Short-term Efficacy and Safety of Intravitreal Brolucizumab Injection for Treatment-Naive Exudate Age-related Macular Degeneration: A Multicenter Study

Purpose@#To compare short-term efficacy and safety of intravitreal brolucizumab injection with aflibercept in treatment-naive neovascular age-related macular degeneration (nAMD) patients. @*Methods@#A total of 59 eyes from 59 treatment-naive nAMD patients in three hospitals were retrospectively reviewed. Of which, 27 patients underwent intravitreal brolucizumab injections and 32 received aflibercept. After monthly consecutive three injections, best-corrected visual acuity (BCVA; in logarithm of minimal angle of resolution [logMAR]), central macular thickness (CMT), dry macula achievement rate, and intraocular inflammation (IOI) incidence were compared. @*Results@#After loading-phase treatment, BCVA was significantly increased from 0.48 ± 0.30 logMAR at baseline to 0.33 ± 0.21 logMAR at 3 months in the brolucizumab group (p = 0.002) and 0.40 ± 0.39 logMAR at baseline to 0.33 ± 0.36 logMAR at 3 months in the aflibercept group (p = 0.007). But there was no significant difference in BCVA improvement at 3 months between the two groups. CMT significantly decreased from 429.67 ± 250.59 μm at baseline to 210.67 ± 93.53 μm at 3 months in the brolucizumab group and from 346.69 ± 159.09 μm to 234.52 ± 83.42 μm in the aflibercept group (both p < 0.001). The amount of CMT reduction was significantly greater in the brolucizumab group after 3 months (p = 0.036). In typical AMD eyes, brolucizumab showed similar BCVA improvement but better CMT reduction at 3 months (p = 0.018). Dry macula achievement rate was not significantly different between the two groups. One IOI was observed in the brolucizumab group. @*Conclusions@#Intravitreal injections of brolucizumab and aflibercept showed similar anatomical and functional outcomes. But CMT reduction was greater in the brolucizumab group. One IOI was identified, which was tolerable for topical agents. These results suggest that brolucizumab could be a novel first line treatment option for treating naive nAMD patients.

Voretigene Neparvovec for the Treatment of RPE65-associated Retinal Dystrophy: Consensus and Recommendations from the Korea RPE65-IRD Consensus Paper Committee

Mutations in the RPE65 gene, associated with Leber congenital amaurosis, early-onset severe retinal dystrophy, and retinitis pigmentosa, gained growing attention since gene therapy for patients with RPE65-associated retinal dystrophy is available in clinical practice. RPE65 gene accounts for a very small proportion of patients with inherited retinal degeneration, especially Asian patients. Because RPE65-associated retinal dystrophy shares common clinical characteristics, such as early-onset severe nyctalopia, nystagmus, low vision, and progressive visual field constriction, with retinitis pigmentosa by other genetic mutations, appropriate genetic testing is essential to make a correct diagnosis. Also, fundus abnormalities can be minimal in early childhood, and the phenotype is highly variable depending on the type of mutations in RPE65-associated retinal dystrophy, which makes a diagnostic difficulty. The aim of this paper is to review the epidemiology of RPE65-associated retinal dystrophy, mutation spectrum, genetic diagnosis, clinical characteristics, and voretigene neparvovec, a gene therapy product for the treatment of RPE65-related retinal dystrophy.

Clinical Manifestations and Genetic Analysis of 5 Korean Choroideremia Patients Initially Diagnosed With Retinitis Pigmentosa

Background@#To investigate the clinical findings of choroideremia patients and perform genetic analysis by whole-exome sequencing (WES). @*Methods@#A total of 94 patients initially diagnosed with retinitis pigmentosa (RP) at another hospital, and who visited our hospital for genetic analysis by WES, were included in the study, along with 64 family members. All subjects underwent comprehensive ophthalmic evaluation, including best-corrected visual acuity, slit lamp examination, fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FAG), visual field (VF), electroretinogram (ERG), and optical coherence tomography (OCT). @*Results@#In six male patients with suspected choroideremia, extensive retinal pigment epithelium (RPE) and severe loss of choroid were observed in the fundus, but not in the macula. CHM gene mutation was confirmed in five patients. A novel single nucleotide variant at a splice site was observed in one patient. OCT showed marked thinning of the outernuclear layer and choroid, except in the macula. FAF showed a small area of hyperfluorescence in the posterior pole. In addition, characteristic interlaminar bridges were observed in four patients. On FAG, hypofluorescence was seen up to the far-peripheral retina in five patients. @*Conclusion@#Of the 94 patients initially diagnosed with RP, CHM mutation was identified in five (5.3%) by WES. Choroideremia should be considered as a differential diagnosis of RP.WES would be useful for identifying the causes of hereditary retinal disease.

