstet Biol Reprod (Paris) 1997; 26: 430–434.Google Scholar
41. Siven, M, Ley, D, Hagerstrand, I, Svenningsen, N. Agenesis of the ductus venosus and its correlation to hydrops fetalis and the fetal hepatic circulation: case reports and review of the literature. Pediatr Pathol Lab Med 1995; 15: 39–50.Google Scholar
42. Smith, JWJ. Absence of the ductus venosus: a case report. Infant 2008; 4: 121–123.Google Scholar
43. Hille, H, Chaoui, R, Renz, S, Hecher, K. Distended azygos and hemiazygos vein without interrupted inferior vena cava in a case of agenesis of the ductus venosus. Ultrasound Obstet Gynecol 2008; 31: 589–591.CrossRefGoogle Scholar
44. Marois, D, van Heerden, JA, Carpenter, HA, Sheedy, PF II. Congenital absence of the portal vein. Mayo Clin Proc 1979; 54: 55–59.Google Scholar
45. Morse, SS, Taylor, KJ, Strauss, EB, Ramirez, E, Seashore, JH. Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome). Pediatr Radiol 1986; 16: 437–439.Google Scholar
46. Grazioli, L, Alberti, D, Olivetti, L, et al. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 2000; 10: 820–825.Google Scholar
47. Yoshidome, H, Edwards, MJ. An embryological perspective on congenital portacaval shunt: a rare anomaly in a patient with hepatocellular carcinoma. Am J Gastroenterol 1999; 94: 2537–2539.Google Scholar
48. Wakamoto, H, Manabe, K, Kobayashi, H, Hayashi, M. Subclinical portal-systemic encephalopathy in a child with congenital absence of the portal vein. Brain Dev 1999; 21: 425–428.Google Scholar
49. Shinkai, M, Ohhama, Y, Nishi, T, et al. Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg 2001; 36: 1026–1031.Google Scholar
50. Kerlan, RK Jr., Sollenberger, RD, Palubinskas, AJ, Raskin, NH, Callen, PW, Ehrenfeld, WK. Portal-systemic encephalopathy due to a congenital portocaval shunt. Am J Roentgenol 1982; 139: 1013–1015.Google Scholar
51. Otake, M, Kobayashi, Y, Hashimoto, D, et al. An inferior mesenteric-caval shunt via the internal iliac vein with portosystemic encephalopathy. Intern Med 2001; 40: 887–890.Google Scholar