Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes

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.