Dr.Nivetha R

Dr. SHIVANAND BUBANALE, Dr. Smitha K.S., DR. AVANEET KUMAR

Abstract

Incontinentia pigmenti is a rare multisystem X-linked dominant disease.Ocular manifestations are seen in 70% of the patients.A 6 month old infant was brought by her parents with complaints of hyperpigmented skin lesions to the dermatologist who diagnosed the baby with incontinentia pigmenti and referred her to the ophthalmologist for further evaluation.The infant had no evidence of blink reflex,did not fixate or follow light.Evaluation under GA under the microscope showed evidence of buphthalmos with increased corneal diameter and hazy corneal in both eyes with shallow anterior chamber and IOP 29mm.There was complicated cataract.Gonioscopy showed closed angles.B scan showed retinal detachment with vitreous haemorrhage.Here, we diagnosed that buphthalmos can be an unusual presentation in cases of incontinentia pigmenti.