Riordan Coleman P, Orbach Darren B, Smith Edward R, Scott R Michael
Departments of1Neurosurgery and.
2Neurointerventional Radiology, Boston Children's Hospital, Boston, Massachusetts.
J Neurosurg Pediatr. 2018 Sep;22(3):244-250. doi: 10.3171/2018.3.PEDS1825. Epub 2018 Jun 1.
OBJECTIVE The most significant adverse outcome of intracranial hemorrhage from an arteriovenous malformation (AVM) is death. This study reviews a single-center experience with pediatric AVMs to quantify the incidence and characterize clinical and radiographic factors associated with sudden death from the hemorrhage of previously undiagnosed AVMs in children. METHODS A single-center database review of the period from 2006 to 2017 identified all patients with a first-time intracranial hemorrhage from a previously undiagnosed AVM. Clinical and radiographic data were collected and compared between patients who survived to hospital discharge and those who died at presentation. RESULTS A total of 57 patients (average age 10.8 years, range 0.1-19 years) presented with first-time intracranial hemorrhage from a previously undiagnosed AVM during the study period. Of this group, 7/57 (12%) patients (average age 11.5 years, range 6-16 years) suffered hemorrhages that led directly to their deaths. Compared to the cohort of patients who survived their hemorrhage, patients who died were 4 times more likely to have an AVM in the posterior fossa. No clear pattern of antecedent triggering activity (sports, trauma, etc.) was identified, and 3/7 (43%) experienced cardiac arrest in the prehospital setting. Surviving patients were ultimately treated with resection of the AVM in 42/50 (84%) of cases. CONCLUSIONS Children who present with hemorrhage from a previously undiagnosed intracranial AVM had a 12% chance of sudden death in our single-institution series of pediatric cerebrovascular cases. Clinical triggers of hemorrhage are unpredictable, but subsequent radiographic evidence of a posterior fossa AVM was present in 57% of fatal cases, and all fatal cases were in locations with high risk of potential herniation. These data support a proactive, aggressive approach toward definitive treatment of AVMs in children.
目的 动静脉畸形（AVM）所致颅内出血最严重的不良后果是死亡。本研究回顾了单中心小儿AVM的治疗经验，以量化发病率，并描述与儿童未诊断出的AVM出血所致猝死相关的临床和影像学因素。方法 对2006年至2017年期间的单中心数据库进行回顾，确定所有首次因未诊断出的AVM发生颅内出血的患者。收集临床和影像学数据，并对存活至出院的患者与就诊时死亡的患者进行比较。结果 在研究期间，共有57例患者（平均年龄10.8岁，范围0.1 - 19岁）首次因未诊断出的AVM发生颅内出血。在这组患者中，7/57（12%）例患者（平均年龄11.5岁，范围6 - 16岁）因出血直接导致死亡。与出血后存活的患者队列相比，死亡患者后颅窝出现AVM的可能性高4倍。未发现明确的前驱触发活动模式（运动、外伤等），3/7（43%）例患者在院前发生心脏骤停。存活患者最终有42/50（84%）例接受了AVM切除术。结论 在我们单机构的小儿脑血管病例系列中，因先前未诊断出的颅内AVM出血就诊的儿童有12%的猝死几率。出血的临床触发因素不可预测，但57%的致命病例随后有后颅窝AVM的影像学证据，且所有致命病例均位于有潜在疝形成高风险的部位。这些数据支持对儿童AVM采取积极主动的确定性治疗方法。
