A Case of Retinal Vasculitis, Uveitis in Myelin Oligodendrocyte Glycoprotein Antibody Associated Optic Neuritis

Purpose@#To report a case of recurrent optic neuritis with uveitis in a patient with myelin oligodendrocyte glycoprotein (MOG)- associated disease.Case summary: A 16-year-old female presented with left eye hyperemia, glare, and headache. The best corrected visual acuity was 1.2 in both eyes with a left relative afferent pupillary defect. Cells in the anterior chamber, optic disc edema, and subretinal fluid were present, and the distal optic nerve was enhanced on orbital magnetic resonance imaging. Although it improved with steroid treatment, it recurred 3 times in 9 months. At the second recurrence, her visual acuity was reduced to light perception, but she responded well to steroids. During the third recurrence, she visited our hospital for the first time and improved with steroid treatment. However, 18 months after treatment, optic neuritis, uveitis, and macular edema occurred, so oral steroids and eye drops were maintained. Serum anti-MOG antibody came out positive, and at the last visit, visual acuity of 1.2 in the left eye and visual function were maintained well. @*Conclusions@#MOG antibody disease (MOGAD) frequently recurs and invades various structures of the eye and various clinical manifestations have been reported. Therefore, if optic neuritis is accompanied by inflammation of the anterior chamber and retinal vessels, the possibility of MOGAD should be considered.

Self-Reported Findings of the Korean Intermittent Exotropia Multicenter Study Questionnaire

Purpose@#To determine subjective symptoms and medical history of patients with intermittent exotropia in a large study population. @*Methods@#The Korean Intermittent Exotropia Multicenter Study (KIEMS) is a nationwide, observational, cross-sectional, multicenter study conducted by the Korean Association for Pediatric Ophthalmology and Strabismus including 5,385 patients with intermittent exotropia. Subjective symptoms and medical history of patients with intermittent exotropia were extracted by a comprehensive survey based on a self-administered questionnaire according to the study protocol of the KIEMS. @*Results@#The mean age of symptom onset was 5.5 years. The most common symptom reported in patients with intermittent exotropia was photophobia (52.1%), followed by diplopia at near fixation (7.3%) and distance fixation (6.2%). Preterm birth was found in 8.8%, and 4.1% had perinatal complications. A family history of strabismus was present in 14.9%, and 5.5% of patients had a family member who underwent strabismus surgery. @*Conclusions@#The KIEMS is one of the largest clinical studies on intermittent exotropia. Intermittent exotropia frequently caused photophobia and diplopia, and patients with a family history was not uncommon.

Clinical Characteristics and Outcomes after Augmented Medial Rectus Muscle Recession in Patients with Acute Acquired Comitant Esotropia

Purpose@#To analyze the clinical characteristics of patients with acute acquired comitant esotropia, evaluate the outcomes of augmented medial rectus muscle recession, and propose indicators for determining the appropriate surgical dosage. @*Methods@#Data of patients with acute acquired esotropia who underwent medial rectus recession between 2016 and 2022 were retrospectively analyzed. The amount of medial rectus muscle recession was 1-2 mm greater than for other types of esotropia. Surgical success was assessed by reference to the angle of deviation and diplopia status 6 months after surgery. We investigated the amount of additional recession required for favorable surgical outcomes, the characteristics of groups that required augmentation, and changes in surgical amount according to the deviation angle. @*Results@#A total of 38 patients were included; the average refractive error was -3.79 ± 3.11 diopters (D). Patient age at the time of surgery was 25.6 ± 18.2 years and the preoperative deviation angle was 38.1 ± 11.5 prism diopters (PD). The amount of medial rectus muscle recession was 6.25 ± 1.4 mm, i.e., 1.2 ± 1.0 mm greater than during other surgeries. The average augmentation was 24.1 ± 18.8%. The motor surgical success rate was 92.1% and the sensory success rate was 97.4%. The required augmentation was 26.9% for those with angles of deviation 40 PD. Patients who required augmentation > 25% were older, and the surgical effect was greater as the angle of deviation increased. @*Conclusions@#To ensure successful surgical outcomes of patients with acute acquired comitant esotropia, it is essential to increase the amount of medial rectus recession compared to that during other surgeries; the average augmentation was 24% in this study.

