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Incidents with Snyders Corneal Dystrophy

1Riebeling P., 1Polz S., 1Tost F., 2Hoeltzenbein M.,
1Ernst-Moritz-Arndt-Universität Greifswald, Klinik und Poliklinik für Augenheilkunde (Greifswald)
2Institut für Humangenetik (Greifswald)

Background: The snyders cristalline corneal dystrophy is characterized by a central corneal clouding with cristalline cholesterol deposits on both eyes. The disease is accompanied by an Arcus lipoides. The inheritage is on an autosomal dominant trait and can be due to a defect of the chromosome 1p34-p36.
Case report: We report about a 66 year old women and her son with a both eye corneal clouding. It has been perfomed a corneal transplantation on the son at the age of 30 years in another clinic. The mother was referred for a penetrating keratoplasty because of a progressive deterioration of vision. The leading hallmarks for the diagnosis Snyders corneal dystrophy were the bothside clouding of the cornae in the superficial layer, the subepithelial cristalline deposits and a big Arcus lipoides. The patient had Xanthelasmen on both palpebraes. We found serological a hyperlipoproteinemia of type 4 and a scanty hypercholesterinemia.
Conclusions: In cases of a suspicion of Snyders cristalline corneal dystrophy it should always excluded a disturbance in the fat metabolism for the prophylaxis of cardiovascular diseases. We recommend an examination of the familiy members and humangenetical diagnostics.