KILT sindrome

A 41-year-old man, with no risk factors, with acute epigastric pain. An emergency CT angiography showed bilateral pulmonary embolism. Admission for anticoagulant treatment and study. Thrombophilia, tumour markers, and venous Doppler ultrasound negative. A body-CT scan (Fig. 1) showed right renal agenesis, inferior vena cava thrombosis extending to the bilateral ilio-femoral sector, and absence of repletion of its hepatic segment (KILT syndrome). A subsequent MRI (Fig. 2) ruled out neoformation and confirmed previous findings. The patient had a satisfactory evolution and was discharged with indefinite oral anticoagulation and elastic compression stockings. At 5-year follow-up: bilateral grade 3 post-thrombotic syndrome.

Fig. 1.

CT scan: Right renal agenesis, and absence of subhepatic and intrahepatic inferior vena cava (IVC). Dilated infrahepatic IVC with thrombosis extending to both iliac and femoral veins.

Fig. 2.

MRI: Thrombosis at the level of the ilio-caval axis. Right renal agenesis and absence of intrahepatic IVC.

DIAGNOSIS: KILT SYNDROME (Kidney and inferior vena cava abnormalities with Leg Thrombosis)