OBJECTIVE: To evaluate the value of fundus autofluorescence (FAF) imaging combined with spectral domain optical coherence tomography (SD-OCT) in diagnosis, prognostic assessment and follow-up observation of acute Vogt-KoyanagiHarada (VKH) disease. METHODS: Clinical data were collected from 12 patients (23 eyes) with acute VKH disease treated in our hospital from May, 2018 to November, 2019, including detailed medical history, best corrected visual acuity (BCVA), and results of slit lamp biomicroscopy, fundus photography, SD-OCT, fundus fluorescein angiography (FFA) and FAF imaging.SDOCT and FAF imaging were repeated after a course of treatment and in follow-up examination, and the results were compared with those at the time of admission. RESULTS: VKH disease involved both eyes in 11 patients (91.7%).Fundus photography showed optic disc edema in 16 eyes (69.6%), and multiple retinal neuroepithelial detachment was detected by SD-OCT in all the involved eyes (100%).IN all the eyes, FFA revealed small and dense fluorescein leakage in the early stage and fluorescein accumulation in advanced stages of VHK disease to form multiple dye pooling in the areas of serous detachment.Hyperauto fluorescence was a common finding in FAF imaging (100%), and the area involved was consistent with that of fluorescein accumulation shown by FAF imaging.Ten eyes (43.5%) showed patches of relative hypoautofluorescence in the hyperauto fl uorescence areas, and granular hyperauto fl uorescence was found in the lesions in 4 eyes (17.4%).During the remission period of VKH disease, FAF imaging showed normal finding in 8 eyes (34.8%) and reduced areas (by 55.2%) and intensity (by 46.5%) of hyperautofluorescence in 9 eyes (39.1%).In 6 eyes (26.1%), only a few hyperautofluorescent spots scattered in the macula were observed.SD-OCT demonstrated significantly reduced (by 69.5% on average) or even disappearance of subretinal fluid in the eyes.The fluorescence intensity in FAF imaging showed a significant positive correlation with the volume of subretinal fluid detected by SD-OCT ( CONCLUSIONS The combination of fluorescein angiography, FAF imaging and SD-OCT can significantly improve the diagnostic accuracy of VKH disease.FAF imaging combined with SD-OCT provides an effective and noninvasive modality for evaluation of remission and monitoring the changes in VKH disease.
Acute Disease ; Fluorescein Angiography ; Follow-Up Studies ; Humans ; Retinal Detachment/diagnostic imaging* ; Tomography, Optical Coherence ; Uveomeningoencephalitic Syndrome/diagnostic imaging*

No abstract available.
Adalimumab ; Adult ; Female ; Humans ; Uveomeningoencephalitic Syndrome

PURPOSE: To report a case of atypical Vogt–Koyanagi–Harada disease that occurred after an acute angle closure glaucoma attack. CASE SUMMARY: A 48-year-old female presented with bilateral visual disturbance accompanied by headache and ocular pain. The patient had no specific past medical or family history except taking oral contraceptives for 10 years. Despite the normalization of intraocular pressure in a local clinic, a shallow-depth anterior chamber and forward displacement of the iris–lens diaphragm remained unresolved. The depth of the anterior chamber had increased in both eyes after laser therapy but without recovery of her visual acuity. B-scans showed ciliochoroidal effusion. Anterior chamber inflammation was observed in both eyes. Optical coherence tomography showed lobulated and serous retinal detachment involving the macula of both eyes. However, fluorescence angiography findings showed no multiple hyperfluorescence, which is unusual for typical cases of Vogt–Koyanagi–Harada disease. The patient was diagnosed with atypical Vogt–Koyanagi–Harada disease and was treated with eyedrops and intravenous steroid pulse therapy, after which she was converted to oral medications with immunosuppressants. After 1 month, no serous retinal detachment was detected. After 3 months, best corrected visual acuity (logMAR) was 0.0 in both eyes, and there has been no recurrence on follow-up. CONCLUSIONS: Atypical Vogt–Koyanagi–Harada disease at presentation can mimic acute attacks of angle closure glaucoma. Therefore, if there is no improvement after treatment for angle closure glaucoma including laser iridotomy, other diseases including Vogt–Koyanagi–Harada disease must be considered and the patient should be closely monitored.
Anterior Chamber ; Contraceptives, Oral ; Diaphragm ; Female ; Fluorescein Angiography ; Follow-Up Studies ; Glaucoma, Angle-Closure ; Headache ; Humans ; Immunosuppressive Agents ; Inflammation ; Intraocular Pressure ; Laser Therapy ; Middle Aged ; Ophthalmic Solutions ; Recurrence ; Retinal Detachment ; Tomography, Optical Coherence ; Uveomeningoencephalitic Syndrome ; Visual Acuity

