the idiopathic retinitis , vasculitis , aneurysms and neuroretinitis ( irvan ) syndrome is a retinal vascular disorder of unknown etiology characterized by three major criteria ( multiple leaking aneurysmal dilations , retinal vasculitis and neuroretinitis ) and three minor criteria ( peripheral capillary nonperfusion , retinal neovascularization and macular exudation ) .
irvan syndrome may progress rapidly to severe visual loss , mainly due to the development of exudative maculopathy and/or retinal ischemia , and its neovascular sequelae such as vitreous hemorrhage and neovascular glaucoma [ 1 , 2 ] .
retinal photocoagulation ( rp ) is the only treatment that has shown evidence to prevent the progression of the disease by acting on proliferative changes [ 1 , 3 ] .
herein , we report a clinical case of irvan syndrome with both retinal neovascularization and macular exudation successfully managed with intravitreal ranibizumab ( lucentis ) as adjunctive therapy to rp .
a 42-year - old man came to our department with a 1-month history of blurred vision in his right eye ( re ) .
best corrected visual acuity ( bcva ) was 20/40 in the re and 20/20 in left eye ( le ) . slit lamp examination was bilaterally unremarkable .
fundus examination of the re evidenced the presence of numerous lipid and soft exudates within the macular area , accompanied by tortuous vessels with aneurysmal changes and marked papillary neovascularization ( fig .
le funduscopy revealed asymptomatic papillary neovascularization , lipid and soft exudates in the vascular arcades , but no macular involvement ( fig .
fluorescein angiography demonstrated bilateral aneurysmal changes of the retinal arteries with staining of their walls , papillary neovascularization with active leakage of the optical nerve head , and extensive areas of retinal ischemia not only in the periphery but also in the posterior pole ( fig .
1c , d ) . optical coherence tomography ( oct ; carl zeiss , dublin , calif . , usa ) showed a subfoveal detachment of the neurosensory retina with prominent reflectance from the accumulation of lipid exudates in the outer retina of the re and a normal foveal contour in the le ( fig .
a complete ocular and systemic examination including chest x - ray , routine blood chemistry , erythrocyte sedimentation rate , angiotensin - converting enzyme , purified protein derivative , venereal disease research laboratory , fluorescent treponemal antibody absorption test , antiphospholipid antibodies , antinuclear antibodies and antineutrophil cytoplasmic antibodies was performed , and the mantoux and pathergy tests were within normal limits . given the clinical findings
optic nerve neovascularization and macular exudation dramatically regressed 1 month after the second injection , and bcva improved to 20/30 in the re ( fig .
panretinal photocoagulation ( prp ) in the re and selective photocoagulation of the ischemic areas in the le were performed ( fig .
2c  f ) . at the 1-year follow - up , bcva was 20/20 bilaterally , with complete regression of neovascularization and exudation and no need for further retreatment .
irvan syndrome is a retinal vascular disease characterized by bilateral multiple leaking aneurysmal dilations , vasculitis , neuroretinitis and peripheral vascular ischemia .
very few cases have been reported in the literature since kincaid and schatz   described the first 2 patients with bilateral retinal arteritis and multiple aneurysmal dilatations in 1983 .
the disease usually presents in the third or fourth decade of life and there are no known systemic associations .
visual loss is caused by exudative maculopathy and/or neovascular sequelae of retinal ischemia so that the prognosis of irvan syndrome depends on prompt treatment [ 1 , 2 ] .
although the best therapy has not yet been established , rp seems to be the most suitable option to control the disease and to prevent its progression [ 1 , 2 , 3 ] .
other treatments have been reported with variable results such as corticosteroids [ 1 , 2 , 3 ] , pars plana vitrectomy [ 2 , 5 ] , anti - tnf alpha agents , antiangiogenic drugs [ 7 , 8 ] or the direct application of laser on aneurysms . a functional staging system based on progressive ischemia and
stage 1 includes macroaneurysms , exudation , neuroretinitis and retinal vasculitis ; stage 2 includes angiographic evidence of capillary nonperfusion ; stage 3 is characterized by the presence of posterior segment neovascularization and/or vitreous hemorrhage ; stage 4 includes anterior segment neovascularization , and stage 5 includes neovascular glaucoma .
samuel et al .   published the most relevant case series of irvan syndrome , including 44 eyes of 22 patients .
all eyes treated with laser in stage 2 maintained a vision of 20/20 without progression of the disease . many of the treated eyes in stage 3 also maintained an acceptable visual acuity of 20/40 or better ; however , 25% of the patients in this stage ended with a visual acuity of 20/200 or worse , with progression of the disease .
half of the patients treated in stages 4 and 5 progressed to a severe vision loss in spite of treatment .
these results show the importance of an early diagnosis and treatment in patients with irvan syndrome ; the treatment of stage 2 cases being the one that achieves better visual results .
vascular endothelial growth factor plays an important role in ocular neovascularization as ischemia is the main stimulus in its production .
antiangiogenic drugs have demonstrated a beneficial effect by controlling neovascularization and macular edema in diseases such as exudative age - related macular degeneration , diabetic retinopathy   and retinal vein occlusion .
the use of these drugs has also been described in retinal vascular diseases that share certain characteristics with irvan syndrome , such as retinal arterial macroaneurysms , eales disease   and coats disease .
previous clinical case reports have shown the efficacy of intravitreal bevacizumab and ranibizumab therapy as adjunctive treatment for irvan syndrome ; however , none of these cases had exudative maculopathy [ 7 , 8 ] .
we reported a clinical case of a stage 3 irvan syndrome with the presence of macroaneurysms , exudation , neuroretinitis , retinal vasculitis and bilateral retinal neovascularization .
prp in the re and selective laser application on the ischemic areas in the le were consecutively performed .
after the 1-year follow - up , visual acuity remained 20/20 in both eyes , with no evidence of progression of the disease and no need for further retreatment .
the case presented underlines that intravitreal ranibizumab alone can cause a reduction of the irvan - associated exudation .
we conclude that ranibizumab constitutes an effective and safe adjunctive treatment for irvan syndrome , contributing to the control and disappearance not only of the neovascularization but also of the macular exudates .
 the idiopathic retinitis , vasculitis , aneurysms and neuroretinitis syndrome is a rare retinal vascular disorder characterized by multiple leaking aneurysmal dilations , retinal vasculitis , neuroretinitis and peripheral vascular ischemia . visual loss mainly occurs due to the development of retinal neovascularization and/or exudative maculopathy . 
 although the treatment of choice has not yet been established , retinal photocoagulation seems to be the best option to control the disease and to prevent its progression . 
 herein , we report a case of idiopathic retinitis , vasculitis , aneurysms and neuroretinitis syndrome with both retinal neovascularization and macular exudation successfully managed with intravitreal ranibizumab ( lucentis ) as adjunctive therapy to retinal photocoagulation . 