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 ocular findings of a mucolipidosis type II patient with novel mutation. CASE SUMMARY: A 10-year-old boy visited our pediatric genetic metabolic clinic for evaluation of his overall developmental delay and short stature. The boy was diagnosed with mucolipidosis type II (I-cell disease) using plasma enzyme assay and DNA sequencing of the GNPTAB gene mutation. An ophthalmologic investigation was then performed, and a depressed nasal bridge, broad nose, and swelling in the upper lid of both eyes were noted. The best corrected visual acuity was 0.32 and 0.1 and the intraocular pressure was 35 mmHg and 24 mmHg in the right and left eyes, respectively. The anterior chamber angles of both eyes were normal and mild cornea opacity in both eyes was observed. Fundus examination revealed retinal atrophy with folds in both eyes, as well as optic disc edema and optic atrophy in the right and left eyes, respectively. Atherosclerotic changes in the retinal vessels and cystoid macular edema in the left eye were observed, and ocular ultrasound revealed increased posterior sclera thickness in both eyes. CONCLUSIONS: Ocular manifestations of mucolipidosis type II are not currently well-known, and differentiation from other metabolic disorders may be difficult. An ophthalmic work-up can assist in diagnosis, and regular ophthalmic examinations should be used to maintain visual function in mucolipidosis patients.
Anterior Chamber ; Atrophy ; Child ; Cornea ; Diagnosis ; Edema ; Enzyme Assays ; Humans ; Intraocular Pressure ; Lysosomal Storage Diseases ; Macular Edema ; Male ; Mucolipidoses* ; Nose ; Optic Atrophy ; Plasma ; Retinal Vessels ; Retinaldehyde ; Sclera ; Sequence Analysis, DNA ; Ultrasonography ; Visual Acuity

PURPOSE: We report a rare case of bilateral macular infarction as an ocular presenting sign of primary antiphospholipid syndrome. CASE SUMMARY: A 29-year-old woman who had undergone a cesarean section for chorioamnionitis in the department of Obsterics was referred to the department of ophthalmology for bilateral visual loss. At examination, best-corrected visual acuity (BCVA) of the right eye was counting fingers, and for the left was 0.05. Fundus examination revealed extensive macular edema and cotton-wool spots in both eyes. We performed hematologic tests including thrombophilia examination. Antinuclear antibody and rheumatoid factor were negative but lupus anticoagulant presented high titers on two occasions 12 weeks apart. She was administered sub-Tenon's injections of triamcinolone acetonide 50 mg/week in both eyes under the diagnosis of bilateral macular arteriolar occlusion in primary antiphospholipid syndrome. Her BCVA remained 0.025 in her right eye and improved to 0.125 in her left eye. CONCLUSIONS: Macular infarction is an uncommon but severe complication of antiphospholipid syndrome. Early and regular fundus exam in patients with antiphospholipid syndrome is necessary to avoid progression of severe ocular complications.
Adult ; Antibodies, Antinuclear ; Antiphospholipid Syndrome* ; Cesarean Section ; Chorioamnionitis ; Diagnosis ; Female ; Fingers ; Hematologic Tests ; Humans ; Infarction* ; Lupus Coagulation Inhibitor ; Macular Edema ; Ophthalmology ; Pregnancy ; Rheumatoid Factor ; Thrombophilia ; Triamcinolone Acetonide ; Visual Acuity

PURPOSE: To report a case of adult-onset Coats' disease which showed decreased retinal edema and improved visual acuity following intravitreal ranibizumab injection. CASE SUMMARY: A 21-year-old woman visited our hospital for decreased visual acuity in left eye from 3 months ago. Her best corrected visual acuity was 1.0 in the right eye, and 0.4 in the left eye. The intraocular pressure was 19 mmHg in the right eye and 16 mmHg in the left eye. At anterior segment examination, no abnormal findings were found. On fundus examination, subretinal exudates, superior retinal edema at posterior pole, and telangiectasia along superortemporal vascular arcade were observed in the left eye. Upon diagnosis as Coats' disease, intravitreal ranibizumab was performed, and laser photocoagulation was done around the retinal telangiectasia and nonperfusion area. Then, the second and third intravitreal ranibizumab injections were performed by a month, and her best visual acuity was improved to 0.8 and optical coherence tomography revealed decreased retinal edema. CONCLUSIONS: We report a case of adult-onset Coats' disease. Intravitreal ranibizumab injection is effective in rapid visual improvement and decrease of retinal edema as combination therapy with laser photocoagulation which was a generalized treatment of choice in Coats' disease.
Diagnosis ; Exudates and Transudates ; Female ; Humans ; Intraocular Pressure ; Light Coagulation ; Macular Edema ; Papilledema ; Ranibizumab* ; Retinaldehyde ; Telangiectasis ; Tomography, Optical Coherence ; Visual Acuity ; Young Adult

