title: Ocular Features and Visual Outcome in Children with Moyamoya Disease and Moyamoya Syndrome: A Case Series.
abstract: 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.
authors.affiliation: Associate Surgeon, Department of Ophthalmology, Christian Medical College , Vellore, Tamil Nadu, India .
authors.forename: Deepa
authors.initials: D
authors.lastname: John
authors.affiliation: Associate Professor, Dept of Neurology, Christian Medical College , Vellore, Tamil Nadu, India .
authors.forename: Karthik
authors.initials: K
authors.lastname: Muthusamy
authors.affiliation: Resident, Department of Ophthalmology, Christian Medical College , Vellore, Tamil Nadu, India .
authors.forename: Bhavagna
authors.initials: B
authors.lastname: Bandla
authors.affiliation: Physician, Department of Radiodagnosis, Christian Medical College , Vellore, Tamil Nadu, India .
authors.forename: Sniya Valsa
authors.initials: SV
authors.lastname: Sudhakar
authors.affiliation: Professor, Department of Neurology, Christian Medical College , Vellore, Tamil Nadu, India .
authors.forename: Maya
authors.initials: M
authors.lastname: Thomas
keywords: Developmental cataract; Functional vision; Seizures in children