Oral management in a patient with fibrodysplasia ossificans progressiva

Abstract

Fibrodysplasia ossificans progressiva (FOP) is an autosomal dominant disorder of connective tissue. FOP results in debilitating heterotopic ossification of the axial and appendicular skeleton. Permanent ankylosis of the temporomandibular joint (TMJ) is a common late finding, but is usually preceded by specific inciting trauma. Extreme care must be exercised when carrying out routine dental care to prevent soft tissue trauma. Oral health care for patients with FOP is usually complex. There are additional considerations when oral surgical procedures, such as extractions, are needed. Maintaining a balance between oral health and disease progression in these patients is challenging, as they frequently present with advanced oral disease. Fiber optic-assisted placement of the nasoendotracheal tube is the standard of care. Intramuscular injections including mandibular blocks must be avoided. Permanent fusion of the TMJ leads to malnutrition, inanition, and aspiration of food. In patients with complete TMJ ankylosis, dental extractions can be safely and effectively performed while accessing teeth from the buccal aspect. This approach has successfully resulted in decreased morbidity in these patients as well as others with limited oral opening.