Moyamoya Vasculopathy in Indian Children: Our Experience

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.