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Outcomes: Recurrence of Disease

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Corneal Transplantation

Abstract

The inherited diseases or true corneal dystrophies tend to recur in the grafts. The frequency and intensity of recurrence vary extensively. With time more sophisticated genetic analyses have made the old clinical classification less reliable and the IC3D classification system welcome. Heredity is part of the definition of a corneal dystrophy something that is rarely found in basement layer corneal dystrophy and in what is generally named Fuchs’ dystrophy. Future studies need to accommodate to strict modern classification. Large variation in recurrence frequency can be found in clinical similar forms of granular dystrophy but having different genetic lesions. Keratoconus is overrepresented within many families, but the mode of inheritance is unclear. The disease can progress within the host cornea, the graft received may harbor keratoconus, and keratoconus may develop within an otherwise healthy graft. The exchange of cells between the host and the graft has been studied, but there are no strict rules of how stromal and endothelial cells carrying the genetic defect from the host will be distributed within the graft and cause a recurrence. A degeneration such as Salzmann’s nodular degeneration can recur in a graft. Well known is also the recurrence of herpes simplex keratitis. Here prophylactic treatment postoperatively using oral acyclovir over long periods has proven beneficial. Corneal dystrophies with a distinct heredity, keratoconus with a presumed heredity, degenerations with no heredity, and infections like herpes simplex are examples of diseases that may recur in the graft.

Interpretation of the literature, especially of the corneal dystrophies, is complicated as scientific progress discloses so much new data. What started as one diagnosis later came to be many different mutations with varying phenotypes. We are presently in the midst of the molecular genetic revolution, and things may change further. It appears more important, in future studies, to carefully characterize both genotype and phenotype in the inherited diseases.

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  1. Department of Ophthalmology, University Hospital, Linköping, Sweden

    Per Fagerholm MD, PhD

  2. Faculty of Health, Institute for Clinical and Experimental Sciences – Ophthalmology, Linköping University, Linköping, 581 83, Sweden

    Per Fagerholm MD, PhD

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    Jesper Hjortdal

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Fagerholm, P. (2016). Outcomes: Recurrence of Disease. In: Hjortdal, J. (eds) Corneal Transplantation. Springer, Cham. https://doi.org/10.1007/978-3-319-24052-7_9

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