Teeth, Noneruption Of, With Maxillary Hypoplasia And Genu Valgum

Clinical Features

Among the 10 children of first-cousin parents, Stoelinga et al. (1976) described 2 males and 2 females with nonerupted permanent teeth, hypoplasia of the alveolar process, maxillozygomatic hypoplasia with underdeveloped maxillary sinuses, and severe genu valgum requiring surgery. All had delayed eruption of the primary teeth, which, however, were eventually shed. The ears were somewhat deformed.

Inheritance

Consanguinity in the family with noneruption of permanent teeth reported by Stoelinga et al. (1976) suggested autosomal recessive inheritance.