Journal of Neuro-Oncology

, Volume 110, Issue 1, pp 1–7 | Cite as

Visual acuity in children with low grade gliomas of the visual pathway: implications for patient care and clinical research

  • Robert A. AveryEmail author
  • Rosalie E. Ferner
  • Robert Listernick
  • Michael J. Fisher
  • David H. Gutmann
  • Grant T. Liu
Topic Review


Low grade gliomas affecting the visual pathway, commonly referred to as optic pathway gliomas (OPGs), have a relatively high survival rate but can cause significant vision loss. While previous treatment outcomes for tumors of the central nervous system have focused primarily on changes in tumor size or patient survival, more recently preservation of vision has also become a primary objective when treating these tumors. Visual acuity (VA) is the most testable and reliable visual parameter in young children with OPGs. Unfortunately, standardized VA assessments have neither been employed to make treatment decisions nor used as primary outcomes in clinical trials. The lack of a standardized VA assessment has also hindered the ability to interpret and compare results between studies. It is essential that all members of the multidisciplinary care team (i.e., pediatric neuro-oncologist, neurologist, neurosurgeon, and ophthalmologist) can accurately interpret VA results and properly use them to guide management decisions. Specifically, determining what constitutes a significant change in VA and the factors that may influence these results should be incorporated into collective team recommendations. This review describes the VA assessment in children with OPGs and proposes a standardized VA testing protocol for future pediatric OPG clinical treatment trials.


Optic nerve glioma Juvenile pilocytic astrocytoma Visual acuity Visual pathways 


Conflict of Interest

All authors declare they have no conflict of interest.


