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.

Non-Arteritic Ischemic Optic Neuropathy Following COVID-19 Vaccination in Korea: A Case Series

Background@#To report the clinical manifestations of non-arteritic anterior ischemic optic neuropathy (NAION) cases after coronavirus disease 2019 (COVID-19) vaccination in Korea. @*Methods@#This multicenter retrospective study included patients diagnosed with NAION within 42 days of COVID-19 vaccination. We collected data on vaccinations, demographic features, presence of vascular risk factors, ocular findings, and visual outcomes of patients with NAION. @*Results@#The study included 16 eyes of 14 patients (6 men, 8 women) with a mean age of 63.5 ± 9.1 (range, 43–77) years. The most common underlying disease was hypertension, accounting for 28.6% of patients with NAION. Seven patients (50.0%) had no vascular risk factors for NAION. The mean time from vaccination to onset was 13.8 ± 14.2 (range, 1–41) days. All 16 eyes had disc swelling at initial presentation, and 3 of them (18.8%) had peripapillary intraretinal and/or subretinal fluid with severe disc swelling. Peripapillary hemorrhage was found in 50% of the patients, and one (6.3%) patient had peripapillary cotton-wool spots. In eight fellow eyes for which we were able to review the fundus photographs, the horizontal cup/ disc ratio was less than 0.25 in four eyes (50.0%). The mean visual acuity was logMAR 0.6 ± 0.7 at the initial presentation and logMAR 0.7 ± 0.8 at the final visit. @*Conclusion@#Only 64% of patients with NAION after COVID-19 vaccination have known vascular and ocular risk factors relevant to ischemic optic neuropathy. This suggests that COVID-19 vaccination may increase the risk of NAION. However, overall clinical features and visual outcomes of the NAION patients after COVID-19 vaccination were similar to those of typical NAION.

Surgical Outcomes of Nonadjustable Modified Harada-Ito Surgery

Purpose@#This study aimed to investigate the surgical outcomes of nonadjustable Harada-Ito surgery under general anesthesia. @*Methods@#Twenty-two patients who underwent nonadjustable modified Harada-Ito surgery under general anesthesia were reviewed retrospectively. Among them, 21 out of the 22 patients who were followed up for 6 months after surgery were included in this study. Subjective cyclotorsion (double Maddox rod test) and objective cyclotorsions (fundus photography) were measured. Success of the surgery was defined as follows: success (the patients do not acknowledge diplopia at any direction), partial (the patients feel diplopia at a specific direction, but they do not feel discomfort in routine life), and fail (the patients feel diplopia in primary gaze, hence requiring a thorough investigation). @*Results@#The mean age of the patients (18 male and 3 female) was 56.5 years (range, 40–77 years). Based on the alternate prism cover test, the patients had 4.2 ± 3.0 prism diopters of vertical deviation. The corrected amounts of cyclotorsion based on the double Maddox rod test and fundus photography were 14.8° ± 7.5° and 9.8° ± 7.9°, respectively, and were significantly different between the two methods (p = 0.006). After the surgery, 20 out of the 21 patients (95.2%) completely recovered from diplopia in the primary gaze. However, among the 20 patients, seven complained of diplopia in the secondary gaze (down gaze, four patients; head tilt gaze, three patients). The success group had a smaller preoperative subjective excyclotorsion than the partial and fail groups (12.6° ± 2.5° and 21.0° ± 8.9°, respectively; p = 0.046). @*Conclusions@#Nonadjustable modified Harada-Ito surgery under general anesthesia has favorable success rate, and preoperative subjective excyclotorsion can be a prognostic factor in patients with bilateral superior oblique palsy.

