Tarango Cristina, Kumar Riten, Patel Manish, Blackmore Anne, Warren Patrick, Palumbo Joseph S
Division of Hematology, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center and the University of Cincinnati College of Medicine, Cincinnati, Ohio.
Division of Hematology, Oncology and Bone Marrow Transplant, Nationwide Children's Hospital and The Ohio State University, Columbus, Ohio.
Pediatr Blood Cancer. 2018 Feb;65(2). doi: 10.1002/pbc.26785. Epub 2017 Aug 29.
Thrombosis in the healthy pediatric population is a rare occurrence. Little is known about the optimal treatment or outcomes of children with unprovoked acute lower extremity (LE) deep vein thrombosis (DVT) associated with atresia of the inferior vena cava (IVC).
We retrospectively analyzed the records of patients with acute LE DVT subsequently found to have IVC atresia who presented to two tertiary pediatric institutions between 2008 and 2016. Data were reviewed for thrombophilia risk factors, treatment, and outcomes.
Eighteen patients, aged 13-18 years (median: 16 years), presenting with acute LE DVT were found to have IVC atresia. Three patients also presented with pulmonary embolism. Fourteen patients underwent site-directed thrombolysis in addition to anticoagulation. Five patients (28%) had confirmed or suspected recurrent thrombosis. Thirteen patients (72%) had no identified provocation for DVT. Ten patients (56%) had post-thrombotic syndrome, and 17 of 18 patients remain on indefinite anticoagulation.
This study suggests that IVC atresia is a risk factor for LE DVT and pulmonary embolism in otherwise healthy children and highlights the importance of dedicated imaging of the IVC in young patients with unprovoked LE DVT. Indefinite anticoagulation may be considered in pediatric patients presenting with unprovoked thrombosis secondary to an atretic IVC.
健康儿童人群中血栓形成较为罕见。对于患有与下腔静脉（IVC）闭锁相关的不明原因急性下肢（LE）深静脉血栓形成（DVT）的儿童，其最佳治疗方法或预后知之甚少。
我们回顾性分析了2008年至2016年间在两家三级儿科机构就诊的急性LE DVT患者的记录，这些患者随后被发现患有IVC闭锁。对血栓形成倾向危险因素、治疗方法和预后进行了数据审查。
18例年龄在13 - 18岁（中位数：16岁）的急性LE DVT患者被发现患有IVC闭锁。3例患者还出现了肺栓塞。14例患者在抗凝治疗的基础上还接受了局部溶栓治疗。5例患者（28%）确诊或疑似复发性血栓形成。13例患者（72%）的DVT未发现诱因。10例患者（56%）出现了血栓后综合征，18例患者中有17例仍在接受长期抗凝治疗。
本研究表明，IVC闭锁是原本健康儿童发生LE DVT和肺栓塞的危险因素，并强调了对不明原因LE DVT的年轻患者进行IVC专项成像检查的重要性。对于因IVC闭锁继发不明原因血栓形成的儿科患者，可考虑长期抗凝治疗。
