Ocular Features and Visual Outcome in Children with Moyamoya Disease and Moyamoya Syndrome: A Case SeriesCorrespondence Address :
Dr. Deepa John,
Associate Surgeon, Department of Ophthalmology, Schell Eye Hospital, Arni Road, Christian Medical College,
Vellore – 632001, Tamil Nadu, India.
Moya Moya Disease (MMD) is characterised by idiopathic vasculopathy affecting the terminal internal carotid arteries resulting in the formation of extensive collaterals at the base of the brain, leptomeninges and parenchymal regions with resultant infarcts and bleeds. Four children presented with clinico-radiological features suggestive of Moyamoya disease/syndrome. This includes global developmental delay, recurrent seizures, transient ischaemic attacks and impaired vision. The first patient had vision of 6/15 in both eyes with bilateral optic disc pallor. Second case also had bilateral optic disc pallor with arteriolar attenuation, but had vision of perception of light only in both eyes. The third child had vision of 6/60 with alternate divergent squint and clinical features suggestive of Neurofibromatosis 1 (NF 1). Fourth patient presented with poor fixation in both eyes with bilateral total cataract. He underwent bilateral cataract surgery with intraocular lens implantation and vision improved to 2/60 with good fixation. We also describe their medical and neurosurgical interventions in this report.
Developmental cataract, Functional vision, Seizures in children
Deepa John, Karthik Muthusamy, Bhavagna Bandla, Sniya Valsa Sudhakar, Maya Thomas. OCULAR FEATURES AND VISUAL OUTCOME IN CHILDREN WITH MOYAMOYA DISEASE AND MOYAMOYA SYNDROME: A CASE SERIES. Journal of Clinical and Diagnostic Research [serial online] 2016 May [cited: 2018 Jan 23 ]; 10:NR01-NR04. Available from
Date of Submission: Jan 29, 2016
Date of Peer Review: Feb 22, 2016
Date of Acceptance: Mar 15, 2016
Date of Publishing: May 01, 2016
Financial OR OTHER COMPETING INTERESTS: None.
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