Chapter 303 Syndromes with Oral Manifestations

Many syndromes have distinct or accompanying facial, oral, and dental manifestations (Apert syndrome, Chapter 585; Crouzon disease, Chapter 585; Down syndrome, Chapter 76).

Osteogenesis imperfecta is often accompanied by effects on the teeth, termed dentinogenesis imperfecta (Chapter 299, Fig. 299-2). Depending on the severity of presentation, treatment of the dentition varies from routine preventive and restorative monitoring to covering affected posterior teeth with stainless steel crowns, to prevent further tooth loss and improve appearance. Dentinogenesis imperfecta can also occur in isolation without the bony effects.

Another syndrome, cleidocranial dysplasia, has orofacial variations such as frontal bossing, mandibular prognathism, and a broad nasal base. Tooth eruption is often delayed. The primary teeth can be abnormally retained and the permanent teeth remain unerupted. Supernumerary teeth are common, especially in the premolar area. Although the erupted teeth are usually free of hypoplasia, variations in the size and shape of the teeth are common. Extensive dental rehabilitation therapy may be needed to maintain effective mastication.

Ectodermal dysplasias (Chapter 641

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