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Published on: October 27, 2023
Demystifying Maxillomandibular Fibrous Dysplasia and Its Impact on Dental Development
Nicholas A Han1, Philip D Tolley1, Theodor B Lenz1
1From the Division of Plastic, Reconstructive, and Oral Surgery, Children's Hospital of Philadelphia.
Background:
Fibrous dysplasia of the jaw (FDJ) is a rare fibroosseous skeletal disorder that affects the maxilla and/or mandible, often resulting in significant dental complications. However, its impact on dentition and occlusion remains poorly characterized. This study aims to analyze the dental and orthodontic sequelae of FDJ to enhance clinical management and improve patient care.
Methods:
A retrospective cohort study was conducted on patients with FDJ seen at the authors' institution from 2015 to 2023. Medical records and computed tomographic imaging were reviewed to assess disease distribution, dental abnormality, occlusion, and orthodontic treatment outcomes.
Results:
Among 39 patients with FDJ, 30 (76.9%) had direct dentoalveolar involvement. Malocclusion was documented in 29 patients (74.4%), with Angle class 1 in 26 (66.7%), Angle class 2 in 10 (25.6%), and Angle class 3 in 3 patients (7.7%). Dental abnormalities included retained primary teeth ( n = 5 [12.8%]), dental caries ( n = 8 [20.5%]), enamel hypoplasia ( n = 1 [2.6%]), and loss of permanent dentition ( n = 3 [7.7%]). Of 15 patients with long-term follow-up after initiation of orthodontics, 12 (80%) experienced long-term stability of occlusion without requiring repeated orthodontic intervention.
Conclusions:
FDJ presents significant dental challenges, with high rates of dentoalveolar involvement and malocclusion. Although some patients experienced tooth pain and rapid disease progression resulting in need for surgical intervention, most patients achieved stable occlusion into adulthood, suggesting that orthodontic treatment is generally successful with appropriate follow-up and multidisciplinary management. Despite concerns about disease progression, adult dentition is typically preserved, and early intervention can be effective when closely monitored.
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