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  • Courtesy of Muhammad Arif Ozir, MD.
    File Size: 154 KB
    Cataract/Anterior Segment, Pediatric Ophth/Strabismus

    A 7-year-old girl presented to the ophthalmology clinic with complaints of painless blurry of vision, photophobia, and glare since birth. There was no history of trauma, infection, or a significant past medical history. Her uncorrected Snellen visual acuity is 1/60 for the right eye and the left eye which had horizontal nystagmus, her visual acuity was counting fingers. Slit lamp examination shows bilateral 360 degree iris hypoplasia. The anterior chamber depth is normal with a clear, crystalline lens. Intraocular pressure is 16 mmHg for both eyes. Bilateral fundus examination is otherwise normal, and there is no other systemic abnormalities detected. The diagnosis of bilateral congenital aniridia was made. Aniridia is a rare genetic disorder in which there is a variable degree of hypoplasia or absence of iris associated with other ocular features, some present from birth and some arising progressively over time. Visual acuity is highly variable, and presence of nystagmus generally worsens vision quality. The autosomal dominant (AD) mode of inheritance accounts for two-thirds of cases and has no systemic implications. Cycloplegic refraction for this patient was done and there is improvement of best corrected visual acuity for the right eye (6/60), and 1/60 for the left eye. She was prescribed photochromic glasses and continues follow-up appointments due to risk of glaucoma and refractive errors.