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Optic pathway glioma: Long‐term visual outcome in children without neurofibromatosis type‐1
Author(s) -
Campagna Marta,
Opocher Enrico,
Viscardi Elisabetta,
Calderone Milena,
Severino Savina Maria,
Cermakova Iveta,
Perilongo Giorgio
Publication year - 2010
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22748
Subject(s) - medicine , neurofibromatosis , radiation therapy , pallor , visual field , visual acuity , chemotherapy , visual impairment , pediatrics , surgery , ophthalmology , radiology , psychiatry
Background Little is known about the visual outcome of children affected by an optic pathway glioma (OPG). Procedures 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. Pediatr Blood Cancer. 2010;55:1083–1088. © 2010 Wiley‐Liss, Inc.