A pair of 19-year-old female identical twins was referred to our hospital with progressive visual loss. They exhibited bilateral chorioretinal atrophy involving the midperiphery on fundoscopy and fluorescein angiography. Bilateral visual field constriction was noted on dynamic Goldmann perimetry, and a markedly impaired response was observed on both photopic and scotopic electroretinograms. Cystoid macular edema was identified in both eyes on optical coherence tomography. Plasma levels of ornithine were elevated. Based on these observations, the patients were diagnosed with gyrate atrophy of the choroid and retina. The clinical diagnosis was confirmed by mutation analysis of the ornithine-δ-aminotransferase (OAT) gene. Patients were treated with a pyridoxine supplement (300 mg/day) and an arginine-restricted diet to lower plasma levels of ornithine, which were successfully reduced without progression of chorioretinal atrophy for 15 months. Our report describes the first case of gyrate atrophy in the Korean population diagnosed by OAT gene analysis and treated with vitamin B6 dietary supplementation.
Gene mutation; Gyrate atrophy; Hyperornithinemia; Ornithine-δ-aminotransferase deficiency; Pyridoxine
To report the surgical outcome of full-thickness sclerotomy in five cases of uveal effusion syndrome (UES). Full-thickness sclerotomy without sclerectomy was performed on five eyes of four patients with UES with or without nanophthalmos. In four of the eyes, exudative retinal detachment associated with UES resolved after the sclerotomy. The subretinal fluid in one eye, which had a normal axial length, was relieved after undergoing three sclerotomy procedures. Full-thickness sclerotomy without vortex vein decompression or sclerectomy is an effective surgical option for the management of significant UES.
Nanophthalmos; Sclerotomy; Uveal effusion syndrome
To evaluate the efficacy of vitrectomy combined with intravitreal injection of triamcinolone acetonide (IVTA) and macular laser photocoagulation for the treatment of nontractional diabetic macular edema (DME) refractory to anti-vascular endothelial growth factor (VEGF) therapy.
Twenty-eight eyes from 28 subjects who were diagnosed with nontractional DME refractory to three or more sequential anti-VEGF injections underwent sequential vitrectomy, IVTA, and macular laser photocoagulation. Changes in best-corrected visual acuity (BCVA) and central subfield thickness (CST) during the six months following vitrectomy were evaluated. Additionally, the CST and BCVA outcomes were compared with those of 26 eyes treated with the same triple therapy for nontractional DME refractory to conventional treatment, such as IVTA or macular laser photocoagulation, or both.
The mean logarithm of the minimum angle of resolution BCVAs before and one, three, and six months after vitrectomy were 0.44 ± 0.15, 0.36 ± 0.18, 0.31 ± 0.14, and 0.34 ± 0.22, respectively. The mean CSTs were 433.3 ± 77.9, 329.9 ± 59.4, 307.2 ± 60.2, and 310.1 ± 80.1 microns, respectively. The values of both BCVA and CST at one, three, and six months were significantly improved from baseline (p < 0.05). The extent of CST reduction during the first month after triple therapy was greater in eyes refractory to conventional treatment than in eyes refractory to anti-VEGF (p = 0.012).
Vitrectomy combined with IVTA and macular laser photocoagulation had a beneficial effect on both anatomical and functional outcomes in eyes with nontractional DME refractory to anti-VEGF therapy.
Anti-vascular endothelial growth factor; Diabetic macular edema; Triple therapy; Vitrectomy
This retrospective observational case series on eyes from three patients was done to elucidate the developmental mechanism of spontaneous reattachment of rhegmatogenous retinal detachment (SRRRD). The study eyes of each patients showed evidence of retinal break and diffuse retinal pigmentary change. Ultrasound biomicroscopic examination revealed vitreous fibers attached to the area around the retinal break. Posterior vitreous attachment was confirmed in each eye. A thin fibrovascular membrane incompletely sealing the retinal break was noted in one case. We suggest that the vitreous attachment around the retinal break and the sealing of the break with adjacent vitreous fibers seem to be involved in the developmental mechanism of SRRRD.
