We report an unusual case of unilateral frosted branch angiitis associated with Behcet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behcet's disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behcet's disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended.
Adult ; Behcet Syndrome/*complications/diagnosis ; Diagnosis, Differential ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Magnetic Resonance Imaging ; Male ; Retinal Artery/*pathology ; Retinal Vasculitis/diagnosis/*etiology

Making accurate and timely diagnosis is often challenging when patients with a systemic disease first present with ocular manifestations. The possibility that vasculitis associated with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) can be misdiagnosed as cysticercosis has not been discussed in the literatures.
Antiphospholipid Syndrome ; diagnosis ; Cysticercosis ; diagnosis ; Diagnosis, Differential ; Eye Diseases ; diagnosis ; Female ; Humans ; Lupus Erythematosus, Systemic ; diagnosis ; Magnetic Resonance Imaging ; Middle Aged ; Retinal Vasculitis ; diagnosis

PURPOSE: To report one case of bilateral acute retinal necrosis (ARN) syndrome in the patient with acquired immunodeficiency syndrome (AIDS). METHODS: Bilateral retinal necrosis, occlusive vasculitis, vitreous opacity and inflammatory reaction in anterior chamber were noted in a 48-years old man with AIDS. RESULTS: The patient was diagnosed as bilateral ARN, but did not respond to intravenous acyclovir therapy. However, the patient was successfully treated with intravenous ganciclovir at a dosage of 5 mg/kg/day, which resulted in resolution of retinal lesion and improvement of visual acuity without complications such as retinal detachment. CONCLUSIONS: CMV retinitis, progressive outer retinal necrosis, ocular syphilis should be considered in the differential diagnosis of ARN syndrome in the AIDS patients and alternative treatment with ganciclovir should be considered promptly when the patient do not respond to acyclovir therapy.
Acquired Immunodeficiency Syndrome* ; Acyclovir ; Anterior Chamber ; Diagnosis, Differential ; Ganciclovir ; Humans ; Middle Aged ; Necrosis ; Retinal Detachment ; Retinal Necrosis Syndrome, Acute* ; Retinaldehyde ; Retinitis ; Syphilis ; Vasculitis ; Visual Acuity

We report a case of post-streptococcal uveitis mainly presenting with bilateral recurrent retinal vasculitis in Korea. A 14-year-old Asian female presented with decreased visual acuity of 20 / 30 in the right eye and 20 / 25 in the left eye. The patient had a history of glomerulonephritis nine months before onset of uveitis. The manifestation of uveitis was predominantly retinal vasculitis. We presumed post-streptococcal uveitis because probable streptococcal infection was confirmed by anti-streptolysin O titer elevation. With topical and oral steroid treatments, the patient experienced complete vision recovery. Post-streptococcal uveitis occurs rarely and mostly involves young patients in the form of non-granulomatous anterior uveitis. However, as this case shows, it may primarily involve the posterior uvea without anterior inflammation and may recur.
Adolescent ; Diagnosis, Differential ; Female ; Humans ; Recurrence ; Retinal Vasculitis/drug therapy/*microbiology ; Steroids/therapeutic use ; Streptococcal Infections/*diagnosis ; Uveitis/drug therapy/*microbiology ; Visual Acuity

PURPOSE: To identify significant fluorescein angiographic (FA) characteristics associated with visual acuity (VA) in Behcet retinal vasculitis. MATERIALS AND METHODS: Retrospective review of 86 eyes of 48 patients (age: 35.6+/-10.2 years) with Behcet retinal vasculitis were performed. VA and FA findings as well as correlation between them were assessed. RESULTS: The mean initial VA of eyes with posterior pole-involved vasculitis (63 eyes; 73.3%) was significantly worse than that of those with peripheral vasculitis (23 eye; 26.7%) (logarithm of the minimum angle of resolution VA: 0.554+/-0.572 vs. 0.078+/-0.148; p<0.0001). Subgroup analysis revealed a more severe and diffuse pattern of vascular leakage in posterior pole-involved vasculitis compared to peripheral vasculitis (p<0.0001). Retinal vascular leakage (beta=0.345; p<0.0001), optic disc hyperfluorescence (beta=0.147; p=0.032), and macular leakage (beta=0.107; p=0.047) were significantly associated with worse initial VA. During the follow up (mean: 33.3+/-17.9 months), the change of leakage showed no significant correlation with change of VA in posterior pole-involved vasculitis (tau=0.199, p=0.092). CONCLUSION: Posterior pole involvement, the degree of retinal vascular leakage, optic disc hyperfluorescence, and macular leakage are significantly associated with VA in Behcet retinal vasculitis.
Adult ; Aged ; Behcet Syndrome/*diagnosis ; Female ; *Fluorescein Angiography ; Fluoresceins ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Regression Analysis ; Retinal Vasculitis/*diagnosis/physiopathology ; Retrospective Studies ; Visual Acuity/*physiology

