Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes.
OBJECTIVE
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.
Rahman
A M A
AM
Discipline of Ophthalmology and Visual Sciences, Royal Adelaide Hospital, University of Adelaide, Adelaide, South Australia.
Madge
S N
SN
Billing
K
K
Anderson
P J
PJ
Leibovitch
I
I
Selva
D
D
David
D
D
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005128:Eye Diseases / Q000209:etiology*; D005147:Facial Bones / Q000000981:diagnostic imaging; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D012189:Retrospective Studies; D012886:Skull / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D014786:Vision Disorders / Q000209:etiology; D014792:Visual Acuity; D055815:Young Adult