Isolated anterior cerebral artery (ACA) territory ischemia in pediatric moyamoya disease (MMD) is rare but has been increasingly recognized, particularly in children manifesting progression of disease in a delayed fashion after middle cerebral artery revascularization surgery. Surgical treatment is complicated by limited graft choices, with the small number of case series largely focused on complex, higher-risk operations (omental flap transfers, large interhemispheric rotational grafts); direct bypass (often untenable in children due to vessel size); or, alternatively, the technically simpler method of multiple burr holes (of limited efficacy outside of infants). Faced with the problem of a growing cohort of pediatric patients with MMD that could benefit from anterior cerebral revascularization, the authors sought to develop a solution that was specifically designed for children and that would be lower risk than the more complex approaches adapted from adult populations but more effective than simple burr holes. In this study, the authors aimed to describe the long-term clinical and radiographic outcomes of a novel approach of pial pericranial dural (PiPeD) revascularization, building on the principles of pial synangiosis but unique in using the pericranium and the dura mater as the primary vascular supply, and employing a larger craniotomy with arachnoid dissection to provide robust full-territory revascularization in all ages with reduced risk relative to more complex procedures.

The medical records of all pediatric patients with MMD who presented at a single center between July 2009 and August 2019 were retrospectively reviewed to identify patients with MMD with anterior cerebral territory ischemia. Clinical characteristics, surgical indications, operative techniques, and long-term clinical and radiographic follow-up data were collected and analyzed.

A total of 25 operations (5.6% of total procedures) were performed in 21 patients (mean age 9.4 years [range 1–16.5 years]; 12 female and 9 male). Almost one-third of the patients had syndromic associations, with no familial cases. Complications included 1 patient (4.7%) with a superficial infection, with no postoperative strokes, hemorrhage, seizures, or deaths. Long-term follow-up was available in 18 of 21 patients (mean 24.9 months [range 4–60 months]). Radiographic engraftment was present in 90.9% (20/22 hemispheres), and no new strokes were evident on MRI on long-term follow-up, despite radiographic progression of the disease.

The use of the pericranium and the dura mater for indirect revascularization provided robust vascularized graft with great flexibility in location and high potential for engraftment, which may obviate more complex and higher-risk operations for ACA territory ischemia. Long-term follow-up demonstrated that PiPeD revascularization conferred durable, long-term radiographic and clinical protection from stroke in pediatric patients with MMD. Based on the results of the current study, the PiPeD technique can be considered an additional tool to the armamentarium of indirect revascularization procedures in select pediatric patients with MMD.

儿童烟雾病 (MMD) 中孤立的大脑前动脉 (ACA) 区域缺血很少见，但已得到越来越多的认可，特别是在大脑中动脉血运重建手术后疾病进展延迟的儿童中。手术治疗因移植物选择有限而变得复杂，少数病例系列主要集中在复杂、高风险的手术（网膜瓣转移、大型半球间旋转移植物）上；直接旁路（由于血管大小，儿童通常无法维持）；或者，或者，技术上更简单的多个钻孔的方法（在婴儿之外​​效果有限）。面对越来越多的 MMD 儿科患者可以受益于前脑血运重建的问题，作者试图开发一种专为儿童设计的解决方案，与适用于成年人群的更复杂方法相比风险更低，但比简单的钻孔更有效。在这项研究中，作者旨在描述一种新的软脑膜颅骨硬膜 (PiPeD) 血运重建方法的长期临床和影像学结果，该方法建立在软脑膜联合血管化的原则基础上，但在使用骨膜和硬脑膜作为主要血管供应，并采用更大的颅骨切开术和蛛网膜解剖，为所有年龄段的人提供强大的全区域血运重建，相对于更复杂的手术，风险更低。

回顾性审查了 2009 年 7 月至 2019 年 8 月期间在单一中心就诊的所有儿科 MMD 患者的医疗记录，以确定患有大脑前部缺血的 MMD 患者。收集和分析了临床特征、手术适应症、手术技术以及长期临床和影像学随访数据。

21 名患者（平均年龄 9.4 岁 [范围 1-16.5 岁]；12 名女性和 9 名男性）共进行了 25 次手术（占全部手术的 5.6%）。几乎三分之一的患者有综合征关联，没有家族性病例。并发症包括 1 名患者 (4.7%) 有浅表感染，没有术后中风、出血、癫痫发作或死亡。21 名患者中有 18 名进行了长期随访（平均 24.9 个月 [范围 4-60 个月]）。90.9%（20/22 个半球）存在放射学植入，尽管疾病的放射学进展，但长期随访的 MRI 上没有明显的新中风。

使用颅骨膜和硬脑膜进行间接血运重建提供了坚固的血管化移植物，在位置上具有很大的灵活性和植入的可能性高，这可能会避免更复杂和更高风险的 ACA 区域缺血手术。长期随访表明，PiPeD 血运重建可为 MMD 儿科患者提供持久的、长期的放射学和临床保护，防止中风。根据当前研究的结果，PiPeD 技术可被视为对特定儿科 MMD 患者进行间接血运重建程序的附加工具。