Abstract
A five-year-old boy showed a typical oculodentodigital dysplasia (microphthalmos, microcornea, abnormal texture of the iris, corectopia, persistent pupillary membrane, cataract, short palpebral fissures and epicanthal folds). the ERG and VER were extinguished. Echography revealed a bilateral persistent hyperplastic primary vitreous. the cases of the literature are reviewed. In none of them this congenital anomaly was mentioned.
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