Intravitreal bevacizumab (Avastin®) in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis

Abstract

Editor, First characterized by Chang et al. (1995), idiopathic retinitis, vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is defined by the association of peripheral retinal vascular occlusion, retinal vasculitis and multiple posterior retinal arterial aneurysms. The severity of the disease results from retinal ischaemia as much as neovascular complications; the consensual treatment is panretinal photocoagulation (PRP) started as quickly as possible (McDonald 2003; Samuel et al. 2007). We report a patient treated with bevacizumab (Avastin®) for a neovascular form of IRVAN syndrome. A 60-year-old woman was referred as an emergency to the XV–XX National Hospital Centre in Paris for sudden vision loss of her right eye. Her best-corrected visual acuity (BCVA) was hand movements in the right eye and 20/20 in the left eye. Ocular examination revealed profuse bilateral rubeosis iridis with an open angle and normal intraocular pressure, total vitreous haemorrhage in the right eye and epipapillary neovascularization in the left eye (Fig. 1). According to the new classification proposed by Samuel, she was classified as stage 4 (Samuel et al. 2007). (A) Gonioscopic view of rubeosis, (B) rubeosis iridis and (C, D) marked regression of rubeosis iridis. On fluorescein angiography, we noted multiple aneurysms, arterial vascular occlusion, late diffuse staining of the optic nerve head and diffuse retinal ischaemic areas in the right eye (Fig. 2). (A) Epipapillary neovascularization, (B) inferotemporal and superonasal aneurysm, ischaemia and temporal arterial occlusion, and (C) regression of neovascularization, panretinal photocoagulation. Systemic examination and laboratory findings ruled out any systemic abnormality. The patient was treated by bilateral intravitreal injection (IVT) of bevacizumab (1.25 mg, 0.05 ml) with 24 hr delay. Detailed informed consent was obtained from the patient. PRP was begun in the left eye; she underwent a pars plana vitrectomy with endolaser photocoagulation in the right eye three days later. Five days after IVT, we noticed the total regression of the rubeosis iridis (Fig. 1) and regression of the epipapillary neovascular vessel. The injection was performed following the standard IVT protocol. Fluorescein angiography carried out 2 months later revealed total regression of retinal neovascularization, complete PRP (Fig. 2). No side-effects were noted. At 6-month follow-up, final visual acuity was 20/25 in the left eye and 20/80 in the right eye. This was explained by a corticonuclear cataract and persistent macular oedema with exudation from an aneurism. Intravitreal injection of bevacizumab has been effective in treating retinal neovascularization and bilateral rubeosis iridis in our patient and others described in the literature (Avery 2006; Avery et al. 2006; Jiang et al. 2008). It has also been used to treat choroidal neovascularization in posterior uveitis such as toxoplasmosis, lupus or sarcoidosis (Guthoff & Goebel 2008; Kurup et al. 2009). As a result of ischaemic sequelae, and despite usual treatment, the poor visual outcome reported in the literature at this level of severity has motivated the use of anti-vascular endothelial growth factor (VEGF) to stop the predictable evolution to neovascular glaucoma or tractional retinal detachment and definitive visual loss. Theoretically, the focal nature of the pathology in this condition is similar to that found in retinal neovascularization associated with diabetes. Anti-VEGF IVT seems to be a new approach in the treatment of all kinds of pathology where a high level of VEGF is produced secondary to large retinal ischaemic areas (Caldwell et al. 2003). Therefore, the favourable results of IVT of anti-VEGF for age-related macular degeneration or diabetes (Avery et al. 2006) may be helpful in deciding whether treatment is indicated for a particular patient with neovascularization associated with IRVAN (stages 3, 4 and 5). As in combined treatment for high-risk proliferative diabetic retinopathy (Tonello et al. 2008), we treated this patient using PRP and bevacizumab successfully. Although PRP remains the gold-standard treatment of IRVAN, intravitreal injection of anti-VEGF could be an option to consider when an IRVAN is discovered at an advanced stage as rubeosis iridis or neovascular glaucoma. Further study with a larger group of patients is required.