The Effect of Physical Eye Examinations on Job Options

Purpose@#To investigate physical ophthalmological parameters relevant to job choice. @*Methods@#I analyzed the ophthalmological demands imposed on those engaged in military service and on applicants for army officer, air force officer, naval officer, railroad officer, firefighting officer, police officer, state public officer, and pilot positions; the visual requirements for a driver’s license were also examined. Visual dysfunction was classified into loss of visual acuity, visual field defects, refractive errors, color vision disorders, strabismus, and other disorders. Additional restrictions imposed by each occupation were investigated. @*Results@#Various standards were imposed on the occupational groups. Applicants to the Air Force Academy required 1.0 visual acuity; the visual acuity for applicants to the military and naval academies were ≥0.6, and those for fire and police personnel ≥0.8. The general public official regulations do not mention visual acuity. In terms of visual field defects, such defects must be <33% for firefighters and railroad drivers; no clear standard was set for other occupations. The importance of color vision differed by job type; pilots and railroad drivers must have full color vision. In terms of refractive error and strabismus, the standards differed among occupations. @*Conclusions@#As the ophthalmological standards vary extensively by occupation, both ophthalmologists and clients must be aware of this information. The scientific basis of the various standards requires attention.

Serial Culture of Eyedrops: Room Temperature vs. under Refrigeration

Purpose@#We investigated the serial culture of multiuse eye drops applied by medical personnel by intentionally touching the tip of the bottle. @*Methods@#Ten different unpreserved and preserved eye drops including eye drops for allergies, artificial tears, antibiotics, and corticosteroids were included. One bottle was stored at room temperature and the other bottle stored under refrigeration. The experimenter intentionally touched the tip of the bottle with a finger twice daily. The first culture test was performed 2 weeks after contamination and the culture were performed six times; 2 weeks apart. @*Results@#Contamination was noted in three types of eye drops (artificial tears without preservatives, corticosteroids, and anti-allergic eye drops) under refrigerated conditions and four types of eye drops (artificial tears without preservatives, artificial tears with preservatives, corticosteroids, and anti-allergic eye drops) under room temperature conditions. The contamination in 13 eye drops was attributable to five microbial species: Staphylococcus epidermidis (m/c), Staphylococcus hominis, Rhodotorula sp., Corynebacterium sp., and Bacillus sp. Under refrigerated conditions, three eyedrop bottles were contaminated, with five positive cultures in 12 weeks. However, at room temperature, four eye drop bottles were contaminated with eight positive cultures. The culture results changed according to culture time. In some cases, the same result was obtained 2 weeks after the first positive culture but in other cases the result was negative or one of two strains disappeared. @*Conclusions@#Eye drops can be contaminated under both room temperature and refrigerated conditions. We should consider serial culture for eye drops because the culture results could be variable according to time.

The 2016 Korea National Health and Nutritional Examination Survey of the Prevalence of Amblyopia

Purpose@#To investigate the prevalence of amblyopia in children between the ages of 5-8 years based on the Korean National Health and Nutrition Examination Survey (2016). @*Methods@#Based on the results of the Korean National Health and Nutrition Examination Survey conducted in 2016, among a total of 443 children, we measured and analyzed the visual acuities of 420 children. The visual acuity was measured using a conventional visual acuity chart. When the uncorrected visual acuity was < 0.8 in the first visual acuity test, a refraction test was performed using the automatic refraction test (KR-8800, Topcon, Tokyo, Japan) and the corrected visual acuity was measured. Unilateral amblyopia and bilateral amblyopia were defined as a ≥ 2 line difference between two eyes and visual acuity in the better eye < 0.8 or < 0.63. The significant refractive errors were defined as follows: the spherical lens was 1.5 diopters (D) or more, astigmatism was 1.0 D or more, or astigmatism of 2.5 D was 4.0 D or more. @*Results@#The prevalence of amblyopia was 5.0% (21 patients, < 0.8 in the better eye) and 3.8% (16 patients, < 0.63 in the better eye) in a total of 420 patients (male:female = 219:201). The prevalence of amblyopia in different age groups was: 10 (10%) at 5 years of age, 6 (5.6%) at 6 years of age, 3.8% at 7 years of age, and 0.9% at 8 years of age. Nine out of 21 patients (42.9%) had significant refractive errors. The regional distribution was highest in Gangwon-do and Chungcheongnam-do provinces and parental income did not affect the prevalence of amblyopia (p = 0.189). @*Conclusions@#The prevalence of amblyopia decreased with age suggesting that early detection and treatment of amblyopia are important. Therefore, systematic eye examinations that can detect amblyopia in the early stage may be important in the National Health Care results.

