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PMID: 32376037 Published · ppublish English Journal Article Review

Fibrous dysplasia of the orbital region: Series of 12 cases and review of the literature.

Journal francais d'ophtalmologie ·Vol. 43 ·No. 6 ·2020-06-00 ·页码 467-476

Bertin H, Huon JF, Guillot P, Longis J, Corre P, Bordereau S, Lebranchu P

Abstract

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. 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. 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. 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.

Keywords
Chirurgie Drug therapy Dysplasie fibreuse de l’os Fibrous dysplasia of bone Orbit Orbite Proptose Proptosis Surgery Thérapie médicamenteuse
MeSH 主题词
Adolescent Adult Aged Bone Diseases, Developmental/diagnosis,drug therapy,pathology Child Cohort Studies Exophthalmos/drug therapy,etiology,surgery Face/surgery Facial Asymmetry/drug therapy,etiology,surgery Female France Humans Male Middle Aged Orbit/drug effects,pathology,surgery Orbital Diseases/complications,drug therapy,surgery Retrospective Studies Young Adult
作者与单位
共 7 位作者,点击展开单位 / ORCID
Bertin H
Service de Chirurgie maxillo-faciale, Centre Hospitalier Universitaire de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France; Laboratoire des sarcomes osseux et remodelage des tissus calcifiés (PhyOs, UMR 1238), faculté de médecine, 1, rue Gaston-Veil, 44035 Nantes cedex, France. Electronic address: [email protected].
Huon J-F
Service de pharmacie Clinique, Centre Hospitalier Universitaire de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France.
Guillot P
Service de rhumatologie, Centre Hospitalier Universitaire de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France.
Longis J
Service de Chirurgie maxillo-faciale, Centre Hospitalier Universitaire de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France.
Corre P
Service de Chirurgie maxillo-faciale, Centre Hospitalier Universitaire de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France.
Bordereau S
Service d'ophtalmologie, Centre Hospitalier Universitaire de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France.
Lebranchu P
Service d'ophtalmologie, Centre Hospitalier Universitaire de Nantes, 1, place Alexis-Ricordeau, 44093 Nantes, France.
Article Info
Journal
Journal francais d'ophtalmologie
Abbr.
J Fr Ophtalmol
ISSN
1773-0597
Corresponding email
Published
2020-06-00
电子出版
2020-00-04
页码
467-476
Language
English
Country/Region
France
NLM ID
7804128
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