Dental and oral manifestations of Sclerosteosis

Aims and objectives: Documentation of the oral and dental manifestations of Sclerosteosis. Setting and participants: Sclerosteosis is a member of the family of genetic craniotubular hyperostoses. This severe progressive scler osing bone dysplasia has important orofacial manifestations and a wide geog raphical distribution. Comprehensive oral and dental evaluation of eight af fected adults in the Afrikaner community of South Africa was undertaken. Re sults: Gross asymmetrical hypertrophy of the mandible was present in all ei ght patients, bilateral or unilateral-facial paralysis with weakness of fac ial muscles due to facial nerve entrapment was present in six. Drooling of saliva and difficulties with mastication were frequent problems in these pe rsons. The teeth were structurally and mechanically normal although there w as partial anodontia in two patients and delayed eruption in another. Maxil lary (palatal) and mandibular tori were present in every affected person. D ue to the hyperostosis of the maxilla and mandible, tooth extraction was of ten a very difficult matter. There were no instances of post-extraction ost omyelitis of the mandible. Conclusions: Accurate differentiation of scleros teosis from the other sclerosing bone dysplasias is crucial for effective d ental prognostication and management.