The detection of COAT’s disease at its early stage and management

R. Shah
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Abstract

Coats disease is the potential leading cause of blindness which is an idiopathic disorder characterized by progressive deposition of intraretinal or subretinal exudates and abnormal development of retinal vasculatures known as telangiectasia that leads to exudative retinal detachment. The case shows unilateral involvement of the eye and primarily at an early age of 12 years. The cause of the disease is idiopathic and if associated with other genetic disorders emphasizes the deposition of a genetic component. The underlying histopathological problem is thought due to the abnormal permeability of capillary endothelial cells of the retina. The complications of long-standing disease may progress to total retinal detachment, leukocoria, painful Glaucoma and can be hard to differentiate from retinoblastoma. The diagnostic tools are indirect ophthalmoscopy, fluorescein angiography, CT scan, and MRI. The treatment depends on the closure of abnormal leaking vessels of the retina which is laser therapy and anti-VEGF at its early stage. The advanced stage requires surgical therapy such as scleral buckling, pars plana vitrectomy, and anterior vitrectomy for reattachment of the retina or pre retinal membrane. A careful selection therapy showed a 70% improvement in the clinical course of the disease
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大衣病的早期发现及治疗
Coats病是一种特发性疾病,其特征是视网膜内或视网膜下渗出物的进行性沉积和视网膜血管的异常发育,即毛细血管扩张,导致渗出性视网膜脱离。该病例显示单侧眼部受累,主要发生在12岁早期。该病的病因是特发性的,如果与其他遗传疾病有关,则强调遗传成分的沉积。潜在的组织病理学问题被认为是由于视网膜毛细血管内皮细胞的异常通透性。长期疾病的并发症可能发展为视网膜完全脱离,白斑,疼痛性青光眼,并且很难与视网膜母细胞瘤区分。诊断工具为间接眼镜检查、荧光素血管造影、CT扫描和MRI。治疗依赖于关闭视网膜的异常渗漏血管,这是激光治疗和早期抗vegf。晚期需要手术治疗,如巩膜扣合、玻璃体切割、前玻璃体切割以重新附着视网膜或视网膜前膜。经过精心选择的治疗方法使临床病程改善了70%
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