Craniofacial XXXX Involving the Orbit: A Case Report and Literature Review.

@entity1 @entity227 @entity82 @entity2694 @entity74
PURPOSE: Craniofacial @entity82 is a @entity74 in which normal craniofacial bones are replaced by immature fibro-osseous tissue. Orbital involvement may present as @entity227 in some @entity1 . The etiology of @entity227 in @entity2694 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 @entity2694 @entity1 . This case report and literature review evaluates the need for early surgical intervention in such @entity1 , 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 @entity1 who developed progressive @entity227 in association with craniofacial @entity82 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 @entity227 is suggested. CONCLUSIONS: Immediate surgical referral and early optic nerve decompression is recommended upon onset of @entity227 . Periodic follow-up is recommended to monitor for recurrence and malignant transformation.
