Purpose: Craniofacial fibrous dysplasia is a benign slowly progressive disease in which normal craniofacial bones are replaced by immature fibro-osseous tissue. Orbital involvement may present as visual loss in some patients. The etiology of visual loss in orbital fibrous dysplasia is still unclear, with many theories proposed in recent literature. This has led to developments in the controversy regarding the role of prophylactic optic nerve decompression in asymptomatic orbital fibrous dysplasia patients. This case report and literature review evaluates the need for early surgical intervention in such patients, as well as highlights other aspects of the condition and its management.
Design: Case report and literature review.
Methods: This was a case report of a 12-year-old Chinese girl who developed progressive visual loss in association with craniofacial fibrous dysplasia involving the orbit. Literature regarding the epidemiology, pathophysiology, clinical features, diagnosis, investigations, and management was searched from the PubMed database.
Results: Extradural decompression of the optic nerve resulted in marked visual improvement, with temporal optic atrophy noted postoperatively. Literature review revealed a number of recent studies recommending against prophylactic optic nerve decompression, but early surgical intervention upon onset of visual loss is suggested.
Conclusions: Immediate surgical referral and early optic nerve decompression is recommended upon onset of visual loss. Periodic follow-up is recommended to monitor for recurrence and malignant transformation.