Recovery of the visual acuity in a family with Reis-Bückler dystrophy

BACKGROUND: The hereditary Reis-Bückler dystrophy is one of the anterior localised corneal dystrophies with superficial reticulated opacities, sometimes in combination with recurrent attacks of corneal erosion. Phototherapeutic keratectomy (PTK) is now the first method to consider for managing this disease when intervention is required.

METHODS: We treated six eyes of four related patients with Reis-Bückler dystrophy, who had a demonstrable reduction in visual acuity due to corneal opacities within their virgin corneas (three eyes), penetrating (one eye) or lamellar (two eyes) grafts. We present the results of change in visual acuity and best corrected spherical spectacle and contact lens refraction at 1 year following surgery. In one case, we had to fit a rigid contact lens to correct the hyperopic shift which caused anisometropia.

RESULTS: The mean spherical refractive change 1 year after PTK surgery was 0.33+/-S.D., 1.8D. A hyperopic shift was observed in four eyes. Visual acuity remained stable in all patients 1 year after surgery. The central corneal area remained clear, and mean best spectacle corrected visual acuity improved from +0.8 logMAR before PTK to +0.15 logMAR after 1 year. There was no significant change (P>0.8) between the mean 1 year best spectacle corrected visual acuity (+0.125 log MAR +/- S.D. 0.15) and the best contact lens corrected visual acuity (+0.108 log MAR +/- S.D. 0.16). After fitting a contact lens in one patient to correct anisometropia, the hyperopic shift did not change significantly during 12 months of follow-up. It was not necessary to adjust the lens parameters. One patient required cataract surgery.

CONCLUSION: PTK corneal surgery is the treatment of first choice when intervention is required in patients with Reis-Bückler dystrophy because it is safe and much less invasive than lamellar or penetrating keratoplasty. Contact lens fitting following PTK has not changed corneal shape.