Reattachment; Rhegmatogenous retinal detachment; Vitreous detachment
To describe the clinical characteristics of idiopathic juxtafoveal telangiectasis (IJT) in Koreans.
Medical records of 16 patients with IJT were analyzed during the period from 1997 to 2009. Diagnosis was based on biomicrosopic and fluorescein angiographic findings and the group was determined according to the Gass and Blodi classification.
We analyzed eight patients in group 1A (50%), two in group 1B (12.5%), and six in group 2A (37.5%). Diverse treatment modalities, such as macular laser photocoagulation, photodynamic therapy, intravitreal antiangiogenic agent, and steroid injection, were applied for macular edema in nine eyes; however, only two eyes showed visual improvement.
In this case series, group 1A was the most common. For macular edema related to IJT, current treatment strategies had no consistent effect.
Idiopathic Juxtafoveal Retinal Telangiectasia; Intravitreal Injection; Macular edema; Photochemotherapy
To describe the clinical and optical coherence tomography (OCT) features of a macular hole (MH) or its precursor lesion in patients treated with systemic antiestrogen agents. We reviewed the medical history of the patient, ophthalmic examination, and both fundus and OCT findings. Three female patients receiving antiestrogen therapy sought treatment for visual disturbance. All of the patients showed foveal cystic changes with outer retinal defect upon OCT. Visual improvement was achieved through surgery for the treatment of MH in two patients. Antiestrogen therapy may result in MH or its precursor lesion, in addition to perifoveal refractile deposits. OCT examination would be helpful for early detection in such cases.
Antiestrogen agent; Foveal cyst; Retinal perforations; Tamoxifen; Toremifen
To assess the diagnostic efficacy of macular and peripapillary retinal thickness measurements for the staging of diabetic retinopathy (DR) and the prediction of disease progression.
In this prospective study, 149 diabetic patients (149 eyes) and 50 non-diabetic control subjects were included. Baseline optical coherence tomography was employed to measure retinal thickness in the macula (horizontal, vertical, and central) and the peripapillary zone (superior, inferior, nasal, and concentric to the optic disc). Seven baseline parameters were correlated with the DR stages identified by fluorescein angiography. Baseline retinal thickness was compared between groups of patients requiring panretinal photocoagulation (PRP) within 6 months (PRP group) and patients not requiring PRP (No-PRP group).
Macular and peripapillary retinal thicknesses in diabetic subjects were significantly greater than that in normal controls (p<0.05). All retinal thickness parameters, and particularly peripapillary circular scans, tended to increase with increasing DR severity (p<0.05). The baseline thicknesses of the peripapillary circular scans were greater in the PRP group than in the no-PRP group (p<0.05).
Peripapillary retinal thickness may prove to be a useful criterion for DR severity and may also serve as an indicator of disease progression.
Diabetic retinopathy; Laser photocoagulation; Retina; Mass screening
Choroidal detachment (CD) associated with rhegmatogenous retinal detachment (RRD) is a rare, but serious condition, which makes the prognosis worse. Previously reported risk factors for CD in RRD patients include high myopia, aphakia, pseudophakia, and advanced age. However, macular hole has not been discussed as an important factor in increasing the risk of CD in RRD patients. The purpose of this study was to evaluate macular hole as a risk factor for CD in eyes evidencing RRD.
The medical records of 480 patients with primary RRD were reviewed. We compared the CD incidence among the RRD patients in accordance with the presence or absence of macular holes. The relationship between gender, age, presence of systemic disease, refractive errors, lens status, intraocular pressure and the development of CD were also analyzed.
The incidence (4/21 eyes, 19.0%) of CD in the RRD with macular hole was significantly higher than that (7/459 eyes, 1.5%) observed in the RRD without macular hole (p=0.010). The preoperative intraocular pressure (mean±SD; 2.5±1.3 mmHg) in the RRD with CD and macular hole was significantly lower than that (7.4±4.4 mmHg) observed in the cases of RRD with CD without macular hole (p=0.035). The eyes complicated by CD evidenced a higher prevalence of diabetes mellitus (p=0.024) than was observed in the
eyes without CD.
The retinal detachment combined with macular hole creates a predisposition toward the development of profound hypotony and CD.
