Dr.Karthikeyan Mahalingam, Dr.Anand Naik Bukke, Prof. Dr.Tanuj Dada

Abstract

A female presented in her middle childhood with complaints of diminished vision in right eye(RE) since 3 years. She had brownish hyperpigmentation over right side of her face along trigeminal nerve distribution, suggestive of Nevus of Ota and over her right chest and back, indicating Nevus of Ito; multiple café-au-lait spots over her abdomen, back and axillary freckles.Head imaging revealed right sphenoid wing dysplasia. Her visual acuity was 1/60 (RE), 6/6 left eye(LE); Intraocular pressure 38mmhg(RE),8 mmHg(LE). Ocular evaluation revealed RE upper eyelid plexiform neurofibroma, Haab’s striae, ectropion uveae, featureless angle, anterior insertion of iris, glaucomatous optic neuropathy; Lisch's nodules in both eyes. She was diagnosed with Neurofibromatosis Type-1 with Nevus of Ota and Nevus of Ito with RE congenital glaucoma and amblyopia. RE 7 clock hours goniotomy was done. Refractive glasses were prescribed; at 3 months followup RE IOP was 10mmHg on two antiglaucoma medications.