成年Coats病的临床特征和诊治

目的观察成年Coats病患者的临床表现及其治疗效果。方法回顾性分析1980年至2006年在中国医学科学院北京协和医院就诊, 经眼底检查和荧光素眼底血管造影检查确诊为Coats病的18例成年患者随诊1年以上的临床资料。所有患者均排除放射治疗史，眼内炎症、视网膜血管闭锁、老年性黄斑变性及渗出较多的糖尿病视网膜病变等类Coats反应疾病。患者中男性14例，女性4例；左眼11只眼，右眼7只眼，均为单眼患病；年龄37~55岁，平均年龄43岁；初诊视力0.02~1.5,视力中值为0.1。对17例17只眼用激光光凝治疗，1例联合视网膜手术放液和冷凝治疗。治疗后随访1.0~15.3年，平均随访3.7年。结果初诊时，18例18只眼中，玻璃体清晰14只眼，仅有少许混浊4只眼。所有患眼视盘正常。病变局限于1~2个象限以内，位于颞侧者为最多（15/18只眼），鼻侧者少（4/18只眼），正上仅有1只眼；有2只眼病变累及2个象限。病变处均有典型的视网膜血管不规则扩张，粟粒状动脉瘤、大动脉瘤、微动脉瘤和毛细血管无灌注区；病变相应区域有黄白色硬性渗出，呈大片块状或成簇的团状。过半数患眼有黄斑水肿或渗出；4只眼有局限性视网膜脱离，其中1只眼视网膜脱离范围较为广泛；3只眼有视网膜出血。治疗后全部患眼眼底渗出和（或）出血吸收，视网膜复位。与治疗前相比, 随访过程中绝大多数(64.3%)患眼视力增进2行或保持在1.2~1.5，无视力减退2行以上者；末诊时视力0.02~1.5,视力中值为0.1。结论成年首次确诊的Coats病患者具有与儿童患者相似的特征性视网膜血管异常和眼底渗出，与之不同的是受累范围较局限，出血少；黄斑受损害轻；随诊过程中病变发展缓慢；视力预后较好。 （中华眼底病杂志，2008，24：279-282）

Clinical characteristics and treatment of Coats' disease in adulthood

Objective To observe the clinical manifestation and treatment effect of Coats' disease in adulthood. Methods The clinical data of 18 adult patients with Coats' disease from 1980 to 2006 at the department of ophthalmology, Peking Union Medical College Hospital which had been diagnosed by ocular fundus examination and fundus fluorescein angiography (FFA), were retrospectively analyzed. The follow-up period was one year. Coats' response diseases in all the patiens were excluded, such as history of radiation therapy, intraocular inflammation, retinal vascular occlusion, age-related macular degeneration (AMD) and more leakage of diabetic retinopathy. The patients, 14 males and 4 females, 11 left eyes and 7 right eyes, all of them occurs in unilateral; aged from 37 to 55 years with the average age of 43 years; the initial diagnostic vision was 0.02 to 1.5, the visual value was 0. 1. 17 patients (17 eyes) were treated by laser photocoagulation, 1 patient was treated by release of retinal operation with condensation. The mean follow up period was 3.7 years (ranged from 1 to 15.3 years). Results In 18 patients (18 eyes), vitreous clear in 14 eyes, vitreous few muddy in 4 years when initial diagnosis. The optic discs in 18 eyes are normal. Lesions confined to one or two quadrant, for the most are in the temporal (15/18 eyes), less are in Bice (4/18 eyes), only one eye at top) two eyes lesions involved in 2 quadrant. There are typical retinal vascular anomalies expansion, miliary aneurysm, arterial aneurysm, macroaneurysms and capillary without perfusion areas; yellow-white hard leakage by a large block or cluster in Lesion corresponding region. More than half of the eyes with macular edema or leakage) limited retinal detachment in 4 eyes, the range was very wide in 1 eye; retinal hemorrhage in 3 eyes. After treatment the exudation or the hemorrhage were absorbed and retinas were reset. Compared the visual acuity with before treatment, mostly (64.3%) improved 2 lines or keep in 1.2-1.5, no one decreased over 2 lines. The last vision was 0.02-1.5, the visual value was 0.1. Conclusions Coats' disease in adulthood diagnosed in first time has similar characteristics with children, such as vascular anomaly of retina, fundus exudation. The differences including limited area of involvement, less hemorrhages, mild damage on macular, slow development of lesions in follow-up period and better visual prognosis.