Case Report
Vici Syndrome In A Mid-eastern Infant
Dr Osama A A M El Hashash, Dr Atiqur Rahman Khan, Dr Hany M Nadi
We describe an infant with Vici syndrome born to healthy, Consanguineous parents, with Oculocutnaeous Hypopigmentation, Agenesis of Corpus Callosum, and cataracts. He had postnatal growth Deficiency and profound developmental retardation. Decreased ejection Fraction of the left ventricle was demonstrated on echocardiographic examination. He also had micrognathia and high-arched palate, and Developed postnatal hydrocephalus (Dandy Walker malformation). The infant died at the age of 4 months. Death was presumably due to Cardiac arrhythmia. These findings strongly suggest the diagnosis of Vici syndrome. Chiyonobu et al [2002] suggeted autosomal recessive inheritance on the basis of occurrence of the syndrome in three pairs of siblings of both sexes to unaffected parents. Positive consanguinity of unaffected parents in our case may support this hypothesis but still needs to be proved.
Hypopigmentation Agenesis of Corpus Callosum Developmental Delay Vici syndrome Hydrocephalus Cataracts.
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