目的 总结小儿颅内静脉窦血栓形成(CVST)的病因、临床表现和影像学特征,以期早期诊断和及时治疗.方法 对复旦大学附属儿科医院于2008年8月至2012年5月收治住院的12例CVST患儿的临床表现、实验室资料、影像学特征、治疗经过及短期预后进行回顾性分析.结果 (1)病因:12例患儿中,7例病因不明,临床表现为亚急性或者慢性头痛进行性或急性加重；2例与感染相关；1例为颅内肿瘤术后；另外2例发生在肾病综合征激素治疗过程中.确诊前有7例发生误诊.(2)诊断:12例患儿均行头颅磁共振及磁共振静脉成像检查,明确诊断为颅内静脉窦血栓.(3)短期预后:所有患儿确诊后均予以抗凝治疗,11例临床症状好转；7例病因不明的患儿中确诊时4例存在不同程度的视力障碍,治疗后短期视力无改善；1例患儿确诊后至外院行数字减影血管造影及球囊扩张,后治疗无效死亡.结论 小儿CVST临床表现缺乏特异性,误诊率高,认识其临床特征并及时行磁共振静脉成像检查是诊断的关键.抗凝治疗是一种安全且有效的方法.%Objective To summarize the etiological,clinical and imaging characteristics of cerebral venous sinus thrombosis (CVST) in children so as to provide the basis for early diagnosis and prompt treatment.Methods All the medical records including clinical manifestations,laboratory data,neuroimaging changes,treatment and short-term prognosis were analyzed retrospectively in 12 cases of CVST hospitalized in Children' s Hospital of Fudan University from Aug 2008 to May 2012.Results (1) Regarding the etiology:of the 12 cases,the causes of CVST were infection (2/12),intracranial tumor (1/12),nephrotic syndrome (2/12),cryptogenic disease (7/12).Seven out of all 12 cases without definite cause were presented subacute or chronic headache associated with progressive or acute exacerbation.Seven cases had been misdiagnosed.(2)Diagnosis:All 12 cases were made a definite diagnosis as CVST after neuroimaging examination of brain magnetic resonance imaging combined with magnetic angiography venography.(3) Short-term prognosis:all the patients were treated with anticoagulation,and 11 cases improved.Four of 7 cases with cryptogenic disease had different degrees of visual impairment,and no improvement were found after the treatment; One patient died although accepted digital subtraction angiography and balloon catheter technique.Conclusions Cerebral venous sinus thrombosis has no specific clinical manifestations and a high rate of misdiagnosis.Increased consideration and prompt magnetic angiography venography play a key role in the accurate diagnosis.Anticoagulation is safe and effective.
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