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Type: Journal article
Title: Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes
Author: Rahman, A.
Madge, S.
Billing, K.
Anderson, P.
Leibovitch, I.
Selva-Nayagam, D.
David, D.
Citation: Eye, 2009; 23(12):2175-2181
Publisher: Nature Publishing Group
Issue Date: 2009
ISSN: 0950-222X
Statement of
AMA Rahman, SN Madge, K Billing, PJ Anderson, I Leibovitch, D Selva and D David
Abstract: Aim: To present the clinical features and management outcomes in a large longitudinal series of patients with craniofacial fibrous dysplasia (CFD). Methods: Retrospective interventional consecutive case series. Main outcome measures included signs and symptoms, radiographic findings, long-term outcomes, and postoperative complications. Results: A total of 42 patients with CFD were identified. The mean age at presentation was 16.7 years; mean follow-up was 12.6 years. Out of these 42 patients, 37 (88.1%) had unilateral involvement and 5 (11.9%) had bilateral involvement, of which 3 (7.1%) had McCune–Albright syndrome. The commonest presenting symptom was facial asymmetry (36 cases, 86%). The frontal bone was the most commonly involved (27 cases, 64.3%), zfollowed by the sphenoid (24 cases, 57.1%). The most common pattern of bone involvement was monostotic (32 cases, 76.2%). Radiological optic canal involvement occurred in 18 eyes of 15 (37.5%) patients, with optic atrophy in 9 eyes (18.8%) of 7 patients (16.7%). Surgical intervention was performed in 30 (71.4%) cases for both functional and reconstructive reasons. Optic canal decompression was performed in three cases, in all of which stabilization of vision was achieved; no patient lost vision as a result of surgery. Conclusions: In this large longitudinal series of CFD, visual loss was not uncommon and occurred insidiously. The presenting clinical and radiological features, surgical interventions, and outcomes are discussed.
Keywords: fibrous dysplasia
optic neuropathy
optic canal decompression
Rights: © 2009 Macmillan Publishers Limited. All rights reserved
DOI: 10.1038/eye.2009.6
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Appears in Collections:Aurora harvest 5
Opthalmology & Visual Sciences publications

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