Table 3.

Guideline recommendations for optic pathway glioma.

Optic pathway glioma
No	Recommendations	Strength
1	Clinical assessment for OPG should begin immediately after diagnosis or suspicion of NF1 in childhood. Baseline ophthalmology assessment should be done at presentation whatever the age.	strong
2	Clinical assessment for OPG should take the form of examination by trained paediatric ophthalmologists or neuro-ophthalmologists or equivalent with experience in the assessment of NF1 related visual changes.	strong
3	Clinical assessment for OPG should include age-appropriate assessment of visual acuity, visual fields, pupillary testing, eye movements, and optic disc appearance.	strong
4	Assessment of retinal nerve fibre layer and retinal ganglion cell layer by optic coherence tomography is helpful and should be conducted whenever feasible.	moderate
5	For children until the age of 8 years without known OPG, ophthalmological assessment (see recommendation 1–3) should be repeated at least every year (every six months if feasible).	moderate
6	In children >8 years without known OPG formal annual visual screening is advised until adulthood. Diagnostic evaluation by an ophthalmologist is also indicated in those with new visual symptoms.	moderate
7	Imaging for OPG with MRI should be performed in people where ophthalmological examination is suggestive for OPG and in children older than 2 years with repeated inconclusive or unreliable ophthalmological exam, e.g. due to age or attention deficit. Abnormal, inconclusive or unreliable ophthalmological exam should be repeated within a short timeframe.	strong
8	Any patient with NF1 diagnosed with an asymptomatic OPG should receive a referral to a unit with expertise (e.g. paediatric, ophthalmology, and/or neuro-oncology) in the monitoring and management of NF1-OPG.	moderate
9	Any patient with NF1 diagnosed with a symptomatic OPG should receive an urgent referral to a unit with expertise (e.g. paediatric, ophthalmology, and/or neuro-oncology) in the management of NF1-OPG.	strong

Note. OPG = optic pathway glioma; NF1 = Neurofibromatosis type 1; MRI = magnetic resonance imaging.