Fibrous dysplasia of the orbit in childhood: clinical features and management

In 16 children with orbitocranial fibrous dysplasia confirmed histopathologically, facial asymmetry, orbital dystopia, and unilateral proptosis were the main presenting problems. Five children had radiological evidence of narrowing of the optic canal but only two developed optic atrophy and visual l...

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Veröffentlicht in:Ophthalmology (Rochester, MN) MN), 1985, Vol.92 (1), p.12-20
Hauptverfasser: MOORE, A. T, BUNCIC, J. R, MUNRO, I. R
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BUNCIC, J. R
MUNRO, I. R
description In 16 children with orbitocranial fibrous dysplasia confirmed histopathologically, facial asymmetry, orbital dystopia, and unilateral proptosis were the main presenting problems. Five children had radiological evidence of narrowing of the optic canal but only two developed optic atrophy and visual loss. One patient had persistent epiphora due to involvement of the bony nasolacrimal duct. Sixteen patients were treated by excision of the dysplastic bone and 15 had immediate craniofacial reconstruction. A good cosmetic and functional result was achieved in the majority of cases although transient vertical diplopia and minor degrees of ptosis occurred postoperatively in about a third. Fibrous dysplasia of the orbit is progressive in childhood and is best managed by radical surgery and reconstruction in a specialized craniofacial unit.
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subjects Adolescent
Biological and medical sciences
Child
Child, Preschool
Exophthalmos - surgery
Female
Fibrous Dysplasia of Bone - pathology
Fibrous Dysplasia of Bone - surgery
Fibrous Dysplasia, Polyostotic - surgery
Follow-Up Studies
Humans
Male
Maxillary Diseases - surgery
Medical sciences
Ophthalmology
Ophthalmoplegia - etiology
Optic Atrophy - surgery
Orbit - pathology
Orbit - surgery
Orbit diseases
Orbital Diseases - pathology
Orbital Diseases - surgery
Postoperative Complications - etiology
Recurrence
title Fibrous dysplasia of the orbit in childhood: clinical features and management
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