Multiple radiopaque mandibular lesions in a patient with Apert syndrome

Abstract

Introduction: Apert syndrome (acrocephalosyndactyly) is a rare congenital malformation characterized by craniosynostosis, craniofacial anomalies, and symmetric syndactyly of the hands and feet. Oral manifestations usually include bifid uvula, a Byzantine arch palate associated with lateral swellings of the palatine processes, severe maxillary dental crowding associated with teeth malposition, severe open bite, dental caries, and gingival and periodontal disorders. Florid osseous dysplasia is an asymptomatic lesion mostly encountered during casual dental radiographic examinations as multiple sclerotic masses in 2 or more quadrants, usually in tooth-bearing regions.

Methods: A 32-year-old woman diagnosed with Apert syndrome was seen in our department for a routine dental examination. Radiographic evaluation showed multiple radiopaque lesions in the mandible. All teeth with radiopaque lesions gave positive responses to vitality tests, and the patient did not report any symptoms. Based on the clinical and radiographic findings, the diagnosis of florid osseous dysplasia in a patient with Apert syndrome was made. Because there were no signs of an intraoral infectious process or endodontic needs, the patient was followed during revisions for Apert syndrome, and the only treatment provided was conservative management of the many carious lesions observed during the clinical examination.

Conclusions: To our knowledge, this is the first study reporting the occurrence of florid osseous dysplasia in a patient with Apert syndrome. Conservative management should be performed in asymptomatic cases. Although rare, our case report highlights the importance of florid osseous dysplasia as a condition that may mimic lesions with an endodontic origin in patients with Apert syndrome.