Table 8.

Guideline Recommendations for orbital and periorbital plexiform neurofibroma.

Orbital and periorbital plexiform neurofibroma
No	Recommendations	Strength
1	The clinical assessment of patients with NF1 suspected of having an orbital and periorbital plexiform neurofibroma, should be physical examination looking for blepharoptosis, proptosis, eyelid oedema, orbital dysplasia and/or dystopia, distortion of the (peri)orbital skeleton, pulsation of the eye, and strabismus. Clinical testing of vision and refractive error, visual field, ocular motility and alignment, and evaluation of the optic disc to exclude glaucoma or optic neuropathy should be basic steps in the examination of patients with NF1 who are suspected of having an orbital and periorbital plexiform neurofibroma.	strong
2	MRI of the brain and orbits should be performed in all children with a suspected orbital and periorbital plexiform neurofibroma. High-resolution MRI sequences with and without contrast should be acquired through the orbit, face, and cavernous sinus. Whenever possible the radiation exposure from CT scans should be avoided in all children with NF1.	strong
3	Symptomatic clinical progression, of known orbital and periorbital plexiform neurofibromas, and new findings should be the primary indication for imaging assessment and follow-up, and this should be by MRI.	strong
4	Given the burden of visible manifestation in patients with NF1 with orbital and periorbital plexiform neurofibroma, people with orbital and periorbital plexiform neurofibroma should be offered psychological support in decisions of management (please see recommendations in the psychosocial needs, Table 15).	weak

Note. NF1 = Neurofibromatosis type 1; MRI = magnetic resonance imaging; CT = computed tomography.