Prevalence of Low Vision in 2017 Based on Korea National Health andNutritional Examination Survey

Purpose@#To investigate the prevalence of low vision and legal blindness based on the Korea National Health and NutritionalExamination Survey (2017). @*Methods@#Based on the results of the National Health and Nutritional Examination Survey conducted in 2017, we analyzed 3,160patients, 40-80 years of age, and measured the visual acuity among the total of 8,127 patients. The visual acuity was measuredusing Jin’s vision chart (for 4 m). When the uncorrected visual acuity was less than 0.8 in the first visual acuity test, vision wasre-evaluated with correction using autorefractor. The analyses of the prevalence of low vision and legal blindness were based onthe criteria of the World Health Organization: moderate visual impairment, 0.32-0.125; severe visual impairment, 0.1-0.025; andlegal blindness, <0.025 in the better eye. We analyzed the occupational reclassification and unemploymentoneconomic activitypopulation status codes of the questionnaire surveys. @*Results@#The prevalence of low vision included 46 (1.46%) patients among a total of 3,160 patients (mean age, 59.4 ± 11.8 years;male:female, 1,328:1,832) and visual impairment including low vision and legal blindness involved 74 (2.34%) patients.Seventy-six percent of the low vision patients were not designated with legal disabilities. In addition, 61.5% of the patients withlow vision and 75.0% of the legally blind patients were unemployed. @*Conclusions@#Based on these results, it is necessary to study the precise condition of the low vision and legally blind patients,and to conduct comprehensive treatments for patients who cannot be covered by the Welfare Act for the Disabled.

The Clinical Course of Superior Oblique Tuck Surgery in Patients with Unilateral Superior Oblique Palsy

PURPOSE: To evaluate the effects of surgery depending on the follow-up duration after superior oblique tuck was performed as the first surgery in unilateral superior oblique palsy patients. METHODS: Sixteen patients who were followed-up for a minimum of 3 months were retrospectively evaluated. The vertical deviation, abnormal head posture, superior oblique underaction, and inferior oblique overaction were evaluated before and at 3, 6, and 12 months after the surgery and at the last follow-up. The angle between the center of the optic disc and fovea (disc-fovea angle) was measured using fundus photography to investigate changes in ocular torsion. RESULTS: The mean follow-up period was 24.9 ± 21.9 months and the mean tuck was 11.4 ± 4.0 mm. Vertical deviation <7 prism diopters in the primary position was observed in 53.9% of patients at 3 months postoperatively, 50.0% at 6 months, 83.3% at 12 months, and 62.5% at the last follow-up (p = 0.55). Head posture was improved in 66.7% of patients at 3 months, 71.4% at 6 months, 50% at 12 months, and 80% at the last follow-up after surgery (p = 0.73). Ocular torsion was decreased in 37.5% of patients at 3 months postoperatively, 66.7% at 6 months, 75% at 12 months, and 80.0% at the last follow-up (p = 0.11). Superior oblique underaction was improved in 100%, 77.8%, 60%, and 75% of the patients and inferior oblique overaction was improved in 100%, 88.9%, 85.7%, and 81.3% of the patients at postoperative month 3, 6, and 12, and at the last follow-up, respectively. CONCLUSIONS: Superior oblique tuck resulted in the maintenance of an improved condition of patients at 3, 6, and 12 months postoperatively, and there was no significant difference in motor measurements between the follow-up periods.

Surgical Treatment of Sensory Cyclic Esotropia

No abstract available.

