Amblyopia, glaucoma and undiagnosed refractive errors
Plexiform neurofibromas of the lid may be accompanied by ptosis, obscuring vision and predisposing to deprivation amblyopia13. The pressure of the lid, on the globe, may cause varying degrees of meridional astigmatism, curvature myopia or other refractive errors, which may remain uncorrected, leading to anisometropic amblyopia if not specifically screened, identified and treated 13. Though rare, ipsilateral glaucoma has been identified in association with a palpebral plexiform neurofibroma. The prevalence of glaucoma in association with plexiform neurofibroma can be as low as 1-2% 13 or as high as 23%40 in some reports. We observed glaucoma in only one case (2.9%). Current guidelines for the follow-up of NF1 patients, recommend an annual assessment for glaucoma 4. Regular assessment for refractive errors and amblyopia should be performed in patients having serial monitoring of a palpebral or craniofacial plexiform neurofibroma, to inform the need for surgical intervention.