Pediatric moyamoya disease: clinical profile, literature review and sixteen year experience from a tertiary care teaching institute.

OBJECTIVE: To assess the clinical spectrum and outcome of pediatric moyamoya disease (MMD) in Indian sub-continent.

METHODS: The authors retrospectively analysed data of 23 patients, diagnosed with pediatric moyamoya disease from a single center during the period of 1996-2011.

RESULTS: There were 18 boys and 5 girls. Mean age at onset of symptoms was 3.8 ± 2.2 y. All patients presented with cerebral ischemic events. Recurrent stroke was the presenting feature in 12(52.2 %) patients. Twenty one patients were definitive case of moyamoya disease and two were probable case of moyamoya disease. Posterior circulation was involved in 26.1 % patients. Three patients underwent indirect surgical revascularisation procedure and rest were managed conservatively. On follow up 66.6 % patients had residual neurological deficit.

CONCLUSIONS: Heightened awareness of this entity among pediatric neurologists is required for early diagnosis of pediatric moyamoya disease.