Horizontal Effects of 10-mm Inferior Oblique Recession versus 14-mm Inferior Oblique Recession

PURPOSE: The aim of this study was to investigate the quantitative effect of inferior oblique (IO) 10- and 14-mm recession on postoperative horizontal deviation. METHODS: Patients (22 men and 18 women) who underwent IO recession were divided into two groups for comparison studies: group 1 (10-mm IO recession, 15 patients) and group 2 (14-mm IO recession, 25 patients). Preoperative and postoperative horizontal deviations were measured, and the resulting horizontal deviations from the 10- and 14-mm IO recession surgeries were compared. The effects of superior oblique underaction, IO overaction, and combined exodeviation on postoperative horizontal deviation were analyzed. RESULTS: Although group 1 did not show a significant horizontal deviation change after surgery (1.9 ± 4.5 prism diopters [PD], p = 0.452), group 2 had a meaningful horizontal change after 14-mm recession (2.2 ± 3.8 PD, p = 0.022). Both groups showed a significant esodrift in horizontal deviation (group 1, p = 0.017; group 2, p = 0.030) in patients with exodeviation over 8 PD. The mean change in horizontal deviation was 6.0 ± 5.4 PD for group 1 and 9.0 ± 5.0 PD for group 2. Although the amount of superior oblique underaction did not affect the extent of change in horizontal deviation, patients with severe IO overaction showed a significant change in horizontal deviation after 14-mm IO recession. CONCLUSIONS: Fourteen-millimeter IO recession could make a statistically significant change in horizontal deviation after surgery. In addition, esodrift should be considered after IO recession in patients with a preoperative exodeviation greater than 8 PD or severe IO overaction.

Cycloplegic Refraction in Hyperopic Children: Effectiveness of a 0.5% Tropicamide and 0.5% Phenylephrine Addition to 1% Cyclopentolate Regimen

PURPOSE: To evaluate the effectiveness of a cycloplegic regimen using 0.5% tropicamide and 0.5% phenylephrine (Tropherine, Hanmi Pharm), in addition to 1% cyclopentolate, in hyperopic children. METHODS: The medical records of hyperopic patients below the age of 14 years who had undergone cycloplegic retinoscopy were retrospectively reviewed. Cycloplegic refractions were performed using one of two cycloplegic regimens. Regimen 1 was a Tropherine-added regimen comprising the administration of one drop of 1% cyclopentolate followed by two to three drops of Tropherine added at 15-minute intervals. Regimen 2 was a cyclopentolate-only regimen comprising the administration of three to four drops of 1% cyclopentolate at 15-minute intervals. The mean difference between noncycloplegic and cycloplegic refraction was compared between the two regimens. RESULTS: A total of 308 eyes of 308 hyperopic children were included. The mean difference (±standard deviation) in the spherical equivalent (SE) between cycloplegic and noncycloplegic refraction was significantly larger in regimen 2 than in regimen 1, with values of +1.70 ± 1.03 diopters (D) and +1.25 ± 0.89 D, respectively (p=0.001). The SE change after cycloplegia was significantly different between the two regimens only in patients aged 5 years or younger (p=0.001), particularly in those with high hyperopia with an SE ≥5 D (p=0.005) or fully accommodative esotropia (p=0.009). There was no significant difference between the two regimens in patients older than 5 years, regardless of the presence of high hyperopia or fully accommodative esotropia. CONCLUSIONS: The Tropherine-added regimen exerted a weaker cycloplegic effect than the cyclopentolate-only regimen, particularly in children under the age of 5 years with high hyperopia or fully accommodative esotropia. However, the difference in refraction between the two regimens was small. A Tropherine-added regimen can be effective in hyperopic children, with less associated discomfort than the instillation of cyclopentolate.

Analysis of Fundus Photography and Fluorescein Angiography in Nonarteritic Anterior Ischemic Optic Neuropathy and Optic Neuritis

