目的:报告Coats病的异常前段和后段特征的临床过程和处理以及它们与患者年龄的关系,以提高对这些目前文献中很少描述的罕见临床特征的认识。
方法:在意大利锡耶纳大学视网膜母细胞瘤转诊中心和眼科部门对45例Coats病患者的45只眼进行了回顾性描述性回顾,分析了2000年至2022年的数据。修改了医疗记录和图像,以发现某些病例在受Coats疾病影响的患者中表现出异常的前后段特征。因此,我们确定了4种异常的临床状况:视网膜大囊肿,前房胆固醇血症,保留中央凹的Coats疾病和继发性血管增殖性肿瘤。
结果:2例视网膜大囊肿患者(2/45=4.4%),一个前房胆固醇血症(1/45=2.2%),2例保留中央凹的Coats病(2/45=4.4%),1例与血管增生性肿瘤相关(1/45=2.2%),总共6例(6/45=13.3%)患者在Coats病中表现出异常的前段或后段特征。
结论:在年龄较小的儿童中,Coats疾病的异常前段和后段特征如视网膜大囊肿和前房胆固醇病的报道更为频繁,而在年龄较大的患者中,中央凹保留和血管增生性肿瘤的报道更为常见。然后,年龄是强有力的预后标记,其允许区分Coats疾病的两种不同表型:年龄小于和大于3岁的患者,分别具有更积极和更温和的表型。
OBJECTIVE: To report the clinical course and management of unusual anterior and posterior segment features of Coats disease and their relation to the age of the patients to increase the awareness towards these rare clinical features rarely described in the current literature.
METHODS: A retrospective descriptive review of 45 eyes of 45 patients affected by Coats disease was conducted at the Retinoblastoma Referral Center and Ophthalmology Unit of the University of Siena in Italy analyzing data from 2000 to 2022. Medical records and images were revised to find some cases presenting unusual anterior and posterior segment features in patients affected by Coats disease.We identified therefore 4 unusual clinical conditions: retinal macrocysts, anterior chamber cholesterolosis, fovea-sparing Coats disease and secondary vasoproliferative tumor.
RESULTS: Two patients presented with retinal macrocyst (2/45 = 4.4%), one with anterior chamber cholesterolosis (1/45 = 2.2%), two with fovea sparing Coats disease (2/45 = 4.4%) and one with vasoproliferative tumor associated (1/45 = 2.2%) for a total of six (6/45 = 13.3%) patients manifesting unusual anterior or posterior segment features in Coats disease.
CONCLUSIONS: Unusual anterior and posterior segment features of Coats disease such as retinal macrocyst and anterior chamber cholesterolosis have been more frequently reported in younger children while fovea-sparing and vasoproliferative tumors have been more commonly described in older patients. Age is then a strong prognostic marker which allows to distinguish two different phenotypes of Coats disease: patients younger and older than 3 years old with more aggressive and milder phenotype respectively.