Bilateral Deep Vein Thrombosis, Vena Cava Agenesis, and Renal Abnormalities: KILT Syndrome-A Case Report and Literature Review

Abstract

KILT (kidney and IVC abnormalities with leg thrombosis) syndrome is a very rare condition that associates inferior vena cava abnormalities, renal defects, and venous thrombosis. These vascular disorders appear in 0.6-2% of patients with cardiovascular events and condition a venous stasis that contributes to the formation of thrombus in the lower limbs. Only a few cases of KILT syndrome have been published in the literature and the genesis, epidemiology, and natural history of the disease are yet unknown. We present a case of a 39-year-old man with no medical background who developed thrombosis of the inferior vena cava in its infrarrenal portion to both common iliac veins, all associated with agenesis of the rest of the vascular structure and an atrophic right kidney. The patient was treated with full anticoagulation, without the development of renal failure, postthrombotic syndrome, nor other complications. Thrombophilia study was normal, including mutations of prothrombin gene, factor V Leiden, and antiphospholipid antibodies. We hypothesize the benefit of an early diagnosis to improve the management of this condition. KILT syndrome must be taken into account in cases of proximal venous thrombosis, especially in young patients without risk factors for the development of thrombosis.

Keywords: Anticoagulants; Inferior vena cava; Venous thrombosis.