Dental characteristics of fibrous dysplasia and McCune-Albright syndrome.
OBJECTIVE
Fibrous dysplasia (FD) is a skeletal disorder often associated with McCune-Albright syndrome, a rare multisystem disorder caused by GNAS1 gene mutation. FD frequently affects the craniofacial bones, including the maxilla and the mandible; nevertheless, its effects on dental tissues and the implications for dental care remain unclear. The aim of this study was to characterize the dental features associated with FD and the reaction of affected bones to routine dental therapy. Study design Thirty-two patients with FD underwent dental evaluation and endocrine testing as part of the diagnosis of FD/McCune-Albright syndrome. Any dental anomalies were recorded, and the associations between endocrinopathies and dental anomalies were analyzed statistically by means of the paired t test.


RESULTS
Eighty-four percent had FD in the maxilla and/or mandible; endocrine dysfunction; and/or renal phosphate wasting. The caries index scores were 2.9 (ages 4-17 years) and 9.6 (ages 18-50 years). Malocclusion (81%) and other prevalent dental anomalies (41%) included tooth rotation, oligodontia, and taurodontism. The expansion of the maxilla or mandible by FD did not distort the dental arch curvature, and routine dental therapies such as extractions, restorations, and orthodontic treatment did not exacerbate FD lesions.


CONCLUSIONS
Maxillomandibular FD was associated with higher rates of caries and malocclusion than were present in healthy patients. Furthermore, patients with FD did not require special dental management and were able to undergo routine dental care without an exacerbation of FD lesions.
Akintoye
Sunday O
SO
National Institute of Dental and Craniofacial Research, National Institute of Health, Bethesda, MD 20892-4320, USA. sakintoye@dir.nidcr.nih.gov
Lee
Janice S
JS
Feimster
Tawana
T
Booher
Susan
S
Brahim
Jaime
J
Kingman
Albert
A
Riminucci
Mara
M
Robey
Pamela G
PG
Collins
Michael T
MT
D000293:Adolescent; D000328:Adult; D000848:Anodontia / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004245:DMF Index; D003724:Dental Arch / Q000473:pathology; D003786:Dental Pulp Cavity / Q000002:abnormalities; D003793:Dental Restoration, Permanent; D005147:Facial Bones / Q000473:pathology*; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000150:complications* / Q000473:pathology; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D008310:Malocclusion / Q000209:etiology; D008336:Mandibular Diseases / Q000473:pathology; D016555:Matched-Pair Analysis; D008439:Maxillary Diseases / Q000473:pathology; D008875:Middle Aged; D009971:Orthodontics, Corrective; D014076:Tooth Diseases / Q000209:etiology*; D014081:Tooth Extraction