儿童脑静脉窦血栓形成的临床特点及治疗

Cerebral venous sinus thrombosis in children:clinical characteristics and treatment

目的分析儿童脑静脉窦血栓形成（CVST）的病因、临床和影像学特点，探讨其治疗方法及预后。方法2000年2月-2006年5月首都医科大学宣武医院共收治32例儿童（年龄从4周至14岁）CVST患儿。对其中22例行肝素抗凝治疗，持续12～18个月；对10例患儿应用血管内治疗方法，行静脉窦内接触性溶栓或机械性碎栓治疗。同时均给予降颅压、抗感染、抗癫痫等对症治疗，并分析患儿的病因、临床和影像学表现、预后情况。结果①患儿以癫痫发作、不同程度的意识障碍、颅内高压综合征和局灶性神经功能缺损症状为主要临床表现；神经影像学检查显示不同程度的脑静脉窦闭塞和脑实质损害征象。②引起CVST的病因中，感染占31．2％（10／32），颅脑外伤占37．5％（12／32），肾病综合征占3．1％（1／32），颅内肿瘤占3．1％（1／32），血液系统疾病占6．2％（2／32），遗传性血栓形成倾向（蛋白C或蛋白S缺乏）占6．2％（2／32），原因不明占12．5％（4／32）。③治疗后，32例患儿中，痊愈17例（53．1％），好转10例（31．2％），死亡5例（15．6％），总有效率为84．4％。24例患儿获得6个月至5年的随访，2例复发，5例仍有不同程度的颅内高压症状和体征，其余疗效稳定。结论儿童脑静脉窦血栓患者病因复杂，临床表现不典型；神经影像学检查能为确诊提供依据；抗凝和静脉窦内溶栓可改善预后。

Objectives To analyze the etiological, clinical, and imaging characteristics of cerebral venous sinus thrombosis （CVST） in children and to investigate its therapeutic approaches and prognosis. Methods A total of 32 children （ aged from 4 weeks to 14 years） with CVST admitted to Xuanwu Hospital, Capital Medical University, Beijing, China from February 2000 to May 2006. Of those 22 patients were treated with heparin anticoagulant therapy for 12 to 18 months. Interventional catheter-directed thrombolysis or mechanical fragulentation of the thrombus in venous sinus were performed in 10 patients. At the same time, the symptomatic treatment such as lowering intracranial pressure, anti-infection, and anti-epilepsy were provided. The etiological, clinical and imaging findings and the prognosis of patients were analyzed. Results （1）The main clinical manifestations of the children included seizures, different levels of consciousness, intracranial hypertension syndrome, and focal neurological defects. Neuroimaging examination showed different degrees of cerebral venous sinus occlusion and signs of brain parenehymal damage. （2）The causes of CVST were infection（31.2% ）, traumatic brain injury（37.5% ）, nephrotie syndrome（3.1% ）, intracranial tmnor（3.1% ）, hematological diseases（6. 2% ）, hereditary tendency to thrombosis （protein C or S deficiency,6. 2% ）, and eryptogenic disease（ 12. 5% ）. （3）After the treatment, 17 （53.1%） of the 32 patients were cured, 10 improved （31.2%） , and 5 died （ 15.6% ）. The total effective rate was 84. 4%. Twenty-two children were followed up for 6 months to 5 years, 2 relapsed and 5 still had different degrees of symptoms and signs of intracranial hypertension. The other patients were stable. Conclusions The etiology of CVST in children is complex, and its clinical manifestations is atypical. Neuroimaging examination may provide evidence for the confirmative diagnosis. Early diagnosis and effective treatment may improve the prognosis.