Iris vessel involvement in iris-nevus syndrome

PURPOSE: To report a case of iris-nevus syndrome accompanied by disruption of the blood-aqueous barrier in the iris which was confirmed angiographically and histopathologically. CASE: The patient was a 39-year-old woman. She noticed blurred vision in the left eye which was diagnosed as left glaucoma. Specular microscopy revealed low endothelial cell density in the left cornea. The left iris showed atrophy with clusters of nodular iris nevus and distorted pupil. The left iridocorneal angle was closed with peripheral anterior synechia. FINDINGS: Indocyanine green iris angiography revealed more vessels on the surface of the left iris than on the right. In fluorescein iris angiography, the dye leaked from the iris vessels in areas where the iris showed advanced atrophy. The trabecular tissue obtained by trabeculectomy from the patient's left eye showed histopathologically a lining of corneal endothelial cells on the surface of the iris. The density of the vessels was high in the iris stroma. Some cells covering the vessel wall showed degeneration with opening of the zonula occludens. Schlemm's canal had narrowed lumina, and the intertrabecular spaces were closed. CONCLUSION: Disruption of the blood-aqueous barrier may occur in iris-nevus syndrome.