absent ductus venosus

Agenesis of the ductus venosus (ADV) is a rare fetal vascular anomaly.

Epidemiology

According to the data obtained from the screening tests performed at 11-14 weeks of pregnancy, its incidence is reported to be 1/2500 .

Pathology

In a large case series, Acherman et al.  defined five drainage patterns in absent ductus venosus:

• the umbilical vein is connected to the systemic venous circulation through the portal sinus
  • via an abnormal venous channel from the portal sinus to the right atrium 
  • presumably via hepatic sinusoids to the hepatic veins
• the umbilical vein bypasses the portal sinus and the liver and connects to the systemic venous circulation via an abnormal venous channel (case 2)
  • from the umbilical vein to the right atrium, directly or through a dilated coronary sinus: considered the most common type  and carries the worst prognosis due to the risk of developing congestive cardiac failure
  • from the umbilical vein to the midportion of the inferior vena cava (IVC)
• from the umbilical vein to the right iliac vein (case 1)

Associations

• development of hydrops fetalis
• fetal aneuploidy
• focal liver necrosis and calcification
• chromosomal anomalies, e.g. Turner syndrome 
• non-aneupliod syndromic anomalies, e.g. Noonan syndrome
• non-syndromic anomalies, e.g. congenital cardiac anomalies

Treatment and prognosis

The prognosis of fetuses with absent ductus venosus diagnosed at 11-13 weeks of gestation depends on the measurement of nuchal translucency thickness, being poor if the thickness is increased and good if it is normal .

Siehe auch:

• Hydrops fetalis
• Herzfehler
• Turner-Syndrom
• Noonan-Syndrom

und weiter:

• Ductus venosus