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J Fr Ophtalmol ; 43(6): 467-476, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32376037

RESUMEN

PURPOSE: Fibrous dysplasia (FD) is a benign fibro-osseous developmental disorder of growing bone, sometimes involving the craniofacial skeleton (CFD). We wish to present a patient series with CFD of the orbital region and discuss treatment modalities. METHODS: Twelve patients were referred for orbital CFD in the Nantes University Hospital between 2000 and 2018 and studied according to the clinical parameters, radiological features, and modalities of treatment. RESULTS: The mean age was 25.6 years. Ten patients exhibited facial asymmetry with vertical globe dystopia (75%), proptosis (58%) and facial bump (50%). The disease was monostotic in 83% of patients, involving the frontal bone (25%), the sphenoidal bone (33%), the fronto-sphenoidal complex (25%), and the skull base (17%). Unilateral radiological proptosis was found in 7 patients, with a mean protrusion 3.9mm. The optic canal was involved in 75% of patients, with no functional impairment. Three patients were treated with bisphosphonate therapy to stop progression of the disease; 6 patients were given a bone remodelling procedure with good aesthetic outcomes. CONCLUSION: The orbit is a rare localization for FD causing aesthetic and functional disabilities. Medical and surgical treatment can be proposed as part of a multidisciplinary approach.


Asunto(s)
Enfermedades del Desarrollo Óseo , Órbita/patología , Enfermedades Orbitales , Adolescente , Adulto , Anciano , Enfermedades del Desarrollo Óseo/diagnóstico , Enfermedades del Desarrollo Óseo/tratamiento farmacológico , Enfermedades del Desarrollo Óseo/patología , Niño , Estudios de Cohortes , Exoftalmia/tratamiento farmacológico , Exoftalmia/etiología , Exoftalmia/cirugía , Cara/cirugía , Asimetría Facial/tratamiento farmacológico , Asimetría Facial/etiología , Asimetría Facial/cirugía , Femenino , Francia , Humanos , Masculino , Persona de Mediana Edad , Órbita/efectos de los fármacos , Órbita/cirugía , Enfermedades Orbitales/complicaciones , Enfermedades Orbitales/tratamiento farmacológico , Enfermedades Orbitales/cirugía , Estudios Retrospectivos , Adulto Joven
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