A Case Report on KILT Syndrome (Kidney and Inferior Vena Cava Abnormalities with Legthrombosis) in A Young Male

Abstract First discovered in 2002, KILT syndrome is an extremely unusual trio consisting of kidney abnormalities, inferior vena cava (IVC) malformations, and deep vein thrombosis (DVT). This disorder, which is more common in younger people, is linked to an increased risk of recurrent thrombotic episodes and typically manifests itself accidentally during imaging. Additionally, it's important to closely observe any signs of possible renal problems.We present the case of a 20-year-old male patient who complained of right flank pain and right lower limb discomfort that had persisted for a period of 10 days. His right loin and lower abdomen pain has been becoming worse. There was nothing noteworthy in his medical background, risk factors, or family tree. Upon inspection, it was found that the right lower limb had edema of variable diameter. Vascular thrombosis was seen in several veins on CT scans, including the common femoral, internal iliac, and right common iliac veins. The inferior vena cava (IVC) below the liver was very small, measuring just 0.8 cm in diameter, and the IVC segment located inside the liver was completely missing. There were enlarged venous collaterals in the lumbar area, retro peritoneum, and pelvis. Additionally, there was enlargement of the azygos and hemiazygos veins. While the left kidney was hypertrophied, the right kidney was hypoplastic. The right renal vein was visible but not filled with blood. No emboli were seen in the pulmonary arterial system. There were no anomalies found in the urinalysis results, and the renal function tests came out normal, with a creatinine level of 92 μmol/L. Nothing came up during the thrombophilia screening. Oral anticoagulation with rivaroxaban was administered after low molecular weight heparin was administered as part of the treatment. Hydration and pain control were part of the supportive care. The patient reported less pain and marked reduction in leg edema upon discharge. Because of its link to recurrent deep vein thrombosis (DVT) and renal problems, KILT syndrome warrants investigation despite its rarity. Inadequate venous drainage increases the risk of deep vein thrombosis (DVT) in intravenous malformations, such as hypoplasia and azygos continuation. Patients presenting with limb symptoms that do not have an obvious cause should be evaluated for KILT syndrome. A multidisciplinary treatment strategy and lifelong anticoagulation are necessary for effective management. Improving treatment techniques and understanding long-term consequences require more research.