Isolated Absent Ductus Venosus with Intrahepatic Shunt: Case Report and Review of Literature

Abstract

A 28-year-old primigravida with spontaneous conception and no complicating medical illness presented to the authors for an early morphology scan. Her scan was unremarkable with normal nuchal translucency for crown rump length, no stigmata for chromosomal abnormalities and no major detectable structural abnormality. A repeated search for ductus venosus revealed non visualization of the ductus with intrahepatic drainage of the umbilical vein. The karyotype was normal. Fetal echocardiogram, anomaly and serial growth scans were also within normal limits. She developed mild hydramnios in the late third trimester and delivered a normal healthy female child at 37 weeks. Postnatal echocardiogram showed a very small muscular ventricular septal defect. Mild pulmonary stenosis has been diagnosed at the age of 6 months. Ductus venosus is the key regulator of oxygenated blood in fetal life. Absent ductus venosus is associated with multiple structural, chromosomal, cardiac abnormalities partial or complete absence of portal venous system, hydrops and fetal death. In the absence of ductus venosus the umbilical vein may drain normally into the liver (intrahepatic shunt) or may drain in an aberrant location into the systemic circulation (e.g., iliac vein, inferior vena cava, right heart and coronary sinus) creating an extrahepatic shunt. In all the cases of absent ductus venosus, the prognosis depends on the presence or absence of associated abnormalities. In cases of extrahepatic aberrant drainage of the umbilical vein, the prognosis depends upon the presence or absence of portal venous system, abnormal shunt site and shunt diameter. In general, intrahepatic variant has a much better prognosis in the absence of other abnormalities.

Keywords

Absent ductus venosus - Intrahepatic shunt - Extrahepatic shunt - Congenital absence of portal venous system - Aberrant drainage of umbilical vein - Turner syndrome

Publication History

Received: 01 September 2014

Accepted: 26 September 2014

Article published online:
08 May 2023