Intravitreal ranibizumab as primary treatment for neovascular membrane associated with angioid streaks

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Editor, Angioid streaks (AS) represent calcified disruptions of Bruch’s membrane and may result in the development of choroidal neovascularization (CNV) with an estimated prevalence of between 72% and 86% (Bhatnagar et al. 2007). The natural history of CNV associated with AS has a poor prognosis. Although various treatments have been assessed, including argon laser photocoagulation, transpupillary thermotherapy, photodynamic therapy and surgical removal, poor or contradictory results are often reported. In recent years, several studies have reported promising results with off-label use of the intravitreal anti-vascular endothelial growth factor-A (VEGF-A) drug bevacizumab (Avastin™; Genentech, South San Francisco, California, USA) for the treatment of CNV related to AS (Bhatnagar et al. 2007; Apte 2008; Derriman et al. 2008; Neri et al. 2009; Wiegand et al. 2009). It has been hypothesized that ranibizumab (Lucentis®; Genentech), which is an approved anti-VEGF treatment for CNV related to age-related macular degeneration (AMD), would offer an outcome at least similar to that of bevacizumab (Derriman et al. 2008). Herein, we report a case of juxtafoveal CNV caused by AS treated successfully with intravitreal ranibizumab. A 70-year-old woman referred from abroad to the retina department of Jules Gonin University Eye Hospital with a 1-month history of visual distortion affecting her left eye. Her ocular history was significant for CNV in the right eye with rapid progression, status post-treatment with intravitreal injection of bevacizumab. Visual acuity was 20/200 in the right eye and 20/20 in the left eye. Fundus examination showed angioid streaks with a grey lesion in both eyes, subretinal haemorrhage and subsensory fluid nasally to the fovea consistent with CNV in the left eye and a disciform scar surrounded by retinal pigment epithelium atrophy in the right eye. Fluorescein angiography (FA) revealed a juxtafoveal classic CNV associated with leakage and hyperfluorescence corresponding to the AS on the left eye and a staining of disciform scarring with no active leakage in the macula of the right eye. Indocyanine green angiography (ICG) highlighted the presence of AS that radiated from the peripapillary area to the macula and midperipheral fundus in both eyes (Fig. 1). Colour photograph and angiography findings of the left eye at baseline and at the last follow-up (27 months). Baseline: colour photograph shows angioid streaks (AS) and a grey lesion with subretinal haemorrhage nasally to the fovea consistent with choroidal neovascularization (CNV) (A); fluorescein angiography (FA) shows staining of the AS radiating from the optic nerve (C) with evidence of active CNV nasally to the macula with late leakage (E). Last follow-up: colour fundus photograph shows the resolution of submacular haemorrhage (B) while FA shows the absence of exudation from CNV (D, F). Note that indocyanine green angiography highlights the presence of AS that radiate from the peripapillary area to the macula (G, H). After informed written consent was provided, three intravitreal injections of ranibizumab (0.5 mg) were administered at monthly intervals in the left eye, despite good visual acuity (VA) considering the history of rapid progression of CNV in her right eye. One month after the third injection, VA remained 20/20 with resolution of subjective visual distortion; FA revealed complete regression of the neovascular complex. VA, FA and ICG findings remained stable until the last follow-up at 27 months. No further injections were required during the follow-up period. As in CNV caused by AMD, VEGF-A may play an important role in the pathogenesis of CNV caused by AS. Ranibizumab is a specific, affinity-mature fragment of a recombinant, humanized immunoglobulin (Ig) G1 monoclonal antibody that neutralizes all active forms of VEGF-A. It has been found to be effective in the treatment of exudative AMD, demonstrating encouraging signs of biological activity, with acceptable safety. However, as far as we are aware, the current medical literature encompasses only one other report of CNV associated with AS treated with ranibizumab, in which Derriman et al. (2008) reported stability of VA for 6 months after three intravitreal injections of ranibizumab (0.5 mg) spaced at monthly intervals. In our case, treatment with three monthly interval intravitreal injections of ranibizumab resulted in complete resolution of CNV for a period of 27 months. Ranibizumab may be an effective treatment of CNV related to AS; nevertheless, further clinical trials are required to determine more accurately its clinical benefit and its safety in this clinical entity.