Stage-related therapy of corneal dystrophies

Corneal dystrophies typically result in a gradual bilateral loss of vision in a primary 'white eye' - often in conjunction with epithelial defects in later stages. Treatment of corneal dystrophies needs to be stage-related. To ensure a stage-related therapeutic approach, an adequate classification based on clinical, histopathological and genetic knowledge is indispensable. In principle, topical medications, contact lenses and various microsurgical approaches are applicable. In case of predominantly superficial dystrophies of the epithelium, basal membrane and/or Bowman's layer (map-dot-fingerprint, Meesmann, Lisch, Reis-Bücklers, Thiel-Behnke), recurrent epithelial defects may complicate the clinical picture. If conservative therapy with gels/ointments, application of therapeutic contact lenses and/or conventional corneal abrasion are not successful, phototherapeutic keratectomy (PTK) using a 193-nm excimer laser is the method of choice today. PTK can be repeated several times, thus post poning corneal transplantation (lamellar or even penetrating) for a long time. Three major goals may be achieved by PTK depending on the diagnosis: (1) to remove superficial opacities; (2) to regularize the surface and treat irregular astigmatism, and (3) to improve the adherence of the epithelium. In dystrophies with depositions predominantly in the stroma (e.g. granular, lattice, macular, recurrence on the graft), PTK may be a reasonable alternative to anterior lamellar or penetrating keratoplasty (PKP) depending on the exact localization of the lesions. Besides exact determination of the depth of depositions using a slit lamp, a preoperative topography analysis is indispensable. The therapy of endothelial dystrophies depends on diagnosis and age: Fuchs endothelial corneal dystrophy will need corneal transplantation (e.g. when visual acuity drops below 0.4). In contrast, transplantation will only be very rarely necessary in posterior polymorphous corneal dystrophy, but the intraocular pressure has to be checked frequently. Especially in elderly patients with reduced compliance, posterior lamellar keratoplasty - preferably in the form of Descemet stripping automated endothelial keratoplasty - may be performed instead of PKP. In case of congenital hereditary endothelial dystrophy, the best time point of PKP has to be determined with regard to amblyopia (surgery too late) and inadequate follow-up (surgery too early) together with parents and pediatric ophthalmologists on an individual basis. In conclusion, for stage-related therapy of corneal dystrophies, besides contact lenses, PTK and PKP, various techniques of lamellar keratoplasties represent an indispensable enrichment of our corneal microsurgical spectrum today.