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.