alexa Homozygous granular corneal dystrophy type II (Avellino corneal dystrophy): natural history and progression after treatment.
Ophthalmology

Ophthalmology

Journal of Clinical & Experimental Ophthalmology

Author(s): Moon JW, Kim SW, Kim TI, Cristol SM, Chung ES,

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Abstract PURPOSE: To describe the clinical features of homozygous granular corneal dystrophy type II (GCDII) with age and with several kinds of treatment in 18 homozygous patients in several different conditions. METHODS: Eighteen homozygous GCDII patients, confirmed with DNA analysis, of 13 families were enrolled. Their clinical features that include age at detection by parents, visual acuity, and disease progression were evaluated. We also studied the recurrence patterns for the 13 patients who underwent phototherapeutic keratectomy, penetrating keratoplasty, lamellar keratoplasty, or deep lamellar keratoplasty. RESULTS: The age at detection by the parents ranged from 3 to 5 years; visual loss begins in childhood with progression into the 20s. All of the patients who had undergone surgeries acquired better vision immediately after surgery. Corneal deposits reappeared soon after treatments. Recurrences became progressively more rapid and severe with treatments. CONCLUSIONS: The clinical features of homozygous GCDII are characterized by a severe granular type of corneal dystrophy with an early onset and rapid progression. After surgical treatment, recurrence is rapid and severe. This article was published in Cornea and referenced in Journal of Clinical & Experimental Ophthalmology

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