P1655: deep venous thrombosis in patient with atresia of inferior vena cava and right kidney hypoplasia (kilt syndrome): case report and systematic review of the literature

Topic: 34. Thrombosis and vascular biology - Biology & Translational Research Background: Inferior vena cava (IVC) anomalies are uncommon congenital causes of deep venous thrombosis (DVT). Their estimated prevalence among patients with DVT is up to 1% in general population, while in those younger than 30 years old it is 5%. It could be associated with other visceral abnormalities or isolated. KILT (Kidney abnormalities, IVC abnormalities, Leg Thrombosis) syndrome associates inferior vena cava with renal abnormalities, and venous thrombosis. Aims: The aim was to systematically review and analyze previously reported cases of KILT syndrome in terms of clinical and radiographic data and treatment. Methods: We performed a literature search of the PubMed, Scopus and Web of Science databases in May 2022. The search was performed in accordance with PRISMA guidelines, with no restrictions regarding publication date (figure). Case reports and case series reporting KILT syndrome were included. Data regarding demographics, clinical (presenting symptoms, presence of thrombophilia and other predisposing factors, comorbidities), radiographic, and therapeutic characteristics were extracted. Moreover, in addition to the previously published cases we included in the analysis our unpublished case of previously healthy 25 years old male with KILT syndrome. Results: After duplicates extraction, a screen of 3914 articles was done. A total of 30 cases were included in the study (including our case). The majority (80%) were males. The median age of this group was 24 years (range: 11-54). Pediatric cases (<18 years) comprised 36.7%. The majority of the patients presented with thrombosis related symptoms (leg swelling and pain), but fifth of the patients presented with abdominal or lumbar pain which was caused by collateral venous systems (e.g. azygos, lumbar). The majority of patients (60%) had right kidney abnormalities. Three patients suffered from arterial hypertension and one from chronic kidney disease. Two patients were smokers, other two had surgery close to thrombosis diagnosis, one used contraceptive pills and one was heterozygous FV Leiden carrier. Bilateral thrombosis was registered in 40%, while in 33% it was localized on the right side. In almost all cases the word was about iliofemoral thrombosis. In 23% of cases thrombosis was recurrent. In the majority of patients (76.7%) long-term anticoagulant therapy was indicated in majority of patients with low molecular heparin and vitamin K antagonists afterwards. Three patients, including the one we reported, received rivaroxaban. Due to persistence of symptoms during medical treatment in 3 patients (10%) invasive procedures (thrombectomy, balloon angioplasty, stent placement) were done. In one patient therapy with oral anticoagulants was ceased after 3 years, however thrombosis reoccurred after 6 years. One patient experienced intracerebral hemorrhage after trauma while on anticoagulant therapy. Summary/Conclusion: Anomalies of IVC, should be suspected in all young patients, especially males, with proximal, recurrent or idiopathic DVT. If IVC anomaly is confirmed, kidneys should be examined in order to monitor and preserve healthy kidney in case of KILT syndrome. In all patients with KILT syndrome long-term anticoagulation and risk factor control should be applied.Figure: Search criteria and PRISMA flowchart Keywords: Thrombosis, Deep venous thrombosis, Venous thrombosis