Cerebral sinus venous thrombosis in Swiss children

Aim To describe the characteristics of paediatric cerebral sinus venous thrombosis (CSVT) in Switzerland. Method Data on clinical features, neuroimaging, risk factors, and treatment were collected for all children in Switzerland younger than 16 years of age who had CSVT between January 2000 and December 2008. A follow‐up examination and a cognitive assessment were performed (mean follow‐up period 26mo). Differences between neonates and children (patients older than 28d) were assessed and predictors of outcome were determined. Results Twenty‐one neonates (14 males, seven females; mean age 9d, SD 8d) and 44 children (30 males, 14 females; mean age 8y 7mo, SD 4y 5mo) were reported. The incidence of paediatric CSVT in Switzerland was 0.558 per 100 000 per year. In neonates, the deep venous system was more often involved and parenchymal injuries were more common. The strongest predictor of poor outcome was neonatal age (odds ratio 17.8, 95% confidence interval 0.847–372.353). Most children showed global cognitive abilities within the normal range, but impairments in single cognitive subdomains were frequent. Interpretation Paediatric CSVT is rare. Its outcome is poor in neonates. Most children have good neurological outcomes, but some patients have individual neuropsychological impairments.     

脑静脉窦血栓形成的临床与治疗

目的:探讨脑静脉窦血栓形成(cerebral venous sinus thrombosis,CVST)的临床特征、影像特点及治疗。方法:分析34例脑静脉窦血栓形成病因、临床表现、影像特征及治疗。结果:本组病因依次为产褥期(58.8%)、感染(23.5%)、脱水(2.9%)、血液病(2.9%)、外伤(2.9%);头痛、恶心、呕吐为首发症状,查体视乳头水肿89.4%,癫痫发作44.1%,脑膜刺激征阳性35.3%,意识障碍26.5%,轻偏瘫5.9%,眼球突出伴活动障碍5.9%;头MRI、MRV均显示脑静脉窦血栓形成,2例行DSA检查发现双侧横窦血栓形成;本组在应用降颅压、抗感染等药物的同时,应用低分子肝素5000u,12小时一次,疗效显著,治愈25例(73.5%),好转3例,死亡6例。结论:产褥期、感染、脱水是CVST常见原因; CVST患者主要表现为高颅压症状;头MRI和MRV能清楚显示血栓形成部位;抗凝治疗是脑静脉窦血栓形成痊愈的关键。     

A prospective outcome study of neonatal cerebral sinovenous thrombosis

Neonatal cerebral sinovenous thrombosis is a frequent contributor to neonatal mortality and morbidity. Treatment is controversial, and reported clinical outcomes vary widely. Newborns with radiologically confirmed neonatal cerebral sinovenous thrombosis from 1992 to 2009 were prospectively followed in our Children's Stroke Clinic for standardized outcomes, including the Pediatric Stroke Outcome Measure. Outcomes were available in 90 of 104 (87%) neonates. Early outcomes included cerebral sinovenous thrombosis-associated death (5) and thrombus propagation (15 [6 associated with new venous infarcts]). Lack of anticoagulation predicted propagation (RR = 13; P = .0007). Complete thrombus recanalization occurred in 90% by 3 months. Late outcomes (median, 2.5 years) were epilepsy (15) and neurological disability (50), which included moderate-severe language (43), sensorimotor (38), and cognitive/behavioral (24) deficits. Overall, 61% had poor outcome (death/any deficit). Concurrent neurological comorbidity at diagnosis (odds ratio = 2.8; P = .029) predicted poor outcome. Clinical trials are urgently needed to establish more effective treatment strategies.     

Risk factors for recurrent venous thromboembolism in the European collaborative paediatric database on cerebral venous thrombosis: a multicentre cohort study

