Agenesis of Inferior Vena Cava Associated with Acute Bilateral Common Iliac Vein Thrombosis and Hypoplastic Right Kidney (KILT Syndrome) in a 54-Year-Old Female
Keywords:
Agenesis of inferior vena cava, deep vein thrombosisAbstract
Background and Objective: Agenesis of the inferior vena cava (AIVC) is a rare anomaly (<1%) with known association with renal anomalies, and an uncommon cause of deep venous thrombosis (DVT). Previous reported cases were young (mostly under 30 years), and predominantly male (82%), but the present case was a 54-year-old female with a thrombophilia sibling, presenting with acute onset DVT.
Material and Method: A 54-year-old female presented with right lower quadrant pain and swelling of both thighs, right more than left, for 12 consecutive days. There was no history of smoking, alcohol drinking, oral contraceptive use, or other underlying diseases. The patient had one brother with thrombophilia. A CT scan of the abdomen demonstrated absence of the entire inferior vena cava (IVC), and bilateral common iliac vein thrombosis with hypoplastic right kidney (KILT syndrome). Assessment for thrombophilia found presence of lupus anticoagulant antibody. The patient was treated with low molecular weight heparin (LMWH), and planned lifelong oral anticoagulant.
Conclusion: AIVC can be found very rarely in female patients over the age of 40 years, with positive family history of thrombophilia. Anticoagulants is the recommended treatment.
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