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.