Intraretinal changes in the Groenblad - Strandberg syndrome WASIF KADRI , EMANUEL

Systemic elastorrhexis is a widespread hereditary degeneration of mesodermal tissues affecting many structures in the body, particularly the dermis, the media of the arteries, and Bruch's membrane in the eye. Pseudoxanthoma elasticum is one form of elastorrhexis mainly affecting the skin. Degeneration of the elastic lamina of Bruch's membrane results in the formation of angioid streaks in the retina. The association of angioid streaks and the cutaneous lesions is called the Groenblad-Strandberg syndrome. The other recognized lesions in the fundus are macular haemorrhagic and pigmentary changes (Scholz, I 94 1); mottling by pigmentary deposits (Smith, Gass, and Justice, I964; Gills and Paton, I965); choroidal atrophy and choroidal sclerosis (Groenblad, 1958); and atrophy of the pigmentary epithelium (Groenblad, 1958; Carlborg, Ejrup, Groenblad, and Lund, I959; Goodman, Smith, Paton, Bergman, Siegel, Ottesen, Shelley, Pusch, and McKusick, I963). The purpose of this paper is to describe a case of Groenblad-Strandberg syndrome which showed intraretinal bands and peripheral retinal degeneration leading to bilateral retinal detachment. The angioid streaks were not visible ophthalmoscopically but were vividly demonstrated by fluorescein studies.