Management of dentin dysplasia and facial disharmony.

D ENTIN DYSPLASIA was first reported in 1922 by Ballschmiedel who described six children in one family whose teeth had short, blunted roots with pulpal occlusion that he called “rootless teeth.” Rushton2 later described a similar condition in an individual without evidence of genetic inheritance that he labeled “dental dysplasia. ” Cases have been reported in the literature that describe dentin dysplasia in association with a variety of congenital syndromes including familial calc i n o ~ i s , ~ Ehlers-Danlos ~ y n d r o m e , ~ and brachio-skeleto-genital syndrome. In 1973, Shields and otherss described two types of dentin dysplasia. They defined type 1 as predominantly affecting the radicular portion of the teeth and generally characterized by foreshortened roots, pulpal occlusion, and periapicai radiolucent areas. Type 2 was reported to be distinguished by opalescent primary teeth, followed by secondary teeth with flame-shaped pulp chambers containing denticles. Morris and Augsburger’ later described dentin dysplasia (type 1) associated with sclerotic bone and skeletal anomalies, and inherited as an autosomal dominant trait in a brother and sister. Eastman and othersB also described several patients with a focal dentinal dysplasia (type 3) in which only isolated teeth were affected. Although both light and scanning electron microscopy have demonstrated characteristic morphologic patterns for dentin dyspla~ia,~ Melnick and otherslO state that the “pathogenesis . . . continues to be an enigma.” From a review of the literature, it seems that dentin dysplasia may appear with or without predictable genetic heritability and that it may vary in clinical expression, both in isolation and with other congenital anomalies. What follows is a report of a case involving combined dentin dysplasia, partial anodontia, and maxillary hypoplasia problems managed by the hospital dentistry team. REPORT OF CASE