Choroidal detachment; Macular hole; Retinal detachment
To evaluate the short-term efficacy and safety of intravitreal bevacizumab injection (IVBI) in patients with retinal angiomatous proliferation (RAP).
Seven eyes of 5 patients with RAP were included in this study. All of the eyes evidenced stage 2 RAP lesions, except for one eye with a stage 3 lesion. IVBI (1.25 mg/0.05 cc) were conducted at 4 or 6-week intervals. Complete ocular examinations, angiographic results and optical coherence tomographic findings before and after the IVBI were analyzed at baseline and upon the follow-up visits.
Seven eyes were studied in 5 patients who had undergone IVBI. Partial (3 eyes) or complete (4 eyes) regression of RAP was noted after IVBI in all of the studied eyes. Visual acuity improved in 5 of the eyes, and was stable in 2 of the eyes. One eye evidenced severe intraocular inflammation after IVBI and a subsequent development of new RAP, which was controlled with vitrectomy and repeat IVBI.
This treatment was effective over 6 months, stabilizing or improving visual acuity and reducing angiographic leakage. These short-term results suggest that IVBI may constitute a promising therapeutic option, particularly in the early stages of RAP.
Intravitreal bevacizumab injection; Retinal angiomatous proliferation
To evaluate the effectiveness of laser photocoagulation as adjuvant therapy in the treatment of large macular holes.
A randomized clinical trial. Thirty-one eyes from 29 subjects with idiopathic macular holes of diameters larger than 400 µm were randomized into a laser group and a control group. All eyes underwent vitrectomy with peeling of the internal limiting membrane. Contrary to the control group eyes, the laser group eyes underwent laser photocoagulation at the center of the macular hole before vitrectomy. Visual acuity and anatomic outcomes assessed by optical coherence tomography (OCT) were analyzed 3 months after surgery.
On postoperative OCT, closure of the macular hole was noted in 17 of the 18 (94.4%) laser group eyes and 10 of the 13 (76.9%) control group eyes. Hole closure without bare retinal pigment epithelium was observed in 16 eyes in the laser group and 6 eyes in the control group (P<0.05). The amount of improvement in logMAR visual acuity 3 months after surgery was 0.40±0.29 in the laser group, and 0.19±0.23 in the control group (P<0.05).
Laser photocoagulation constitutes a potent adjuvant therapy that may improve anatomical and visual outcomes of surgery for macular holes larger than 400 µm.
Laser photocoagulation; Macular hole; Optical coherence tomography
We investigated the demographic characteristics and risk factors of Korean patients with naÏve central or branch retinal vein occlusion (CRVO or BRVO). This study enrolled 41 clinical sites throughout Korea and included 557 consecutive patients with retinal vein occlusion (RVO) from May through November 2010. A total of 557 patients with new-onset RVO participated in this study. Two hundred and three (36.4%) patients were diagnosed with CRVO and 354 (63.6%) patients were diagnosed with BRVO. Comparisons between the two groups showed that the prevalence of diabetes mellitus was significantly higher in CRVO patients and hypertension was significantly higher in BRVO patients (P = 0.001 and 0.002, respectively). Poor baseline visual acuity was significantly associated with female and old age in BRVO patients (P = 0.002 and 0.013, respectively), whereas the wide intraretinal hemorrhage (CRVO, P = 0.029; BRVO, P < 0.001) and the macular ischemia (CRVO, P < 0.001; BRVO, P < 0.001) were associated with both groups. The study results show the clinical features of RVO in Korean patients. Hypertension is strongly associated with BRVO and diabetes mellitus is more strongly associated with CRVO in Korean patients with RVO. As the first nationwide study performed by the Korean Retinal Society, the results of this study can be applied to future studies on RVO.
