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104. Jahrestagung der Deutschen Ophthalmologischen Gesellschaft 2006

Abstract
Abstract

DO.13.03

Cornea farinata – dystrophy or degeneration?

Löffler K. U.
Universitäts-Augenklink Bonn

Objective: A 43-year-old female patient with bilateral keratoconus received a perforating keratoplasty in her left eye. In addition, she also revealed the typical clinical findings of cornea farinata with fine whitish granular deposits next to Descemet's membrane. Based on the first documented clinico-pathologic correlation in this condition, the origin and development of this disease process shall be discussed.
Methods: The removed corneal button was investigated using light and electron microscopy. Findings were compared with “normal” keratoconus cornea and with those findings described in the literature as pre-Descemet corneal dystrophies.
Results: Besides features characteristic of keratoconus, special stains for lipofuscin revealed a subtle dot-like labeling between the posterior lamellae of an otherwise unremarkable stroma. By electron microscopy, a prominent intracellular vacuolization of keratocytes not restricted to the most posterior lamellae but extending up to the mid-stroma was seen. Only very few of these granules were found in corneas with comparable keratoconus but no clinical diagnosis of cornea farinata, and they were too few to be demonstrated by histochemistry. No other significant changes were detected. The endothelium and Descemet's membrane itself were unremarkable.
Conclusions: Apparently cornea farinata is – similar to the pre-Descemet dystrophies – due to an increased deposition of lipofuscin but should probably be regarded as a degeneration rather than a dystrophy. However, the cause and in particular the unique distribution pattern as well as the pathogenesis remain unexplained.


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