title: Photophobia in a blind eye after removal of a progressive orbital optic glioma with denervation.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Pediatric Hematology and Oncology, Center of Pediatrics and Adolescent Medicine, University Medical Center Freiburg, Germany; Pediatric Hematology and Oncology, Department of Pediatrics, Universiti Kebangsaan Malaysia Medical Center, Kuala Lumpur, Malaysia.
authors.forename: C Khai
authors.initials: CK
authors.lastname: Loh
authors.affiliation: Pediatric Hematology and Oncology, Center of Pediatrics and Adolescent Medicine, University Medical Center Freiburg, Germany.
authors.forename: Barbara
authors.initials: B
authors.lastname: Weis
authors.affiliation: Department of Neurosurgery, University Medical Center Freiburg, Germany.
authors.forename: Vera
authors.initials: V
authors.lastname: van Velthoven
authors.affiliation: Eye Center, University Medical Center Freiburg, Germany.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Reiff
authors.affiliation: Pediatric Hematology and Oncology, Center of Pediatrics and Adolescent Medicine, University Medical Center Freiburg, Germany. Electronic address: jochen.roessler@uniklinik-freiburg.de.
authors.forename: Jochen
authors.initials: J
authors.lastname: Rössler
mesh_terms: D001766:Blindness / Q000209:etiology*; D002675:Child, Preschool; D003714:Denervation; D005094:Exophthalmos / Q000209:etiology*; D006801:Humans; D008297:Male; D009900:Optic Nerve / Q000601:surgery*; D020339:Optic Nerve Glioma / Q000150:complications* / Q000601:surgery; D009915:Orbit / Q000601:surgery; D020795:Photophobia / Q000503:physiopathology*; D014276:Trigeminal Nerve / Q000503:physiopathology
keywords: Blindness; Exophthalmos; Optic glioma; Photophobia; Trigeminal nerve