PURPOSE: To evaluate the influence of retinal collateral vessels in eyes with macular edema secondary to branch retinal vein occlusion (BRVO) treated with intravitreal bevacizumab (IVB). METHODS: We reviewed the medical records of patients with BRVO who were followed up for 12 months. To compare formations of collateral vessels, the patients were divided into 2 groups. The treatment group included 20 patients (20 eyes) treated with IVB, and the control group included 41 patients (41 eyes) without treatment. RESULTS: In the treatment group, the mean age was 58.4 ± 9.5 years. The average number of IVB injections performed during the 12 months was 4.5 ± 2.5 (range 2 to 8). After 12 months from diagnosis, 13 eyes (65%) presented with collateral vessels. In the control group, the mean age was 60.6 ± 9.3 years and 28 eyes (68.3%) presented with collateral vessel after 12 months. There was no difference in incidence of collateral vessel formation between the treatment group and the control group (p = 0.574). CONCLUSIONS: IVB does not influence the formation of retinal collateralization in eyes treated for macular edema secondary to BRVO.
Diagnosis ; Humans ; Incidence ; Macular Edema ; Medical Records ; Retinal Vein Occlusion* ; Retinal Vein* ; Retinaldehyde* ; Bevacizumab

PURPOSE: To evaluate the influence of retinal collateral vessels in eyes with macular edema secondary to branch retinal vein occlusion (BRVO) treated with intravitreal bevacizumab (IVB). METHODS: We reviewed the medical records of patients with BRVO who were followed up for 12 months. To compare formations of collateral vessels, the patients were divided into 2 groups. The treatment group included 20 patients (20 eyes) treated with IVB, and the control group included 41 patients (41 eyes) without treatment. RESULTS: In the treatment group, the mean age was 58.4 ± 9.5 years. The average number of IVB injections performed during the 12 months was 4.5 ± 2.5 (range 2 to 8). After 12 months from diagnosis, 13 eyes (65%) presented with collateral vessels. In the control group, the mean age was 60.6 ± 9.3 years and 28 eyes (68.3%) presented with collateral vessel after 12 months. There was no difference in incidence of collateral vessel formation between the treatment group and the control group (p = 0.574). CONCLUSIONS: IVB does not influence the formation of retinal collateralization in eyes treated for macular edema secondary to BRVO.
Diagnosis ; Humans ; Incidence ; Macular Edema ; Medical Records ; Retinal Vein Occlusion* ; Retinal Vein* ; Retinaldehyde* ; Bevacizumab

PURPOSE: To identify the factors related to repeatability of intravitreal bevacizumab injections in patients with macular edema secondary to branch retinal vein occlusion (BRVO). METHODS: The present study included 26 patients with macular edema secondary to BRVO occurring within 1 month of diagnosis. Initial best corrected visual acuity, type of BRVO according to the involved vein branch, distance between fovea and occlusion vein, central macular thickness, type of macular edema, presence of macula hemorrhage, and presence of capillary nonperfusion were evaluated at the time of diagnosis. The patients received an intravitreal bevacizumab injection at the time of diagnosis and reinjections when macular edema and visual acuity were aggravated. According to the factors considered, the reinjection-free rate which was considered a survival rate was evaluated for 1 year after the first injection. RESULTS: The patients with initial visual acuity lower than log MAR 0.5 showed 52.6% survival rate within 1 year compared with 14.3% of the patients with visual acuity higher than log MAR 0.5 (p < 0.01). The patients with occluded vein closer than a distance of 2.5 disc diameters (DD) from the foveal center had a 57.1% survival rate and the patients with occluded vein farther than a distance of 2.5 DD from the foveal center had a 25.0% survival rate (p = 0.04). Macular BRVO and major BRVO patients had 64.3% and 16.7% survival rates, respectively (p = 0.01). CONCLUSIONS: The patients with BRVO may have less chance of repetitive intravitreal bevacizumab injections due to macular edema when initial visual acuity is lower than log MAR 0.5, occluded vein is closer than 2.5 DD from the foveal center, and macular branch is involved at the initial diagnosis. These factors can be utilized to predict the prognosis of BRVO patients and the probability of repetitive intravitreal bevacizumab injections.
Capillaries ; Diagnosis ; Edema* ; Hemorrhage ; Humans ; Macular Edema ; Prognosis ; Retinal Vein Occlusion* ; Retinal Vein* ; Retinaldehyde* ; Survival Rate ; Veins ; Visual Acuity ; Bevacizumab