Development of a Post-vitrectomy Injection of N-methyl-N-nitrosourea as a Localized Retinal Degeneration Rabbit Model

Since genetic models for retinal degeneration (RD) in animals larger than rodents have not been firmly established to date, we sought in the present study to develop a new rabbit model of drug-induced RD. First, intravitreal injection of N-methyl-N-nitrosourea (MNU) without vitrectomy in rabbits was performed with different doses. One month after injection, morphological changes in the retinas were identified with ultra-wide-field color fundus photography (FP) and fundus autofluorescence (AF) imaging as well as spectral-domain optical coherence tomography (OCT). Notably, the degree of RD was not consistently correlated with MNU dose. Then, to check the effects of vitrectomy on MNU-induced RD, the intravitreal injection of MNU after vitrectomy in rabbits was also performed with different doses. In OCT, while there were no significant changes in the retinas for injections up to 0.1 mg (i.e., sham, 0.05 mg, and 0.1 mg), outer retinal atrophy and retinal atrophy of the whole layer were observed with MNU injections of 0.3 mg and 0.5 mg, respectively. With this outcome, 0.2 mg MNU was chosen to be injected into rabbit eyes (n=10) at two weeks after vitrectomy for further study. Six weeks after injection, morphological identification with FP, AF, OCT, and histology clearly showed localized outer RD - clearly bordered non-degenerated and degenerated outer retinal area - in all rabbits. We suggest our post-vitrectomy MNU-induced RD rabbit model could be used as an interim animal model for visual prosthetics before the transition to larger animal models.

Simultaneous Recording of Flash Electroretinography and Visual Evoked Potential in Vitreous Hemorrhage with Diabetic Retinopathy

PURPOSE: To determine whether the simultaneous recording of photopic electroretinography (ERG) and flash visual evoked potential (VEP) can predict the postoperative outcome in diabetic cases where massive vitreous hemorrhage precludes fundus observation. METHODS: The photopic ERG and flash VEP were recorded simultaneously on 20 eyes of 20 normal subjects, and 23 eyes of 23 patients who were diagnosed with Grade IV vitreous hemorrhage d/t diabetic retinopathy. Of the 23 patients, fellow eyes were diagnosed with proliferative diabetic retinopathy and they underwent pars plana vitrectomy after the test. Three groups were analyzed the responses of photopic ERG and flash VEP. Best corrected visual acuity was also checked before and after the surgery. After the 8 weeks after the vitrectomy, two groups were formed, based on the outcome of surgery and these two groups were analyzed the preoperative response of photopic ERG and flash VEP. RESULTS: When comparing the groups between proliferative diabetic retinopathy and normal eyes, Grade IV vitreous hemorrhage and fellow eyes, there was a statistically significant (p < 0.05) difference in a wave amplitude, a wave implicit time, b wave amplitude, b wave implicit time of photopic ERG and P2 peak time of flash VEP. In addition, a wave amplitude of photopic ERG showed the best predictive ability (area under receiver operating characteristic [AUROC] curve value of 0.88) when comparing improved visual acuity group to the unimproved visual acuity group. CONCLUSIONS: Simultaneous recordings of photopic ERG and flash VEP showed the decreased function of retina and optic pathway on eyes with vitreous hemorrhage precluding inspection of the fundus. In addition, preoperative photopic ERG and flash VEP can safely predict the outcome of vitrectomy in dense vitreous hemorrhage of diabetics.

