Department of Neurological Surgery, University of Virginia Health System, Charlottesville, VA, 22908, USA.
Acta Neurochir (Wien). 2024 Jan 17;166(1):21. doi: 10.1007/s00701-024-05923-4.
OBJECTIVE: Brain arteriovenous malformations (AVMs) in patients with hereditary hemorrhagic telangiectasia (HHT) present different characteristics from sporadic AVMs, and they have lower initial bleeding rates. Conservative management is usually preferred for the treatment of these lesions. In this case study, we present the largest series of HHT patients treated with stereotactic radiosurgery to date. METHODS: We identified eight patients with HHT and 14 AVMs. We retrospectively collected clinical, radiographic, and treatment characteristics of the patients and each AVM. RESULTS: Most patients in our sample presented with small AVMs. The median volume of these AVMs was 0.22 cm (IQR 0.08-0.59). Three out of eight patients presented with initial intracerebral hemorrhage (ICH). The majority of lesions had low (12/14) Spetzler-Martin grades (I-II). Median maximum and margin doses used for treatment were 36.2 (IQR 35.25-44.4) and 20 (IQR 18-22.5) Gy, respectively. The overall obliteration rate after SRS was 11/14, and the median time to obliteration across all 11 obliterated AVMs was 35.83 months (IQR, 17-39.99). Neurological status was favorable with all patients having a mRS of 0 or 1 at the last follow-up. Symptomatic radiation-induced changes (RIC) after SRS were low (7.1%), and there were no permanent RIC. CONCLUSIONS: Patients with HHT who present with multiple brain AVMs are generally well served by SRS. Obliteration can be achieved in the majority of HHT patients and with a low complication rate. In the current study, initial hemorrhage rates prior to SRS were noticeable which supports the decision to treat these AVMs. Future studies are needed to better address the role of SRS for HHT patients harboring ruptured and unruptured AVMs.
目的:与散发性动静脉畸形(AVM)相比,遗传性出血性毛细血管扩张症(HHT)患者的脑动静脉畸形(AVM)具有不同的特征,且初始出血率较低。通常,这些病变采用保守治疗。在本病例研究中,我们报告了迄今为止接受立体定向放射外科治疗的最大系列 HHT 患者。
方法:我们确定了 8 例 HHT 患者和 14 个 AVM。我们回顾性地收集了患者和每个 AVM 的临床、影像学和治疗特征。
结果:我们样本中的大多数患者存在小的 AVM。这些 AVM 的中位体积为 0.22cm(IQR 0.08-0.59)。8 例患者中有 3 例出现初始颅内出血(ICH)。大多数病变的 Spetzler-Martin 分级较低(12/14,I-II)。治疗时使用的最大和边缘剂量中位数分别为 36.2(IQR 35.25-44.4)和 20(IQR 18-22.5)Gy。SRS 后总的闭塞率为 11/14,11 个闭塞的 AVM 中闭塞的中位时间为 35.83 个月(IQR,17-39.99)。所有患者的最后随访 mRS 均为 0 或 1,神经状态良好。SRS 后症状性放射性诱导变化(RIC)发生率较低(7.1%),无永久性 RIC。
结论:有多发性脑 AVM 的 HHT 患者一般适合接受 SRS 治疗。大多数 HHT 患者可实现闭塞,且并发症发生率较低。在本研究中,SRS 前的初始出血率较高,这支持了治疗这些 AVM 的决定。需要进一步的研究来更好地阐明 SRS 对 HHT 患者伴破裂和未破裂 AVM 的作用。
Acta Neurochir (Wien). 2024-1-17
Neurosurg Clin N Am. 2022-4