[A case of Waardenburg-Klein syndrome observed at the Cotonou NUHC]. / Un cas de syndrome de Waardenburg-Klein observé au C.N.H.U. de Cotonou.

ABSTRACT

Waardenburg-Klein syndrome is an "Oculo-dermato-auditif" dysplasia described in 1947 by Waardenburg and by Klein in 1950. Canthus dystopia and congenital deafness are the main symptoms. Three clinical types have been reported type I presents the full symptomatology; type II without canthus dystopia; type III presents not only the complete syndrome but also an orthro-osteomyodysplasia of the upper limbs. This clinical case in a small 3.5-year-old boy with congenital deafness, bilateral iris hypochromia and retina albinism without canthus dystopia was classed as type II Waardenburg-Klein syndrome. The patient had a second apparently fortuitous hereditary affection hemoglobinopathy (Hb AS). But this seems to be fortuitous.