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.

Ductus Venosus Agenesis: A Case Report

“…The anomaly scan done at 18 weeks of gestational age was suggestive of ductus venosus agenesis with intrahepatic shunting of the umbilical vein (Figure 1) along with a tiny echogenic focus in the left ventricle. 2 Quadruple scan for Down’s syndrome was within normal limits (Figure 2). Fluorescence in situ hybridization done to look for aneuploidy of chromosomes 13, 18, 21, and sex chromosomes also came out to be within normal limits (Figure 3A) and, while TORCH titres done for the mother were strongly positive for CMV IgG (Figure 3B).…”