  1. 1.
    Fisher MJ, Loguidice M, Gutmann DH, Listernick R, Ferner RE, Ullrich NJ, Packer RJ, Tabori U, Hoffman RO, Ardern-Holmes SL, Hummel TR, Hargrave DR, Bouffet E, Charrow J, Bilaniuk LT, Balcer LJ, Liu GT (2012) Visual outcomes in children with neurofibromatosis type 1-associated optic pathway glioma following chemotherapy: a multicenter retrospective analysis. Neuro Oncol 14:790–797 Google Scholar
  2. 2.
    Campagna M, Opocher E, Viscardi E, Calderone M, Severino SM, Cermakova I, Perilongo G (2010) Optic pathway glioma: long-term visual outcome in children without neurofibromatosis type-1. Pediatr Blood Cancer 55(6):1083–1088PubMedCrossRefGoogle Scholar
  3. 3.
    Listernick R, Ferner RE, Liu GT, Gutmann DH (2007) Optic pathway gliomas in neurofibromatosis-1: controversies and recommendations. Ann Neurol 61(3):189–198PubMedCrossRefGoogle Scholar
  4. 4.
    Ferner RE (2007) Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol 6(4):340–351PubMedCrossRefGoogle Scholar
  5. 5.
    Kluwe L, Hagel C, Tatagiba M, Thomas S, Stavrou D, Ostertag H, von Deimling A, Mautner VF (2001) Loss of NF1 alleles distinguish sporadic from NF1-associated pilocytic astrocytomas. J Neuropathol Exp Neurol 60(9):917–920PubMedGoogle Scholar
  6. 6.
    Wimmer K, Eckart M, Meyer-Puttlitz B, Fonatsch C, Pietsch T (2002) Mutational and expression analysis of the NF1 gene argues against a role as tumor suppressor in sporadic pilocytic astrocytomas. J Neuropathol Exp Neurol 61(10):896–902PubMedGoogle Scholar
  7. 7.
    Dobson V, Quinn GE, Biglan AW, Tung B, Flynn JT, Palmer EA (1990) Acuity card assessment of visual function in the cryotherapy for retinopathy of prematurity trial. Invest Ophthalmol Vis Sci 31(9):1702–1708PubMedGoogle Scholar
  8. 8.
    Mayer DL, Dobson V (1980) Assessment of vision in young children: a new operant approach yields estimates of acuity. Invest Ophthalmol Vis Sci 19(5):566–570PubMedGoogle Scholar
  9. 9.
    McDonald MA, Dobson V, Sebris SL, Baitch L, Varner D, Teller DY (1985) The acuity card procedure: a rapid test of infant acuity. Invest Ophthalmol Vis Sci 26(8):1158–1162PubMedGoogle Scholar
  10. 10.
    Drover JR, Wyatt LM, Stager DR, Birch EE (2009) The Teller acuity cards are effective in detecting amblyopia. Optom Vis Sci 86(6):755–759PubMedCrossRefGoogle Scholar
  11. 11.
    Dobson V, Quinn GE, Tung B, Palmer EA, Reynolds JD (1995) Comparison of recognition and grating acuities in very-low-birth-weight children with and without retinal residua of retinopathy of prematurity. Cryotherapy for Retinopathy of Prematurity Cooperative Group. Invest Ophthalmol Vis Sci 36(3):692–702PubMedGoogle Scholar
  12. 12.
    Adoh TO, Woodhouse JM, Oduwaiye KA (1992) The Cardiff test: a new visual acuity test for toddlers and children with intellectual impairment. A preliminary report. Optom Vis Sci 69 (6):427–432Google Scholar
  13. 13.
    Dobson V, Quinn GE, Siatkowski RM, Baker JD, Hardy RJ, Reynolds JD, Trese MT, Tung B (1999) Agreement between grating acuity at age 1 year and Snellen acuity at age 5.5 years in the preterm child. Cryotherapy for Retinopathy of Prematurity Cooperative Group. Invest Ophthalmol Vis Sci 40(2):496–503PubMedGoogle Scholar
  14. 14.
    Kushner BJ, Lucchese NJ, Morton GV (1995) Grating visual acuity with Teller cards compared with Snellen visual acuity in literate patients. Arch Ophthalmol 113(4):485–493PubMedCrossRefGoogle Scholar
  15. 15.
    Getz LM, Dobson V, Luna B, Mash C (1996) Interobserver reliability of the Teller acuity card procedure in pediatric patients. Invest Ophthalmol Vis Sci 37(1):180–187PubMedGoogle Scholar
  16. 16.
    Mash C, Dobson V (1998) Long-term reliability and predictive validity of the Teller acuity card procedure. Vision Res 38(4):619–626PubMedCrossRefGoogle Scholar
  17. 17.
    Rice ML, Leske DA, Holmes JM (2004) Comparison of the amblyopia treatment study HOTV and electronic-early treatment of diabetic retinopathy study visual acuity protocols in children aged 5 to 12 years. Am J Ophthalmol 137(2):278–282PubMedCrossRefGoogle Scholar
  18. 18.
    Moke PS, Turpin AH, Beck RW, Holmes JM, Repka MX, Birch EE, Hertle RW, Kraker RT, Miller JM, Johnson CA (2001) Computerized method for visual acuity testing: adaptation of the amblyopia treatment study visual acuity testing protocol. Am J Ophthalmol 132:903–909PubMedCrossRefGoogle Scholar
  19. 19.
    Liu GT, Volpe NJ, Galetta SL (2010) Neuro-ophthalmology: diagnosis and management. 2nd edn. Saunders Elsevier, LondonGoogle Scholar
  20. 20.
    Beck R, Maguire M, Bressler N, Glassman A, Lindblad A, Ferris F (2007) Visual acuity as an outcome measure in clinical trials of retinal diseases. Ophthalmology 114(10):1804–1809PubMedCrossRefGoogle Scholar
  21. 21.
    North KN, Riccardi V, Samango-Sprouse C, Ferner R, Moore B, Legius E, Ratner N, Denckla MB (1997) Cognitive function and academic performance in neurofibromatosis. 1: consensus statement from the NF1 cognitive disorders task force. Neurology 48(4):1121–1127PubMedCrossRefGoogle Scholar
  22. 22.
    Lambert SR, Buckley EG, Drews-Botsch C, DuBois L, Hartmann E, Lynn MJ, Plager DA, Wilson ME (2010) The infant aphakia treatment study: design and clinical measures at enrollment. Arch Ophthalmol 128(1):21–27PubMedCrossRefGoogle Scholar
  23. 23.
    Avery RA, Liu GT, Fisher MJ, Quinn GE, Belasco JB, Phillips PC, Maguire MG, Balcer LJ (2011) Retinal nerve fiber layer thickness in children with optic pathway gliomas. Am J Ophthalmol 151(3):542–549PubMedCrossRefGoogle Scholar
  24. 24.
    Chang L, El-Dairi MA, Frempong TA, Burner EL, Bhatti MT, Young TL, Leigh F (2010) Optical coherence tomography in the evaluation of neurofibromatosis type-1 subjects with optic pathway gliomas. J AAPOS 14(6):511–517PubMedCrossRefGoogle Scholar
  25. 25.
    Phillips PH (2010) Is optical coherence tomography indicated for the evaluation of patients with neurofibromatosis type 1? J AAPOS 14(6):467–468PubMedCrossRefGoogle Scholar
  26. 26.
    Davidson S, Quinn GE (2011) The impact of pediatric vision disorders in adulthood. Pediatrics 127(2):334–339PubMedCrossRefGoogle Scholar
  27. 27.
    Chadha RK, Subramanian A (2011) The effect of visual impairment on quality of life of children aged 3–16 years. Br J Ophthalmol 95(5):642–645PubMedCrossRefGoogle Scholar
  28. 28.
    Frick KD, Kymes SM, Lee PP, Matchar DB, Pezzullo ML, Rein DB, Taylor HR (2010) The cost of visual impairment: purposes, perspectives, and guidance. Invest Ophthalmol Vis Sci 51(4):1801–1805PubMedCrossRefGoogle Scholar
  29. 29.
    Rahi JS, Cumberland PM, Peckham CS (2009) Visual function in working-age adults: early life influences and associations with health and social outcomes. Ophthalmology 116(10):1866–1871PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC. 2012

Authors and Affiliations

  • Robert A. Avery
    • 1
    Email author
  • Rosalie E. Ferner
    • 2
  • Robert Listernick
    • 3
  • Michael J. Fisher
    • 4
  • David H. Gutmann
    • 5
  • Grant T. Liu
    • 6
  1. 1.Department of Neurology, Department of Pediatrics and Gilbert Neurofibromatosis CenterChildren’s National Medical CenterWashingtonUSA
  2. 2.Neurofibromatosis Centre, Department of Neurology, Guy’s and St. Thomas’ NHS Foundation Trust and Institute of PsychiatryKing’s CollegeLondonUK
  3. 3.Department of PediatricsAnn & Robert H. Lurie Children’s Hospital of Chicago, Feinberg School of Medicine, Northwestern UniversityChicagoUSA
  4. 4.Division of Oncology, Children’s Hospital of Philadelphia, and Department of PediatricsThe Perelman School of Medicine at The University of PennsylvaniaPhiladelphiaUSA
  5. 5.Neurofibromatosis Center, and Department of NeurologyWashington University, School of MedicineSt. LouisUSA
  6. 6.Neuro-Ophthalmology Service, Children’s Hospital of Philadelphia, and Departments of Neurology and OphthalmologyThe Perelman School of Medicine at The University of PennsylvaniaPhiladelphiaUSA

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