目的:纵向评估镰状细胞病(SCD)儿童的黄斑厚度和微血管变化。
方法:回顾性连续系列。
方法:1998年1月至2022年8月在波士顿儿童医院进行眼科检查的年龄在18岁或18岁以下的SCD儿童。
方法:对光学相干断层扫描(OCT)和OCT血管造影(OCTA)图像进行定性和定量分析。
方法:在黄斑OCT上测量的总视网膜厚度,以及浅表毛细血管丛(SCP)和深毛细血管丛(DCP)血管密度(VD),和在6x6mmOCTA扫描上测得的中央凹无血管区(FAZ)面积。
结果:ICD-10代码搜索确定了303名在研究期间接受眼科检查的小儿SCD患者。在就诊时采集104只(17.2%)和60只(9.9%)眼睛的OCT和OCTA图像,在最后一次就诊时采集159只(26.2%)和100只(16.5%)眼睛的OCT和OCTA图像。总的来说,有35.6%的SCD患者在就诊时和39.6%的患者在最后一次就诊时定性地发现颞部视网膜变薄.在那些黄斑变薄的患者中,94.6%和90.5%在就诊和最后一次就诊时患有外周镰状细胞视网膜病变(SCR)。在定量OCT分析中,与HbSC相比,HbSS眼的中央凹和颞侧副凹的视网膜厚度较低,P<0.05。与没有周边SCR的眼睛相比,周边SCR的眼睛在表现时具有更大的FAZ(P=0.004),在颞下黄斑最后一次就诊时DCPVD较低(P=0.03),最后一次访视时,上鼻黄斑的DCPVD较高(P=0.01)。40名患者的80只眼具有OCT,20名患者的34只眼具有在初始和最终访问时获得的OCT和OCTA图像。在最后一次访问中,视网膜厚度在中央凹减少,下中央凹,与演示相比,P<0.05。并行,最终访视鼻上象限的VDDCP增加,P=0.03。
结论:黄斑视网膜变薄是进行性的,在有和没有周边SCR的眼中都观察到。随着时间的推移,OCTA的鼻黄斑中DCPVD有代偿性增加。
OBJECTIVE: To longitudinally assess macular thickness and microvascular changes in children with sickle cell disease (SCD).
METHODS: A retrospective consecutive series.
METHODS: Children with SCD aged ≤ 18 years who had an ophthalmic examination at Boston Children\'s Hospital between January 1998 and August 2022.
METHODS: Qualitative and quantitative analyses of both OCT and OCT angiography (OCTA) images were performed.
METHODS: Total retinal thickness measured on macular OCT, superficial capillary plexus and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) area measured on 6- × 6-mm OCTA scans.
RESULTS: International Classification of Diseases, 10th Revision, code search identified 303 pediatric SCD patients who underwent ophthalmic examination during the study period. OCT and OCTA images were acquired on 104 (17.2%) and 60 (9.9%) eyes at presentation and on 159 (26.2%) and 100 (16.5%) eyes at final visit, respectively. Overall, temporal retinal thinning was noted qualitatively in 35.6% of SCD patients at presentation and 39.6% at final visit. Of those patients with macular thinning, 94.6% and 90.5% had peripheral sickle cell retinopathy (SCR) at presentation and final visit. On quantitative OCT analysis, HbSS eyes had a lower retinal thickness in the fovea and temporal parafovea compared with HbSC (P < 0.05). Eyes with peripheral SCR had a larger FAZ at presentation compared with eyes without peripheral SCR (P = 0.004), a lower DCP VD at final visit in the inferior temporal macula (P = 0.03), and a higher DCP VD at final visit in the superior nasal macula (P = 0.01). Eighty eyes of 40 patients had OCT, and 34 eyes of 20 patients had both OCT and OCTA images acquired at both initial and final visits. At final visit, retinal thickness decreased at the fovea, inferior perifovea, and temporal perifovea compared with presentation (P < 0.05). In parallel, VD DCP in the superonasal quadrant increased at final visit (P = 0.03).
CONCLUSIONS: Macular retinal thinning was progressive and observed in eyes with and without peripheral SCR. Over time, there was a compensatory increase in DCP VD in the nasal macula on OCTA.
BACKGROUND: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.