title: Fibrodysplasia ossificans progressiva (FOP) in South Africa: dental implications in 5 cases.
abstract: Fibrodysplasia ossificans progressiva (FOP) is a severe genetic disorder in which progressive ossification of subcutaneous tissues leads to immobility and profound physical handicap. Dental management of affected persons may be constrained by impaired mobility of the temporomandibular joints. Equally, the traumatic aspects of dental intervention can exacerbate the ossification process. In the later stages of the disorder, thoracic immobility impairs pulmonary ventilation and compromises dental anesthesia. We have undertaken dental appraisal and treatment of 5 South Africans with FOP, with ages ranging from 2 to 52 years. The age relationship of the manifestations of FOP was apparent in these persons, as were the dental problems that emerged. These dental observations illustrate the importance of accurate diagnosis and the awareness of potential complications that may be encountered in dental management. Our observations are presented and discussed in this article.
authors.affiliation: Faculty of Dentistry, University of the Western Cape, Cape Town, South Africa.
authors.forename: Tina
authors.initials: T
authors.lastname: Roberts
authors.forename: Lawrence
authors.initials: L
authors.lastname: Stephen
authors.forename: Chris
authors.initials: C
authors.lastname: Scott
authors.forename: M
authors.initials: M
authors.lastname: Urban
authors.forename: Stephen
authors.initials: S
authors.lastname: Sudi
authors.forename: Peter
authors.initials: P
authors.lastname: Beighton
mesh_terms: D000328:Adult; D000367:Age Factors; D000844:Ankylosis / Q000175:diagnosis; D002648:Child; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000175:diagnosis; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008334:Mandible / Q000002:abnormalities; D008865:Microstomia / Q000175:diagnosis; D009221:Myositis Ossificans / Q000175:diagnosis*; D009999:Ossification, Heterotopic / Q000175:diagnosis; D010510:Periodontal Diseases / Q000175:diagnosis; D016059:Range of Motion, Articular / Q000502:physiology; D013019:South Africa; D013705:Temporomandibular Joint Disorders / Q000175:diagnosis