Neurology and Pediatrics, Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Penn HHT Center of Excellence and Children's Hospital of Philadelphia Hereditary Hemorrhagic Telangiectasia Program, 3401 Civic Center Boulevard, Philadelphia, 19104, PA, USA.
Radiology, Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
Childs Nerv Syst. 2024 Jul;40(7):2101-2108. doi: 10.1007/s00381-024-06366-z. Epub 2024 Mar 22.
The brain arteriovenous malformation (BAVM) nidus compactness score (CS), determined on angiography, predicts BAVM recurrence after surgical resection among children with sporadic BAVMs. We measured the angiographic CS for BAVMs among children with hereditary hemorrhagic telangiectasia (HHT) to determine CS characteristics in this population.
A pediatric interventional neuroradiologist reviewed angiograms to determine the CS of BAVMs in children with HHT recruited to the BVMC. CS is based on overall nidus and perinidal anomalous vessel compactness. CS categories included 1 = diffuse nidus, 2 = intermediate nidus, and 3 = compact nidus.
Forty-eight of 78 children (61.5%) with HHT and brain vascular malformations had a conventional angiogram; 47 (97.9%) angiograms were available. Fifty-four BAVMs were identified in 40 of these 47 children (85.1%). Of 54 BAVMs in children with HHT, CS was 1 in 7 (13%), 2 in 29 (53.7%), and 3 in 18 BAVMs (33.3%) compared with CS of 1 in six (26.1%), 2 in 15 (65.2%), and 3 in 2 BAVMs (8.7%) among 23 previously reported children with sporadic BAVMs, p = 0.045 (Fisher's exact). Seven children with HHT had intracranial hemorrhage: 4 had CS = 3, 1 had CS = 2, and 2 had CS = 1.
A range of CSs exists across HHT BAVMs, suggesting it may be an angiographic measure of interest for future studies of BAVM recurrence and hemorrhage risk. Children with HHT may have more compact niduses compared to children with sporadic BAVMs. Additional research should determine whether CS affects hemorrhage risk or post-surgical recurrence risk in HHT-associated BAVMs, which could be used to direct BAVM treatment.
在接受手术切除的散发性脑动静脉畸形（BAVM）患儿中，通过血管造影确定的脑动静脉畸形病灶致密评分（CS）可以预测 BAVM 的复发。我们测量了遗传性出血性毛细血管扩张症（HHT）患儿的脑动静脉畸形血管造影 CS，以确定该人群中 CS 的特征。
一名儿科介入神经放射科医生回顾了血管造影图像，以确定招募至 BVMC 的 HHT 患儿的 BAVM 的 CS。CS 基于病灶整体和周围异常血管的致密程度。CS 类别包括 1=弥漫性病灶，2=中间病灶，3=致密病灶。
78 例 HHT 伴脑血管畸形患儿中有 48 例（61.5%）接受了常规血管造影检查；其中 47 例（97.9%）的血管造影结果可用。在这 47 例患儿中的 40 例（85.1%）中发现了 54 个 BAVM。在 HHT 患儿的 54 个 BAVM 中，CS 为 1 的有 7 个（13%），CS 为 2 的有 29 个（53.7%），CS 为 3 的有 18 个（33.3%），而在 23 例以前报道的散发性 BAVM 患儿中，CS 为 1 的有 6 个（26.1%），CS 为 2 的有 15 个（65.2%），CS 为 3 的有 2 个（8.7%），p=0.045（Fisher 确切概率）。7 例 HHT 患儿发生颅内出血：CS=3 的 4 例，CS=2 的 1 例，CS=1 的 2 例。
HHT 的 BAVM 存在多种 CS，这表明它可能是 BAVM 复发和出血风险的未来研究的一种血管造影测量指标。与散发性 BAVM 患儿相比，HHT 患儿的病灶可能更致密。进一步的研究应确定 CS 是否会影响 HHT 相关 BAVM 的出血风险或术后复发风险，这可能有助于指导 BAVM 的治疗。
