CTICasesCongenital Absence of the Intrahepatic Inferior Vena Cava (IVC)
Imaging findings
Contrast-enhanced CT of the abdomen reveals complete thrombosis of the infrarenal systemic iliac veins with some contrast layering at the edges, and reconstitution of flow at the level of the renal arteries through extensive retroperitoneal and subcutaneous collaterals. There is a complete absence or atresia of the intrahepatic segment of the inferior vena cava (IVC). The hepatic veins drain retrogradely back down into the infrahepatic IVC, which then flows back through collaterals and eventually drains into the superior vena cava system. Massive collateral vessels are seen extending from intra-abdominal spaces, penetrating the musculature, and coursing through a small defect into the subcutaneous soft tissues of the abdominal wall. Marked splenomegaly, hepatomegaly, and ascites are also present.
Key takeaways
Congenital absence of the intrahepatic portion of the IVC is a rare vascular anomaly where the hepatic segment of the IVC fails to develop (arising instead from embryonic vitelline vein remnants), often replaced by a ligamentous band of tissue. While patients can remain asymptomatic for decades due to rich collateralization, they are highly prone to progressive lower extremity and deep venous thrombosis, severe edema, hepatic congestion, and eventual hepatic failure. Any surgical attempt to reconstruct the intrahepatic portion may fail, leaving the patient dependent on massive subcutaneous and retroperitoneal collateral networks that pose a high risk of hemorrhage if injured.
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