Congenital absence of the IVC

Case contributed by Bradley Kliewer
Diagnosis almost certain

Presentation

Patient presented with history of ETOH cirrhosis, Hep C, ESRD with altered mental status secondary to hepatic encephalopathy. History of frequent DVTs and found to have DVT on present admission. IR consult for IVC filter placement.

Patient Data

Age: 55
Gender: Female
dsa

Unable to visualize the right common femoral vein with ultrasound so decision was made to access left common femoral.  Unable to advance a wire so hand injection was preformed showing multiple collateral veins.  No external or common iliac vein visualized.

Inferior vena cava below the intrahepatic portion is not visualized. In the region of the inferior vena cava, there are numerous collaterals.

Case Discussion

The infrahepatic IVC develops from three paired embryonic veins at around 6-8 weeks of embryogenesis:

  • posterior cardinal
  • subcardinal
  • supracardinal

Absence of the entire IVC would result from failure of all three embryonic veins to develop properly. 

Absence of the infrarenal IVC would be the result of failure of the posterior cardinal and supracardinal veins to develop.

Controversy exists as to whether these conditions are the result of true anomalies or perinatal IVC thrombosis. Patients usually present with multiple DVTs, often at a young age.

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