Pahl Daniel A, Green Nancy S, Bhatia Monica, Lee Margaret T, Chang Jonathan S, Licursi Maureen, Briamonte Courtney, Smilow Elana, Chen Royce W S
Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, Columbia University Medical Center, New York, New York.
Department of Ophthalmology, Columbia University Medical Center, New York, New York.
Am J Ophthalmol. 2017 Nov;183:91-98. doi: 10.1016/j.ajo.2017.08.010. Epub 2017 Aug 30.
Based on standard screening techniques, sickle retinopathy reportedly occurs in 10% of adolescents with sickle cell disease (SCD). We performed a prospective, observational clinical study to determine if ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCT-A) detect more-frequent retinopathy in adolescents with SCD.
Cross-sectional study.
Setting: Institutional.
Sixteen adolescents with SCD, aged 10-19 years (mean age 14.9 years), and 5 age-equivalent controls (mean age 17.4 years).
Examinations including acuity, standard slit-lamp biomicroscopy, UWFA, SD-OCT, and OCT-A were performed.
Sickle retinopathy defined by biomicroscopic changes, Goldberg stages I-V, Penman scale, flow void on OCT-A, or macular thinning on SD-OCT.
While 22 of 32 SCD eyes (68.8%) had retinopathy on biomicroscopy, by UWFA 4 of 24 (16.7%) SCD eyes had peripheral arterial occlusion (Goldberg I), and 20 of 24 eyes (83.3%) had peripheral arteriovenous anastomoses (Goldberg II) in addition. No patients had Goldberg stages III-V. By SD-OCT and OCT-A, thinning of the macula and flow voids in both the superficial and deep retinal capillary plexus were found in 6 of 30 (20%) eyes.
All 24 eyes with adequate UWFA studies demonstrated sickle retinopathy. SD-OCT and OCT-A, which have not been previously reported in the adolescent population, detected abnormal macular thinning and flow abnormalities undetected by biomicroscopy. These findings suggest that pediatric sickle retinopathy may be more prevalent than previously suspected. If these findings are confirmed with larger cross-sectional and prospective analyses, these approaches may enhance early screening for sickle retinopathy.
据报道,基于标准筛查技术,镰状细胞病(SCD)青少年中10%会发生镰状视网膜病变。我们进行了一项前瞻性观察性临床研究,以确定超广角荧光血管造影(UWFA)、光谱域光学相干断层扫描(SD-OCT)和光学相干断层扫描血管造影(OCT-A)是否能检测出SCD青少年中更常见的视网膜病变。
横断面研究。
研究地点:机构研究。
16名年龄在10 - 19岁(平均年龄14.9岁)的SCD青少年,以及5名年龄相仿的对照者(平均年龄17.4岁)。
进行包括视力、标准裂隙灯生物显微镜检查、UWFA、SD-OCT和OCT-A在内的检查。
通过生物显微镜检查变化、戈德堡分期I - V、彭曼量表、OCT-A上的血流缺失或SD-OCT上的黄斑变薄来定义镰状视网膜病变。
在32只SCD眼睛中,22只(68.8%)通过生物显微镜检查发现有视网膜病变,通过UWFA,24只SCD眼睛中有4只(16.7%)出现周边动脉阻塞(戈德堡I期),另外20只眼睛(83.3%)出现周边动静脉吻合(戈德堡II期)。没有患者处于戈德堡III - V期。通过SD-OCT和OCT-A,30只眼睛中有6只(20%)发现黄斑变薄以及视网膜浅层和深层毛细血管丛中的血流缺失。
所有24只进行了充分UWFA检查的眼睛均显示有镰状视网膜病变。SD-OCT和OCT-A在青少年人群中此前未见报道,它们检测到了生物显微镜检查未发现的黄斑异常变薄和血流异常。这些发现表明儿童镰状视网膜病变可能比之前怀疑的更为普遍。如果这些发现通过更大规模的横断面和前瞻性分析得到证实,这些方法可能会加强对镰状视网膜病变的早期筛查。