Department of Ophthalmology, Institute of Health Biosciences, The University of Tokushima Graduate School, 3-18-15, Kuramoto-cho, Tokushima, 770-8503, Japan.
Department of Ophthalmology, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima, 890-8520, Japan.
BMC Ophthalmol. 2015 Mar 7;15:19. doi: 10.1186/s12886-015-0014-2.
To report a thicker choroid and larger choroidal luminal area in an eye with Wyburn-Mason syndrome. To the best of our knowledge, this is the first report demonstrating an increase in the choroidal thickness and the luminal area in a case of Wyburn-Mason syndrome. In addition, we report the changing appearance of retinal arteriovenous malformations over a 16-year period.
A 27-year-old woman, who was diagnosed with Wyburn-Mason syndrome at age 11 years, visited our clinic. Her best-corrected visual acuity was 20/12.5 in the right eye and light perception in the left eye. Severely dilated, tortuous vascular loops were distributed from the optic disc over all four quadrants of the left fundus. The vascular loops in some areas were more dilated and tortuous than 16 years earlier. Optical coherence tomography (OCT) showed retinal edema with cystic changes and enlarged choroidal vessel lumens in the left eye. The subfoveal choroidal thickness was manually measured by the caliper function in the enhanced depth imaging OCT (EDI-OCT) images. Binarization of the EDI-OCT images was performed with publicly accessible ImageJ software. The examined area of the subfoveal choroid was 1,500 μm wide, and the dark areas representing the luminal areas were traced by the Niblack method. After determining the distance of each pixel, the luminal area was automatically calculated. The subfoveal choroidal thickness was 250 μm in the right eye and 462 μm in the left eye. The luminal area of the 1,500-μm-wide subfoveal choroid was computed to be 307,165.6 μm(2) in the right eye and 545,780.7 μm(2) in the left eye.
The EDI-OCT images showed a thicker choroid, and binarization of the EDI-OCT images showed that the luminal areas were significantly larger in the affected eye, suggesting a dilatation of the choroidal vessels. The results demonstrated that conversion of EDI-OCT images to binary images was a useful method to quantify the choroidal structure.
报告一例 Wyburn-Mason 综合征患者的脉络膜增厚和脉络膜管腔面积增大。据我们所知,这是首例报告显示 Wyburn-Mason 综合征患者脉络膜厚度和管腔面积增加的病例。此外,我们还报告了 16 年来视网膜动静脉畸形外观变化的情况。
一名 27 岁女性,11 岁时被诊断为 Wyburn-Mason 综合征,来我院就诊。她右眼最佳矫正视力为 20/12.5,左眼光感。左眼视盘周围的四个象限均分布着严重扩张、扭曲的血管环。一些区域的血管环比 16 年前更扩张和扭曲。光学相干断层扫描(OCT)显示左眼视网膜水肿伴囊样改变和脉络膜大血管管腔增大。左眼中心凹下脉络膜厚度通过增强深度成像 OCT(EDI-OCT)图像的卡尺功能手动测量。EDI-OCT 图像采用公共可用的 ImageJ 软件进行二值化。用 Niblack 方法追踪代表管腔区域的暗区来检测 1500μm 宽的中心凹下脉络膜的受检区域。确定每个像素的距离后,自动计算管腔面积。右眼中心凹下脉络膜厚度为 250μm,左眼为 462μm。计算出 1500μm 宽的中心凹下脉络膜的管腔面积,右眼为 307165.6μm(2),左眼为 545780.7μm(2)。
EDI-OCT 图像显示脉络膜增厚,EDI-OCT 图像的二值化显示患眼的管腔面积明显增大,提示脉络膜血管扩张。结果表明,将 EDI-OCT 图像转换为二值图像是一种量化脉络膜结构的有用方法。