Dear Editor Coats’ disease is a nonhereditary condition of primary retinal telangiectasia that is characterized by dilation and tortuosity of retinal blood vessels, multiple aneurysms, and exudates. Coats’ disease is usually unilateral, affects young boys, and causes severe visual loss due to exudative retinal detachment. Routine photocoagulation, cryotherapy, and even surgery may have poor therapeutic effects for total retinal detachment. We report a case of Coats’ disease with total retinal detachment that demonstrated a remarkable response to intravitreal bevacizumab injection. A 3-year-old Chinese boy presented with a 2-year history of left eye squinting. The corneal light reflection test showed a 15° exotropia of the left eye, which could not be corrected. The grating visual acuity of the right eye was 0.2, and the left eye did not respond to stimuli. Examination under anesthesia showed total retinal detachment of the left eye, with extensive subretinal exudates accompanied by dilated tortuous vessels (Fig. 1a), and the intraocular pressures were 14 mmHg bilaterally. Type-B ultrasonography indicated total retinal detachment of the left eye (Fig. 2a). A diagnosis of Coats’ disease stage 3B was made via Shields’ classification [1]. After receiving consent from the boy’s parents for a trial of off-label use of bevacizumab, we performed intravitreal bevacizumab injection (1.25 mg/0.05 ml) three times at 6week intervals. Eight weeks after the last injection, the dilated vessels were alleviated, and the subretinal exudates and fluid were decreased (Fig. 1b). Type-B ultrasonography revealed a significant anatomical improvement of the retina compared with pre-injection, with only a small range of superior retinal detachment. Within 15 weeks after the last injection, the corrected visual acuity of the left eye was 20/ 125 and Type-B ultrasonography showed further resolution of the retinal detachment (Fig. 2b). Diagnosing Coats’ disease is a lengthy process, and there is still confusion about the definition. The most widely accepted diagnostic criteria of Coats’ disease include idiopathic retinal telangiectasia, intraretinal and subretinal exudation, and frequent exudative retinal detachment without vitreous or retinal traction. Coats’ disease has no clear etiopathogenisis, but it is widely accepted that it is related to a defect in the development of the retinal vessels. Vascular endothelial growth factor (VEGF) plays an important role in the development of retinal vessels. Although basic investigations of Coats’ disease are rare, it is believed that VEGF induced by hypoxia may be part of the pathogenesis, considering the evidence of elevated intravitreal VEGF levels of a patient with stage 4 Coats’ disease [2, 3]. The hypothesis suggests anti-VEGF therapies, which have been used in tumors and some neovascular diseases in ophthalmology, as possible therapies for Coats’ disease. Bevacizumab, a full-length humanized recombinant antibody against VEGF, is one of the anti-VEGF agents under consideration. There have been several encouraging reports about treating Coats’ disease with bevacizumab combined with triamcinolone injection or laser treatment [4–6]. To our knowledge, this is the first report of treating total retinal detachment caused by Coats’ disease using Financial support None.
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Jan 1, 2024
Danna A Alvirde-Ayala + 3
Open Access