A 43-year-old woman (case II-2) with dystopia canthorum, white foreloc
k, blue iris in the left eye, hypopigmented fundi, and systemic hypert
ension complained of visible floaters in the left eye. Whitish branch
retinal vein, preretinal hemorrhages and neovascularization were found
in the left fundus. Her intraocular pressure in the left eye was some
times elevated to 22 mm Hg. The patient's brother (case II-3) was repo
rted to have blue irises in both eyes. We believe that these patients
have Waardenburg syndrome, that branch retinal vein occlusion in case
II-2 may be caused chiefly by systemic hypertension, and that elevated
intraocular pressure associated with the blue iris may be partly invo
lved in the pathogenesis of vein occlusion in the left eye.