PURPOSE: To report a case of Vogt-Koyanagi-Harada (VKH) disease with chronic recurrence, which was treated with intravitreal dexamethasone implantation. CASE SUMMARY: A 39-year-old female presented with decreased visual acuities in both eyes accompanied by headaches and tinnitus the previous week. On fundus examination and optical coherence tomography, multiple serous retinal detachments with subretinal septa were observed in both eyes, and she was diagnosed with VKH disease. After undergoing high dose intravenous steroid therapy at another hospital, she was treated with oral prednisolone and immunosuppressant drugs. However, she showed frequent recurrences, and also complained of side effects from prolonged systemic steroids. We then performed intravitreal injections of dexamethasone implants in both eyes at 3-week intervals. One month following the injections, the inflammations in the anterior chamber and vitreous opacities were improved. Until the last visit at 6 months after the injection, there was no significant recurrence of inflammation, although oral prednisolone had been tapered. CONCLUSIONS: In VKH disease, intravitreal dexamethasone implantation may be an effective treatment option if chronic recurrence occurs, despite systemic steroid and immunosuppressant therapy or sustained treatment being difficult to maintain because of side effects.
Adult ; Anterior Chamber ; Dexamethasone ; Female ; Headache ; Humans ; Inflammation ; Intravitreal Injections ; Prednisolone ; Recurrence ; Retinal Detachment ; Steroids ; Tinnitus ; Tomography, Optical Coherence ; Uveitis ; Uveomeningoencephalitic Syndrome ; Visual Acuity

PURPOSE: To report clinical features of patients with retinal and choroidal diseases presenting with acute visual disturbance during pregnancy. METHODS: In this retrospective case series, patients who developed acute visual loss during pregnancy (including puerperium) and visited a tertiary hospital from July 2007 to June 2015, were recruited by searching electronic medical records. Patients were categorized according to the cause of visual loss. Clinical features and required diagnostic modalities were analyzed in the retinal and choroidal disease group. RESULTS: Acute visual loss occurred in 147 patients; 49 (38.9%) were classified into the retinal and choroidal group. The diagnoses included central serous chorioretinopathy (22.4%), hypertensive retinopathy with or without pre-eclampsia (22.4%), retinal tear with or without retinal detachment (18.4%), diabetic retinopathy progression (10.2%), Vogt-Koyanagi-Harada disease (4.1%), retinal artery occlusion (4.1%), multiple evanescent white dot syndrome (4.1%), and others (14.3%). Visual symptoms first appeared at gestational age 25.9 ± 10.3 weeks. The initial best-corrected visual acuity (BCVA) was 0.27 ± 0.39 logarithm of the minimum angle of resolution (logMAR); the final BCVA after delivery improved to 0.13 ± 0.35 logMAR. Serious visual deterioration (BCVA worth than 20 / 200) developed in two patients. Differential diagnoses were established with characteristic fundus and spectral-domain optical coherence tomography findings in all cases. CONCLUSIONS: In pregnant women with acute visual loss, retinal and choroidal diseases are common and could be vision threatening. Physicians should be aware of pregnancy-associated retinal and choroidal diseases and their clinical features. The differential diagnosis can be established with non-invasive techniques.
Central Serous Chorioretinopathy ; Choroid Diseases* ; Choroid* ; Diabetic Retinopathy ; Diagnosis ; Diagnosis, Differential ; Electronic Health Records ; Female ; Gestational Age ; Humans ; Hypertensive Retinopathy ; Pre-Eclampsia ; Pregnancy ; Pregnant Women ; Retinal Artery Occlusion ; Retinal Detachment ; Retinal Diseases ; Retinal Perforations ; Retinaldehyde* ; Retrospective Studies ; Tertiary Care Centers ; Tomography, Optical Coherence ; Uveomeningoencephalitic Syndrome ; Visual Acuity

