Inherited Idiopathic Retinal Venous Beading with Branch Vein Occlusion
Althaus C., Birnbaum F.
Augenklinik, Heinrich-Heine-Universität, Düsseldorf
Purpose: Venous beading usually is a secondary phenomenon in eyes with chronic alterations of the retinal vasculature or in hyperviscosity syndrome. Up to now, only very few patients were reported with primary venous beading and involvement of other family members with different severity. Known complications are central or branch vein occlusions and preretinal bleeding.
Method: We report a 17 year old female Caucasian patient with a sudden decrease of vision in the left eye due to branch vein occlusion supero-temporally on the basis of bilateral venous beading in all quadrants with massive irregularities of central and peripheral retinal veins.
Results: Fluorescein-angiography confirmed this and showed a normal arm-retina time and arterio-venous passage time in the other venous branches. Family examination showed similar changes of the retinal vasculature of her father but to a lesser degree. Both patients showed a leucocytosis and otherwise normal laboratory studies.
Conclusions: Inherited primary venous beading is rare. Extreme forms of irregularities seem to predispose to vasoocclusive complications. This justifies to discuss anticoagulation therapy.
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