PURPOSE: We evaluated fundus and fluorescein angiography (FAG) findings and characteristics that can help distinguish nonarteritic anterior ischemic optic neuropathy (NAION) from optic neuritis (ON). METHODS: Twenty-three NAION patients and 17 ON with disc swelling patients were enrolled in this study. We performed fundus photography and FAG. The disc-swelling pattern, hyperemia grade, presence of splinter hemorrhages, cotton-wool spots, artery/vein ratio and degree of focal telangiectasia were investigated. The FAG findings for each patient were compared with respect to the following features: the pattern of disc leakage in the early phase, arteriovenous (artery/vein) transit time (second), and the presence and pattern of the filling delay. RESULTS: Cotton-wool spots, focal telangiectasia, and venous congestion were more common in the affected eyes of NAION patients. Upon FAG, 76.5% of the patients in the ON group exhibited normal choroidal circulation. However, 56.5% of patients in the NAION group demonstrated abnormal filling defects, such as peripapillary, generalized, or watershed zone filling delays. CONCLUSIONS: Fundus findings, including cotton-wool spots, focal telangiectasia, and venous congestion in the affected eye, may be clues that can be used to diagnose NAION. In addition, choroidal insufficiencies on FAG could be also helpful in differentiating NAION from ON.

Differences between Non-arteritic Anterior Ischemic Optic Neuropathy and Open Angle Glaucoma with Altitudinal Visual Field Defect

PURPOSE: To investigate the differences in retinal nerve fiber layer (RNFL) change and optic nerve head parameters between non-arteritic anterior ischemic optic neuropathy (NAION) and open angle glaucoma (OAG) with altitudinal visual field defect. METHODS: Seventeen NAION patients and 26 OAG patients were enrolled prospectively. The standard visual field indices (mean deviation, pattern standard deviation) were obtained from the Humphrey visual field test and differences between the two groups were analyzed. Cirrus HD-OCT parameters were used, including optic disc head analysis, average RNFL thickness, and RNFL thickness of each quadrant. RESULTS: The mean deviation and pattern standard deviation were not significantly different between the groups. In the affected eye, although the disc area was similar between the two groups (2.00 +/- 0.32 and 1.99 +/- 0.33 mm2, p = 0.586), the rim area of the OAG group was smaller than that of the NAION group (1.26 +/- 0.56 and 0.61 +/- 0.15 mm2, respectively, p < 0.001). RNFL asymmetry was not different between the two groups (p = 0.265), but the inferior RNFL thickness of both the affected and unaffected eyes were less in the OAG group than in the NAION group. In the analysis of optic disc morphology, both affected and unaffected eyes showed significant differences between two groups. CONCLUSIONS: To differentiate NAION from OAG in eyes with altitudinal visual field defects, optic disc head analysis of not only the affected eye, but also the unaffected eye, by using spectral domain optical coherence tomography may be helpful.

Normative Data of Videonystagmography in Young Healthy Adults under 40 Years Old

PURPOSE: The purpose of this study was to establish a set of normative data values for saccade movements using videonystagmography and to evaluate the effects of manual correction on this data. METHODS: We examined 25 healthy subjects (9 men and 16 women). All tests were carried out by one well-instructed physician. Errors such as the wrong detection of the inflection point, missing movement, and prediction occurred during some tests. Thus, the same physician manually corrected the data by deleting error data from row results. RESULTS: We established a set of normative data for horizontal saccade movements (amplitude size 15 and 30 degrees) for mean peak velocity, latency, and accuracy. Manual correction only impacted latency and accuracy at 30 degrees horizontal, which is likely related to possible errors during the test. CONCLUSIONS: The present study provides clinically useful videonystagmography-based normative data for clinicians regarding saccade movements in Korean individuals.

The Parameters of Pattern Visual Evoked Potential in the Severe Visual Loss Patients in Korean

PURPOSE: To compare the characteristics of the pattern visual evoked potential (PVEP) in patients with severe visual loss and normal controls, and to demonstrate the range of PVEP parameters in normal Koreans. METHODS: The patients were divided into three groups according to visual acuity: group 1, ranging from no light perception to less than 0.02; group 2, ranging from 0.02 to 0.1; and group 3, ranging from 0.125 to 0.25. Group 4 was established as a healthy control group. The 95% confidence intervals (CIs) of the PVEP parameters were calculated for group 4. The PVEP parameters were compared among these four groups, and the amplitudes were evaluated with respect to the 95% CIs. We used the area under the curve to integrate the sensitivity and the specificity of the PVEP parameter quantitative values (7.01 to 9.57 microV and 6.75 to 10.11 microV). RESULTS: A total of 101 eyes were investigated. The 95% CIs of the P100 and N135 amplitudes of group 4 were 7.01 to 9.57 microV and 6.75 to 10.11 microV, respectively. The amplitudes of P100 and N135 were significantly higher in group 4 (p < 0.001). The P100 and N135 amplitude were below the 95% CI in all group 1 patients. The area under the curve of the P100 amplitude was the highest (0.789). CONCLUSIONS: No legally blind patient in the present study exhibited a value within the 95% CI of the controls. The P100 amplitude may be the best parameter for defining blindness in patients.