Vogt-Koyanagi-Harada disease is a rare multisystemic granulomatous autoimmune disorder affecting pigmented tissues such as the choroid, meninges, inner ear, and the skin. Neurologic symptoms are usually mild. Clinical manifestations include generalized muscle weakness, headache, meningismus, vertigo, decreased visual acuity, hearing loss and mental changes ranging from mild confusion to psychosis, hemiparesis, dysarthria, and aphasia. Seizures are very rare. We describe a case of ¹⁸F-fluorodeoxyglucose (F-18 FDG) positron emission tomography (PET) and software-fused PET-magnetic resonance imaging (MRI) in Vogt-Koyanagi-Harada disease with seizure.
Aphasia ; Choroid ; Dysarthria ; Ear, Inner ; Headache ; Hearing Loss ; Magnetic Resonance Imaging ; Meninges ; Meningism ; Muscle Weakness ; Neurologic Manifestations ; Paresis ; Positron-Emission Tomography ; Psychotic Disorders ; Seizures ; Skin ; Uveomeningoencephalitic Syndrome ; Vertigo ; Visual Acuity

Ocular inflammation from various causes may have similar clinical presentation thus careful clinical evaluations are mandatory particularly when the disease appear to be resistant to treatment. This paper reports a case of ocular lymphoma which was initially treated as Vogt Koyanagi Harada disease at a different centre. A 65-year-old Sudanese man complained of gradual worsening left eye vision. Careful ocular and slit lamp examination revealed a conjunctival lesion with choroidal infiltration as well as exudative retinal detachment. Computed tomography scan (CT scan) showed left eye axial proptosis, and a homogenous enhancing mass at the posterior coat of the globe. Tissue biopsy of the conjunctival lesion revealed marginal Zone B cell (MALT type), low grade, non-Hodgkin’s lymphoma. AS the ocular signs and symptoms progressed, chemotherapy was initiated. The proptosis, exudative retinal detachment, disc hyperemia and swelling improved after chemotherapy. The post treatment CT scan showed reduction of the posterior ocular coat mass. The possibility of malignant lymphoma should be considered in patients with resistant uveitis despite administration of corticosteroid. Ocular lymphoma is an indolent tumour with good prognosis. Careful ocular examination, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis.
Uveomeningoencephalitic Syndrome

PURPOSE: To evaluate the therapeutic effect and safety of mycophenolate mofetil (MMF) on chronic uveitis in Korean patients. METHODS: This study included 25 patients with chronic uveitis who used MMF and were followed up more than 6 months in 2 referral centers from 2010 to 2014. The medical records were analyzed retrospectively. The therapeutic effect was assessed based on control of inflammation, corticosteroid sparing effects, and discontinuation of MMF, and the safety was assessed based on side effects. Control of inflammation was defined as no active inflammation observed on at least 2 consecutive visits 28 days apart or more. RESULTS: The 25 patients consisted of 18 males and 7 females. The mean age of the patients was 47.52 years. The etiology of uveitis was as follows: Behcet's disease in 15 patients (60%), Vogt-Koyanagi-Harada disease in 4 (16%), sympathetic ophthalmia in 2 (8%), systemic lupus erythematosus in 1 (4%), and idiopathic uveitis in 3 (12%). Anatomic classification was anterior uveitis in 20% and posterior uveitis or panuveitis in 80% of patients. Complete control of inflammation was achieved in 44% and 50% of patients within 6 months and 1 year, respectively. Systemic corticosteroid dosage was reduced to 10 mg of prednisone or less while maintaining sustained control of inflammation in 36% and 45% of patients for 6 months and 1 year, respectively. MMF was discontinued in 3 patients (12%) due to side effects and in 2 patients (8%) due to lack of effectiveness. CONCLUSIONS: MMF was effective and side effects were uncommon when managing chronic uveitis in Korean patients.
Classification ; Female ; Humans ; Inflammation ; Lupus Erythematosus, Systemic ; Male ; Medical Records ; Ophthalmia, Sympathetic ; Panuveitis ; Prednisone ; Referral and Consultation ; Retrospective Studies ; Uveitis* ; Uveitis, Anterior ; Uveitis, Posterior ; Uveomeningoencephalitic Syndrome