OBJECTIVE: Tuberculosis is a chronic infection caused by Mycobacterium tuberculosis that can appear throughout the entire body; however, intraocular tuberculosis is rare. We report a case of tuberculous neuroretinitis accompanied by periphlebitis. METHODS: A 17-year-old female was referred with the diagnosis of retinal vasculitis. In the fundus of her right eye, an edematous disc, serous detachment of the macula, multiple hard exudates, and vascular sheathing around the veins were noted. Also, she had no direct light reflex in her right eye. Although her chest X-ray showed no abnormalities, the Tuberculin test was positive; therefore, the condition was diagnosed with a neuroretinitis accompanied by periphlebitis caused by Mycobacterium tuberculosis. RESULTS: High-dose steroid treatment decreased peripapillary edema but did not improved visual acuity. Treatment with antituberculosis medicine resulted in the recovery of her visual acuity and in fundus changes. CONCLUSIONS: Ocular tuberculosis commonly appears as perivasculitis; however, it can cause a visual disturbance by invading the retina and optic nerve simultaneously.
Adolescent ; Diagnosis ; Edema ; Exudates and Transudates ; Female ; Humans ; Mycobacterium tuberculosis ; Optic Nerve ; Phlebitis ; Reflex ; Retina ; Retinal Vasculitis ; Retinitis* ; Thorax ; Tuberculin Test ; Tuberculosis ; Tuberculosis, Ocular ; Veins ; Visual Acuity

Behcet's disease is a chronic, relapsing, inflammatory multisystem disorder characterized by recurrent oral and genital ulcerations along with eye lesions (anterior uveitis, posterior uveitis, cells in vitreous on slit lamp examination, retinal vasculitis observed by ophthalmologist), skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions, acneiform nodules), arthralgia, central nervous system and vascular involvement, and gastrointestinal manifestations. The etiology of Behcet's disease remains unknown. We report a case of a 48-year-old male with a 25-year-history of blindness, recurrent ulcers on mucosal surfaces, and papulopustular lesions on the trunk. Dapsone is an easily available, cheap and relatively safe drug, useful particularly in the management of the mucocutaneous symptoms of Behcet's disease. Dapsone 100 mg was given daily for one month which resulted in improvement of skin and mucosal lesions. Early diagnosis and treatment of this condition is important to prevent irreversible organ damage and mandatory to reduce mortality and morbidity.

Human ; Male ; Middle Aged ; Arthralgia ; Behcet Syndrome ; Blindness ; Dapsone ; Early Diagnosis ; Erythema Nodosum ; Ophthalmologists ; Retinal Vasculitis ; Skin ; Slit Lamp Microscopy ; Ulcer ; Uveitis, Anterior ; Uveitis, Posterior

PURPOSE: To report a case of frosted branch angiitis (FBA) associated with Epstein-Barr virus (EBV) infection in a child. CASE SUMMARY: A 7-year-old boy presented with bilateral blurred vision. On ophthalmic examination, his best-corrected visual acuity was 20/25 in the right eye and 20/32 in the left eye. The pupils were equal, round, and reactive to light without a relative afferent pupillary defect. He had normal color vision in both eyes. Slit-lamp examination revealed no abnormalities in the anterior parts of the eyes. Fundoscopic examination revealed prominent white sheathing retinal vasculitis predominantly on the veins in all quadrants, as well as macular edema and irregular foveal reflex in both eyes. Fluorescein angiography showed normal blood flow, but late diffuse staining and leakage of the affected vessels. Spectral domain optical coherence tomography (SD-OCT) showed thickening of the vessel walls, swelling due to hyperreflective material, and hyperreflective retinal depositions. Serological tests and the serum polymerase chain reaction for EBV were positive. A diagnosis of FBA associated with EBV was made. He was treated with systemic acyclovir and steroids. The response was rapid, with improvement in visual acuity to 20/20 in both eyes by day 3. After 7 weeks, all clinical signs resolved and SD-OCT examination showed normal vessel wall thickness and the absence of hyperreflective depositions. CONCLUSIONS: EBV may present with FBA even in the absence of a systemic sign of primary EBV infection. Thus, EBV should be considered as the etiology of FBA.
Acyclovir ; Child ; Color Vision ; Diagnosis ; Epstein-Barr Virus Infections ; Fluorescein Angiography ; Herpesvirus 4, Human ; Humans ; Macular Edema ; Male ; Polymerase Chain Reaction ; Pupil ; Pupil Disorders ; Reflex ; Retinal Vasculitis ; Retinaldehyde ; Serologic Tests ; Steroids ; Tomography, Optical Coherence ; Vasculitis ; Veins ; Visual Acuity