BACKGROUND: The relative importance of previous diagnosis and hereditary prothrombotic risk factors for cerebral venous thrombosis (CVT) in children in determining risk of a second cerebral or systemic venous thrombosis (VT), compared with other clinical, neuroimaging, and treatment variables, is unknown. METHODS: We followed up the survivors of 396 consecutively enrolled patients with CVT, aged newborn to 18 years (median 5.2 years) for a median of 36 months (maximum 85 months). In accordance with international treatment guidelines, 250 children (65%) received acute anticoagulation with unfractionated heparin or low-molecular weight heparin, followed by secondary anticoagulation prophylaxis with low-molecular weight heparin or warfarin in 165 (43%). RESULTS: Of 396 children enrolled, 12 died immediately and 22 (6%) had recurrent VT (13 cerebral; 3%) at a median of 6 months (range 0.1-85). Repeat venous imaging was available in 266 children. Recurrent VT only occurred in children whose first CVT was diagnosed after age 2 years; the underlying medical condition had no effect. In Cox regression analyses, non-administration of anticoagulant before relapse (hazard ratio [HR] 11.2 95% CI 3.4-37.0; p<0.0001), persistent occlusion on repeat venous imaging (4.1, 1.1-14.8; p=0.032), and heterozygosity for the G20210A mutation in factor II (4.3, 1.1-16.2; p=0.034) were independently associated with recurrent VT. Among patients who had recurrent VT, 70% (15) occurred within the 6 months after onset. CONCLUSION: Age at CVT onset, non-administration of anticoagulation, persistent venous occlusion, and presence of G20210A mutation in factor II predict recurrent VT in children. Secondary prophylactic anticoagulation should be given on a patient-to-patient basis in children with newly identified CVT and at high risk of recurrent VT. Factors that affect recanalisation need further research.     

脑静脉窦血栓形成的诊断与血管内治疗

目的 探讨脑静脉窦血栓形成的诊断和血管内溶栓治疗。方法 对8例以顽固性头痛、呕吐伴或不伴局灶性神经功能缺损或癫发作为主要表现的患者，采用颅脑MRI和脑血管造影检查确定诊断，并施行经颈内动脉、静脉窦内接触性溶栓或静脉窦内支架成形术协同抗凝治疗。结果 MRI显示相应脑静脉窦区出现长T2WI，短T1WI信号，DSA显示脑静脉窦闭塞、静脉侧支循环建立和脑动静脉循环时间延长。治疗后静脉窦完全或部分再通，脑循环时间接近正常，临床病情显著缓解。结论 血管内介入治疗可能是脑静脉窦血栓形成最有效的治疗方法之一，有待大样本研究证实。     

颅内静脉窦血栓形成后昏迷患者预后相关因素研究

目的探讨影响颅内静脉窦血栓形成(CVST)后昏迷患者预后的相关因索。 方法对梧州市工人医院神经内科自2001年2月至2010年2月收治的以昏迷就诊或在入院6h内出现不同程度昏迷的30例CVST患者的临床资料进行回顾性分析。 结果随访0.5～10年,本组患者中12例无明显颅内压增高、脑深静脉血栓及相关脑血管意外,8例死亡,2例重残,8例遗留不同程度的偏瘫或智力语言障碍。CVST后昏迷患者伴有中枢神经系统感染、恶性肿瘤、脑深静脉血栓形成、癫痫及颅内出血等改变时患者预后差,其中恶性肿瘤患者重残或死亡率高达50％。 结论CVST后发生昏迷的患者预后差,病死率高,及早诊治并合理治疗有助于改善预后。     

Recanalisation and outcome cerebral venous thrombosis

Little is known of the natural history and rate of sinus recanalisation after cerebral venous thrombosis (CVT). Although acute anticoagulation is effective, the duration of therapy remains speculative. We aimed to determine the relationship between sinus recanalisation and clinical outcome. We studied 12 consecutive patients with aseptic CVT with evidence of sinus thrombosis on initial magnetic resonance imaging, followed up 5-68 months after onset, using 15 repeat magnetic resonance scans in 9 of the patients to assess recanalisation. All patients initially had one or more thrombosed sinuses and were treated with anticoagulants for at least 6 months, including 3 with haemorrhagic infarction. Residual neurological deficits were present in only one patient. No patient had a recurrent thrombosis. Recanalisations was incomplete in 6 of the 9 cases. Sinus recanalisation after cerebral venous thrombosis does not correlate with clinical outcome. Although empirical, the general recommendation of 6 months anticoagulant therapy is appropriate.     

