The aim of this study was to evaluate whether indirect revascularization in pediatric patients with moyamoya disease leads to periventricular anastomosis (PVA) regression, which is markedly developed in moyamoya vessels and is regarded as a risk factor for hemorrhage.

Pediatric patients with moyamoya disease treated with indirect revascularization from 2011 to 2021 were included in this study. Magnetic resonance angiography and arterial spin labeling images acquired before and 1 year after surgery were assessed to obtain a visual scale of postoperative collateral artery formation, moyamoya vessels, PVA, and quantitative values of cerebral blood flow (CBF). The relationship between background information (age, sex, RNF213 p.R4810K variant status, and preoperative CBF) and postoperative collateral artery formation, as well as postoperative CBF improvement and regression of moyamoya vessels and PVA, was evaluated.

Of 89 hemispheres in 58 patients (34 females; mean [SD] patient age 8.0 [3.4] years), 74.2% showed good postoperative collateral artery formation and a significant increase in CBF (p < 0.001). Postoperative PVA showed significant regression (postoperative score 1.46 [1.06] vs 2.02 [1.69], p = 0.001), especially in those arising from choroidal arteries (postoperative score 0.28 [0.50] vs 0.72 [0.67], p < 0.001). Compared with hemispheres without good collateral artery formation, those with good collateral artery formation were more likely to show a higher increase in CBF (9.74 [12.44] ml/min/100 g vs −4.86 [9.68] ml/min/100 g, p < 0.001) and regression of PVA (54.5% [36/66] vs 30.4% [7/23], p = 0.015). Although not statistically significant, patients with postoperative PVA progression were younger than those with regression (6.75 [3.03] years vs 8.18 [3.17] years, p = 0.188), and patients with the RNF213 p.R4810K variant were more likely to show regression (28/57 [49.1%] hemispheres vs 5/13 [38.5%] hemispheres, p = 0.069).

Indirect revascularization in pediatric patients with moyamoya disease resulted in good collateral extracranial artery formation and an increase in CBF and PVA regression, especially of vessels arising from choroidal arteries. With good postoperative collateral artery development, patients were more likely to show improved CBF and regression of moyamoya vessels, including PVA. Whether postoperative PVA changes reduce future hemorrhage risk requires further investigation.

本研究的目的是评估烟雾病儿科患者的间接血运重建是否会导致脑室周围吻合术（PVA）消退，这种现象在烟雾病血管中显着发展，被认为是出血的危险因素。

本研究纳入了 2011 年至 2021 年接受间接血运重建治疗的烟雾病儿科患者。对术前和术后 1 年采集的磁共振血管造影和动脉自旋标记图像进行评估，以获得术后侧支动脉形成、烟雾血管、PVA 和脑血流 (CBF) 定量值的视觉尺度。评估背景信息（年龄、性别、 RNF213 p.R4810K 变异状态和术前 CBF）与术后侧支动脉形成、术后 CBF 改善以及烟雾血管和 PVA 消退之间的关系。

在 58 名患者（34 名女性；平均 [SD] 患者年龄 8.0 [3.4] 岁）的 89 个半球中，74.2% 的患者术后侧支动脉形成良好，CBF 显着增加（p < 0.001）。术后 PVA 显示显着回归（术后评分 1.46 [1.06] vs 2.02 [1.69]，p = 0.001），尤其是来自脉络膜动脉的患者（术后评分 0.28 [0.50] vs 0.72 [0.67]，p < 0.001）。与没有良好侧支动脉形成的半球相比，侧支动脉形成良好的半球更有可能表现出更高的 CBF 增加（9.74 [12.44] ml/min/100 g vs -4.86 [9.68] ml/min/100 g，p < 0.001）和 PVA 回归（54.5% [36/66] vs 30.4% [7/23]，p = 0.015）。虽然不具有统计学意义，但术后 PVA 进展的患者比出现退化的患者更年轻（6.75 [3.03] 岁 vs 8.18 [3.17] 岁，p = 0.188），并且具有 RNF213 p.R4810K 变异的患者更有可能出现退化（ 28/57 [49.1%] 半球 vs 5/13 [38.5%] 半球，p = 0.069）。

烟雾病儿科患者的间接血运重建导致颅外侧支动脉形成良好，CBF 和 PVA 消退增加，尤其是脉络膜动脉产生的血管。术后侧支动脉发育良好，患者更有可能表现出 CBF 改善和烟雾血管（包括 PVA）消退。术后 PVA 变化是否会降低未来出血风险需要进一步研究。