Deep Venous Thrombosis of the Leg, Associated with Agenesis of the Infrarenal Inferior Vena Cava and Hypoplastic Left Kidney (KILT Syndrome) in a 14-Year-Old Child

Abstract

Agenesis of the inferior vena cava (IVC) is a rare anomaly which can be identified as incidental finding or can be associated with iliofemoral vein thrombosis. IVC agenesis has a known association with renal anomalies which are mainly confined to the right kidney. We describe a case of a 14-year-old male who presented with left leg swelling and pain. Ultrasonography confirmed the presence of left leg deep vein thrombosis (DVT). No underlying hematologic risk factors were identified. A CT scan was obtained which demonstrated absent infrarenal IVC and extensive thrombosis in the left deep venous system and development of collateral venous flow into the azygous/hemiazygous system, with extension of thrombus into paraspinal collaterals. An additional finding in the patient was an atrophic left kidney and stenosis of an accessory left renal artery. Agenesis of the IVC should be considered in a young patient presenting with lower extremity DVT, especially in patients with no risk factors for thrombosis. As agenesis of the IVC cannot be corrected, one should be aware that there is a lifelong risk of lower extremity DVT.

Figure 1

Grayscale ultrasound transverse image demonstrating a thrombus on the left common femoral vein. White arrow is pointing to the intraluminal echogenic material and noncompressibility, findings compatible with deep vein thrombosis.

Figure 2

Contrast enhanced abdominopelvic CT scan. Two contiguous coronal reformations showing patent suprarenal inferior vena cava (white arrow). Note absence of the infrarenal inferior vena cava in the right image.

Figure 3

There is thrombus noted within a paraspinal collateral vein (white arrow).

Figure 4

Contrast enhanced abdominopelvic CT scan. Coronal reformation demonstrating left renal hypoplasia with compensatory right renal hypertrophy. The right adrenal gland is heavily calcified.

Figure 5

(a) Abdominal CTA coronal reformation and (b) three-dimensional reconstruction showing the left kidney supplied by two hypoplastic renal arteries originating from the aorta. The origin of the inferior left renal artery has a focal area of stenosis proximally, depicted by the white arrow.