The Task Force recommends the adoption of the uniform terminology “Orbital-Periorbital Plexiform Neurofibroma” or OPPN for plexiform neurofibromas involving the eyelid, orbit, periorbital, and facial structures. Children with OPPN are at highest risk for rapid growth of OPPN before the age of 8. Comprehensive ophthalmic evaluation is recommended every 6 months until visual maturity. After that, frequency of examination should be guided by the clinical course. Patients with OPPN confined to the upper eyelid may not need to undergo neuroimaging. For patients with orbital, periorbital, or facial involvement, high resolution MRI scanning with and without contrast of the orbit, face, and cavernous sinus should be performed. Treatment for related ophthalmic issues, such as ptosis, lacrimal involvement, or amblyopia is supportive. Early intervention is recommended with the exception of strabismus surgery. Strabismus caused by orbital or periorbital tumor involvement while the tumor is in its rapid growth phase carries a high risk for recurrence after strabismus surgery. Associated problems such as amblyopia and refractive error should be managed aggressively and surgery deferred until the tumor growth has stabilized, if clinically appropriate to do so. Debulking surgery may be indicated for the following:
Debulking is more successful in older patients and adults. Younger patients have a high risk of recurrent progression and need for more surgery. Clinical trials using biologic agents (i.e., MEK inhibitors) are underway but no definitive recommendations can be made at this time.
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