Rughani Prita, Yeung Frances, Halgren Camilla Raya, Cada Michaela, Schwartz Sarah
Department of Paediatrics, Whipps Cross Hospital, Barts Health NHS Trust, London, UK.
Department of Pediatric Medicine, Victoria Hospital and Children's Hospital, London, Ontario.
Paediatr Child Health. 2020 Aug;25(5):273-275. doi: 10.1093/pch/pxy170. Epub 2019 Apr 10.
Venous thromboembolism (VTE) is now increasingly recognized within paediatrics. A Canadian VTE registry has estimated the incidence as 0.7 to 1.0 per 100,000 population, with a peak in infancy and adolescence. Congenital inferior vena cava agenesis (IVCA) is an important risk factor that may be unfamiliar to paediatricians. Several case reports have since described an association between IVCA, VTE, and renal hypoplasia, which has been referred to as KILT syndrome (Kidney and IVC abnormalities with Leg Thromboses). We describe the first reported paediatric case of KILT syndrome in Canada. In any young patient presenting with a spontaneous DVT, particularly, if it is bilateral in nature with no co-existing risk factors for thrombus formation, we recommend investigating for the possibility of an underlying congenital vena cava anomaly. The use of prolonged anticoagulant therapy is supported by the inherent life-long risk of recurrent thrombosis associated with IVC anomalies.
静脉血栓栓塞症（VTE）如今在儿科领域越来越受到关注。加拿大的一个VTE登记处估计其发病率为每10万人中有0.7至1.0例，在婴儿期和青春期达到高峰。先天性下腔静脉发育不全（IVCA）是一个重要的风险因素，儿科医生可能对此并不熟悉。此后有几例病例报告描述了IVCA、VTE和肾发育不全之间的关联，这被称为KILT综合征（伴有腿部血栓形成的肾脏和IVC异常）。我们报告了加拿大首例KILT综合征的儿科病例。对于任何出现自发性深静脉血栓形成（DVT）的年轻患者，特别是如果血栓是双侧性的且不存在血栓形成的并存风险因素，我们建议调查是否存在潜在的先天性腔静脉异常。由于IVC异常存在复发性血栓形成的终身固有风险，因此支持使用延长抗凝治疗。
