The Natural History of Fibrous Dysplasia of the Orbit

Orbital fibrous dysplasia (FD) is a disease of disordered fibro-osseous proliferation secondary to altered osteogenesis, with potential sequelae including compressive neuropathy and irreversible vision loss. The purpose of this study was to evaluate the natural history and longitudinal outcomes of 37 patients with orbital FD who underwent stratified surgical management.

All patients treated for FD from 2015 to 2021 were identified, yielding 185 patients, 39 with orbital involvement. Impressions from head computed tomographic scans were analyzed by a craniofacial radiologist to evaluate location and timing of tumor growth. Operative records were reviewed to determine surgical approach (partial excision/contouring, complete excision, or partial excision with optic nerve decompression).

The average patient age at diagnosis was 10.7 ± 4.5 years, and the average follow-up was 5.2 ± 4.7 years. Of the 37 patients with orbital involvement, 28 (75.7%) had optic canal involvement. Of those with optic canal involvement, 13 (46.4%) required partial excision with optic nerve decompression, whereas 15 (53.6%) did not. Of those without optic canal involvement, two patients (22.2%) underwent partial excision/contouring of the anterior orbit to correct dystopia and/or proptosis, and four patients (44.4%) underwent complete excision of the orbital component and reconstruction with bone graft or mesh. Younger age at diagnosis was associated with an increased number of surgical interventions ( P = 0.011), younger age at first optic canal decompression ( P = 0.003), and worse visual outcomes ( P = 0.009).

In the authors' cohort, patients diagnosed at a younger age required more operations, underwent decompression earlier, and had worse visual outcomes.

Risk, III.