Coats-like Exudative Vitreoretinopathy in Retinitis Pigmentosa

Multimodal treatment of Coats-like exudative vitreoretinopathy in Goldmann-Favre syndrome

“…The etiological and pathophysiologic mechanisms that lead to a Coats-like exudative vitreoretinopathy in various retinal dystrophies remains unclear. 12 Early theories of the origin of the exudative vasculopathy include primary endothelial cell dysfunction with a secondary increase in vascular permeability. 13 Other secondary changes include RPE dysfunction, subretinal fluid accumulation, chronic inflammation, hypoxia and neovascularization.…”

“…13 , 19 Even though early intervention may decrease the degree of vision loss, there is limited evidence of complete disease resolution post treatment. 12 …”

“…Laser photocoagulation has been reported as the most effective alternative by some authors. 12 , 26 , 27 , 28 , 29 However, no randomized prospective studies evaluating treatment for Coats-like exudative vitreoretinopathy has been performed. Intravitreal anti-vascular endothelial growth factors (anti-VEGF) agents have been recently utilized as adjuvant treatment to modulate the acute exudative response seen after primary therapy.…”

Coats-like exudative vitreoretinopathy (CLEVER) in CEP290 inherited retinal degeneration

“…1 7 It may be noted synchronously with RP diagnosis (33%) or later in the disease course. 1 The retinal telangiectasias and subretinal exudation are typically inferotemporal. 1 CLEVER may be detected earlier (ie, presymptomatically) in patients undergoing clinical review for existing conditions (ie, RCD) than in isolated Coats disease, thus, CLEVER may have a more favourable prognosis due to earlier initiation of intervention.…”

“…1 The retinal telangiectasias and subretinal exudation are typically inferotemporal. 1 CLEVER may be detected earlier (ie, presymptomatically) in patients undergoing clinical review for existing conditions (ie, RCD) than in isolated Coats disease, thus, CLEVER may have a more favourable prognosis due to earlier initiation of intervention. FFA may be beneficial in confirming diagnosis and guiding treatment which relies on laser photocoagulation or cryotherapy of leaking telangiectatic vessels with response rates similar to cases of isolated Coats disease.…”