Abstract

Lattice corneal dystrophy type I is an autosomal dominant corneal dystrophy caused by allelic mutations of the BIGH3 gene. Type I dystrophy is recognized clinically by the characteristic net of linear opacities within the corneal stroma that results from an accumulation of amyloid. This study was designed to evaluate the therapeutic potential of phototherapeutic keratectomy (PTK) for the treatment of lattice corneal dystrophy type I. PTK was performed with the Chiron Technolas Chiron Keracor 217c on a series of 19 eyes of 13 patients with lattice dystrophy type I. Mean patient age was 38.9 years. The mean follow-up period was 36 months. Localization of central opacities was determined by analyzing Scheimpflug images. The changes in spherical equivalent and best corrected visual acuity were evaluated at 1, 3, 6, 9, 12, 18, 24 and 36 months. The central depth of the deposits measured with the Scheimpflug camera was on average 74.14+/-31.03 microm in the primary dystrophies and 30.1+/-10 microm in graft recurrence. We noted a clear improvement in visual acuity, which increased by 0.257+/-0.120 to 0.600+/-0.178 as of the 3rd month and stabilized at 0.684+/-0.257 until the 36th month. A disappearance of repeating ulcerations was observed at month 30. We found a statistically significant correlation (R=0.6776; p=0.0109) between the improvement in vision (in lines) and the depth of opacities (with the Scheimpflug camera). The mean hyperopic shift caused by photoablation (69+/-15 microm) was +0.71+/-1 D at 36 months. These results confirm that PTK is an effective method of managing corneal lattice dystrophy type I.

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