Optic pathway glioma: long-term visual outcome in children without neurofibromatosis type-1.
BACKGROUND
Little is known about the visual outcome of children affected by an optic pathway glioma (OPG).


METHODS
We evaluated the long-term visual outcome of 32 consecutive children affected by OPG without neurofibromatosis type-1 referred to the Pediatric Department of Padua University and managed according to standardized strategies.


RESULTS
Eight children received chemotherapy, 10 radiotherapy, 7 both chemotherapy and radiotherapy, whereas 7 were untreated. At presentation, visual acuity (VA) was normal in 22 children (13 unilaterally and 9 bilaterally), and reduced in 10. At follow-up, VA had improved in 6 patients; it was stable in 8 and worse in 18. Visual field, assessed in 29 children, was normal in 9 and reduced in 20. The number of children with some grade of visual impairment increased from 7 to 10 during follow-up. Of the 17 children in whom the tumor became significantly smaller, VA improved in 6, was stable in 3, and worse in 8. Of the 6 children with improved VA, 5 received radiotherapy, and their papilla was normal or mildly pale. After a median follow-up of 6 years, 26 patients are alive with stable disease.


CONCLUSIONS
The visual prognosis of children with OPG is unsatisfactory. Older children treated with radiotherapy seem to have a better visual outcome than younger children. Severe optic pallor at diagnosis or during follow-up may be indicative of a negative prognosis.
Campagna
Marta
M
Department of Pediatrics, University of Padua, Padua, Italy.
Opocher
Enrico
E
Viscardi
Elisabetta
E
Calderone
Milena
M
Severino
Savina Maria
SM
Cermakova
Iveta
I
Perilongo
Giorgio
G
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009456:Neurofibromatosis 1 / Q000503:physiopathology; D020339:Optic Nerve Glioma / Q000503:physiopathology* / Q000628:therapy; D019574:Optic Nerve Neoplasms / Q000503:physiopathology* / Q000628:therapy; D011879:Radiotherapy Dosage; D016896:Treatment Outcome; D014792:Visual Acuity / Q000502:physiology*; D014794:Visual Fields / Q000502:physiology*; D014795:Visual Pathways / Q000503:physiopathology*