The natural history of cerebral cavernous malformations in children
The natural history of cerebral cavernous malformations in children
复制标题
DOI：
10.3171/2015.2.peds14541
复制
发表时间：
2016-02-01
影响因子：
1.9
通讯作者：
Smith, Edward R.
中科院分区：
文献类型：
--
作者：
Gross, Bradley A.;Du, Rose;Smith, Edward R.
目的脑海绵状血管畸形（CM）是由占位效应和出血引起的癫痫发作和局灶性神经功能缺损导致的神经系统疾病的一个来源。虽然有几个自然历史的报告存在与CM的成人，类似的数据为儿科patients.METHODS的作者回顾了医院的数据库，以确定儿童CM谁没有接受手术治疗，谁有临床和放射学随访。年出血率计算病变年，和危险因素进行了评估，使用考克斯比例风险model.Results在一个队列的167例患者222 CM，平均患者年龄在诊断时为10.1岁（SD 6.0）。90例患者（54%）为男性。104名患者（62%）至少有1个CM出血，58名（35%）癫痫发作伴或不伴CM出血，43名（26%）有偶发病变。25例患者（15%）有多发性CM，17例（10%）有CM家族史，33例（20%）有放射学上明显的发育性静脉异常（DVA）。总体年出血率为3.3%。每次出血的永久性神经系统发病率为29%，脑干、丘脑或基底节CM的永久性神经系统发病率增加至45%，幕上脑叶或小脑病变的永久性神经系统发病率降低至15%。偶发性CM的年出血率为0.5%;出血性CM的年出血率为11.3%，在前3年内增加至18.2%。3年内的出血聚集具有统计学显著性（HR 6.1，95% CI 1.72-21.7，p = 0.005）。多变量分析显示，出血性表现（HR 4.63，95% CI 1.53-14.1，p = 0.007），脑干位置（HR 4.42，95% CI 1.57-12.4，p = 0.005）和相关的放射学明显DVA（HR 2.91，95%CI 1.04-8.09，p = 0.04）出现出血的显着危险因素，而年龄，性别，CM多样性，CM家族史doesn 't.CONCLUSIONS先前出血，脑干位置，和相关的DVA是有症状的出血在CM儿童的显着危险因素。在出血的前3年内可能发生出血聚集，这是患者管理和咨询中的一个潜在重要因素。
OBJECTIVE Cerebral cavernous malformations (CMs) are a source of neurological morbidity from seizures and focal neurological deficits due to mass effect and hemorrhage. Although several natural history reports exist for adults with CMs, similar data for pediatric patients are limited.METHODS The authors reviewed hospital databases to identify children with CMs who had not been treated surgically and who had clinical and radiological follow-up. Annual hemorrhage rates were calculated in lesion-years, and risk factors were assessed using the Cox proportional hazards model.RESULTS In a cohort of 167 patients with 222 CMs, the mean patient age at the time of diagnosis was 10.1 years old (SD 6.0). Ninety patients (54%) were male. One hundred four patients (62%) presented with hemorrhage from at least 1 CM, 58 (35%) with seizures with or without CM hemorrhage, and 43 (26%) with incidental lesions. Twenty-five patients (15%) had multiple CMs, 17 (10%) had a family history of CMs, and 33 (20%) had radiologically apparent developmental venous anomalies (DVAs). The overall annual hemorrhage rate was 3.3%. Permanent neurological morbidity was 29% per hemorrhage, increasing to 45% for brainstem, thalamic, or basal ganglia CM and decreasing to 15% for supratentorial lobar or cerebellar lesions. The annual hemorrhage rate for incidental CMs was 0.5%; for hemorrhagic CMs, it was 11.3%, increasing to 18.2% within the first 3 years. Hemorrhage clustering within 3 years was statistically significant (HR 6.1, 95% CI 1.72-21.7, p = 0.005). On multivariate analysis, hemorrhagic presentation (HR 4.63, 95% CI 1.53-14.1, p = 0.007), brainstem location (HR 4.42, 95% CI 1.57-12.4, p = 0.005), and an associated radiologically apparent DVA (HR 2.91, 95% CI 1.04-8.09, p = 0.04) emerged as significant risk factors for hemorrhage, whereas age, sex, CM multiplicity, and CM family history did not.CONCLUSIONS Prior hemorrhage, brainstem location, and associated DVAs are significant risk factors for symptomatic hemorrhage in children with CMs. Hemorrhage clustering within the first 3 years of a bleed can occur, a potentially important factor in patient management and counseling.