PURPOSE: In the present study, a case of repeated intravitreal dexamethasone implantation for a suspected idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome associated with recurrent exudative retinal detachment and macular edema is reported. CASE SUMMARY: A 39-year-old female who underwent steroid pulse therapy due to Vogt-Koyanagi-Harada disease in the left eye was referred for exudative retinal detachment and macular edema. Best corrected visual acuity (BCVA) was 1.0 in the right eye and 0.5 in the left eye. Cystoid macular edema combined with serous retinal detachment was observed on spectral-domain optical coherence tomography. Fluorescein angiography revealed neovascularization and multiple macroaneurysms with fluorescein leakage in the left peripapillary area. Severe peripheral capillary non-perfusion and fluorescein leakage were also observed in both eyes. Intravitreal dexamethasone implantation was performed in the left eye and macular edema showed wax-and-wane pattern. No edema was observed after 4 additional dexamethasone implantations, however, preretinal hemorrhage occurred in the peripapillary area during treatment. Seventeen months after initiation of treatment, BCVA was 0.6 in the left eye and dry macula was maintained. CONCLUSIONS: Repeated intravitreal dexamethasone implantation was effective for recurrent macular edema in a patient suspected with IRVAN syndrome.
Adult ; Aneurysm ; Capillaries ; Dexamethasone* ; Edema ; Female ; Fluorescein ; Fluorescein Angiography ; Hemorrhage ; Humans ; Macular Edema ; Retinal Detachment ; Retinal Vasculitis ; Retinitis ; Tomography, Optical Coherence ; Uveomeningoencephalitic Syndrome ; Visual Acuity

PURPOSE: To report characteristics of Vogt-Koyanagi-Harada (VKH) disease as observed on spectral-domain optical coherence tomography (SD-OCT) images. CASE SUMMARY: (Case 1) A 38-year-old female presented with visual impairment in both eyes. On fundus examination, multifocal serous retinal detachment in the posterior pole was observed in both eyes. On enhanced SD-OCT, serous retinal detachment and cystoid macular edema in the outer retina were observed and the intraretinal fluid space was divided by a membranous structure forming an intraretinal compartmentalized cystic space. Under the diagnosis of VKH disease, the patient was treated with intravenous administration of methylprednisolone. After treatment, enhanced SD-OCT images showed decreased serous retinal detachment. (Case 2) A 58-year-old male presented with visual impairment in both eyes. On fundus examination, multifocal serous retinal detachment in the posterior pole was observed in both eyes. On enhanced SD-OCT, choroidal folds were observed and the membranous structure showed continuity with ellipsoid zone, suggesting the membranous structure was part of the outer photoreceptor layer of the adjacent attached retina. The patient was treated with intravenous administration of methylprednisolone. After treatment, enhanced SD-OCT images showed cystic space was decreased. CONCLUSIONS: SD-OCT images of VKH disease demonstrated multiple serous retinal detachments and intraretinal compartmentalized cystic space divided by a membranous structure. The membranous structure may be considered a part of the outer photoreceptor layer of the attached retina.
Administration, Intravenous ; Adult ; Choroid ; Diagnosis ; Female ; Humans ; Macular Edema ; Male ; Methylprednisolone ; Middle Aged ; Retina ; Retinal Detachment ; Tomography, Optical Coherence* ; Uveomeningoencephalitic Syndrome* ; Vision Disorders