Demographic Factors; Retinal Vein Occlusions; Risk Factors
As younger preterm infants are able to survive, more extremely preterm infants are at risk of developing retinopathy of prematurity (ROP). To investigate the incidence, progression and risk factors of ROP in extremely preterm infants in Korea, the medical records of infants born before 25 weeks gestation were retrospectively reviewed. The criteria for laser treatment agreed with type 1 ROP as defined by the Early Treatment for Retinopathy of Prematurity study. Of the 121 infants included in the analysis, 119 (98.4%) infants developed any stage ROP, including 78 infants (64.5%) with type 1 ROP. The mean postmenstrual age (PMA) at the onset of any ROP and type 1 ROP were 33.5 and 36.1 weeks, respectively. All but one infant developed type 1 ROP after 31 weeks PMA. Univariate analysis showed that duration of total parenteral nutrition and onset of any ROP (PMA) were associated with the development of type 1 ROP. In conclusion, this study shows high incidence of ROP in extremely preterm infants and suggests that, although current screening protocols are feasible for most preterm infants born before 25 weeks gestation, earlier screening before 31 weeks PMA may be necessary in infants with an unstable clinical course.
Retinopathy of Prematurity; Infant, Premature; Infant, Low Birth Weight
To report the three-year outcomes of macular laser photocoagulation following intravitreal injection of triamcinolone acetonide (IVTA) for diffuse diabetic macular edema (DME).
A prospective, randomized controlled study was completed. Eighty-six eyes of 74 patients with diffuse DME were randomized into two groups. Eyes assigned to the combination group (n = 48) were subjected to macular laser photocoagulation three weeks after IVTA. Eyes in the IVTA group (n = 38) underwent IVTA alone. Central macular thickness was measured by optical coherence tomography, and the number of additional treatments and mean time to recurrence were assessed.
Thirty-seven eyes in the combination group and 26 eyes in the IVTA group completed the three-year follow-up. Recurrence of DME after initial treatment was not observed for nine of the 37 (24.3%) eyes in the combination group or for one of the 26 (3.9$) eyes in the IVTA group (p = 0.028). DME was absent for 19.9 months after treatment in the combination group compared to 10.3 months in the IVTA group (p = 0.027). The mean number of additional treatments was 0.92 in the combination group and 1.88 in the IVTA group (p = 0.001).
Results in the subset of subjects who completed the three-year follow-up demonstrated that laser photocoagulation following IVTA is more effective than IVTA monotherapy for diffuse DME. Combination therapy required fewer additional treatments and resulted in a lower recurrence rate than IVTA monotherapy.
Diabetic retinopathy; Intravitreal injections; Laser therapy; Macular edema; Triamcinolone
Acute retinal necrosis (ARN), a viral retinal disease with poor visual prognosis, following herpes simplex encephalitis (HSE) are uncommonly seen, and there has been no case yet reported of the reverse situation. We herein present the reverse situation, an immune-competent patient with HSE following ARN. A 57-year-old man who had been under steroid therapy for retinal vasculitis the prior two weeks, presented with abrupt confusion and high fever. His cerebrospinal fluid study and brain magnetic resonance imaging revealed typical HSE. Ophthalmic examination and polymerase chain reaction of the vitreous specimen revealed ARN by herpes simplex virus type 2. Intravenous acyclovir treatment improved his encephalitis symptoms and retinal necrosis. This case implies that ARN may be a risk factor for HSE and the virus may reach the brain from the eye. Inappropriate administration of a systemic steroid may exacerbate herpes viral infection in the retina, with subsequent spread to the brain.
Acute retinal necrosis syndrome; Herpetic simplex encephalitis; Herpes simplex; Steroids
To identify the correlation between preoperative optical coherence tomography (OCT) features and postoperative visual outcomes in eyes with idiopathic macular holes (MHs).
Data from 55 eyes with idiopathic MHs which had been sealed by vitrectomy were retrospectively reviewed. Correlation analysis was conducted between postoperative visual acuity (Vpostop, logarithm of the minimum angle of resolution [logMAR]) and preoperative factors, including four OCT parameters: the anticipated length (A) devoid of photoreceptors after hole closure, MH height (B), MH size (C), and the grading (D) of the viability of detached photoreceptors. Additionally, the formula for the prediction of visual outcome was deduced.
Vpostop was determined to be significantly correlated with the preoperative visual acuity (Vpreop) and OCT parameters A, C, and D (p<0.001). Based on the correlation, the formula for the prediction of Vpostop was derived from the most accurate regression analysis: Vpostop=0.248×Vpreop+1.1×10-6×A2-0.121×D+0.19.