Nestin Expression in the Adult Mouse Retina with Pharmaceutically Induced Retinal Degeneration

The present study investigated the temporal pattern and cellular localization of nestin in the adult mouse retina with pharmaceutically induced retinal degeneration using N-methyl-N-nitrosourea (MNU). After a single intraperitoneal injection of MNU in 8-week-old C57BL/6 mice, the animals were sacrificed at 1, 3, 5, 7, and 21 days (n = 6, in each stage). The eyes were examined by means of immunohistochemical tests using nestin, ionized calcium-binding adaptor molecule (Iba-1), CD11b, F4/80, and glial fibrillary acidic protein (GFAP). Western blot analysis and manual cell counting were performed for quantification. Nestin expression was increased after MNU administration. Nestin+/Iba-1+ cells were migrated into outer nuclear layer (ONL) and peaked at day 3 post injection (PI). Nestin+/CD11b+ cells were also mainly identified in ONL at day 3 PI and peaked at day 5. Nestin+/F4/80+ cells were shown in the subretinal space and peaked at day 3 PI. Nestin+/GFAP+ cells were distinctly increased at day 1 PI and peaked at day 5 PI. The up-regulation of nestin expression after MNU administration in adult mouse retinal microglia, and monocyte/macrophage suggests that when retinal degeneration progresses, these cells may revert to a more developmentally immature state. Müller cells also showed reactive gliosis and differentiational changes.

Preferences and Trends in the Treatment of Diabetic Retinopathy in Korea and Japan

PURPOSE: To evaluate preferences and trends in the management of diabetic retinopathy in Korea and Japan. METHODS: An Internet survey comprised of 49 questions was sent to the members of Korean Retina Society (KRS) and Japanese Society of Ophthalmic Diabetology (JSOD). The survey was conducted during the period between June 2012 and July 2012. RESULTS: Ninety-one of 210 members of the KRS (43%) and 120 of 754 members of the JSOD (16%) participated in the survey. For diffuse diabetic macular edema, 'intravitreal injection of anti-vascular endothelial growth factor (anti-VEGF) followed by focal laser treatment' was the most preferred treatment in Korea (48%), while 'sub-tenon steroid injection followed by focal laser treatment' was the most common procedure in Japan (33%). Vitrectomy was the second most common procedure in Japan (18%). In contrast, none of the KRS members preferred vitrectomy in this situation. For refractory diabetic macular edema, however, vitrectomy with or without the use of anti-VEGF was chosen in 75% of the KRS members. In Japan, vitrectomy without the use of intravitreal injection of anti-VEGF or steroid was relatively more preferred. Small-gauge (G) vitrectomy using either a 23 G or 25 G needle was popular in both countries (90% in Korea, 64% in Japan). CONCLUSIONS: Although the trends in diagnostic and surgical environments were similar in Korea and Japan, the preferred treatment approaches for diabetic macular edema were different. Sub-tenon steroid injection and vitrectomy were preferred in Japan, while anti-VEGF injection was most commonly employed in Korea.

Clinical Usefulness of Fresnel Ganzfeld Stimulator

PURPOSE: To compare electroretinogram (ERG) waveforms acquired using a ganzfeld stimulator and a Fresnel ganzfeld stimulator. METHODS: ERGs were recorded with a ganzfeld stimulator and a Fresnel ganzfeld stimulator from both eyes of 25 volunteers. Peak-to-trough amplitudes and peak implicit times were compared between both eyes and between a ganzfeld stimulator and a Fresnel ganzfeld stimulator. ERGs taken from the sitting and supine positions were also compared using the Fresnel ganzfeld stimulator. RESULTS: There was no statistically significant difference between the Fresnel ganzfeld stimulator and dome-shaped ganzfeld stimulator in amplitude and implicit time of dark adapted 0.01, 3.0 ERG, photopic 3.0 ERG, photopic 3.0 flicker. The differences in amplitude and implicit time between the right and left eyes were not influenced by the Fresnel ganzfeld stimulator. Additionally, no differences were observed in ERGs obtained from the sitting and supine positions using the Fresnel ganzfeld stimulator. CONCLUSIONS: The newly developed ganzfeld stimulator with a sealed Fresnel lens can be considered as a reliable alternative method for measuring ERGs. Fresnel ganzfeld stimulator is useful for patients having problems with mobility or uncooperative children.

