Coats’’ disease : classification and current treatment

Purpose Coats’ disease is characterized by idiopathic telangiectasia with lipid exudates and retinal ischemia. Advanced cases develop total retinal detachment (RD)(stage 3B) and neovascular glaucoma (stage 4), resulting in enucleation in 7% and 78% of cases respectively. Methods Nine consecutive cases of Coats’ disease stage 3B (n=5) and 4 (n=4) received 1 or more intravitreal injections of 0.5 ml ranibizumab. In 3 cases, subretinal fluid drainage by sclerotomy was performed. Mean age at diagnosis was 17.9 months (range 1‐38). At the end of treatment, ERG was recorded (n=4) and amblyopia treatment was attempted in 6 patients. Mean follow‐up was 42.9 months. Results Rubeosis disappeared during the first week with the retina reattaching within 4 months, rendering pathological vessels and ischemic retina accessible to conventional therapy. A single injection was sufficient in 6 cases, 2 injections were necessary in 1 patient and another needed 4. One patient developed 10 months after complete retinal reattachment a severe fibrous vitreo‐retinopathy evolving to subtotal tractional RD. Amblyopic treatment was performed in 4 patients and restored measurable visual acuity in two patients. Scotopic ERG was unrecordable with a severely reduced photopic b wave in 3 patients. The last patient had a reduced scotopic b wave (50%) and a normal photopic ERG with slightly increased implicit time. Conclusion There is an increasing interest in anti‐VEGF therapy in Coats' disease. To our knowledge, this is the largest series of stage 4 and 3B Coats' disease treated by ranibizumab, demonstrating that it is a well‐tolerated and safe therapy in young children with advanced disease. Ranibizumab facilitates the management by suppressing rubeosis in stage 4 and decreasing exudation.