Moorfields Eye Hospital, City Road, EC1V2PD, London, UK.
Graefes Arch Clin Exp Ophthalmol. 2010 Dec;248(12):1719-28. doi: 10.1007/s00417-010-1437-3. Epub 2010 Jul 17.
The aim of this study is to image and describe the in vivo choroidal changes in various retinal dystrophies using the technique of enhanced depth imaging (EDI) optical coherence tomography (OCT) and to correlate these findings with the clinical appearance. Associations between choroidal change and genotype, visual acuity and results of retinal electrophysiology are also explored.
Retrospective observational case series.
Twenty patients attending the medical retina clinics at Moorfields Eye Hospital underwent EDI OCT choroidal scans as part of the scanning protocol when they underwent OCT imaging with the Spectralis HRA and OCT. The choroidal images were obtained by moving the Spectralis camera close enough to obtain an inverted image of the retina. The scans were read by two experienced OCT readers assessing the choroidal thickness as well as the choroidal contour for focal areas of choroidal thinning corresponding to the areas of RPE/outer retinal atrophy. The spectrum of patients included those with Stargardt macular dystrophy, macular dystrophies secondary to known mutations such as peripherin/RDS, uncharacterised macular dystrophies, Best disease, bifocal chorioretinal atrophy, Bietti crystalline retinal dystrophy and choroideraemia.
The choroidal appearance was symmetrical in all patients who had both eyes scanned. Ten patients showed no choroidal thinning, five had focal mild to moderate choroidal thinning, three had focal severe choroidal thinning, and two patients had diffuse severe choroidal thinning. There was no association between choroidal thinning and visual acuity [Fisher's exact test, p = 0.350 (right eye), p = 1.000 (left eye)], or extent of retinal dysfunction on electrophysiology (Fisher's exact test, p = 1.000).
Enhanced depth imaging using spectral domain OCT can be used to identify choroidal changes in inherited retinal disease. The pattern of choroidal change correlates well with the clinical appearance. It appears that the extent and pattern of choroidal thinning is dependent on the stage of the disease in some cases, and in others the causative gene defect.
本研究旨在使用增强深度成像（EDI）光学相干断层扫描（OCT）技术对各种视网膜营养不良患者的脉络膜进行成像和描述，并将这些发现与临床表现相关联。还探讨了脉络膜变化与基因型、视力和视网膜电生理结果之间的关系。
回顾性观察性病例系列。
20 名在 Moorfields 眼科医院就诊的视网膜疾病患者在接受 Spectralis HRA 和 OCT 成像时，作为扫描方案的一部分接受了 EDI OCT 脉络膜扫描。通过将 Spectralis 相机移近到足以获得视网膜倒置图像的位置来获取脉络膜图像。两名经验丰富的 OCT 阅读器对脉络膜厚度以及脉络膜轮廓进行评估，以评估与 RPE/外视网膜萎缩区域相对应的局灶性脉络膜变薄区域。患者谱包括斯特格（Stargardt）黄斑营养不良、已知突变引起的黄斑营养不良（如 peripherin/RDS）、特征不明的黄斑营养不良、Best 病、双焦点脉络膜视网膜萎缩、Bietti 结晶状视网膜营养不良和脉络膜痣。
所有接受双眼扫描的患者的脉络膜外观均对称。10 例患者无脉络膜变薄，5 例患者有局灶性轻度至中度脉络膜变薄，3 例患者有局灶性重度脉络膜变薄，2 例患者有弥漫性重度脉络膜变薄。脉络膜变薄与视力之间没有关联（Fisher 确切检验，右眼 p = 0.350，左眼 p = 1.000），或电生理上视网膜功能障碍的严重程度（Fisher 确切检验，p = 1.000）。
使用光谱域 OCT 的增强深度成像可用于识别遗传性视网膜疾病中的脉络膜变化。脉络膜变化的模式与临床表现很好地相关。在某些情况下，脉络膜变薄的程度和模式似乎取决于疾病的阶段，而在其他情况下则取决于致病基因缺陷。
