Department of Paediatrics and Adolescent Medicine, Faculty of Medicine, Medical Centre - University of Freiburg, Baden-Württemberg, Germany.
Institute of Clinical Chemistry and Laboratory Medicine, Faculty of Medicine, Medical Centre - University of Freiburg, Baden-Württemberg, Germany.
Hamostaseologie. 2023 Jun;43(3):188-195. doi: 10.1055/a-1848-3649. Epub 2022 Nov 10.
Cerebral sinus venous thrombosis (CSVT) is a rare disease, especially in children. Therefore, thrombophilia markers, risk factors, treatment strategy, and MRI, as well as clinical outcome need further investigation to support future diagnostic and therapeutic guidelines for children.
We retrospectively identified all children with CSVT treated in our center between January 1, 2000, and December 31, 2015. Risk factors and laboratory findings were investigated. Furthermore, outcome and treatment satisfaction were evaluated using magnetic resonance imaging (MRI) analyses and a modified questionnaire.
All 43 patients, who agreed to participate, were treated with therapeutic levels of heparin; 86% of children had an increased risk for thromboembolic events upon onset of CSVT (acute disease: 58.1%, perinatal risk factors: 9.3%, medical intervention/immobility: 14%, chronic disease: 16.3%). Thrombophilia markers showed positive results (e.g., reduced values for protein C/S, factor-V-Leiden mutation) in 58% of children at the time of CSVT diagnosis but dropping to 20.9% over the course of the disease. Forty-two of 43 patients received MRI follow-ups and the outcome showed complete recanalization in 69% of the patients and partial recanalization in 31%. At the onset of CSVT, 88% of patients reported restrictions in everyday life due to CSVT; at follow-up this percentage declined to 18%. Satisfaction with the outcome among parents/patients according to the questionnaire was high with 1.7 (German school grades from 1 to 6).
All 42 children with MRI follow-up demonstrated complete or partial recanalization under anticoagulation. This positive result underlines the need for future studies on anticoagulation to optimize therapy regimens of pediatric CSVT.
脑静脉窦血栓形成（CSVT）是一种罕见的疾病，特别是在儿童中。因此，血栓形成倾向标志物、危险因素、治疗策略、磁共振成像（MRI）以及临床结局需要进一步研究，以支持未来针对儿童的诊断和治疗指南。
我们回顾性地确定了 2000 年 1 月 1 日至 2015 年 12 月 31 日期间在我们中心接受治疗的所有 CSVT 患儿。调查了危险因素和实验室检查结果。此外，还通过 MRI 分析和改良问卷评估了预后和治疗满意度。
所有 43 名同意参与的患儿均接受了治疗剂量的肝素治疗；86%的患儿在 CSVT 发病时存在血栓栓塞事件的高风险（急性疾病：58.1%，围产期危险因素：9.3%，医疗干预/制动：14%，慢性疾病：16.3%）。血栓形成倾向标志物在 CSVT 诊断时呈阳性结果（例如，蛋白 C/S 值降低，因子 V Leiden 突变），占 58%，但在疾病过程中降至 20.9%。43 例患儿中有 42 例接受了 MRI 随访，结果显示 69%的患儿完全再通，31%的患儿部分再通。CSVT 发病时，88%的患儿因 CSVT 而在日常生活中受限；随访时，这一比例下降到 18%。根据问卷，父母/患儿对结果的满意度很高，为 1.7（德国学校成绩从 1 到 6）。
所有接受 MRI 随访的 42 例患儿在抗凝治疗下均实现完全或部分再通。这一积极结果强调了未来需要开展抗凝治疗研究，以优化儿童 CSVT 的治疗方案。
