title: Visual acuity of children treated with chemotherapy for optic pathway gliomas.
abstract: BACKGROUND
  Chemotherapy is the most common primary treatment modality for pediatric optic pathway gliomas (OPGs). Due to the risk of severe visual impairment, visual acuity (VA) has become a clinical parameter of fundamental importance for children with OPGs. Despite this reality, most studies omit crucial information necessary for analysis of the effect of chemotherapy on VA in patients with cerebral gliomas. The principal goal of this study was to determine the immediate and long-term visual outcome of children treated first with chemotherapy for OPGs.
  
  
  METHODS
  Retrospective, non-comparative, case series of children with OPGs treated initially with chemotherapy. VA was measured prior to chemotherapy, directly following chemotherapy, as well as at last follow-up.
  
  
  RESULTS
  Seven children (14 eyes) were positive for the neurofibromatosis type-1 (NF1) mutation and 10 children (20 eyes) were without the NF1 mutation (sporadic). Three deaths, all in the sporadic cohort, occurred as a result of their OPG. Median follow-up time of survivors was 10.54 ± 4.36 (SD) years. Both NF1 mutation positive and sporadic cohorts had deterioration in VA over time; however, deterioration was only statistically significant in the sporadic population. The percentage of eyes with vision weaker than 20/200 prior to chemotherapy, directly following chemotherapy and at last follow-up grew from 18% to 24% to 38%, respectively.
  
  
  CONCLUSIONS
  In both NF1 mutant and sporadic OPGs, VA deteriorated directly following chemotherapy as well as at long-term follow-up. Despite chemotherapy, eyes with severe functional impairment gradually increased over time.
authors: CHU Sainte Justine, University of Montreal, Montreal, Quebec, Canada.
authors: Evan
authors: E
authors: Kalin-Hajdu
authors: Jean-Claude
authors: JC
authors: Décarie
authors: Monia
authors: M
authors: Marzouki
authors: Anne-Sophie
authors: AS
authors: Carret
authors: Luis H
authors: LH
authors: Ospina
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D016514:Genes, Neurofibromatosis 1; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009154:Mutation / Q000235:genetics*; D009367:Neoplasm Staging; D009456:Neurofibromatosis 1 / Q000235:genetics*; D020339:Optic Nerve Glioma / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D011379:Prognosis; D012189:Retrospective Studies; D014786:Vision Disorders / Q000139:chemically induced / Q000209:etiology* / Q000473:pathology; D014792:Visual Acuity
keywords: chemotherapy; optic pathway glioma; visual acuity