The length and viability of detached photoreceptors are significant preoperative OCT features for predicting visual prognosis. This suggests that, regardless of the MH size and symptom duration, active surgical intervention should be encouraged, particularly if the MH exhibits good viability in the detached photoreceptor layer.
Optical coherence tomography; Photoreceptor; Visual acuity
A 43-year-old male presented with a painless left testicular mass. The pathologic diagnosis of the radical orchiectomy specimen was peripheral T-cell lymphoma, unspecified (PTCL-u). According to the Ann Arbor staging system, his initial stage was III because of the right nasopharyngeal involvement. After first-line chemotherapy with four courses of the CHOP regimen and this was followed by involved-field radiotherapy, he achieved complete remission. Two months later, disease recurred to the left ciliary body of the left eye without evidence of involvement at other sites. Although the patient received intensive chemotherapy with autologous hematopoietic stem cell transplantation, he ultimately died of leptomeningeal seeding. Because both the central nervous system (CNS) and the orbit are sanctuary sites for chemotherapy, orbital infiltration of lymphoma should prompt physicians to evaluate involvement of the CNS and to consider performing prophylactic intrathecal chemotherapy as a treatment option.
Non-Hodgkin's lymphoma; T cell lymphoma; Testes; Eye neoplasm
Intraocular (IO) retinoblastoma (RB) has traditionally been treated with enucleation (ENU) or external beam radiotherapy (EBRT). Recently, clinical trials are in progress to cure RB without ENU or EBRT in order to salvage the globe and to avoid unacceptable side effects of EBRT. We performed a pilot study to treat patients with advanced Reese-Ellsworth (RE) stage IO RB with initial chemotherapy (CRx) followed by local therapy (LT) and adjuvant CRx. Ten eyes (8 RE group V, 2 RE group IV) from 9 patients were enrolled from March 2001 to November 2001. All tumors responded to CRx. In 5 of 10 eyes, the RB was enough to be treated with LT after chemoreduction. One patient who underwent LT is waiting for ENU due to post-cryotherapy complication. For a median follow-up of 13 months (8-16 mo), 4 eyes that received LT and adjuvant CRx were relapse-free. A patient with bilateral RB who failed to be a candidate for LT was rescued with high-dose CRx and hematopoietic stem cell transplantation. Consequently, by treating patients according to our strategy, we were able to salvage 6 out of 10 eyes without ENU or EBRT. These results suggest that chemoreduction followed by LT and adjuvant CRx might offer the opportunity to salvage the globe and vision even in patients with advanced stage IO RB.
The aim of this study is to report on preliminary data regarding the prevalence of major eye diseases in Korea.
We obtained data from the Korea National Health and Nutrition Examination Survey, a nation-wide cross-sectional survey and examinations of the non-institutionalized civilian population in South Korea (n = 14,606), conducted from July 2008 to December 2009. Field survey teams included an ophthalmologist, nurses, and interviewers, traveled with a mobile examination unit and performed interviews and ophthalmologic examinations.
The prevalence of visual impairment, myopia, hyperopia and astigmatism in participants over 5 years of age was 0.4 ± 0.1%, 53.7 ± 0.6%, 10.7 ± 0.4%, and 58.0 ± 0.6%, respectively. The prevalence of strabismus and blepharoptosis in participants over 3 years of age was 1.5 ± 0.1% and 11.0 ± 0.8%, respectively. In participants over 40 years of age, the prevalence of cataract, pterygium, early and late age-related macular degeneration, diabetic retinopathy and glaucoma was 40.2 ± 1.3%, 8.9 ± 0.5%, 5.1 ± 0.3%, 0.5 ± 0.1%, 13.4 ± 1.5%, and 2.1 ± 0.2%, respectively.
This is the first nation-wide epidemiologic study conducted in South Korea for assessment of the prevalence of eye diseases by both the Korean Ophthalmologic Society and the Korea Center for Disease Control and Prevention. This study will provide preliminary information for use in further investigation, prevention, and management of eye diseases in Korea.
Epidemiology; Eye diseases; Korea; Korea National Health and Nutrition Examination Survey; Prevalence