Foveal Hypoplasia in Patients with Stickler Syndrome
Foveal Hypoplasia in Patients with Stickler Syndrome
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DOI：
10.1016/j.ophtha.2017.01.046
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发表时间：
2017-06-01
期刊：
影响因子：
13.7
通讯作者：
Kondo, Hiroyuki
中科院分区：
文献类型：
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作者：
Matsushita, Itsuka;Nagata, Tatsuo;Kondo, Hiroyuki
目的：应用光谱域光学相干断层扫描(SD-OCT)和扫描源光学相干断层扫描(SCOCT)技术对Stickler综合征患者的黄斑中心凹微结构进行研究。设计：回顾性病例系列研究。研究对象：研究对象：Stickler综合征患者25例39眼。对OCT断层图像、OCT血管造影(OCTA)和面部OCT图像进行评估。测定中心凹内视网膜层厚度(FIRL)与中心凹旁内层厚度(PIRL)之比、中心凹外视网膜外层(ForL)与中心凹外视网膜外层(POIL)厚度之比、中心凹无血管区(FAZ)的大小。主要观察指标：Stickler综合征患者的黄斑中心凹发育不良程度及最佳矫正视力。光学相干断层血管成像显示17例(25眼)OCTA患者的FAZ小于正常眼，范围为0~0.19 mm(2)。视力与FIRL/PIRL比值无明显相关性。结论：Stickler综合征患者的特点是黄斑中心凹轻度发育不良并持续存在IRL。视力与FIRL/pIRL比值无相关性。(C)2017年度美国眼科学会
Purpose: To determine the microstructure of the fovea in patients with Stickler syndrome using imaging by spectral-domain optical coherence tomography (SD OCT) and swept-source OCT.Design: Retrospective case series study.Participants: A total of 39 eyes of 25 patients with genetically confirmed Stickler syndrome were studied.Methods: All of the patients had mutations in the COL2A1 gene and were diagnosed with Stickler syndrome. Cross-sectional OCT images, OCT angiography (OCTA), and en face OCT images were assessed. The ratio of the foveal inner retinal layer (fIRL) thickness to the parafoveal inner retinal layer (pIRL) thickness, the ratio of the foveal outer retinal layer (fORL) thickness to the parafoveal outer retinal layer (pORL) thickness, and the size of the foveal avascular zone (FAZ) were determined.Main Outcome Measures: The degree of foveal hypoplasia and the best-corrected visual acuity in patients with Stickler syndrome.Results: A persistence of the inner retinal layers in the fovea with an fIRL/pIRL ratio >0.2 was present in 32 of the 39 eyes (82%). Optical coherence tomography angiography showed that the FAZ was smaller, 0 to 0.19 mm(2), than that of normal eyes, in 25 eyes of 17 patients who underwent OCTA. There was no significant correlation between the visual acuities and the fIRL/pIRL ratios.Conclusions: A mild foveal hypoplasia with a persistence of the IRL is characteristic of eyes with Stickler syndrome. The visual acuities were not correlated with the fIRL/pIRL ratios. (C) 2017 by the American Academy of Ophthalmology