The aim of current study was to evaluate the incidence and characteristics of ocular sarcoidosis in a Korean population. We conducted a retrospective study of 104 consecutive patients with biopsy-proven sarcoidosis seen at Asan Medical Center in Seoul, Korea, from 1993 to 2007. Medical records, photographs, and fluorescein angiograms were reviewed. Of 104 patients, 22 (21%) had intraocular involvement with female predominance (86%, M:F=3:19). Of the 39 eyes with ocular involvement, 16 (41%) eyes had isolated anterior uveitis, 12 (31%) eyes had intermediate uveitis, 6 eyes (15%) had panuveitis with retinal vasculitis, and 5 (13%) eyes had panuveitis with punched multifocal choroiditis. Mean duration of ophthalmologic follow-up was 62 months. All ocular inflammation was well managed with topical steroid and/or systemic steroid with relatively good final visual outcomes. Ocular complications such as cataract (12 eyes, 30%), glaucoma (6 eyes, 15%), vitreous opacity (1 eye, 3%), cystoid macular edema (3 eyes, 7%), neovascularization (2 eye, 5%), and epiretinal membrane (4 eye, 10%) were related to ocular sarcoidosis. In Korea, where sarcoidosis is very rare, our study indicates relatively low ocular and predominantly non posterior segment involvement with relatively good visual prognosis.
Adult ; Age Factors ; Asian Continental Ancestry Group ; Choroiditis/diagnosis ; Eye Diseases/*diagnosis/epidemiology/therapy ; Female ; Fluorescein Angiography ; Humans ; Male ; Middle Aged ; Retinal Vasculitis/diagnosis ; Retrospective Studies ; Sarcoidosis/*diagnosis/epidemiology/therapy ; Steroids/therapeutic use ; Uveitis, Anterior/diagnosis ; Uveitis, Intermediate/diagnosis

The aim of current study was to evaluate the incidence and characteristics of ocular sarcoidosis in a Korean population. We conducted a retrospective study of 104 consecutive patients with biopsy-proven sarcoidosis seen at Asan Medical Center in Seoul, Korea, from 1993 to 2007. Medical records, photographs, and fluorescein angiograms were reviewed. Of 104 patients, 22 (21%) had intraocular involvement with female predominance (86%, M:F=3:19). Of the 39 eyes with ocular involvement, 16 (41%) eyes had isolated anterior uveitis, 12 (31%) eyes had intermediate uveitis, 6 eyes (15%) had panuveitis with retinal vasculitis, and 5 (13%) eyes had panuveitis with punched multifocal choroiditis. Mean duration of ophthalmologic follow-up was 62 months. All ocular inflammation was well managed with topical steroid and/or systemic steroid with relatively good final visual outcomes. Ocular complications such as cataract (12 eyes, 30%), glaucoma (6 eyes, 15%), vitreous opacity (1 eye, 3%), cystoid macular edema (3 eyes, 7%), neovascularization (2 eye, 5%), and epiretinal membrane (4 eye, 10%) were related to ocular sarcoidosis. In Korea, where sarcoidosis is very rare, our study indicates relatively low ocular and predominantly non posterior segment involvement with relatively good visual prognosis.
Adult ; Age Factors ; Asian Continental Ancestry Group ; Choroiditis/diagnosis ; Eye Diseases/*diagnosis/epidemiology/therapy ; Female ; Fluorescein Angiography ; Humans ; Male ; Middle Aged ; Retinal Vasculitis/diagnosis ; Retrospective Studies ; Sarcoidosis/*diagnosis/epidemiology/therapy ; Steroids/therapeutic use ; Uveitis, Anterior/diagnosis ; Uveitis, Intermediate/diagnosis