A young girl with painless, progressive, and permanent visual loss

We present a case of vision loss in a young girl with craniofacial fibrous dysplasia (FD). She had normal pubertal development, with menarche at the age of 9 years and no other system involvement. Other family members were not affected. Clinically she had facial asymmetry, a café-au-lait macule on the right upper back, and only perception of light present from both eyes with optic atrophy. She had renal phosphate wasting. Noncontrast computed tomography of the orbit and paranasal sinuses revealed generalized FD lesions involving craniofacial bones, with encroachment on bilateral optic nerves. Surgical optic nerve decompression was offered but refused by the patient in view of surgical risks and less chances of improvement in vision. This shows that craniofacial FD should be managed by multidisciplinary specialties from the diagnosis, and immediate referral for surgical evaluation should be made once there is evidence of visual deterioration.