中文摘要：

目的探讨不同病变阶段回旋状脉络膜视网膜萎缩的临床发展转归特征。方法回顾分析2006年1月至2015年5月在云南省第二人民医院眼科就诊的22例不同病变阶段回旋状脉络膜视网膜萎缩的眼底特征，并复习相关文献，对该病发展转归中特征性的指标进行总结。结果12例处于病变早期的眼底表现为周边视网膜斑片状、扇形样脉络膜毛细血管萎缩，8例中、晚期患者可见眼底萎缩灶的数量和面积增大、融合，2例患者病变发展晚期眼底萎缩灶完全融合。视网膜色素发生在眼底萎缩区域，病变未累及区域可见轻度色素脱失的改变。12例病变早期患者视神经颜色正常，随病变进展，视神经颜色逐渐变淡，到后期苍白萎缩。视网膜血管管径在病变的早期及中期阶段均未见明显变化，处于病变较为晚期的患者，可见视网膜血管变细，粗大的脉络膜血管清晰可辨。16例患者具有明显的并发性白内障，且白内障伴随眼底脉络膜视网膜萎缩病变呈进行性发展，早期主要表现为后极部后囊下白内障，逐步发展成广泛的皮质混浊。结论认识了解回旋状脉络膜视网膜萎缩的发展转归的规律性特征可为该病的诊断、鉴别诊断及视功能的预后判断等提供客观依据和指标。

英文摘要：

Objective To investigate clinical characteristics of Gyrate Atrophy of the choroids and retina. Methods Twemty-two cases of Gyrate Atrophy of the choroids and retina were collected, the characteristics of fundus, visual conditions and other clinical data were analyzed. Results Peripheral retina patchy, fan-like choriocapillaris atrophy were observed in 12 cases in the early stage. The number and size of lesions increased and integrated in 8 cases with advanced choroidal and retinal atrophy; 2 patients with advanced retinal atrophy lesions developed fully integrated. Depigmentation was seen in the atrophy area, not involving the region of the normal area. The optic nerve of 12 cases with early disease was normal, with the lesions progress, optic color gradually faded, paled in the late atrophy stage. Retinal vascular caliber in the early and mid-stage lesions showed no significant changes in patients, with advanced disease was more visible retinal blood vessels became thin, thick veins envelope membrane vessels legible; 16 cases of patients with significant concurrent cataract and cataract associated with retinal choroidal lesions had progressive retinal atrophy development, mainly for early stage posterior pole of posterior subcapsular cataracts, and gradually developed into a widespread cortical opacities. Conclusions Recognizing Gyrate Atrophy of the choroids and retina can provide patients with genetic counseling, diagnosis and differential diagnosis, determine the prognosis of visual function.