The Parameters of Pattern Visual Evoked Potential in the Severe Visual Loss Patients in Korean

PURPOSE: To compare the characteristics of the pattern visual evoked potential (PVEP) in patients with severe visual loss and normal controls, and to demonstrate the range of PVEP parameters in normal Koreans. METHODS: The patients were divided into three groups according to visual acuity: group 1, ranging from no light perception to less than 0.02; group 2, ranging from 0.02 to 0.1; and group 3, ranging from 0.125 to 0.25. Group 4 was established as a healthy control group. The 95% confidence intervals (CIs) of the PVEP parameters were calculated for group 4. The PVEP parameters were compared among these four groups, and the amplitudes were evaluated with respect to the 95% CIs. We used the area under the curve to integrate the sensitivity and the specificity of the PVEP parameter quantitative values (7.01 to 9.57 microV and 6.75 to 10.11 microV). RESULTS: A total of 101 eyes were investigated. The 95% CIs of the P100 and N135 amplitudes of group 4 were 7.01 to 9.57 microV and 6.75 to 10.11 microV, respectively. The amplitudes of P100 and N135 were significantly higher in group 4 (p < 0.001). The P100 and N135 amplitude were below the 95% CI in all group 1 patients. The area under the curve of the P100 amplitude was the highest (0.789). CONCLUSIONS: No legally blind patient in the present study exhibited a value within the 95% CI of the controls. The P100 amplitude may be the best parameter for defining blindness in patients.

Long-Term Results of Intermittent Exotropia Surgery: Comparison between Motor and Functional Success

PURPOSE: To evaluate the long-term surgical success rate (>3 years) and contributing success factors considering motor and sensory criteria for patients with intermittent exotropia. METHODS: Fifty-four patients who received surgery for intermittent exotropia and were followed-up for a minimum of 3 years, including reoperation, were retrospectively evaluated. The first procedure was unilateral recession and resection in 50 patients and bilateral lateral rectus recession in 4 patients. Patients were classified as achieving a good, fair or poor outcome based on motor and sensory criteria. Success rate and associated factors such as postoperative alignment, exotropia type, first surgical procedure, and proportion of patients receiving reoperations were analyzed. RESULTS: The mean follow-up period after the first surgery was 4.81 +/- 1.30 years. The good outcome was achieved in 32 patients (59.3%), fair in 14 patients (25.9%), and poor in 8 patients (14.8%) considering motor criteria only. The good outcome was achieved in 29 patients (51.9%), fair in 12 patients (22.2%), and poor in 14 patients (25.9%) when considering combined motor/sensory (functional) criteria. No patient who received bilateral lateral rectus recession as the first procedure belonged to the good outcome group (p = 0.03 by motor criteria and p = 0.044 by functional criteria). Patients who received reoperation were significantly more likely to be in the good and fair groups (p = 0.009 by motor criteria and p = 0.02 by functional criteria). CONCLUSIONS: Long-term surgical results of intermittent exotropia in this Korean population revealed 85.2% motor success rate and 74.1% functional success rate. Early postoperative overcorrection was not associated with long-term success. Recession and resection procedure and reoperations were significantly associated with better outcome based on motor and functional criteria. Diligent reoperations after the first surgery could possibly contribute to good long-term functional outcome in intermittent exotropia patients.

