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Optical coherence tomography (OCT) features of cystoid spaces in choroideremia (CHM).
Graefe's Archive for Clinical and Experimental Ophthalmology ( IF 2.7 ) Pub Date : 2019-10-28 , DOI: 10.1007/s00417-019-04508-7
Vittoria Murro 1 , Dario Pasquale Mucciolo 1 , Dario Giorgio 1 , Andrea Sodi 1 , Ilaria Passerini 2 , Giacomo Bacci 3 , Sara Bargiacchi 4 , Gianni Virgili 1 , Stanislao Rizzo 1
Affiliation  

PURPOSE To investigate the prevalence and features of cystoid spaces (CS) in patients with confirmed genetic diagnosis of choroideremia (CHM) using swept source optical coherence tomography (OCT). METHODS We retrospectively reviewed CHM patients examined at the Regional Reference Center for Hereditary Retinal Degenerations at the Eye Clinic in Florence. We took into consideration genetically confirmed CHM patients with ophthalmological and swept source optical coherence tomography (OCT) examinations. The presence/absence and location of cystoid spaces in the retina of each eye were reported. RESULTS A total of 42 eyes of 21 CHM patients were included in our series. The average age of the patients was 36.5 ± 20.1 (range, 13-73 years). The average best-corrected visual acuity (BCVA) for all patients was 0.63 ± 1.00 logMar (range, 0-2,80). CS were present in 15 eyes of eight patients (8/21, 38%). In all cases, CS were located in inner nuclear layer (INL); in five eyes of three patients, CS were detected also in ganglion cell layer (GCL). CS appeared as microcistoyd abnormalities and were detected in retinal areas characterized by retinal pigment epithelium (RPE) and outer retinal layers atrophy at the transition zone. CONCLUSIONS Cystoid spaces in choroideremia showed peculiar features; they are clusters of small-size extrafoveal degenerative cysts mainly located in inner nuclear layer at the transition zone where outer retinal layers and RPE are severely damaged.

中文翻译:

脉络膜血友病(CHM)中囊样空间的光学相干断层扫描(OCT)特征。

目的通过扫描源光学相干断层扫描(OCT)研究确诊为脉络膜血症(CHM)的遗传诊断患者的囊状间隙(CS)的患病率和特征。方法我们回顾性分析了在佛罗伦萨眼科诊所遗传性视网膜变性区域参考中心检查的CHM患者。我们考虑了经过眼科和扫频光学相干断层扫描(OCT)检查的基因确诊CHM患者。报道了每只眼睛的视网膜中是否存在和存在囊状间隙。结果本研究共纳入21例CHM患者的42眼。患者的平均年龄为36.5±20.1(范围13-73岁)。所有患者的平均最佳矫正视力(BCVA)为0.63±1.00 logMar(范围0-2,80)。CS出现在8例患者的15眼中(8/21,38%)。在所有情况下,CS都位于内核层(INL)中。在三名患者的五只眼中,在神经节细胞层(GCL)中也检测到CS。CS表现为微胞胎异常,并在以视网膜色素上皮(RPE)和过渡区视网膜外层萎缩为特征的视网膜区域中检测到。结论脉络膜血肿中的囊性间隙表现出独特的特征。它们是小凹小凹退行性囊肿的簇,主要位于过渡区的内核层,视网膜外层和RPE受到严重破坏。CS表现为微胞胎异常,并在以视网膜色素上皮(RPE)和过渡区视网膜外层萎缩为特征的视网膜区域中检测到。结论脉络膜血肿中的囊性间隙表现出独特的特征。它们是小凹小凹退行性囊肿的簇,主要位于过渡区的内核层,视网膜外层和RPE受到严重破坏。CS表现为微鞘细胞异常,并在以视网膜色素上皮(RPE)和过渡区视网膜外层萎缩为特征的视网膜区域中检测到。结论脉络膜血肿中的囊性间隙表现出独特的特征。它们是小凹小凹退行性囊肿的簇,主要位于过渡区的内核层,视网膜外层和RPE受到严重破坏。
更新日期:2019-10-26
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