Absent infrarenal inferior vena cava

An absent infrarenal inferior vena cava can be congenital, due to the failure of development of the posterior cardinal and supracardinal veins, or acquired, as a result of intrauterine or perinatal inferior vena cava thrombosis.

Epidemiology

It is an extremely rare anomaly.

Clinical presentation

The patient may present with symptoms of lower extremity venous insufficiency or idiopathic deep venous thrombosis, particularly in younger patients.

Radiographic features

The non-invasive modalities of choice are contrast enhanced CT or MRI, which are preferred to ultrasound. The gold standard is venography, sometimes performed for surgical planning.

General features include:

• absent infrarenal inferior vena cava with preservation of the suprarenal segment
• common iliac veins may be absent
• external and internal iliac veins drain into the ascending lumbar veins, which drain into the azygos-hemiazygos system
• collateral paraspinal circulation may be present