A Case of Ocular Myasthenia Gravis Presenting as Double Depressor Palsy

A 65-year-old man who had been experiencing diplopia in front and down gaze for 15 days visited our hospital. Hypertropia was noted in the patient's left eye, and limitation of depression was found in the adduction, primary gaze, and abduction. Brain magnetic resonance imaging showed no remarkable findings. Two weeks after the first visit, the patient complained of ptosis in the left eye. An ice test was performed and the ptosis was resolved after the test. Then, anti-acetylcholine receptor binding antibody levels were checked and found to be slightly elevated. We prescribed methylprednisolone per os 24 mg for 2 weeks, and his symptoms improved after the 2-week treatment. Five weeks after his first visit, the patient showed an ortho result in the alternate prism cover test and normal ocular movements. This may be the first case in which ocular myasthenia gravis presented as double depressor palsy, and in such cases, the possibility of ocular myasthenia gravis should be considered to rule out double depressor palsy.

Using ImageJ to Evaluate Optic Disc Pallor in Traumatic Optic Neuropathy

PURPOSE: To evaluate optic disc pallor using ImageJ in traumatic optic neuropathy (TON). METHODS: This study examined unilateral TON patients. The optic disc was divided into 4 quadrants (temporal, superior, nasal, and inferior), consistent with the quadrants on optical coherence tomography (OCT) retinal nerve fiber layer (RNFL) thickness maps. Optic disc photography was performed and disc pallor was quantified using gray scale photographic images imported into ImageJ software. The correlation between optic disc pallor and RNFL thickness was examined in each quadrant. RESULTS: A total of 35 patients (31 male, 4 female) were enrolled in the study. The mean participant age was 34.8 +/- 15.0 years (range, 5 to 63 years). Overall RNFL thickness decreased in 6 patients, with thinning most often occurring in the inferior quadrant (28 of 35 eyes). There was a significant correlation between optic disc pallor and RNFL thickness (superior, rho = -0.358, p = 0.04; inferior, rho = -0.345, p = 0.04; nasal, rho = -0.417, p = 0.01; temporal, rho = -0.390, p = 0.02). The highest level of correspondence between disc pallor and RNFL thickness values outside of the normative 95th percentiles was 39.3% and occurred in the inferior quadrant. CONCLUSIONS: Optic disc pallor in TON was quantified with ImageJ and was significantly correlated with RNFL thickness abnormalities. Thus, ImageJ evaluations of disc pallor may be useful for evaluating RNFL thinning, as verified by OCT RNFL analyses.

Symptom Based Diagnosis of Infant under One Year in Outpatient Clinic

PURPOSE: We investigated chief complaints and ocular disorders in infants under one year of age and analyzed the association between complaints and disorders. METHODS: The medical records of 815 infants were reviewed. Chief complaints were grouped into the following 11 categories: eyelid drooping, epiphora (tearing), ocular discharge, lid swelling, eyelashes touching the eyeball, abnormalities of the anterior segment, abnormal eyeball movement (e.g., squint or nystagmus), red eye, trauma, poor eye contact, and miscellaneous. We performed ophthalmologic examinations at the doctors' discretion and analyzed the relationship between chief complaint and final diagnosis. RESULTS: Mean age of visiting the outpatient clinic was 6.09 +/- 3.07 months. The most common complaint was ocular discharge, followed by abnormal eyeball movement, epiphora, and eye redness. Among infants with ocular discharge, most were attributed to congenital nasolacrimal duct obstruction (155 infants) and conjunctivitis (75 infants). A total of 160 guardians complained of abnormal eyeball movement and 86 of 160 infants (51.9%) suffered from pseudoesotropia. Additionally, exodeviation and esodeviation was found in 17.9% and 16.4% of infants, respectively. Eight guardian complained of poor eye contact in their infants and these infants were diagnosed with retinal disorders (3), congenital optic nerve disorders (2), cataract (1), glaucoma (1), and high hyperopia (1). CONCLUSIONS: The most common ocular symptoms in infants under one year of age included ocular discharge, epiphora, and eye redness. However, infants with poor eye contact or suspected abnormal eye movement should undergo a full ophthalmologic examination.