Optic glioma (OPG) accounts for 4-8% of all
brain tumors in children. En-block removal of intraorbital
tumor is recommended in cases with disfiguring
exophthalmos and impaired vision. Surgical resection of intraorbital optic nerve (ON) poses the risks of permanent ptosis and globe
atrophy. We present here the case of a 4-year-old boy with
exophthalmos and near
blindness due to an intraorbital OPG. Despite
chemotherapy he showed progressive
exophthalmos and vision loss. Bony orbital
decompression with ON transection temporally reduced his
exophthalmos. OPG resection was required later for recurrence of his
exophthalmos secondary to
tumor progression. Post operatively, he had preserved oculomotor nerve functions but developed globe
ischemia. Unusually, his ischemic globe caused him to have
pain and severe
photophobia, which later lead to enucleation.
Photophobia has been reported in blind patients. Animal models and MRI functional imaging showed activation of trigeminal pathway during
photophobia in completely transected ON. However, the exact neuro-ophthalmology pathway requires further study.
CONCLUSION: This is the first described case of
photophobia after excision of OPG with ON
denervation.
Photophobia can be a serious side effect that significantly lowers the patient's quality of life.