Two Cases of Retinal Hemorrhage in Alcoholic Cirrhosis

PURPOSE: To report 2 cases of retinal hemorrhage due to anemia and thrombocytopenia in patients with alcoholic cirrhosis. CASE SUMMARY: (Case 1) A 45-year-old female with alcoholic cirrhosis who was treated in the gastroenterology department presented with reduced vision in both eyes. Fundus examination showed multiple preretinal and subretinal hemorrhages with macular involvement in both eyes. Hematological findings revealed severe anemia and thrombocytopenia. One month after the transfusion treatment her visual acuity was improved and retinal hemorrhages resolved. (Case 2) A 47-year-old male presented with painless loss of vision in the left eye 3 days after orthotopic liver transplantation for the treatment of alcoholic cirrhosis. Fundus examination showed preretinal hemorrhages in both eyes with macular involvement in the left eye. During the transplantation, hematological findings revealed severe anemia and thrombocytopenia. Three months after the transfusion treatment his visual acuity was improved and retinal hemorrhages nearly completely resolved. CONCLUSIONS: Hematological abnormalities due to alcoholic cirrhosis can cause retinal hemorrhage. In the present cases the retinal hemorrhages were resorbed and the visual acuity recovered.

Spectral-Domain Optical Coherence Tomography Findings of Vogt-Koyanagi-Harada Disease

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.

Combined Operation Assosciated with Ahmed Glaucoma Valve Implantation

PURPOSE: To evaluate surgical results after combined operation associated with Ahmed glaucoma valve (AGV) implantation. METHODS: A retrospective review was performed for 71 eyes of 67 patients who underwent combined operation associated with AGV implantation from June 2003 to August 2012. RESULTS: Pars plana vitrectomy or phacoemulsification combined with AGV implantation were performed for 16 eyes and AGV implantation alone was performed in 55 eyes. Mean IOP (mm Hg) and the number of antigluacoma agents for both the combined and single groups showed a statistically significant decrease (combined/single; p = 0.008/0.000, 0.002/ 0.000). Visual acuity (log MAR) was improved, but these differences were not statistically significant (combined/single; p = 0.309/0.052). CONCLUSIONS: Combined vitrectomy or phacoemulsification and AGV implantation for intractable glaucoma with vitreoretinal disease or cataract is considered to be the primary procedure because of its equivalent efficacy in lowering IOP, improving visual acuity and decreasing the number of glaucoma agents.

Expression Profiles of F4/80 and Nestin in Ocular Immune Cells Following Pharmaceutically Induced Retinal Degeneration in Adult Mice

PURPOSE: To evaluate the expression patterns of F4/80 and nestin in the ciliary body and the optic nerve following N-methyl-N-nitrosourea (NMU)-induced retinal degeneration in adult mice. METHODS: After intraperitoneal injection of MNU (60 mg/kg) in adult mice, the eyes were enucleated at 2, 4, 7 and 30 days. Hematoxylin and eosin (H&E) stain, terminal deoxyribonucleotidyl transferase-mediated dUTP nick end labeling (TUNEL) stain and immunohistochemical stains of F/80 and nestin were performed. RESULTS: After MNU treatment, the photoreceptors were destroyed by cell apoptosis. According to immunohistochemistry, F4/80 and nestin were not co-expressed in the control group, but F4/80 was expressed within the ciliary body and optic nerve in the MNU-treated group; the expression of nestin also increased. In the outer nuclear layer, F4/80 and nestin co-expressing cells were observed. CONCLUSIONS: In response to retinal damage, the F4/80 and nestin co-expressing cells migrated to the retina from the ciliary body and optic nerve and were activated.

Specific Spectral Domain Optical Coherence Tomographic Findings of Oguchi Disease

PURPOSE: To report specific spectral domain OCT findings of Oguchi disease diagnosed with fundoscopic examination and electrophysiological study. CASE SUMMARY: A 14-year-old patient visited our clinic with a complaint of night blindness for ten years. Fundoscopic examination showed a golden-yellow fundus reflex. After three hours of dark adaptation, the fundus color returned to normal (Mizuo-Nakamura phenomenon). In full-field ERG, rod b-wave was not detectable. The a-wave amplitude in maximal combined response increased after three hours of dark adaptation, although the b-wave amplitude was similar to the amplitude before dark adaptation, demonstrating a negative waveform. In the spectral domain OCT images of the perifoveal area, no gap between the retinal pigment epithelium and the inner segment/outer segment (IS/OS) junction was detected before prolonged dark adaptation, and a highly reflective band was shown. However, the gap appeared after three hours of dark adaptation, and two highly reflective bands were detected in the OCT images. CONCLUSIONS: The characteristic OCT finding in addition to the specific fundoscopic finding and full-field ERG results may be useful to diagnose Oguchi disease.

