Vici syndrome is a condition associated with agenesis of the corpus callosum, congenital cataract, incomplete albinism, ± immunodeficiency and cardiomyopathy. Individuals show profound hypotony and severe neuro abnormalities. This paper reports a case of Vici syndrome. A 38-month-old female had bilateral nuclear and anterior polar cataracts and bilateral optic nerve atrophy. Visual interaction improved after left cataract extraction and anterior vitrectomy. This child was quiet, placid and profoundly hypotonic. She had fair skin and lightly pigmented blond hair, blue irides and normal sclera, long eyelashes, mild bitemporal narrowing, narrow palate and parallel transverse palmar creases bilaterally. VEP demonstrated misrouting of optic nerve pathways. OCT showed a poorly defined fovea with lesser degree of foveal depression. VEP was reported to aid the diagnosis of the case.
Vici syndrome
Reviewed by Fiona Rowe
Ophthalmologic features of Vici syndrome.
CONTRIBUTOR
Fiona Rowe (Prof)
Institute of Population Health, University of Liverpool, UK.
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