Die Behandlung des Goldmann-Favre-Syndroms mit Cyclosporin A und Bromocriptin

Abstract

Up to now several reports have described a disturbance of humoral and cellular immune response in patients with tapetoretinal degeneration, but a pathogenetic relation of the described immunological changes to the retinal disease was not established. Therefore immunosuppressive treatment has not been advocated for degenerative vitreoretinal disease. We now report about the natural course of Goldmann-Favre's vitreo-retinal degeneration and the results of a therapeutical trial with cyclosporin A and bromocriptine in two patients. Without therapy we saw a slowly progressive flat central retinoschisis with a serous macular exsudation in fluorescein-angiography. Additionally we found peripheral vitreoretinal changes, mild cellular vitreal infiltration, extinguished electro-retinogram and disturbed dark adaptation. Visual acuity and visual field were quite stabile. Under therapy with cyclosporin A we found a regression of the macular edema and flattening of the retinoschisis. One of the two cases showed a good improvement of visual acuity, in the other case a clear subjective improvement did not correlate with an increase in visual acuity. An additional therapy with bromocriptine did not bring further success, but we were able to reduce the cyclosporin A to a blood concentration of 70-100 ng per ml.

Clinical features of Goldmann-Favre syndrome

“…He also found that ERG was abnormal in both siblings. Further changes have been described in later reports in which the clinical features vary among patients (Fishman et al 1976, Schmidt & Weinberg 1976, Peyman et al 1977, Izumi & Matsuhashi 1987, Nasr et al 1990, Garweg et al 1991.…”

“…Cataract surgery is necessary in some cases. Medical treatment and retinal surgery have been tested (Garweg et al 1991), but still, the visual prognosis is poor.…”