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Raffaele Parrozzani, Maurizio Clementi, Gloria Orlando, Giacomo Miglionico, Eva Trevisson, Edoardo Midena; Screening of Optic Pathway Gliomas in Children with Neurofibromatosis Type-1. Invest. Ophthalmol. Vis. Sci. 2013;54(15):5889.
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© ARVO (1962-2015); The Authors (2016-present)
The aim of this study was to compare visual function assessment (VA), optic disc evaluation by indirect ophthalmoscopy and retinal nerve fibres layer (RNFL) analysis by optical coherence tomography (OCT) as screening tools for optic pathway gliomas (OPGs) in pediatric patients (2-15 years) affected by neurofibromatosis type-1 (NF1).
Fifty-seven consecutive pediatric patients affected by NF1 with recent (<6 moths) orbital/brain MRI were included. Patients underwent VA (Hyvarinen symbols chart, HOTV or Snellen charts in patients aged 2-4, 4-6 and 6-15 years respectively) and optic disc evaluation by indirect ophthalmoscopy by experienced, masked paediatric ophthalmologist. Spectral domain-OCT (SD-OCT; Spectralis™, Heidelberg, Germany) was performed by a single masked operator to assess RNFL (16 to 100 averaged images by Automatic Real Time-ART).
Fifteen of fifty-seven enrolled patients were affected by MRI-proven ONGs. VA was judged feasible and reliable in ten (66%) of fifteen OPGs patients (40%, 60% and 100% in patients aged <5, 5-10 and 10-15 years respectively) versus thirty-for of forty-two (81%) no-OPGs patients (57%, 86% and 100% respectively). VA was clinically judged to be suspected of OPGs in six of ten (60%) OPGs examined patients versus five of thirty-four (15%) no-OPGs examined patients (p=0.01). Optic disc evaluation was judged clinically feasible in fourteen of fifteen (93%) OPGs patients (100%, 80% and 100% respectively) versus forty of forty-two (95%) no-OPGs patients (93%, 93% and 100% respectively). Optic disc aspect was clinically judged to be suspected of OPGs in four of fourteen (40%) OPGs examined patients versus four of forty (10%) no-OPGs examined patients (p>0.05). SD-OCT analysis was clinically feasible in twelve (80%) of fifteen OPGs patients (60%, 80% and 100% respectively) versus thirty-nine (93%) no-OPGs patients (79%, 93% and 100% respectively). SD-OCT analysis documented RNFL loss in eleven of twelve patients affected by OPGs (92%) and in no-one of thirty-nine no-OPGs patients (p<0.001).
RNFL assessment using SD-OCT is superior to visual function assessment and optic disc evaluation as a clinical screening tool of OPGs in NF1pediatric patients. The use of SD-OCT to detect RNFL loss under the age of 5 years may be questionable due to the low rate of children compliance, although more feasible and reliable than standard visual function assessment.
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