孕产妇合并颅内静脉窦血栓形成的诊疗策略

Treatment Strategies for Pregnancy with Cerebral Venous Sinus Thrombosis

目的探讨孕产妇合并颅内静脉窦血栓形成可能的病因、临床表现、影像学特征、诊断,总结诊疗策略。方法回顾性分析北京协和医院神经内科1993-2013年连续收治的孕产妇合并危重颅内静脉窦血栓形成患者12例,平均年龄（26±5）岁（19~34岁）。12例患者均在急性/亚急性期行计算机断层扫描（computed tomography,CT）或磁共振成像（magnetic resonance imaging,MRI）检查;9例行磁共振静脉成像（magnetic resonance venography,MRV）,6例行数字减影血管造影（digital subtraction angiography,DSA）检查诊断静脉窦血栓形成。分析可能的病因、临床表现、影像学特征、诊断及治疗策略。结果发病时期：孕早期7例,孕晚期1例,产后2周内4例,人工流产后1例;合并疾病包括：蛋白S缺乏1例,高同型半胱氨酸1例,特发性血小板减少性紫癜1例,全身感染1例,贫血1例;临床特征：均为急性或亚急性起病。头痛12例,痫性发作5例,其他局灶性神经系统体征5例（包括肢体无力、病理征、麻木、共济失调、周围性面瘫等）,视力下降1例,不同程度意识障碍4例。12例患者中9例存在脑实质异常,其中脑梗死或出血性梗死7例,脑出血1例,脑组织明显肿胀1例。受累静脉窦：横窦、乙状窦9例,上矢状窦8例,直窦、下矢状窦2例,颈静脉2例。治疗：6例妊娠期发病患者均及时终止妊娠,1例因家属不同意终止妊娠,病情加重,自动放弃回家。10例积极尽早给予肝素化抗凝治疗,所有患者均病情好转出院。结论孕产妇出现急性/亚急性头痛、抽搐及其他局灶体征时,需警惕危重颅内静脉窦血栓形成可能;影像学提示符合静脉窦引流区域的脑实质异常信号（出血性梗死为主）,可作为及时诊断的重要线索;病情危重时及时选择终止妊娠去除病因可能与良好预后相关,同时需尽早开始抗凝治疗。

Objective To discuss the possible cause, clinical symptoms, radiologic characteristics, diagnosis and our treatment strategies of cerebral venous sinus thrombosis （SCVT） in pregnancy. 〈br〉 Methods We presented 12 cases of pregnancy with emergent SCVT admitted between 1993 and 2013 in the Department of Neurology, Peking Union Medical College （PUMC） hospital. The average age was （26＆#177;5） （19~34）. Computed tomography （CT）/magnetic resonance imaging （MRI） was performed in all 12 patients in acute/subacute period; magnetic resonance venography （MRV） in 9 and digital subtraction angiography （DSA） in 6. The possible pathogenesis, clinical symptoms, radiological ifndings, diagnosis and treatment strategies were discussed. 〈br〉 Results Of the 12 pregnant or maternity patients with SCVT, 7 got the disease in early pregnancy, 1 in late pregnancy, 4 within 2 weeks after delivery, 1 after abortion. The accompanied diseases were：one with protein S deficiency, one with hyperhomocystinemia, one with idiopathic thrombocytopenia purpura, one with infection, one with anemia. Clinical onset was acute or subacute. Twelve patients had headache, 5 had epilepsy, 5 had focal neurological signs （including weakness, Babinski signs, numbness, ataxia, peripheral facial paralysis）, 1 had visual loss, 4 had disturbance of consciousness. All 12 patients had MRI during acute or subacute period, 9 had abnormal parenchymal signals--7 had infarction or hemorrhagic infarction, 1 had cerebral hemorrhage, and 1 had cerebral edema. Transverse sinus and sigmoid sinus were involved in 9 cases, superior sagittal sinus in 8, straight sinus and inferior sagittal sinus in 2, jugular vein in 〈br〉 2. Treatment：pregnancy was timely terminated in 6 pregnant patients; 1 patient whose family disagreed to terminate pregnancy aggravated and went back home. Ten patients accepted heparinized anti-coagulation as soon as possible, and all of them got better when they were discharged. 〈br〉 Conclusion When pregnant women had acute/subacute headache, epilepsy and other local signs, the possibility of SCVT should be suspected. Abnormal parenchymal signals in draining area of venous sinus （especially hemorrhagic infarction） could be a clue to diagnosis. Timely termination of pregnancy and anti-coagulation could lead to a good prognosis.