Broadhead G K, Grigg J R, McCluskey P, Korsakova M, Chang A A
Save Sight Institute, The University of Sydney, Sydney, NSW, Australia.
Sydney Institute for Vision Science, Sydney, NSW, Australia.
Doc Ophthalmol. 2016 Oct;133(2):139-143. doi: 10.1007/s10633-016-9555-9. Epub 2016 Jul 16.
We present a case of enhanced S-cone syndrome (ESCS)-associated choroidal neovascularisation (CNV) treated successfully with intravitreal bevacizumab therapy.
METHODS/CASE REPORT: A 14-year-old with a known retinal dystrophy presented with acute visual deterioration. Fluorescein angiography demonstrated CNV, and treatment was initiated with an anti-vascular endothelial growth factor (anti-VEGF) agent, with significant improvement in vision. Subsequent electroretinogram examination of the patient and her younger sister showed severely reduced rod responses with accentuated fast cone (S-cone only) response, confirming the diagnosis of ESCS as the underlying dystrophy.
CNV is a rare complication of ESCS that is responsive to anti-VEGF therapy. Although cystic retinal lesions may develop in patients with retinal dystrophies due to multiple possible aetiologies, CNV is a known cause of macula oedema in these patients that requires treatment with different agents, namely anti-VEGF therapy. Rapid visual loss in patients with inherited retinal disorders should prompt immediate clinical assessment to exclude CNV, and if CNV is detected, anti-VEGF therapy can preserve vision.
我们报告一例通过玻璃体内注射贝伐单抗成功治疗的与增强型S锥体综合征（ESCS）相关的脉络膜新生血管（CNV）病例。
方法/病例报告：一名患有已知视网膜营养不良的14岁患者出现急性视力下降。荧光素血管造影显示有CNV，遂开始使用抗血管内皮生长因子（抗VEGF）药物进行治疗，视力有显著改善。随后对该患者及其妹妹进行的视网膜电图检查显示，视杆细胞反应严重降低，快速视锥细胞（仅S视锥细胞）反应增强，从而确诊潜在的营养不良为ESCS。
CNV是ESCS的一种罕见并发症，对抗VEGF治疗有反应。尽管由于多种可能的病因，视网膜营养不良患者可能会出现视网膜囊性病变，但CNV是这些患者黄斑水肿的已知病因，需要使用不同药物进行治疗，即抗VEGF治疗。遗传性视网膜疾病患者的快速视力丧失应促使立即进行临床评估以排除CNV，如果检测到CNV，抗VEGF治疗可以保留视力。
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