Tawara A, Nakamura T, Yoshida A, Kubota T, Ohnishi Y, Inomata H
Department of Ophthalmology, Wakayama Medical College, Japan.
Nippon Ganka Gakkai Zasshi. 1999 Mar;103(3):259-67.
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.
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.
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.
Disruption of the blood-aqueous barrier may occur in iris-nevus syndrome.
报告一例虹膜痣综合征伴有虹膜血-房水屏障破坏的病例，该病例经血管造影和组织病理学证实。
患者为一名39岁女性。她发现左眼视力模糊，被诊断为左青光眼。镜面显微镜检查显示左眼角膜内皮细胞密度低。左眼虹膜显示萎缩，伴有结节状虹膜痣簇和瞳孔变形。左眼虹膜角膜角因周边前粘连而关闭。
吲哚菁绿虹膜血管造影显示左眼虹膜表面的血管比右眼多。在荧光素虹膜血管造影中，染料在虹膜出现严重萎缩的区域从虹膜血管渗漏。从患者左眼小梁切除术中获得的小梁组织在组织病理学上显示虹膜表面有一层角膜内皮细胞。虹膜基质中的血管密度很高。一些覆盖血管壁的细胞出现变性，紧密连接开放。施莱姆管管腔变窄，小梁间隙关闭。
虹膜痣综合征可能发生血-房水屏障破坏。
