Birmingham Women's & Children's Hospital, Birmingham, UK.
Queen Elizabeth Hospital, University Hospitals Birmingham, Birmingham, UK.
Childs Nerv Syst. 2023 Apr;39(4):975-982. doi: 10.1007/s00381-022-05800-4. Epub 2022 Dec 29.
PURPOSE: Intracranial arteriovenous-malformation (AVM) is a relatively rare condition in pediatrics, yet is a major cause of spontaneous intracranial hemorrhage with a risk of fatal hemorrhage reported to be between 4 and 29%. Little is known about vessel morphology and optimum treatment modalities including multimodality combination therapy and prognosis in children. METHODS: A retrospective review of all children presenting to our institution from 2006 to 2020 that had an AVM was undertaken. RESULTS: A total of 50 children were identified with median age of 11 (range 1-16) years. The mean follow-up was 7.6 years. Forty-one children presented as an emergency and of those, 40 had hemorrhage identified on initial brain imaging. The average nidus size was 25 mm, drainage was superficial in 51% of cases, and located in eloquent cortex in 56%. The supplemental Spetzler-Martin grading indicated 78% (39/50) were grade 4 and above (moderate to high risk). Primary treatment modalities included embolization in 50% (25) or SRS in 30% (15) and surgery in 20% (10).The AVM was obliterated on follow-up DSA in 66% children. Three children had post-treatment hemorrhage, two related to embolization and one the day following SRS, giving a re-bleed rate of 6%. The GOSE was available for 32 children at long term follow and 94% had a good outcome (GOSE 5-8). Two children died due to acute hemorrhage (4%). CONCLUSION: The majority of children with AVM present with hemorrhage. The rebleed rate during definitive treatment is low at 6% over the study period. The selective use of the 3 modalities of treatment has significantly reduced mortality and severe disability.
目的:颅内动静脉畸形(AVM)在儿科中相对少见,但却是自发性颅内出血的主要原因,据报道其致命性出血风险为 4%至 29%。儿童的血管形态和最佳治疗方式(包括多模态联合治疗)以及预后知之甚少。
方法:对 2006 年至 2020 年期间我院收治的所有颅内 AVM 患儿进行回顾性研究。
结果:共 50 例患儿纳入研究,中位年龄为 11 岁(范围 1-16 岁)。平均随访 7.6 年。41 例患儿以急症就诊,其中 40 例患儿的初始脑影像学检查显示有出血。平均病灶大小为 25mm,引流为表浅型的占 51%,位于功能区皮质的占 56%。补充的斯皮茨勒-马丁分级表明,78%(39/50)为 4 级及以上(中高危)。主要治疗方式包括栓塞 50%(25 例)、立体定向放射外科 30%(15 例)和手术 20%(10 例)。66%的患儿在随访时 DSA 显示 AVM 完全闭塞。3 例患儿在治疗后出现出血,其中 2 例与栓塞相关,1 例在 SRS 后当天发生,再出血率为 6%。32 例患儿在长期随访时获得了格拉斯哥预后评分(GOSE),94%的患儿预后良好(GOSE 5-8)。2 例患儿因急性出血死亡(4%)。
结论:大多数颅内 AVM 患儿以出血为首发症状。在研究期间,明确治疗过程中的再出血率为 6%,相对较低。这三种治疗方式的选择性使用显著降低了死亡率和严重残疾的发生。
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