to report a case of recurrent idiopathic frosted branch angiitis ( fba ) successfully treated with adalimumab . a 14-year - old otherwise healthy boy was referred to the uveitis clinic for bilateral panuveitis with diffuse retinal vascular sheathing and severe macular edema . two years ago , a similar episode had been treated with oral prednisolone , however it was complicated by adverse psychiatric effects . the progressive course of the condition mandated considering other therapeutic measures ; adalimumab was chosen based on its purported efficacy for treatment of childhood uveitis and a favorable safety profile . the patient responded dramatically to a single subcutaneous injection of adalimumab without any side effect during and after injection . to the best of our knowledge , this is the first case of idiopathic fba treated successfully with adalimumab without adjunctive steroid therapy . frosted branch angiitis ( fba ) is a rare disease characterised by visual disturbance associated with anterior chamber and vitreous inflammation and severe sheathing of retinal vessels resembling the appearance of frosted branches of a tree . it was originally described in a six - year - old child by ito in 1976.1 fba can be associated with ocular and systemic diseases . cytomegalovirus retinitis , acquired immunodeficiency syndrome , and toxoplasma chorioretinitis are frequent ocular associations , while systemic lupus erythematosus , crohn 's disease , behcet s disease , large cell lymphoma , and acute lymphoblastic leukemia have been described as systemic associations.2 the idiopathic type typically occurs in young and healthy persons and is associated with good recovery of vision . a 14-year - old boy , who was otherwise healthy , was referred to the uveitis clinic for progressive blurring of vision in both eyes since 1 month before . on examination , anterior segment examination revealed mild ciliary injection and anterior chamber reaction ( 2 + cells ) but no keratic precipitates or posterior synechiae . both eyes had inflammatory reaction in the vitreous ( 2 + cells ) but there were no snow balls or snow bank formation . fundus examination disclosed extensive retinal vascular sheathing and scattered intraretinal hemorrhages with severe macular edema in both eyes ( fig . 1 ) . systemic work - up and investigations , including a complete blood count ( cbc ) , erythrocyte sedimentation rate ( esr ) , c - reactive protein ( crp ) titer , angiotensin converting enzyme ( ace ) titer , tuberculin skin test ( tst ) , urine analysis and chest x - ray were unremarkable . rheumatologic tests including antinuclear antibodies ( ana ) , anti double strand dna antibody ( anti ds - dna ) , rheumatic factor ( rf ) , antiphospholipid antibody , anti - neutrophil cytoplasmic antibodies ( ancas ) , and cryoglobulin assay were all negative . blood cd4 and cd8 counts were within normal range and serology for the human immunodeficiency virus was also negative . blood serology for possible infectious causes including herpes family viruses , i.e. herpes simplex virus ( hsv ) type 1 and 2 , varicella zoster virus ( vzv ) , cytomegalovirus ( cmv ) , and epstein - barr virus ( ebv ) , hepatitis b and c viruses , treponema , borrelia , and toxoplasma were checked . immunoglobulin g ( igg ) levels against hsv type 2 and cmv ( but not igm ) were elevated . we tested the aqueous specimen after an anterior chamber paracentesis to investigate the presence of deoxyribonucleic acid ( dna ) of hsv type 1 and cmv viruses . the result of polymerase chain reaction ( pcr ) on aqueous samples was negative for both viruses . until the results of blood tests and aqueous pcr became ready , vision continued to deteriorate down to 20/1000 and 20/800 in the right and left eyes , respectively . based on the inconclusive work - up a diagnosis of idiopathic fba the patient s parents mentioned that two years ago , for treatment of a similar condition , oral prednisolone 1 mg / kg had been commenced but soon after initiation , the patient developed severe mood changes which persisted long after discontinuation of treatment . the progressive nature of the condition mandated considering other therapeutic measures to prevent macular scarring . due to the safety profile of anti - tumor necrosis factor ( tnf ) agents and their rapid onset of effect , adalimumab was considered as an alternative to conventional treatment for which the parents provided written informed consent . a single dose of 40 mg pre - filled adalimumab ( humira , abbott laboratories , north chicago , il , usa ) was injected subcutaneously in the patient s right thigh . no local or systemic adverse effect was noted at the time of injection or later on . three weeks after injection , visual acuity improved to 20/200 and 20/120 in the right and left eyes , respectively . anterior chamber and vitreous reaction decreased to 1 + cell , and vascular sheathing and macular edema resolved dramatically . repeat fluorescein angiography and optical coherence tomography ( oct ) confirmed the improvement ( figs . 2 and 3 ) . 10 weeks after injection , visual acuity improved to 20/40 and 20/30 in the right and left eyes , respectively . in both eyes , clinical improvement continued up to the last visit ( 6 months after the injection ) when visual acuity reached 20/25 in both eyes with no sign of active inflammation . fba is basically divided into three different subgroups.3 the first comprises patients affected by lymphoma and leukemia presenting with a frosted branch - like appearance in the fundus . the second group includes patients with associated autoimmune or viral disease which can present with fba in association with the underlying disease . the characteristic feature of this latter type is its occurrence in otherwise healthy young individuals with good recovery of vision . clinically , there is severe sheathing of retinal vessels appearing like the frosted branches of a tree , and acute visual disturbance associated with anterior chamber and vitreous inflammation ; fluorescein angiography shows leakage from sheathed vessels without signs of occlusion or stasis.4,5 systemic steroids are the main and only suggested treatment for this condition . in our patient however , prior adverse psychiatric effects of steroid treatment encouraged us to employ another therapeutic option : adalimumab , a fully humanized monoclonal anti - tnf antibody , was chosen based on its suggested effect for treatment of refractory childhood uveitis including juvenile idiopathic arthritis.6 - 8 our patient responded dramatically to a single subcutaneous injection of adalimumab . the improvement was rapid and long lasting and the patient did not report any side effect during follow up . review of the literature revealed no other therapeutic option for treatment of idiopathic fba except systemic steroids . to our best knowledge , this is the first case of idiopathic fba treated successfully with an anti - tnf agent without adjunctive steroid therapy .
purposeto report a case of recurrent idiopathic frosted branch angiitis ( fba ) successfully treated with adalimumab.case reporta 14-year - old otherwise healthy boy was referred to the uveitis clinic for bilateral panuveitis with diffuse retinal vascular sheathing and severe macular edema . 
 extensive work - up including aqueous sampling for detection of viral causes was inconclusive . 
 two years ago , a similar episode had been treated with oral prednisolone , however it was complicated by adverse psychiatric effects . 
 the progressive course of the condition mandated considering other therapeutic measures ; adalimumab was chosen based on its purported efficacy for treatment of childhood uveitis and a favorable safety profile . 
 the patient responded dramatically to a single subcutaneous injection of adalimumab without any side effect during and after injection . 
 the therapeutic effect was rapid and relatively long-lasting.conclusionto the best of our knowledge , this is the first case of idiopathic fba treated successfully with adalimumab without adjunctive steroid therapy .