Emergency Optic Canal Decompression for Vision Salvage in Fibrous Dysplasia

The most common neurologic manifestations of fibrous dysplasia (FD) are vision and hearing loss. Optic decompression for progressive vision loss has been shown to yield positive results in terms of visual outcomes; however, emergency optic decompression surgery for sudden loss of vision in FD has not to date been reported in the pediatric population. We report the first case of FD presenting with sudden vision loss and successfully managed with emergency optic decompression.

A 10-year-old male patient known to have FD with a 2-month history of progressive vision loss in his left eye presents with sudden blindness. Emergency decompression surgery restored vision in his left eye to 20/40, which remained stable on long-term follow-up.

There is no consensus on the benefit and safety of prophylactic optic decompression, yet therapeutic decompression has been shown to prevent vision deterioration. Our findings suggest that therapeutic decompression even when done in the emergency setting yields positive results, while prophylactic decompression carries an inherent risk for loss of vision in a seeing eye.