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.

Changes in Hypertropia in Patients with Asymmetric Inferior Oblique Overaction after Symmetric Inferior Oblique Myectomy

Purpose@#This study assessed the effects of bilateral inferior oblique myectomy for hypertropia on the preoperative vertical deviation angle in patients with asymmetric primary inferior oblique overaction (IOOA). @*Methods@#This study included patients who underwent bilateral inferior oblique myectomy and lateral rectus recession due to asymmetric primary IOOA and intermittent exotropia, and were followed up for at least 6 months postoperatively. Pre- and post-operative vertical deviation angles were compared. The correlation between the extent of correction of vertical deviation after surgery and residual hypertropia, according to the preoperative degree of vertical deviation and difference between bilateral IOOA, was evaluated. @*Results@#This study included 178 eyes from 89 patients. The angle of hypertropia in the primary position was reduced from 3.2 ± 2.2 prism diopters (PD) preoperatively to 0.5 ± 2.5 PD postoperatively (Wilcoxon signed-rank test, p < 0.001). No significant correlation was observed between the preoperative interocular difference in IOOA and postoperative extent of correction of the vertical deviation (r = 0.044, p = 0.684), or between the preoperative difference in bilateral IOOA and residual hypertropia (Spearman's rank-order correlation, r = -0.084, p = 0.432). Increased preoperative hypertropia correlated with a greater extent of surgical correction of the vertical deviation (r = 0.733, p < 0.001). Preoperative hypertropia had no significant correlation with residual hypertropia (Spearman's rank-order correlation, r = 0.182, p = 0.087). @*Conclusions@#In symmetric bilateral inferior oblique myectomy with bilateral lateral rectus recession for asymmetric bilateral primary IOOA with V-type intermittent exotropia, a positive correlation between the degree of preoperative vertical deviation and extent of correction of the vertical deviation was observed. Additionally, IOOA and hypertropia were significantly improved postoperatively.

Phakomatous Choristoma of the Orbit with Inferior Oblique Muscle Involvement

Purpose@#We report a case of phakomatous choristoma presenting as an orbital tumor with involvement of the inferior oblique muscle. Case summary: A 2-month-old male infant presented to our clinic with a right orbital mass that had been present since birth. Magnetic resonance imaging demonstrated a homogenous enhanced well-defined mass located in the inferomedial portion of the right orbit without bone erosion. By transconjunctival orbitotomy, the orbital tumor invading the inferior oblique muscle was identified and resected. Histopathology showed a thick basement membrane surrounding pseudoglandular structures embedded in a collagenous stroma, psammomatous calcific foci in the stroma, and eosinophilic material in the lumen. Immunohistochemical analysis showed positive staining for S-100 and cytokeratin. On histopathological evaluation, the tumor was diagnosed as phakomatous choristoma. @*Conclusions@#To our knowledge, this is the first report in South Korea of phakomatous choristoma of the orbit with involvement of the inferior oblique muscle. Although rare, phakomatous choristoma should be included in the differential diagnosis of tumors occurring on the inferomedial side of the orbit.

Phakomatous Choristoma of the Orbit with Inferior Oblique Muscle Involvement

Purpose@#We report a case of phakomatous choristoma presenting as an orbital tumor with involvement of the inferior oblique muscle. Case summary: A 2-month-old male infant presented to our clinic with a right orbital mass that had been present since birth. Magnetic resonance imaging demonstrated a homogenous enhanced well-defined mass located in the inferomedial portion of the right orbit without bone erosion. By transconjunctival orbitotomy, the orbital tumor invading the inferior oblique muscle was identified and resected. Histopathology showed a thick basement membrane surrounding pseudoglandular structures embedded in a collagenous stroma, psammomatous calcific foci in the stroma, and eosinophilic material in the lumen. Immunohistochemical analysis showed positive staining for S-100 and cytokeratin. On histopathological evaluation, the tumor was diagnosed as phakomatous choristoma. @*Conclusions@#To our knowledge, this is the first report in South Korea of phakomatous choristoma of the orbit with involvement of the inferior oblique muscle. Although rare, phakomatous choristoma should be included in the differential diagnosis of tumors occurring on the inferomedial side of the orbit.

