Homozygous granular corneal dystrophy type II (Avellino corneal dystrophy): Natural history and progression after treatment

Jong Wook Moon, Sun Woong Kim, Tae Im Kim, Stephen M. Cristol, Eui Sang Chung, Eung Kweon Kim

Research output: Contribution to journalArticle

22 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1095-1100
Number of pages6
JournalCornea
Volume26
Issue number9
DOIs
Publication statusPublished - 2007 Oct 1

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Natural History
Corneal Transplantation
Recurrence
Parents
Penetrating Keratoplasty
Therapeutics
Visual Acuity
Disease Progression
Corneal dystrophy Avellino type
DNA

All Science Journal Classification (ASJC) codes

  • Ophthalmology

Cite this

Moon, Jong Wook ; Kim, Sun Woong ; Kim, Tae Im ; Cristol, Stephen M. ; Chung, Eui Sang ; Kim, Eung Kweon. / Homozygous granular corneal dystrophy type II (Avellino corneal dystrophy) : Natural history and progression after treatment. In: Cornea. 2007 ; Vol. 26, No. 9. pp. 1095-1100.
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Homozygous granular corneal dystrophy type II (Avellino corneal dystrophy) : Natural history and progression after treatment. / Moon, Jong Wook; Kim, Sun Woong; Kim, Tae Im; Cristol, Stephen M.; Chung, Eui Sang; Kim, Eung Kweon.

In: Cornea, Vol. 26, No. 9, 01.10.2007, p. 1095-1100.

Research output: Contribution to journalArticle

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AU - Chung, Eui Sang

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N2 - 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.

AB - 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.

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