Diagnostic value of combined conventional magnetic resonance imaging and magnetic resonance angiography in sinus venous thrombosis

25 patients are presented, who were diagnosed with cerebral sinus venous thrombosis by a combination of Magnetic Resonance Angiography (MRA) and conventional Spin-Echo sequences. An angiography in DSA technique was carried out additionally in 12 cases. In 24 patients MR-angiographic control examinations were conducted. In seven cases MRA revealed an isolated thrombosis of the superior sagittal sinus (SSS) whereas in eleven patients there was an additional occlusion of other sinuses or cerebral veins. The transverse sinus (ST) was the second most affected. DSA essentially confirmed the MR-imaging data. Despite anticoagulation follow up MRA's showed complete recanalisation in only 7 patients, in 8 cases partial recanalisation was observed and in 9 cases a complete occlusion persisted. In contrast to these findings neurological examinations during the follow up period were unremarkable in 16 patients and the remainder revealed only mild residual symptoms. In general no further morphological alterations of the cerebral sinus system were detectable by MRA after 6 months. Follow up imaging beyond 6 months therefore appears unnecessary, unless a relapse is suspected.     

多途径介入治疗进展性颅内静窦血栓初步分析

目的 探讨血管内治疗急性进展性颅内静脉窦血栓的疗效和安全性。 方法 选择2012年1月-2015年8月在首都医科大学附属北京天坛医院脑血管病中心经抗凝、脱水降颅 内压等药物治疗后病情仍进行性加重的24例急性静脉窦血栓形成的患者,依据病情实施了微导管置 管溶栓术、球囊和微导丝机械碎栓联合微导管溶栓术以及支架拉栓联合微导管溶栓术等3种血管内 治疗术式。分析患者手术治疗前后的一般状况、临床特征、围手术并发症等资料。 结果 24例患者中,单独微导管置入溶栓12例,导丝、球囊机械联合微导管溶栓8例,支架拉栓联 合微导管溶栓4例。术后11例（45.8%）患者静脉窦完全再通,10例（41.7%）患者静脉窦部分再通,3例 （12.5%）患者静脉窦未见通畅。出院时痊愈（不遗留神经功能障碍）14例（58.3%）,遗留轻度神经功 能障碍8例（33.3%）,死亡2例（8.3%）。 结论 对于内科治疗不能控制的进展性急性颅内静脉窦血栓形成患者,血管内治疗可能是一种安 全、有效的治疗手段。     

脑静脉窦血栓形成的早期诊断与治疗

目的探讨脑静脉窦血栓形成的早期临床特点及治疗方法。方法回顾性分析16例脑静脉窦血栓形成患的发病年龄、发病形式、首发症状、临床表现、影像学特征及脑脊液等检查结果与治疗方法。结果发病年龄以20～60岁多见，多数患为急性发病，早期临床表现为头痛、呕吐，伴或不伴局灶性神经功能缺损或癫痫发作。影像学检查表现为静脉窦闭塞及局限性梗死灶。脑脊液压力明显增高，白细胞数及蛋白质含量正常或增高，但感染性或癌性脑静脉窦血栓形成除外。应用脱水、抗凝、局部溶栓及病因治疗等方法后，16例患中6例基本痊愈，7例症状好转，1例无变化，2例死亡。结论脑静脉窦血栓形成的临床表现复杂多样，无特异性，对临床急性发病的头痛、呕吐、伴或不伴局灶性神经功能缺损及乏癫痫发作的年轻患，应高度怀疑脑静脉窦血栓形成，及时行磁共振成像(MRI)及磁共振血管造影(MRA)联合检查，以明确诊断。确诊后应尽早开始治疗。     

脑静脉及静脉窦血栓形成16例临床分析

目的：探讨脑静脉及静脉窦血栓形成（CVST）的病因及临床特点。方法：回顾性调查16例CVST患者的临床资料。结果：侧窦血栓（87.5%）和上矢状窦血栓形成（37.5%）最常见,70%患者有2个以上部位血栓形成。最常见的症状为头痛14例（87.5%）,其次为癫癎、偏瘫、嗜睡及反应迟钝等。头颅CT有14例（87.5%）发现特征性改变,为枕部近颅板下或后纵裂条索状高密度影。头颅MRI结合MRV阳性率为94%。结论：头痛是CVST患者最常见的主诉,CT可作为一种诊断CVST有效的初始检查,MRI结合MRV有诊断价值。     

Deep cerebral venous sinus thrombosis often presents with neuropsychologic symptoms.

The outcome of cerebral venous sinus thrombosis (CVST) depends on rapid diagnosis and initiation of effective anticoagulation. We report seven cases of a subgroup with deep cerebral venous sinus thrombosis (DCVST) treated in our institution since 1990. Six of our seven patients presented with early neuropsychological deficits (mental obtundation, bradyphrenia or apathia). This clinical presentation, in combination with headache, and focal neurological deficits, aids the early diagnosis of DCVST. Thalamic hyperintensities on T2-weighted MRI images, previously considered infarctions, were fully reversible during treatment with heparin. This indicates that early in the course of the disease they correspond to vasogenic oedema.     

