Long-term visual outcomes of optic pathway gliomas in pediatric patients without neurofibromatosis type 1.
Sporadic optic pathway gliomas (OPGs) have been reported to cause more vision loss than OPGs associated with neurofibromatosis type-1, but long-term visual outcome data are limited. The purpose of this study was to report the visual outcomes of a cohort of pediatric patients with sporadic OPGs. This was a retrospective, cohort study at a tertiary care pediatric hospital and cancer institute. The study included all patients with sporadic OPGs evaluated from 1990 to 2014. The primary outcome was visual acuity at final follow-up. Secondary outcomes were risk factors for a poor visual outcome and the rate of progression. There were 59 pediatric patients included in the study. Median age at presentation was 2.5 years old and median follow-up was 5.2 years. In the worse eye at final follow-up, 16 patients (27 %) were 20/30 or better, 9 patients (15 %) were between 20/40 and 20/80, and 34 patients (58 %) were 20/100 or worse. In the better eye at final follow-up, 33 patients (56 %) were 20/30 or better, 11 patients (19 %) were between 20/40 and 20/80, and 15 patients (25 %) were 20/100 or worse. Risk factors for a poor visual outcome included younger age at presentation, optic nerve pallor, and tumor extent. Of the 54 patients (92 %) who received treatment, 40 (74 %) experienced disease progression during or after treatment. A majority of pediatric patients with sporadic OPGs had significant long-term visual impairment. In spite of treatment, tumor progression is common. Serial ophthalmic examinations with quantitative vision measurements are essential in the management of sporadic OPGs.
Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Fegan 4, Boston, MA, 02115, USA.
Michael J
MJ
Wan
Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, 02115, USA.|Department of Hematology/Oncology, Boston Children's Hospital, Harvard Medical School, Boston, MA, 02115, USA.|Pediatric Neuro-Oncology, Dana-Farber/Boston Children's Hospital Center for Cancer and Blood Disorders, Boston, MA, 02115, USA.
Nicole J
NJ
Ullrich
Department of Hematology/Oncology, Boston Children's Hospital, Harvard Medical School, Boston, MA, 02115, USA.|Pediatric Neuro-Oncology, Dana-Farber/Boston Children's Hospital Center for Cancer and Blood Disorders, Boston, MA, 02115, USA.
Peter E
PE
Manley
Department of Hematology/Oncology, Boston Children's Hospital, Harvard Medical School, Boston, MA, 02115, USA.|Pediatric Neuro-Oncology, Dana-Farber/Boston Children's Hospital Center for Cancer and Blood Disorders, Boston, MA, 02115, USA.
Mark W
MW
Kieran
Department of Neurosurgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, 02115, USA.
Liliana C
LC
Goumnerova
Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Fegan 4, Boston, MA, 02115, USA. Gena.Heidary@childrens.harvard.edu.
Gena
G
Heidary
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*; D020339:Optic Nerve Glioma / Q000150:complications*; D019574:Optic Nerve Neoplasms / Q000150:complications*; D012189:Retrospective Studies; D012307:Risk Factors; D014786:Vision Disorders / Q000209:etiology*; D014792:Visual Acuity; D014795:Visual Pathways / Q000473:pathology*
Glioma; Juvenile pilocytic astrocytoma; Neurofibromatosis 1; Optic nerve glioma; Visual acuity; Visual pathways