PURPOSE: To analyze the correlation between microperimetric parameters and optical coherent tomographic findings in the eyes of patients with macular diseases. METHODS: A total of 64 eyes were included in this retrospective cross-sectional study. Differences in the macular integrity index and microperimetric parameters were analyzed according to types of outer retinal band defects. Correlations between average threshold values and retinal thickness in the corresponding areas were analyzed. Finally, microperimetric parameters were compared between inner and outer retinal lesions. RESULTS: Measures of best-corrected visual acuity, macular integrity index, and average threshold values were significantly worse in eyes with defects in the ellipsoid portion of the photoreceptor inner segment (ISe), the cone outer segment tip (COST), and the external limiting membrane (ELM) than in eyes without ISe, COST, and ELM defects. Also, visual functionality is more significantly impacted by ELM defects than by other hyper-reflective band defects. There was a significant negative correlation between retinal thickness and the average threshold of the corresponding area in the Early Treatment Diabetic Retinopathy Study grid. Microperimetric parameters in the eyes of patients with inner retinal lesions were better than in the eyes of patients with outer retinal lesions. CONCLUSIONS: The macular integrity index may be a useful factor for reflecting the functional aspects of macular diseases. Specifically, ELM, ISe, and COST defects are significantly associated with poor retinal sensitivity and macular integrity index values that suggest abnormalities. Among eyes with these particular defects, the visual functionality of patients is most sensitively impacted by ELM defects. This finding suggests that microperimetric parameters are sensitive and useful for evaluating functional abnormalities in the eyes of patients with macular disease, particularly in patients with outer retinal pathology.
Cross-Sectional Studies ; Female ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Macula Lutea/*pathology ; Macular Edema/*diagnosis ; Male ; Middle Aged ; Retrospective Studies ; Tomography, Optical Coherence/*methods ; Visual Field Tests/*methods

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

PURPOSE: To report transient corneal edema after phacoemulsification as a predictive factor for the development of pseudophakic cystoid macular edema (PCME). METHODS: A total of 150 eyes from 150 patients (59 men and 91 women; mean age, 68.0 ± 10.15 years) were analyzed using spectral domain optical coherence tomography 1 week and 5 weeks after routine phacoemulsification cataract surgery. Transient corneal edema detected 1 week after surgery was analyzed to reveal any significant relationship with the development of PCME 5 weeks after surgery. RESULTS: Transient corneal edema developed in 17 (11.3%) of 150 eyes 1 week after surgery. A history of diabetes mellitus was significantly associated with development of transient corneal edema (odds ratio [OR], 4.04; 95% confidence interval [CI], 1.41 to 11.54; p = 0.011). Both diabetes mellitus and transient corneal edema were significantly associated with PCME development 5 weeks after surgery (OR, 4.58; 95% CI, 1.56 to 13.43; p = 0.007; and OR, 6.71; CI, 2.05 to 21.95; p = 0.003, respectively). In the 8 eyes with both diabetes mellitus and transient corneal edema, 4 (50%) developed PCME 5 weeks after surgery. CONCLUSIONS: Transient corneal edema detected 1 week after routine cataract surgery is a predictive factor for development of PCME. Close postoperative observation and intervention is recommended in patients with transient corneal edema.
Adult ; Aged ; Aged, 80 and over ; Cornea/*pathology ; Corneal Edema/*diagnosis/etiology ; Female ; Fluorescein Angiography ; Follow-Up Studies ; Fundus Oculi ; Glucosinolates ; Humans ; Macular Edema/diagnosis/*etiology ; Male ; Middle Aged ; *Phacoemulsification ; Pseudophakia/*complications/diagnosis ; Retrospective Studies ; Tomography, Optical Coherence