Sir,
We are pleased that the publications have provoked open debate and thank Gutmann et al for their comments.1
We concur that the current recommendations of ophthalmology appointments for children with NF1 do not constitute screening, and should not be labelled as such. A recent audit of the NF1 OPG screening service in Manchester revealed that screening appointments were poorly attended and no OPGs were detected during annual ophthalmology screening over a 7-year, 128-episode period.2
The term ‘age appropriate screening’ remains problematic on several levels. At least 40% children with NF1 have developmental delay or learning disability and have difficulty performing the tests.3 Learning disability is associated with cerebral visual impairment (CVI); CVI and amblyopia reduce vision. If there are no ‘normal’ data for visual development in children with NF1, how are we to define the ‘reduced vision’ that is ‘highly predictive of OPG’?1
Perhaps what we should all be working towards is ‘developmentally appropriate screening’ and use the ongoing national project to collect the ‘normal’ data we need, so that we may be better informed when making the next round of recommendations.
References
Gutmann DH, Listernick R, Ferner RE . Screening for symptomatic optic pathway glioma in children with neurofibromatosis type 1 Eye 2011; 25: 818.
Pilling RF, Lloyd IC, Huson SM . Utility of optic pathway glioma screening in young children with neurofibromatosis type I: questions generated by a clinical audit. Eye 2010; 24 (10): 1603–1605.
Krab LC, Aarsen FK, de Goede-Bolder A, Catsman-Berrevoets CE, Arts WF, Moll HA et al. Impact of neurofibromatosis type 1 on school performance. J Child Neurol 2008; 23 (9): 1002–1010.
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Pilling, R., Taylor, R. Response to Gutmann et al. Eye 25, 818–819 (2011). https://doi.org/10.1038/eye.2010.230
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DOI: https://doi.org/10.1038/eye.2010.230