Abstract：
OBJECTIVE: Posterior cerebral artery involvement (PCAi) has been identified as an important factor related to poor prognosis in moyamoya disease (MMD). This study summarized the characteristics of children with MMD and PCAi, clarified the clinical course, identified prognostic predictors, and investigated the long-term effect of encephaloduroarteriosynangiosis for posterior circulation (EDAS-p).
METHODS: We retrospectively reviewed all our pediatric MMD cases with follow-up angiograms from November 2003 to December 2016. PCAi was classified as early-onset at initial diagnosis and delayed-onset after anterior circulation revascularization. Multivariable data including clinical features, radiographic findings, and surgical outcomes were analyzed.
RESULTS: Among 570 children with MMD, 246 (43.2%) had PCAi, with 176 (30.9%) classified as early-onset PCAi. During a median follow-up period of 10 years, 17.8% (70/394) of patients without initial PCAi developed delayed-onset PCAi. The median time to detection of a new PCA lesion was 15.5 (range 7-110) months from initial diagnosis, with a median age of 10.5 (3-22). Younger age at onset, familial occurrence, advanced Suzuki stages, and preoperative infarctions were predictors of delayed-onset PCAi. EDAS-p was performed on 294 hemispheres of 195 patients with PCAi. Stroke-free survival was significantly higher in the EDAS-p group than in the non-EDAS-p group (99.0% vs 90.2%; p < 0.001 [Breslow test]; p = 0.001 [log-rank test]; median follow-up: 101 months).
CONCLUSIONS: PCAi is not uncommon in children with MMD, underscoring the need for long-term close clinical monitoring, especially in patients with high-risk factors for PCA progression. EDAS-p may be a safe and effective procedure for preventing subsequent stroke in children with MMD and PCAi.
METHODS: We retrospectively reviewed all our pediatric MMD cases with follow-up angiograms from November 2003 to December 2016. PCAi was classified as early-onset at initial diagnosis and delayed-onset after anterior circulation revascularization. Multivariable data including clinical features, radiographic findings, and surgical outcomes were analyzed.
RESULTS: Among 570 children with MMD, 246 (43.2%) had PCAi, with 176 (30.9%) classified as early-onset PCAi. During a median follow-up period of 10 years, 17.8% (70/394) of patients without initial PCAi developed delayed-onset PCAi. The median time to detection of a new PCA lesion was 15.5 (range 7-110) months from initial diagnosis, with a median age of 10.5 (3-22). Younger age at onset, familial occurrence, advanced Suzuki stages, and preoperative infarctions were predictors of delayed-onset PCAi. EDAS-p was performed on 294 hemispheres of 195 patients with PCAi. Stroke-free survival was significantly higher in the EDAS-p group than in the non-EDAS-p group (99.0% vs 90.2%; p < 0.001 [Breslow test]; p = 0.001 [log-rank test]; median follow-up: 101 months).
CONCLUSIONS: PCAi is not uncommon in children with MMD, underscoring the need for long-term close clinical monitoring, especially in patients with high-risk factors for PCA progression. EDAS-p may be a safe and effective procedure for preventing subsequent stroke in children with MMD and PCAi.
摘要：
目的：大脑后动脉受累（PCAi）已被确定为烟雾病（MMD）预后不良的重要因素。本研究总结了MMD和PCAi患儿的特点,阐明了临床过程，确定的预后预测因子，并研究了脑性动脉共病对后循环的长期影响（EDAS-p）。
方法：我们回顾性回顾了2003年11月至2016年12月的所有儿科MMD病例并进行了血管造影随访。PCAi在最初诊断时被分类为早发性和前循环血运重建后延迟性。多变量数据，包括临床特征，射线照相结果，并对手术结果进行分析。
结果：在570名患有MMD的儿童中，246（43.2％）患有PCAi，176例（30.9%）被归类为早发性PCAi。在10年的中位随访期间，17.8％（70/394）无初始PCAi的患者出现延迟发作的PCAi。从最初诊断开始，发现新的PCA病变的中位时间为15.5（范围7-110）个月，年龄中位数为10.5（3-22）。发病年龄较小，家族性事件，先进的铃木阶段，术前梗死是延迟性PCAi的预测因素。对195例PCAi患者的294个半球进行了EDAS-p。EDAS-p组的无卒中生存率明显高于非EDAS-p组（99.0％vs90.2％；p<0.001[Breslow检验]；p=0.001[log-rank检验]；中位随访时间：101个月）。
结论：PCAi在患有MMD的儿童中并不少见，强调需要长期密切的临床监测，尤其是有PCA进展高危因素的患者。EDAS-p可能是预防MMD和PCAi患儿后续卒中的安全有效方法。
方法：我们回顾性回顾了2003年11月至2016年12月的所有儿科MMD病例并进行了血管造影随访。PCAi在最初诊断时被分类为早发性和前循环血运重建后延迟性。多变量数据，包括临床特征，射线照相结果，并对手术结果进行分析。
结果：在570名患有MMD的儿童中，246（43.2％）患有PCAi，176例（30.9%）被归类为早发性PCAi。在10年的中位随访期间，17.8％（70/394）无初始PCAi的患者出现延迟发作的PCAi。从最初诊断开始，发现新的PCA病变的中位时间为15.5（范围7-110）个月，年龄中位数为10.5（3-22）。发病年龄较小，家族性事件，先进的铃木阶段，术前梗死是延迟性PCAi的预测因素。对195例PCAi患者的294个半球进行了EDAS-p。EDAS-p组的无卒中生存率明显高于非EDAS-p组（99.0％vs90.2％；p<0.001[Breslow检验]；p=0.001[log-rank检验]；中位随访时间：101个月）。
结论：PCAi在患有MMD的儿童中并不少见，强调需要长期密切的临床监测，尤其是有PCA进展高危因素的患者。EDAS-p可能是预防MMD和PCAi患儿后续卒中的安全有效方法。
