Reich Michael, Glatz Andreas, Cakir Bertan, Böhringer Daniel, Lang Stefan, Küchlin Sebastian, Joachimsen Lutz, Lagreze Wolf, Agostini Hansjuergen T, Lange Clemens
Eye Center, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
BMJ Open Ophthalmol. 2019 Nov 2;4(1):e000318. doi: 10.1136/bmjophth-2019-000318. eCollection 2019.
To describe vascular changes in different stages of Stargardt disease (STGD) via double swept-source optical coherence tomography angiography.
Prospective, cross-sectional case-control study. Twenty-three patients (45 eyes) with ABCA4 mutations graded according to the Fishman STGD classification and 23 controls (23 eyes) were included. Two independent investigators quantified the foveal avascular zone (FAZ) in the superficial and deep capillary plexus (SCP/DCP) and the areas presenting rarefied flow and complete vascular atrophy in the outer retina to choriocapillaris (ORCC) and choriocapillaris (CC) slab.
The mean age at first diagnosis of STGD was 24.0 years (range 9-50) and 37.9 years (range 18-74) at the time of examination. Eleven patients were assigned to the Fishman STGD classification stage (S) 1, three to S2, eight to S3 and one to S4. The FAZ in SCP and DCP was increased in all stages compared with controls (p<0.01). Areas with rarefied flow signal and vascular atrophy were detected in the ORCC and the CC layer and grew with increasing stage of disease (p<0.01). The duration of disease correlated with the extent of the enlarged FAZ in the SCP/DCP and with the area of reduced flow in the ORCC and CC layer (p<0.01). Best corrected visual acuity correlated negatively with the extent of the enlarged FAZ in the SCP/DCP (p<0.0001), as well as with enlarged atrophic area in the ORCC and CC layer (p=0.026 and p=0.074).
Patients with STGD reveal vascular changes in the retina and CC in all disease stages. The avascular zone in the SCP/DCP and areas with rarefied flow signal in the ORCC/CC increase with the duration and stage of disease, indicating progressive vascular decay most likely secondary to retinal pigment epithelium and neuronal loss. Furthermore, increased vascular damage is associated with decreased vision.
通过双扫频源光学相干断层扫描血管造影描述斯塔加特病（STGD）不同阶段的血管变化。
前瞻性横断面病例对照研究。纳入23例ABCA4基因突变患者（45只眼），根据Fishman STGD分类进行分级，以及23名对照者（23只眼）。两名独立研究者对浅表和深层毛细血管丛（SCP/DCP）中的黄斑无血管区（FAZ）以及外视网膜至脉络膜毛细血管层（ORCC）和脉络膜毛细血管层（CC）板层中呈现血流稀疏和完全血管萎缩的区域进行量化。
STGD首次诊断时的平均年龄为24.0岁（范围9 - 50岁），检查时为37.9岁（范围18 - 74岁）。11例患者被归入Fishman STGD分类的1期（S1），3例为S2期，8例为S3期，1例为S4期。与对照组相比，所有阶段SCP和DCP中的FAZ均增大（p<0.01）。在ORCC和CC层检测到血流信号稀疏和血管萎缩区域，且随疾病阶段增加而扩大（p<0.01）。病程与SCP/DCP中扩大的FAZ范围以及ORCC和CC层血流减少区域相关（p<0.01）。最佳矫正视力与SCP/DCP中扩大的FAZ范围呈负相关（p<0.0001），也与ORCC和CC层扩大的萎缩区域相关（p = 0.026和p = 0.074）。
STGD患者在所有疾病阶段视网膜和CC均有血管变化。SCP/DCP中的无血管区以及ORCC/CC中血流信号稀疏区域随病程和疾病阶段增加，表明血管渐进性衰退，很可能继发于视网膜色素上皮和神经元丢失。此外，血管损伤增加与视力下降相关。
