Prevalence of cystic macular lesions in patients with Usher II syndrome

Prevalence of cystic macular lesions in patients with Usher II syndrome
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DOI:
10.1038/eye.2008.105
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发表时间:
2009-05-01
期刊:
EYE
影响因子:
3.9
通讯作者:
Hajali, M.
Hajali, M.
中科院分区:
医学3区
文献类型:
--
作者:
Walia, S.;Fishman, G. A.;Hajali, M.

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目的评估Usher II综合征患者黄斑囊状病变的患病率。方法收集2002年1月至2007年12月在芝加哥伊利诺伊大学遗传性眼病门诊就诊的Usher II型患者76例。每位参与患者均接受详细的临床检查,包括最佳矫正视力、裂隙灯生物显微镜检查和散瞳眼底检查。结果76例患者中有19例(25%)至少有1只眼出现黄斑囊样改变,13例(13只眼)以OCT为基础,5例使用FFA(2例仅使用FFA,3例基于临床记录和FFA结果),1例仅基于临床记录。在18例CME患者中,通过OCT或FFA确定,5例(27.8%)显示眼底镜下正常外观黄斑(n = 4)或萎缩性外观黄斑变化(n = 1)。结论我们的Usher II患者总队列的四分之一有囊性黄斑病变。此外,在OCT和/或FFA检测的18例囊性黄斑病变患者中,22%(n = 4)的眼底镜下观察到正常黄斑。基于我们队列中囊性黄斑病变的合理高患病率,评估Usher II患者是否存在囊样黄斑水肿似乎是谨慎的。Eye(2009)23,1206-1209; doi:10.1038/eye.2008.105; 2008年4月18日在线发表
Purpose To evaluate the prevalence of cystic macular lesions in patients with Usher II syndrome.Methods All Usher type II patients seen in the inherited eye disease clinic at the University of Illinois at Chicago between January 2002 and December 2007 were included (n = 76). Each participating patient underwent a detailed clinical examination, including best-corrected visual acuity, slit-lamp biomicroscopy and dilated fundus examination. The presence of cystoid lesions was determined by optical coherence tomography (OCT), fundus fluorescein angiogram (FFA), fundus photographs and/or clinical examination.Results A cystic-appearing macular change was observed in at least one eye in 19 out of the 76 patients (25%), 13 on the basis of OCT, five using FFA (two solely with the use of FFA and three based on clinical notes and FFA findings) and one based solely on clinical notes. Of the 18 patients with CME, determined by OCT or FFA, five (27.8%) showed either a funduscopically normal-appearing macula (n = 4) or an atrophic appearing macular change (n = 1).Conclusions One-fourth of our total cohort of Usher II patients had cystic macular lesions. Moreover, a funduscopically normal-appearing macula was observed in 22% (n = 4) of our 18 patients with cystic-appearing macular lesions on OCT and/or FFA testing. On the basis of the reasonably high prevalence of cystic macular lesions in our cohort, it would seem prudent to evaluate Usher II patients for the presence of cystoid macular oedema. Eye (2009) 23, 1206-1209; doi:10.1038/eye.2008.105; published online 18 April 2008