A Single Isolated Cotton-Wool Spot not Associated with Systemic Diseases

PURPOSE: To present a case of a single isolated cotton-wool spot in a healthy patient. CASE SUMMARY: A 43-year-old woman with no systemic disease complained of sudden inferior field defect of the right eye. On fundoscopic examination of the right eye, a single soft white spot was observed in the superotemporal vascular arcade. An elevated hyper-reflective area in the inner retina corresponding to a white spot was observed on optical coherence tomography. Automated perimetry revealed an arcuate field defect which was wider than the cotton-wool spot. A complete workup for systemic diseases was within normal range except a positive rheumatoid factor. At the 2-week follow-up, the patient's subjective symptom improved and the white spot decreased. After 6 months, the cotton-wool spot disappeared and slit-like retinal nerve fiber layer (RNFL) defects corresponding to the location of the cotton-wool spot was noticed. One year later, disc and RNFL defect were unchanged but the previous field defect disappeared. CONCLUSIONS: Isolated cotton-wool spots can occur without serious systemic diseases in healthy patients. A single isolated cotton-wool spot in the present case disappeared spontaneously leaving permanent structural damage on the retina over time.

A Single Isolated Cotton-Wool Spot not Associated with Systemic Diseases

PURPOSE: To present a case of a single isolated cotton-wool spot in a healthy patient. CASE SUMMARY: A 43-year-old woman with no systemic disease complained of sudden inferior field defect of the right eye. On fundoscopic examination of the right eye, a single soft white spot was observed in the superotemporal vascular arcade. An elevated hyper-reflective area in the inner retina corresponding to a white spot was observed on optical coherence tomography. Automated perimetry revealed an arcuate field defect which was wider than the cotton-wool spot. A complete workup for systemic diseases was within normal range except a positive rheumatoid factor. At the 2-week follow-up, the patient's subjective symptom improved and the white spot decreased. After 6 months, the cotton-wool spot disappeared and slit-like retinal nerve fiber layer (RNFL) defects corresponding to the location of the cotton-wool spot was noticed. One year later, disc and RNFL defect were unchanged but the previous field defect disappeared. CONCLUSIONS: Isolated cotton-wool spots can occur without serious systemic diseases in healthy patients. A single isolated cotton-wool spot in the present case disappeared spontaneously leaving permanent structural damage on the retina over time.

Vogt-Koyanagi-Harada disease occurring during pegylated interferon-alpha2b and ribavirin combination therapy for chronic hepatitis C / 대한간학회지

Vogt-Koyanagi-Harada (VKH) disease is a multisystem syndrome characterized by ocular (uveitis and retinal detachment), neurological (headache, tinnitus, and meningitis), and integumentary (vitiligo, alopecia, and poliosis) involvement. Although the pathogenesis of VKH disease is not well understood, an autoimmune T-cell response to a melanocyte-associated antigen is considered to be a cause of VKH disease. The complex immunological response to interferon and ribavirin may induce or exacerbate the autoimmune condition; however, VKH disease is a very rare complication associated with interferon therapy in chronic hepatitis C. We report a case of VKH disease occurring during pegylated interferon-alpha2b and ribavirin combination therapy for chronic hepatitis C.

Electroretinographic Assessment of the Retinal Function during Intravitreal Bevacizumab Injections in Central Retinal Vein Occlusion

PURPOSE: To investigate the changes of electroretinogram (ERG) at baseline and during the first 3 injections of intravitreal bevacizumab in central retinal vein occlusion (CRVO) patients. METHODS: Thirteen eyes of 13 CRVO patients who received 3 injections of intravitreal bevacizumab at 6-week intervals and who could be examined for visual acuity, optical coherence tomography and ERG at 4 weeks after each injection were included in the present study. In addition, the ERG results of the unaffected fellow eyes group at 4 weeks after each injection were compared with the CRVO eyes group. RESULTS: Amplitudes of rod b wave, a and b waves of maximal combined response and cone response, oscillatory potentials and 30-Hz flicker significantly decreased in the CRVO eyes group than the fellow eyes group at 4 weeks after each injection (p 0.05). CONCLUSIONS: After intravitreal bevacizumab injection, macular functions significantly improved and amplitudes of all ERG parameters gradually increased in CRVO patients.

