Morbidity after Hemorrhage in Children with Untreated Brain Arteriovenous Malformation.
Morbidity after Hemorrhage in Children with Untreated Brain Arteriovenous Malformation.
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未经治疗的脑动静脉畸形儿童出血后的发病率
DOI：
10.1159/000458731
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发表时间：
2017
期刊：
影响因子：
--
通讯作者：
Zhao Y
中科院分区：
文献类型：
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作者：
Ma L;Kim H;Chen XL;Wu CX;Ma J;Su H;Zhao Y
背景：未经治疗的脑动静脉畸形（bAVM）儿童在生命早期就面临着危及生命的出血的风险。侵入性治疗的主要目的是减少与 bAVM 破裂相关的不良后果。更好地了解 bAVM 出血的发病率可能有助于权衡未经治疗的 bAVM 和侵入性治疗的风险。我们的目的是评估 bAVM 破裂后的临床结果并确定预测儿童严重出血的特征。方法：我们确定了 2009 年 7 月至 2014 年 12 月期间因 bAVM 入院的所有连续儿童。使用儿童改良 Rankin 量表 (mRS) 评估出血表现和随后出血后的临床结果。使用单变量和多变量回归分析研究人口统计学特征和 bAVM 形态与严重出血（mRS > 3 或需要紧急血肿清除）的关联。制定基于多变量分析的列线图来预测个体患者的严重出血风险。结果：共有 134 名患者，平均无治疗随访期为 2.1 年。 83 名 (62%) 儿童的 bAVM 破裂：82 名儿童就诊时出现出血，其中 6 名在随访期间出现复发性出血； 1 名患者有其他诊断症状，但在随访期间出现出血。其中，49%（41/83）有严重出血；其中28%（23/83）需要紧急血肿清除，24%（20/83）在末次随访时仍处于残疾状态（mRS≥3）。百分之四十六 (38/82) 的出血儿童严重残疾 (mRS > 3)。百分之四十三 (3/7) 在随后的出血后严重残疾。总体队列中随后严重出血的年发生率为 1%，破裂儿童的年发生率为 3.3%。随后的严重出血事件均发生在有严重出血史的儿童中（7%，3/41）。脑室周围位置、非颞叶位置和长引流静脉是儿童未经治疗的 bAVM 严重出血的预测因素。基于bAVM形态的列线图被用来预测个体患者的严重出血风险，该列线图经过良好校准并且具有良好的判别能力（调整后的C统计量，0.72）。结论：评估 bAVM 发病率和形态可能有助于权衡儿科患者未经治疗的 bAVM 风险。
Background: Children with untreated brain arteriovenous malformations (bAVM) are at risk of encountering life-threatening hemorrhage very early in their lives. The primary aim of invasive treatment is to reduce unfavorable outcome associated with a bAVM rupture. A better understanding of the morbidity of bAVM hemorrhage might be helpful for weighing the risks of untreated bAVM and invasive treatment. Our aim was to assess the clinical outcome after bAVM rupture and identify features to predict severe hemorrhage in children. Methods: We identified all consecutive children admitted to our institution for bAVMs between July 2009 and December 2014. Clinical outcome after hemorrhagic presentation and subsequent hemorrhage was evaluated using the modified Rankin Scale (mRS) for children. The association of demographic characteristics and bAVM morphology with severe hemorrhage (mRS >3 or requiring emergency hematoma evacuation) was studied using univariate and multivariable regression analyses. A nomogram based on multivariable analysis was formulated to predict severe hemorrhage risk for individual patients. Results: A total of 134 patients were identified with a mean treatment-free follow-up period of 2.1 years. bAVM ruptured in 83 (62%) children: 82 had a hemorrhage at presentation and 6 of them experienced a recurrent hemorrhage during follow-up; 1 patient had other diagnostic symptoms but bled during follow-up. Among them, 49% (41/83) had a severe hemorrhage; emergency hematoma evacuation was required in 28% of them (23/83), and 24% (20/83) remained as disabled (mRS ≥3) at last follow-up. Forty-six percent (38/82) of children with hemorrhagic presentation were severely disabled (mRS >3). Forty-three percent (3/7) were severely disabled after subsequent hemorrhage. The annual rate of severe subsequent hemorrhage was 1% in the overall cohort and 3.3% in children with ruptured presentation. All the subsequent severe hemorrhage events occurred in children with severe hemorrhage history (7%, 3/41). Periventricular location, non-temporal lobe location, and long draining vein were predictors for severe hemorrhage in pediatric untreated bAVMs. A nomogram based on bAVM morphology was contracted to predict severe hemorrhage risk for individual patients, which was well calibrated and had a good discriminative ability (adjusted C-statistic, 0.72). Conclusions: Evaluating bAVM morbidity and morphology might be helpful for weighing the risks of untreated bAVM in pediatric patients.