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Abstract #22996 Published in IGR 11-1

Bilateral aniridia lenticular coloboma and snowflake retinal degeneration

Doganay S; Emre S; Firat P
Ophthalmic Surgery Lasers and Imaging 2009; 40: 54-56

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A 6-year-old boy presented with bilateral aniridia associated with lens coloboma and snowflake retinal degeneration. Ophthalmologic examination revealed bilateral corneal peripheral epithelial thickening and aniridia. Additionally, the patient had lenticular colo-boma and snowflake retinal degeneration in both eyes. Intraocular pressure was 22 mmHg bilaterally. The patient also had pendular nystagmus. Uncorrected visual acuity was counting fingers at 2 meters for both eyes, but improved to 0.2 and 0.05, respectively, with correction. Congenital aniridia has been reported with various ophthalmic pathologies, but this is the first case to display bilateral lenticular coloboma and snowflake retinal degeneration associated with aniridia.

Dr. S. Doganay, B. Huseyin Bey Mah. Yunus Emre Cad., Mehmet Ozgungor T, R-44100 Malatya, Turkey

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Classification:

9.1.3 Syndromes of Axenfeld, Rieger, Peters, aniridia (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)

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