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钱德勒综合征作为原发性虹膜萎缩的一种变体:一项临床病理研究

Chandler's syndrome as a variant of essential iris atrophy. A clinicopathologic study.

作者信息

Rodrigues M M, Streeten B W, Spaeth G L

出版信息

Arch Ophthalmol. 1978 Apr;96(4):643-52. doi: 10.1001/archopht.1978.03910050339009.

Abstract

Trabeculectomy and peripheral iridectomy specimens from one male and two female patients with Chandler's syndrome (age, 30 to 42 years) showed that all had unilateral corneal endothelial "dystrophy," corneal edema, mild to moderate iris atrophy without holes, peripheral anterior synechiae, and glaucoma. In one, fluorescein angiography of the iris disclosed a sector filling delay of limbal and conjunctival vessels and pupillary and extrapupillary leakage. Histopathologic examination showed a layer of degenerated corneal endothelium and Descemet's membrane extending across the inner uveal trabeculum. Descemet's membrane displayed irregular, nodular, scroll-like excrescences in some cases, and thinner placoid configurations with abnormal widely spaced collagen (100 nm) in others. Corneal endothelial cells exhibited increased microvilli, widened cellular interdigitations, and occasional shrunken cells with enlarged or disrupted cytoplasmic blebs. Peripheral iris specimens displayed mild to moderate stromal atrophy without vascular occlusions. Pigment epithelium was normal.

摘要

对一名30岁男性和两名30至42岁女性钱德勒综合征患者的小梁切除术和周边虹膜切除术标本进行检查,结果显示,所有患者均患有单侧角膜内皮“营养不良”、角膜水肿、轻度至中度虹膜萎缩(无破孔)、周边前粘连和青光眼。其中一名患者的虹膜荧光素血管造影显示,角膜缘和结膜血管扇形充盈延迟,瞳孔和瞳孔外渗漏。组织病理学检查显示,一层变性的角膜内皮和后弹力层延伸穿过葡萄膜小梁内侧。在某些病例中,后弹力层呈现不规则、结节状、卷曲状赘生物,而在其他病例中,则呈现较薄的板状结构,胶原间距异常增宽(100纳米)。角膜内皮细胞微绒毛增多,细胞交错连接增宽,偶尔可见细胞皱缩,胞质小泡增大或破裂。周边虹膜标本显示轻度至中度基质萎缩,无血管闭塞。色素上皮正常。

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