Fig. 1

Ocular findings of a patient with homozygous congenital protein C deficiency. a In the right eye thin dysplastic retina and peripheral avascularization with a fibrovascular band from optic disc to superior arcade is visible (arrow). b In the left eye leukoma and irregular pupil with posterior synechia and dilated iris vessels and cataract is visible. c Total funnel shaped retinal detachment of left eye is visible in B-scan (arrow). d and e Splinter and patchy abnormal fluorescences of the skin at the time of fluorescein angiography are shown (arrow). f-h Fluorescein angiography with early leakage (arrow) from mid-peripheral vessels all over the retina and later leakage around the optic nerve head due to the tractional effect of fibrovascular band. The leakage increases during less than 1 min with severe vitreous fluorescence