Prolonged direct catheter thrombolysis of cerebral venous sinus thrombosis in children: a case series

Cerebral venous sinus thrombosis is a rare condition with potentially devastating neurologic outcome--death and severe disability are common in advanced cases. In adults, protocols for mechanical clot disruption and direct thrombolysis are established; no guidance exists for children. We present our experience of 6 children with cerebral venous sinus thrombosis and ominous clinical progression. We found that effective thrombolysis required substantially longer infusion, more rounds of mechanical disruption, and higher doses of thrombolytics than are commonly practiced. Despite pervasive thrombosis, prethrombolysis hemorrhage, coma, and other predictors of death and disability, our patients survived and 4 of 6 had no functional deficits. One patient had moderate, and one had severe deficits. We report these cases to illustrate that hemorrhage may not be a contraindication to thrombolysis for cerebral venous sinus thrombosis, that prolonged infusion may be required to restore perfusion, and that good neurologic outcomes can be achieved despite dire clinical presentations and extensive sinus thrombosis.     

脑静脉窦血管内治疗脑静脉窦血栓形成临床研究

目的对比静脉肝素抗凝与脑介入治疗脑静脉窦血栓(CVST)的有效性和安全性。方法回顾性分析我院14例脑静脉窦血栓患者,按治疗方法分为介入组(脑静脉窦血管内治疗)和抗凝组(静脉肝素抗凝),每组7例,比较2组平均住院时间、血管完全再通率、mRS及并发症发生率。结果脑静脉窦介入组住院时间较抗凝组短,血管完全再通率高于抗凝组,mRS平均评分低于抗凝组,差异均有统计学意义(P0.05)。2组并发症发生率无显著差异(P0.05)。结论脑静脉窦血管内治疗可改善CVST患者的转归,疗效优于静脉肝素溶栓保守治疗。     

联合治疗方案与全身抗凝治疗脑静脉窦血栓形成

目的 比较脑静脉窦血栓形成的联合治疗方案与全身抗凝在临床应用中的有效性和安全性.方法 网顾性连续收集脑静脉窦血栓形成116例患者资料,根据治疗方式不同分为联合治疗组30例和全身抗凝组86例.其中联合治疗组在全身抗凝的基础上,接受改良溶栓方案介入治疗,即脑动脉及静脉窦造影明确诊断后行机械性破栓及吸栓术,于静脉窦内留置微导管行尿激酶微量泵点接触性溶栓.采用NIHSS评分对两组患者治疗前后神经功能缺损评分,应用改良Rankin量表(mRS)评价出院时情况.结果 联合治疗组30例,男9例;全身抗凝组86例,男23例.治疗前神经功能缺损:联合治疗组0～19分,全身抗凝组0～17分,差异无统计学意义(Z=-0.474,P=0.636);治疗后联合治疗组神经功能缺损程度减轻,出院时mRS评分较低,两组颅内出血发生率差异无统计学意义.结论 联合治疗较全身抗凝有利于神经功能恢复.两种治疗方法颅内出血发生率无明显差异.     

Superior sagittal sinus thrombosis associated with Evans' syndrome of haemolytic anaemia

Summary A case of superior sagittal sinus thrombosis associated with Evans' syndrome of immune haemolytic anaemia is reported. The neurological symptoms and signs were headaches, right quadrant hemianopia, dyslexia without agraphia, motor aphasia, numbness in and weakness of the right upper extremity, papilloedema and coma. The cerebral venous sinus thrombosis, involving cerebral veins, superior sagittal sinus and straight sinus, was diagnosed by cerebral angiography. It is noteworthy that the superior sagittal sinus thrombosis occurred during a haematological recovery period, with rapid responses to treatment with 6-mercaptopurine and high doses of adrenocorticosteroids. Following a reduction in the doses of these drugs, the symptoms and signs related to the superior sagittal sinus thrombosis gradually subsided, and the haematological pictures remained in remission.     