Clinical Features of Duane Retraction Syndrome: A New Classification

Purpose@#We sought to provide a new classification system for Duane retraction syndrome (DRS) according to type and angle of strabismus during primary gaze and to analyze the clinical features of each DRS type. @*Methods@#The medical records of 65 DRS patients who visited the department of pediatric ophthalmology at Seoul National University Children's Hospital between 2010 and 2017 were retrospectively analyzed. Patients whose angle of exotropia at primary gaze exceeded 3 prism diopters (PDs) were classified as “Exo-Duane,” those whose angle of strabismus at primary gaze did not exceed 3 PDs were classified as “Ortho-Duane,” and those whose angle of esotropia at primary gaze exceeded 3 PDs were classified as “Eso-Duane.” @*Results@#Among 65 DRS patients, Ortho-Duane was the most common (53.8%) type, followed by Eso-Duane (33.8%) and Exo-Duane (12.3%). The mean age at diagnosis was significantly higher in the Exo-Duane group than the Ortho-Duane or Eso-Duane group (p = 0.003 and p < 0.001, respectively). A predominance of left eye involvement was observed in the Ortho-Duane (62.9%) and Eso-Duane (90.9%) groups. The frequencies of upshoot, downshoot, fissure narrowing, and globe retraction were not significantly different among the subgroups. Head-turn was more frequent in Eso-Duane patients than in Exo-Duane or Ortho-Duane patients (p = 0.001 and p < 0.001, respectively). Myopia accounted for the most common refractive error among Exo-Duane patients (71.4%), while hyperopia was found more often in both Ortho-Duane (64.7%) and Eso-Duane (85.0%) patients. The majority of patients showed gross stereoacuity (93.1%), and a large proportion had good stereoacuity (Exo-Duane 60.0%, Ortho-Duane 81.3%, Eso-Duane 87.5%). @*Conclusions@#Our newly proposed classification of DRS according to type and angle of strabismus at primary gaze was practically useful and showed potential for use as an objective guideline in the clinical setting.

Long-term Lens Complications Following Removal of Persistent Pupillary Membrane

PURPOSE: We evaluated the long-term surgical outcome and lens complications in children with persistent pupillary membrane following removal using vitreous scissors. METHODS: Patients diagnosed with persistent pupillary membrane who received surgical treatment from 1987 to 2012 were retrospectively reviewed. The removal was performed using vitreous scissors after instillation of miotics. The minimum follow-up period after surgery was four years. Factors of age, sex, visual acuity, refractive errors, and complications during or after surgery were evaluated. RESULTS: A total of 32 eyes of 26 patients were included. The mean age at the initial visit was 22.6 ± 34.7 (range, 0.9 to 141.2) months, and the mean age at surgery was 43.7 ± 36.0 (range, 1.0 to 142.5) months. There were no intraoperative complications using vitreous scissors, and all lesions were completely removed. After a mean follow-up period of 6.5 ± 3.3 (range, 4.0 to 14.8) years, the best corrected visual acuity at the final visit was 0.6 ± 0.9 logarithm of the minimum angle of resolution, and two eyes (6.3%) presented with lens opacity during follow-up. CONCLUSIONS: In children with persistent pupillary membrane, there were no intraoperative complications, and only two patients presented with lens change during the long-term postoperative follow-up period. Surgical removal should be considered a safe and effective treatment for patients with visually significant persistent pupillary membrane.

Clinical Characteristics of Retinoblastoma Patients whose Diagnosis was Difficult due to Atypical Ocular Manifestation

