颅内静脉窦血栓形成的临床特征研究

Clinical Features of Cerebral Venous Sinus Thrombosis

背景颅内静脉窦血栓形成(CVST)发病原因复杂多样、临床表现变化多端且缺少特异性,漏诊率及误诊率均较高,而提高临床对CVST的认识有利于及时诊断、治疗CVST,改善CVST患者预后。目的分析CVST患者的临床特征。方法选取2010—2018年在南京大学医学院附属鼓楼医院住院的CVST患者126例,回顾性分析其人口学特征、首诊情况及危险因素、临床表现、D-二聚体检测结果、颅脑影像学检查结果、治疗及预后。结果人口学特征:男41例(32.5%),女85例(67.5%),男女比例为1:2.1;发病年龄为16~80岁,平均年龄(37±15)岁,以21~35岁者居多(48.4%)。首诊情况及危险因素:首诊于急诊者65例(52.6%),首诊于专科门诊者61例(48.4%)。排在前五位的危险因素:妊娠及产褥相关疾病45例(35.7%),免疫系统疾病、病因不明各18例(14.3%),中枢神经系统感染、鼻窦炎/中耳炎/乳突炎各11例(8.7%),血液系统疾病8例(6.3%),败血症5例(4.0%)。临床表现:头痛101例(80.2%)、恶心呕吐57例(45.2%)、意识障碍37例(29.4%)、抽搐36例(28.6%)、偏瘫16例(12.7%)、视物模糊10例(7.9%)、言语不清8例(6.3%)。D-二聚体检测结果:D-二聚体为0.10~55.32 mg/L,平均D-二聚体为(4.15±3.07)mg/L,其中D-二聚体升高(≥0.50 mg/L)者109例(86.5%),另17例(13.5%)D-二聚体正常。颅脑影像学检查结果:本组126例CVST患者均首选颅脑CT检查,怀疑CVST者再行颅脑磁共振成像(MRI)或静脉窦成像(MRV)及增强MRV检查确诊。血栓部位:横窦109例(86.5%)、乙状窦91例(72.2%)、上矢状窦52例(41.3%)、窦汇13例(10.3%)、直窦6例(4.8%);累及静脉窦数量:1个31例(24.6%)、2个53例(42.1%)、3个29例(23.0%)、4个及以上8例(6.3%);脑实质病变:脑梗死24例(19.0%),脑出血22例(17.5%),无脑实质病变80例(63.5%)。治疗及预后:本组126例CVST患者均给予低分子量肝素皮下注射,7~10 d后改为口服华法林治疗并使国际标准化比值(INR)维持在2~3至少3个月以上;4例患者因脑出血量大而行开颅血肿清除术;入院时轻症105例(83.3%)、重症21例(16.7%),入院21 d轻症116例(92.1%)、重症10例(7.9%),其中死亡4例,病死率为3.2%。结论CVST患者以青年女性多见,临床表现复杂多变且缺少特异性,诊断较困难;妊娠及产褥相关疾病、免疫系统疾病及感染性疾病是CVST的主要危险因素;颅脑MRI、MRV检查尤其是颅脑增强MRV检查是目前最佳的无创CVST诊断手段,抗凝治疗是CVST患者急性期首选治疗方案,同时也是基础治疗方案,而经治疗后CVST患者总体预后良好。

Background Aetiological agent of cerebral venous sinus thrombosis(CVST)is with complexity and diversify,and clinical manifestation of CVST is changeful and nonspecific,thus its rate of missed diagnosis and misdiagnosis rate are relatively high,however it is helpful to improve the clinical understanding of CVST for timely diagnosis and treatment as well as improving the prognosis.Objective To analyze the clinical features of CVST.Methods From 2010 to 2018,a total of 126 inpatients with CVST were selected in Nanjing Drum Tower Hospital,the Affiliated Hospital of Nanjing University Medical School,and their clinical data was retrospectively analyzed,mainly including demographic characteristics,situation for the first time to see a doctor,risk factors,clinical manifestations,D-dimer examination results,craniocerebral imaging examination results,treatment and prognosis.Results Demographic characteristics:there were 41 males(32.5%)and 85 females(67.5%),with the male and female ratio of 1:2.1;age of onset was from 16 to 80 years old,with average age of(37±15)years old and 21 to 35 years old as the majority(48.4%).Situation for the first time to see a doctor and risk factors:65 cases(52.6%)first saw a doctor in the Department of Emergency Medicine,the other 61 cases(48.4%)in the Specialist Clinic;the top five risk factors:45 cases(35.7%)with pregnancy related and puerperal diseases,18 cases(14.3%)with immune system disease or etiology obscure,11 cases(8.7%)with central nervous system infection or nasosinusitis/otitis media/mastoiditis,8 cases(6.3%)with hematological system diseases,5 cases(4.0%)with sepsis.Clinical manifestations:101 cases(80.2%)with headache,57 case(s 45.2%)with nausea and vomiting,37 case(s 29.4%)with disturbance of consciousness,36 case(s 28.6%)with convulsion,16 cases(12.7%)with hemiplegia,10 cases(7.9%)with blurred vision,8 cases(6.3%)with alalia.D-dimer examination results:D-dimer ranged from 0.10 to 55.32 mg/L,with an average of(4.15±3.07)mg/L,including 109 cases(86.5%)with elevation of D-dimer(≥0.50 mg/L)and 17 cases(13.5%)with normal D-dimer.Craniocerebral imaging examination results:craniocerebral CT examination was the first choice in all of the 126 patients,and they were subsequently confirmed as CVST by craniocerebral MRI or MRV/CE-MRV.Thrombotic locus:109 cases(86.5%)in transverse sinus,91 cases(72.2%)in sigmoid sinus,52 cases(41.3%)in superior sagittal sinus,13 cases(10.3%)in confluence of sinus,6 cases(4.8%)in straight sinus;number of involved venous sinuses:31 cases(24.6%)with single involved venous sinus,53 cases(42.1%)with two,29 cases(23.0%)with three,8 cases(6.3%)with four or more;Brain parenchymal lesions:24 cases(19.0%)with cerebral infarction,22 cases(17.5%)with cerebral hemorrhage,80 cases(63.5%)without brain parenchymal lesions.Treatment and prognosis:all of the 126 patients first received subcutaneous injection of low molecular weight heparin and then oral warfarin after 7 to 10 days,which need to maintain INR in 2-3 for 3 months at least;4 cases underwent craniotomy for evacuation of hematoma due to large amount of cerebral hemorrhage;105 cases(83.3%)were mild and 21 cases(16.7%)were severe at admission,116 cases(92.1%)were mild and 10 cases(7.9%)were severe 21 days after admission,thereinto 4 cases died,with an fatality rate of 3.2%.Conclusion CVST patients are mostly young women,however clinical manifestations of CVST are complicated,changeable and non-specific,which is relatively difficult to diagnose;pregnancy related and puerperal diseases,immune system disease and infectious diseases are major risk factors of CVST;craniocerebral MRI or MRV in especial CE-MRV is the best non-invasive diagnostic method of CVST,anticoagulant therapy is the basic and first choice for treatment in the acute phase of CVST,however the overall prognosis of CVST patients is good after treatment.