Patil Varsha Anant, Kulkarni Shilpa Dattaprasanna, Deopujari Chandrashekhar E, Biyani Naresh K, Udwadia-Hegde Anaita H, Shah Krishnakumar N
Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital, Mumbai, Maharashtra, India.
J Pediatr Neurosci. 2017 Oct-Dec;12(4):320-327. doi: 10.4103/jpn.JPN_65_17.
Moyamoya vasculopathy is a chronic progressive vaso-occlusive disease affecting the distal intracranial carotid arteries and their proximal branches. It is an important cause of recurrent strokes in children. Surgical revascularization procedures are now considered as the treatment option for moyamoya vasculopathy. The data from Indian children with moyamoya vasculopathy are limited to a very few studies.
We analyzed the records of children with moyamoya vasculopathy treated at our tertiary care center from 2000 to 2014. Our study population included all patients (aged 0-18 years) with moyamoya disease/syndrome (MMD/MMS). The demographic data, clinical characteristics, imaging, treatment details, and surgical procedures performed were reviewed.
A total of 41 patients (females-19, males-22) were identified. Thirty-three (80.48%) had MMD and eight (19.5%) had MMS. The mean age (±standard deviation) at presentation was 6.26 ± 3.79 years (range: 6 months-14 years). Majority had ischemic events at onset; none had hemorrhagic manifestations. Twenty-eight (68.29%) patients underwent surgery (a total of 33 surgical procedures, bilateral in five and unilateral in 23) and 13 (31.7%) were managed conservatively. The median duration of follow-up was 2.2 ± 1.85 years (range: 4 months-7 years). Two/thirteen patients (15%), who were managed conservatively, had recurrent strokes as against none (0/28) in the operated patients. No mortality was observed in our cohort.
We agree with previous studies that Indian patients with moyamoya vasculopathy differ from their Asian and European counterparts. The availability of expertise in revascularization surgeries in various centers should prompt surgery as an efficient and safe treatment option.
烟雾病血管病变是一种慢性进行性血管闭塞性疾病，影响颅内远端颈动脉及其近端分支。它是儿童复发性中风的重要原因。手术血运重建术目前被认为是烟雾病血管病变的治疗选择。来自印度烟雾病血管病变儿童的数据仅限于极少数研究。
我们分析了2000年至2014年在我们三级医疗中心接受治疗的烟雾病血管病变儿童的记录。我们的研究人群包括所有患有烟雾病/综合征（MMD/MMS）的患者（年龄0 - 18岁）。回顾了人口统计学数据、临床特征、影像学、治疗细节以及所进行的外科手术。
共确定了41例患者（女性19例，男性22例）。33例（80.48%）患有MMD，8例（19.5%）患有MMS。就诊时的平均年龄（±标准差）为6.26 ± 3.79岁（范围：6个月至14岁）。大多数患者起病时有缺血性事件；无出血表现。28例（68.29%）患者接受了手术（共33次手术，双侧手术5例，单侧手术23例），13例（31.7%）接受保守治疗。随访的中位时间为2.2 ± 1.85年（范围：4个月至7年）。13例接受保守治疗的患者中有2例（15%）发生复发性中风，而手术患者中无一例（0/28）发生。我们的队列中未观察到死亡病例。
我们同意先前的研究，即印度烟雾病血管病变患者与亚洲和欧洲患者不同。各中心血运重建手术专业知识的可得性应促使手术成为一种有效且安全的治疗选择。