Postoperative Visual Field Outcomes in Patients Showing Visual Field Defects due to Pituitary Adenoma

PURPOSE: To evaluate visual field (VF) changes in patients with pituitary adenoma after surgical treatment. METHODS: The present study retrospectively evaluated 96 eyes of 48 patients with pituitary adenoma who received surgical tumor removal between July 2001 and February 2010. Preoperative and postoperative clinical data including age, tumor volume, logMAR BCVA, surgical technique (transsphenoidal surgery and transcranial surgery), static perimetry scores (mean deviation [MD], pattern standard deviation [PSD], and visual field defect [VFD] scores) were reviewed. RESULTS: The MD (15.79%, p = 0.001) and PSD (3.98%, p = 0.003) improved postoperatively (mean postoperative follow-up period 1.85 months). Transsphenoidal surgery for tumor removal showed significant MD (26.99%, p = 0.000) and PSD (12.92%, p = 0.003) improvements. A multivariate regression analysis of the transsphenoidal surgery patient group revealed that the preoperative MD was related to the postoperative MD (Pearson = 0.762, p = 0.000), but negatively correlated to the amount of postoperative improvement in MD score (Pearson = -0.231, p = 0.046). Transcranial surgery did not significantly improve the MD (p = 0.419), PSD (p = 0.562), VFD score (p = 0.135), or logMAR BCVA (p = 0.708). CONCLUSIONS: Visual filed defects in patients with pituitary adenoma improved after neurosurgical treatment. Better postoperative visual field outcomes were achieved in patients who had smaller preoperative visual field defects. Transsphenoidal surgery significantly improved the visual field defects and visual acuity in patients with non-functioning pituitary adenoma, compared to the transcranial surgery patients.

Use of Spectral-Domain Optical Coherence Tomography to Analyze Macular Thickness According to Refractive Error

PURPOSE: To investigate the correlation of macular retinal thickness and refractive error using spectral-domain optical coherence tomography (SD-OCT). METHODS: A total of 120 eyes with no posterior abnormalities were enrolled in the present study. Subjects were divided into 3 groups based on their spherical equivalent. Visual acuity, refraction, slit lamp examination, tonometry and fundus examination were performed. Retinal thickness between the RPE and IS/OS junction was measured at the fovea, 1 mm (inner ring) and 2 mm (outer ring) superiorly, inferiorly, nasally and temporally using SD-OCT. Overall average thickness, average foveal thickness, and the inner and outer ring macular thickness were measured. RESULTS: The average foveal thickness was significantly greater in the high myopic eyes than in the low to moderate myopic and emmetropic eyes (p = 0.001). However, the RPE-IS/OS junction thickness of the foveola and the outer macular thickness were significantly lower (p = 0.001, p = 0.002) in the high myopic eyes. There was a weak, but significant negative correlation between refractive error and average foveal thickness (r = -0.38, p = 0.001). A positive correlation was found between refractive error and the RPE-IS/OS junction thickness (r = 0.40, p = 0.001). CONCLUSIONS: Macular retinal thickness is related to refractive error in normal subjects. Effects of eyeball elongation are more apparent in high myopic eyes than in low to moderate myopic eyes. A significant decline in the RPE-IS/OS junction thickness suggests the photoreceptor outer segments in the foveola are damaged in high myopic eyes.

Leukemic Infiltration of the Optic Nerve Head as the Initial Manifestation of Leukemic Relapse

PURPOSE: To present a case of leukemic infiltration of the optic nerve head as the initial manifestation of leukemic relapse. CASE SUMMARY: A 65-year-old woman was diagnosed with acute myeloid leukemia. Complete remission was achieved after 4 complete courses of chemotherapy. She complained of a sudden decrease in visual acuity in her left eye. Fundus examination showed severe optic disc edema with peripapillary hemorrhage and serous retinal detachment. Visual acuity and fundus continued to aggravate and high-dose intravenous steroid therapy was instituted. Visual acuity and fundus deteriorated more after treatment. Brain magnetic resonance imaging and CSF study were normal but intrathecal chemotherapy and focal irradiation were performed on account of the suspected CNS involvement of leukemia. Morphologic improvement in the retinal structure was achieved, however, optic atrophy remained and her vision did not recover. CONCLUSIONS: The present case shows the involvement of the optic nerve head as the initial isolated manifestation for the relapse in a patient with complete remission. CNS involvement is rare in acute myeloid leukemia and in particular, the optic nerve is rarely reported as the initial isolated presentation for the relapse. Moreover, the disease progression relatively aggravated after treatment. In the atypical aspects of leukemic relapse, the present case was noticeable.