颅内静脉窦血栓形成（9例临床报道）

目的:探讨脑静脉血栓形成(CVT)的临床特点与影像学表现。方法:回顾性分析本院9例CVT的临床及影像学资料。结果:①临床表现:头痛(77%)、癫样抽搐(66%)、局灶性神经功能缺失(66%)、视乳头水肿(44%)、不同程度意识障碍(22%)。②影像学资料及治疗转归:1例头颅CT示弥漫性脑水肿,蛛网膜下隙出血合并多发出血性脑梗死,因颅内压持续增高,予外科手术行去骨板减压术,见大脑表面浅静脉怒张呈绳索状并多数点片状出血灶。8例行头颅MRI和MRV,显示直接征像8例、间接征像6例。其中2例同时行DSA,均显示多个静脉和静脉窦充盈缺损。静脉血栓部位:上矢状窦合并浅静脉血栓1例,上矢状窦合并侧窦及多处静脉受累7例,直窦血栓1例。7例行肝素抗凝等对症治疗、1例行血管内局部溶栓、1例行矢状窦手术取栓并抗凝治疗。症状缓解至痊愈7例,病死2例。结论:首诊时诊断不明确,可先作头颅CT,排除颅内非CVT性病变,临床已拟诊CVT时,应首选MRI+MRV,进行综合判断是目前诊断和随访CVT的最好方法,婴幼儿CVT常表现为癫发作起病,提示CVT是婴幼儿重要的未被识别的性发作原因之一。     

静脉侧支回流在多发性脑静脉窦血栓病中的作用(附89例分析)

目的 探讨静脉侧支回流在多发性脑静脉窦血栓病程中的作用。方法对我科收治的89例多发性脑静脉窦血栓病人采用经颈动脉溶栓、静脉窦内留置微导管连续溶栓等治疗方法，同时辅以全身抗凝治疗，观察临床效果和侧支循环的变化。结果所有病例治疗后颅内压均明显下降，DSA示残留静脉窦有回流63例，海绵窦代偿性引流75例，椎静脉丛引流35例，头皮静脉代偿引流14例。结论血管内溶栓和抗凝是治疗脑静脉血栓较为可靠和安全的方法：静脉侧支回流的建立和加强对于多发性脑静脉窦血栓的治疗和预后判断意义重大。     

Randomized, placebo-controlled trial of anticoagulant treatment with low-molecular-weight heparin for cerebral sinus thrombosis

BACKGROUND AND PURPOSE: Treatment of cerebral sinus thrombosis with heparin is controversial. We conducted a double-blind, placebo-controlled multicenter trial to examine whether anticoagulant treatment improves outcome in patients with sinus thrombosis. METHODS: Patients were randomized between body weight-adjusted subcutaneous nadroparin (180 anti-factor Xa units/kg per 24 hours) and matching placebo for 3 weeks (double-blind part of trial), followed by 3 months of oral anticoagulants for patients allocated nadroparin (open part). Patients with cerebral hemorrhage caused by sinus thrombosis were also included. RESULTS: Sixty patients were enrolled, and none were lost to follow-up. In 1 patient the diagnosis proved wrong after randomization. After 3 weeks, 6 of 30 patients (20%) in the nadroparin group and 7 of 29 patients (24%) in the placebo group had a poor outcome, defined as death or Barthel Index score of <15 (risk difference, -4%; 95% CI, -25 to 17%; NS). After 12 weeks, 4 of 30 patients (13%) in the nadroparin group and 6 of 29 (21%) in the placebo group had a poor outcome, defined as death or Oxford Handicap Score of >/=3 (risk difference, -7%; 95% CI, -26% to 12%; NS). There were no new symptomatic cerebral hemorrhages. One patient in the nadroparin group had a major gastrointestinal hemorrhage, and 1 patient in the placebo group died from clinically suspected pulmonary embolism. CONCLUSIONS: Patients with cerebral sinus thrombosis treated with anticoagulants (low-molecular-weight heparin followed by oral anticoagulation) had a favorable outcome more often than controls, but the difference was not statistically significant. Anticoagulation proved to be safe, even in patients with cerebral hemorrhage.     

颅内静脉系统血栓形成的临床特征与疗效分析

目的 探讨颅内静脉系统血栓形成的临床特征以及治疗原则.方法 分析14例颅内静脉系统血栓形成患者的发病年龄、发病形式、临床表现、影像学特征、脑脊液检查结果,并比较阿加曲班和低分子肝素对神经功能改善程度及预后的影响.结果 颅内静脉系统血栓形成可见于任何年龄段,中青年女性多见,以妊娠、感染、免疫性疾病等为常见发病原因.急性或...