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

PURPOSE: To report a rare case of Aspergillus endophthalmitis as a preceding symptom of central nervous system (CNS) lymphoma. CASE SUMMARY: A 66-year-old female was admitted to our clinic with mental change for 3 days. Seven months earlier, she had been diagnosed with retinal vasculitis in an ophthalmology clinic because of blurred vision in both eyes and was administered steroid therapy. Three months earlier, because of progressive symptoms, vitreous fluid culture had been performed and showed Aspergillus endophthalmitis. She was treated with intravitreous voriconazole injection and oral voriconazole. Initial brain magnetic resonance imaging (MRI) and cerebrospinal fluid study was normal. Two months later, a second MRI showed multiple enhancing lesions, which were aggravated on the third MRI at admission to our clinic. Although brain biopsy was not performed due to the poor condition of the patient, CNS lymphoma was suspected based on the neuroimaging. After steroid pulse therapy and whole brain radiation, follow-up neurologic examination showed improved mental state, and follow-up MRI showed remarkable shrinkage of multiple lesions. CONCLUSIONS: As Aspergillus endophthalmitis is an opportunistic infection in those with an immune-compromised state and the orbit is near the central nervous system, the clinician should be alert to concomitant disorders in CNS. For a prompt and accurate diagnosis of CNS disorder, early evaluation of neurologic symptoms beyond symptoms of endophthalmitis and neuroimaging is essential.
Aged ; Aspergillus* ; Biopsy ; Brain ; Central Nervous System* ; Cerebrospinal Fluid ; Diagnosis ; Endophthalmitis* ; Female ; Follow-Up Studies ; Humans ; Lymphoma* ; Magnetic Resonance Imaging ; Neuroimaging ; Neurologic Examination ; Neurologic Manifestations ; Ophthalmology ; Opportunistic Infections ; Orbit ; Retinal Vasculitis

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

PURPOSE: To Report A Case Of Atypical Acute Retinal Necrosis (Arn) Observed Using Ultra-wide-field Imaging. CASE SUMMARY: A 50-year-old male with recurrent oral ulcer presented with visual loss in his right eye. On the initial visit, slit-lamp examination showed inflammatory cells (3+) in the anterior chamber and vitreous of the right eye. Funduscopic examination and ultra-wide-field fundus images showed severe occlusive vasculitis with perivascular hemorrhage. No necrotic lesion was observed at the peripheral retina in the left eye. Under the impression of Behcet's uveitis, immunosuppressive therapies including oral steroid, intravenous cyclophosphamide, anti-tumor necrosis factor (TNF) alpha agent and intravitreal triamcinolone were administered, but without improvement. Subsequently, a necrotic lesion appeared at the far peripheral retina in the right eye and varicella-zoster virus was identified using the polymerase chain reaction test. After the patient was diagnosed with atypical ARN, intravenous acyclovir was administered, resulting in eventual suppression of the necrotic lesion progression. Due to retinal detachment, pars plana vitrectomy with silicone oil infusion was performed in the right eye. At that time, a whitish lesion at the far peripheral retina in the ultra-wide-field photography of the fellow eye was found and 3 intravitreal ganciclovir injections were administered. Finally, the whitish lesion regressed after 1 month. CONCLUSIONS: Ultra-wide-field imaging might be useful for diagnosis and follow-up of atypical ARN patients.
Acyclovir ; Anterior Chamber ; Cyclophosphamide ; Diagnosis ; Ganciclovir ; Hemorrhage ; Herpesvirus 3, Human ; Humans ; Male ; Middle Aged ; Necrosis ; Oral Ulcer ; Photography ; Polymerase Chain Reaction ; Retina ; Retinal Detachment ; Retinal Necrosis Syndrome, Acute* ; Silicone Oils ; Triamcinolone ; Uveitis ; Vasculitis ; Vitrectomy

PURPOSE: Polyarteritis nodosa (PAN) is the necrotic vasculitis affecting middle and small-sized arteries throughout the body including ocular tissue. We report an atypical PAN case of unilateral central retinal artery occlusion in which the ocular involvement occurred before systemic symptoms. CASE SUMMARY: A 58-year-old male visited the ophthalmology department due to abruptly decreased visual acuity. Best corrected visual acuity (BCVA) was 0.05 (in the right eye) and 1.0 (in the left eye) basedon the Snellen chart. He complained of acute decreased vision occurring ten days prior and intermittent migraine on the right side with no underlying diseases, such as hypertension or diabetes mellitus. Relative afferent pupillary defect was observed in the right eye. Generalized edema was found around the optic disc and fovea on fundus examination and optical coherence tomography. The patient was diagnosed with central retinal artery occlusion (CRAO) based on a fluorescein angiography. Subsequently, PAN was diagnosed based on clinical features, laboratory test results and imaging studies. The treatment was started with an immunosuppressive agent. One month later, the BCVA was 0.05 based on the Snellen chart. CONCLUSIONS: In PAN patients, decreased vision can occur as a first symptom due to CRAO. If the patient visits the ophthalmology clinic, history taking and laboratory tests for PAN can aid in early diagnosis and treatment, as well as preventing additional complications of PAN.
Arteries ; Diabetes Mellitus ; Early Diagnosis ; Edema ; Fluorescein Angiography ; Humans ; Hypertension ; Male ; Middle Aged ; Migraine Disorders ; Ophthalmology ; Polyarteritis Nodosa* ; Pupil Disorders ; Retinal Artery Occlusion ; Tomography, Optical Coherence ; Vasculitis ; Visual Acuity

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

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

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

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

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