Fibrodysplasia Ossificans Progressiva

A Case Report

Abstract

Fibrodysplasia ossificans progressiva is a rare genetic disorder characterized by progressive heterotopic ossification, which may lead to severe functional impairment. Orofacial involvement can result in marked restriction of jaw movement up to extra-articular temporomandibular ankylosis, severely complicating oral hygiene, diagnostics and dental treatment. This case report describes a 25-year-old male patient with advanced fibrodysplasia ossificans progressiva and complete jaw immobility with a maximum interincisal opening of 0 mm. He presented himself with daily dental pain, poor oral hygiene and recurrent swelling episodes. Conventional dental radiography was not feasible. Computed tomography revealed a permanent dentition except tooth 38, marked crowding and several malpositioned, non-occluding posterior teeth without obvious periapical pathology, marked periodontal bone loss or gross carious lesions. After interdisciplinary planning, including anesthesiologic consultation and expert advice in fibrodysplasia ossificans progressiva care, removal of the posterior teeth was performed to improve oral cleanability, reduce the risk of inflammatory complications in inaccessible posterior regions, facilitate nutrition within the existing limitations and improve emergency access for suction in case of vomiting. Local injection techniques were avoided because of the risk of iatrogenic heterotopic bone formation. The procedure was performed under general anesthesia with awake fibreoptic nasotracheal intubation and standby tracheostomy, using a strict soft-tissue-sparing approach and meticulous avoidance of positioning trauma. The patient was monitored for two nights and recovered without complications. At 11-month follow-up, he remained free of dental pain and recurrent swelling episodes and reported improved food intake with a weight gain of 8 kg. This case highlights the importance of preventive dentistry, strict indication for invasive procedures, multidisciplinary planning, airway preparedness, maximal trauma avoidance and long-term dental follow-up in specialized centers.