Craniofacial fibrous dysplasia: Clinical characteristics and long-term outcomes

A. M.A. Rahman, S. N. Madge, K. Billing, Peter Anderson, I. Leibovitch, D. Selva, D. David

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

AimTo present the clinical features and management outcomes in a large longitudinal series of patients with craniofacial fibrous dysplasia (CFD).MethodsRetrospective interventional consecutive case series. Main outcome measures included signs and symptoms, radiographic findings, long-term outcomes, and postoperative complications.ResultsA 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.ConclusionsIn 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.

LanguageEnglish
Pages2175-2181
Number of pages7
JournalEye
Volume23
Issue number12
DOIs
Publication statusPublished - 1 Jan 2009

Keywords

  • Fibrous dysplasia
  • Optic canal decompression
  • Optic neuropathy

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems

Cite this

Rahman, A. M. A., Madge, S. N., Billing, K., Anderson, P., Leibovitch, I., Selva, D., & David, D. (2009). Craniofacial fibrous dysplasia: Clinical characteristics and long-term outcomes. Eye, 23(12), 2175-2181. https://doi.org/10.1038/eye.2009.6
Rahman, A. M.A. ; Madge, S. N. ; Billing, K. ; Anderson, Peter ; Leibovitch, I. ; Selva, D. ; David, D. / Craniofacial fibrous dysplasia : Clinical characteristics and long-term outcomes. In: Eye. 2009 ; Vol. 23, No. 12. pp. 2175-2181.
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Rahman, AMA, Madge, SN, Billing, K, Anderson, P, Leibovitch, I, Selva, D & David, D 2009, 'Craniofacial fibrous dysplasia: Clinical characteristics and long-term outcomes', Eye, vol. 23, no. 12, pp. 2175-2181. https://doi.org/10.1038/eye.2009.6

Craniofacial fibrous dysplasia : Clinical characteristics and long-term outcomes. / Rahman, A. M.A.; Madge, S. N.; Billing, K.; Anderson, Peter; Leibovitch, I.; Selva, D.; David, D.

In: Eye, Vol. 23, No. 12, 01.01.2009, p. 2175-2181.

Research output: Contribution to journalArticle

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AU - Rahman, A. M.A.

AU - Madge, S. N.

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AU - Selva, D.

AU - David, D.

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N2 - AimTo present the clinical features and management outcomes in a large longitudinal series of patients with craniofacial fibrous dysplasia (CFD).MethodsRetrospective interventional consecutive case series. Main outcome measures included signs and symptoms, radiographic findings, long-term outcomes, and postoperative complications.ResultsA 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.ConclusionsIn 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.

AB - AimTo present the clinical features and management outcomes in a large longitudinal series of patients with craniofacial fibrous dysplasia (CFD).MethodsRetrospective interventional consecutive case series. Main outcome measures included signs and symptoms, radiographic findings, long-term outcomes, and postoperative complications.ResultsA 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.ConclusionsIn 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.

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Rahman AMA, Madge SN, Billing K, Anderson P, Leibovitch I, Selva D et al. Craniofacial fibrous dysplasia: Clinical characteristics and long-term outcomes. Eye. 2009 Jan 1;23(12):2175-2181. https://doi.org/10.1038/eye.2009.6