• Bone dysplasia;
  • dental genetics;
  • 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 sclerosing bone dysplasia has important orofacial manifestations and a wide geographical distribution. Comprehensive oral and dental evaluation of eight affected adults in the Afrikaner community of South Africa was undertaken.

Results: Gross asymmetrical hypertrophy of the mandible was present in all eight patients, bilateral or unilateral facial paralysis with weakness of facial muscles due to facial nerve entrapment was present in six. Drooling of saliva and difficulties with mastication were frequent problems in these persons. The teeth were structurally and mechanically normal although there was partial anodontia in two patients and delayed eruption in another. Maxillary (palatal) and mandibular tori were present in every affected person. Due to the hyperostosis of the maxilla and mandible, tooth extraction was often a very difficult matter. There were no instances of post-extraction ostomyelitis of the mandible.

Conclusions: Accurate differentiation of sclerosteosis from the other sclerosing bone dysplasias is crucial for effective dental prognostication and management.