Shields J A, Shields C L, Honavar S G, Demirci H
Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.
Am J Ophthalmol. 2001 May;131(5):561-71. doi: 10.1016/s0002-9394(00)00883-7.
The purpose of this report is to review the clinical variations and natural course of Coats disease, using strict diagnostic guidelines.
In a retrospective, consecutive series, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with the clinical features, complications, and diagnostic approaches to Coats disease.
In 150 consecutive patients (158 eyes), Coats disease was diagnosed at a median age of 5 years (range, 1 month to 63 years), occurred in 114 males (76%), and was unilateral in 142 patients (95%). There was no predilection for race or laterality. The most common referral diagnoses were Coats disease in 64 cases (41%) and retinoblastoma in 43 (27%). The first symptom or sign was decreased visual acuity in 68 cases (34%), strabismus in 37 (23%), leukocoria in 31 (20%), and 13 patients (8%) were asymptomatic. Visual acuity at presentation was 20/200 to no light perception in 121 eyes (76%). The anterior segment was normal in 142 eyes (90%). The retinal telangiectasia involved the midperipheral or peripheral fundus in 156 of the 158 eyes (99%) and was restricted to the macular area in two eyes (1%); involved mainly the temporal fundus in 66 eyes (42%), inferior fundus in 41 eyes (26%), and more than one sector in 34 eyes (22%). Retinal exudation was present in all 12 clock hours in 86 eyes (55%) and six or more clock hours in 115 eyes (73%). There was a total retinal detachment in 74 eyes (47%) and neovascular glaucoma in 12 (8%). Retinal macrocysts were present in 18 eyes (11%), a vasoproliferative tumor in nine eyes (6%) and retinal neovascularization in four eyes (3%). Fluorescein angiography in 49 of the 158 eyes (37%) disclosed early hyperfluorescence of the telangiectasias and macular edema in 18 of eyes (37%). Ultrasonography typically showed a retinal detachment but no solid mass.
Coats disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation. It is usually unilateral, occurs mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment. The clinician should follow strict criteria in making the diagnosis, to avoid confusing Coats disease with other forms of exudative retinopathy.
本报告旨在使用严格的诊断标准，回顾Coats病的临床变异和自然病程。
在一项回顾性连续系列研究中，Coats病被定义为特发性视网膜毛细血管扩张症，伴有视网膜内或视网膜下渗出，且无明显的视网膜或玻璃体牵拉迹象。我们回顾了我们在Coats病的临床特征、并发症和诊断方法方面的经验。
在150例连续患者（158只眼）中，Coats病的诊断中位年龄为5岁（范围1个月至63岁），114例为男性（76%），142例患者（95%）为单眼发病。该病无种族或偏侧性倾向。最常见的转诊诊断为Coats病64例（41%），视网膜母细胞瘤43例（27%）。首发症状或体征为视力下降68例（34%），斜视37例（23%），白瞳症31例（20%），13例患者（8%）无症状。就诊时视力为20/200至无光感的有121只眼（76%）。142只眼（90%）眼前节正常。158只眼中156只眼（99%）的视网膜毛细血管扩张累及中周部或周边眼底，2只眼（1%）局限于黄斑区；主要累及颞侧眼底66只眼（42%），下方眼底41只眼（26%），累及一个以上象限34只眼（22%）。86只眼（55%）的视网膜渗出累及所有12个钟点，115只眼（73%）累及6个或更多钟点。74只眼（47%）发生完全性视网膜脱离，12只眼（8%）发生新生血管性青光眼。18只眼（11%）存在视网膜大囊肿，9只眼（6%）存在血管增生性肿瘤，4只眼（3%）存在视网膜新生血管。158只眼中49只眼（37%）的荧光素血管造影显示毛细血管扩张早期高荧光，18只眼（37%）存在黄斑水肿。超声检查通常显示视网膜脱离，但无实性肿块。
Coats病是一种独特的临床实体，其特征为特发性视网膜毛细血管扩张和视网膜渗出。它通常为单眼发病，多见于年轻男性，可因渗出性视网膜脱离导致严重视力丧失。临床医生在做出诊断时应遵循严格标准，以避免将Coats病与其他形式的渗出性视网膜病变相混淆。
