Unusually severe limitation of the jaw attributable to fibrodysplasia ossificans progressiva: a case report with cone-beam computed tomography findings

Abstract

Fibrodysplasia ossificans progressiva (FOP) is a rare hereditary connective tissue disease characterized by the progressive ectopic ossification of ligaments, tendons, and facial and skeletal muscles throughout life. Symptoms begin in childhood as localized soft tissue swellings. Immobility and articular dysfunction appear with involvement of the spine and proximal extremities. The temporomandibular joint (TMJ) is a critical component involved in the maxillofacial region, resulting in severe limitation of masticatory function, although TMJ involvement is rare. We report a case of FOP presenting as severely limited TMJ movements owing to ectopic calcification of the left coronoid process. In addition to the clinical examination, panoramic radiography and cone-beam computed tomography images were obtained. The case is presented and the clinical and imaging findings, differential diagnosis, and treatment modalities are discussed.