PURPOSE: To report the clinical characteristics of retinoblastoma patients whose diagnosis was difficult due to atypical ocular manifestations. METHODS: Among retinoblastoma patients who were diagnosed and treated from January 1999 to December 2014 at Seoul National University Children's Hospital, 6 patients whose diagnosis was difficult were retrospectively reviewed. Factors including age, sex, family history, initial findings, time to final diagnosis, histopathologic examination, additional treatment, and survival rate were evaluated. RESULTS: Among 6 patients, 5 were male, and the mean age at the initial visit was 32.9 ± 19.1 months. None of the patients had family history, and all presented with unilateral lesion at the initial visit. The initial diagnoses were Coats' disease and uveitis in 2 patients, respectively, and persistent hyperplastic primary vitreous and traumatic hyphema in 1 patient, respectively. During an intensive short-term follow-up of 8.3 ± 5.3 weeks, 2 patients showed malignant cells after external subretinal fluid drainage procedure, and 4 patients demonstrated increasing ocular size or calcification in imaging. These patients received enucleation under suspicion of malignancy and were finally diagnosed with retinoblastoma after histopathologic examination. There were 2 patients with optic nerve involvement, and 3 patients underwent additional systemic chemotherapy. Five patients were followed-up for 7.6 ± 6.3 years after enucleation, and the mean age at final follow-up was 10.6 ± 7.4 years. CONCLUSIONS: Retinoblastoma is one of the diseases in which early diagnosis and treatment are important. However, some cases are difficult to diagnose, even for experienced clinicians. If there are no typical manifestations such as mass or calcification and early findings show retinal detachment, glaucoma, pseudohypopyon, or hyphema, intensive short-term follow-up to exclude retinoblastoma is needed.

Oil Droplet Cataract: Cause of Decreased Vision of Unknown Etiology

PURPOSE: To report cases of oil droplet cataract, one cause of decreased vision of unknown etiology. METHODS: We performed a retrospective chart review analysis of patients referred to the neuro-ophthalmology clinic due to unknown etiology of decreased visual acuity and diagnosed with oil droplet cataract. Clinical features including history, result of ophthalmologic examinations, and clinical course were evaluated. RESULTS: Among the patients referred to the neuro-ophthalmology clinic due to unknown etiology of decreased visual acuity, 6 patients were diagnosed with oil droplet cataract. The patients ranged from 38 to 63 years of age and their best corrected visual acuities at their first visits were between 0.1 and 0.7. Ophthalmologic examinations including neuro-ophthalmologic tests were normal except for changes in lens nucleus and peculiar fundus reflexes were observed using retinoscopy in all patients. Five eyes of 4 patients underwent cataract surgery and all 5 eyes achieved the best corrected visual acuity of 1.0 or higher. CONCLUSIONS: Oil droplet cataract is a cause of decreased visual acuity of unknown etiology that can be missed. The disease abnormalities are difficult to observe because only subtle changes in lens nucleus are apparent on slit lamp examination; however characteristic fundus reflexes can be identified using retinoscopy. Ophthalmologists should thoroughly understand the oil droplet cataract and diagnose it in the early stages to avoid misdiagnosis and unnecessary costs.

Combined Cataract Extraction and Vitrectomy for Macula-sparing Retinal Detachment: Visual Outcomes and Complications

PURPOSE: To evaluate the visual outcome of combined phacoemulsification, intraocular lens implantation, and vitrectomy for macula-sparing rhegmatogenous retinal detachment. METHODS: The results of combined vitrectomy with cataract extraction were retrospectively analyzed in patients with preexisting cataracts and new-onset rhegmatogenous retinal detachment. To qualify, patients must also have had macular sparing in a region 6,000 microm in diameter on optical coherence tomography. The anatomical success rate, visual outcomes, and postoperative complications relating to visual acuity were evaluated. RESULTS: In 56 patients followed postoperatively for more than 12 months, the initial and final surgical success rate was 96.4% and 100%, respectively. The mean preoperative logarithm of the minimum angle of resolution visual acuity was 0.05 and decreased to 0.11 postoperatively (p < 0.001). Of the 56 patients, 20 (35.7%) had worse visual acuity postoperatively, compared with preoperatively (0.06 vs. 0.27, p < 0.001); these cases were comprised of six patients with epiretinal membranes, 12 patients with a posterior capsule opacity, and two patients with cystoid macular edema. In the remaining 36 patients, there were no significant differences in visual acuity preoperatively and postoperatively (0.04 vs. 0.03, p = 0.324). CONCLUSIONS: In patients with cataracts who develop macula-sparing rhegmatogenous retinal detachment and whose visual prognosis is excellent assuming the retina can be reattached successfully, combined phacoemulsification, intraocular lens implantation, and vitrectomy might be an effective treatment. However, the visual prognosis is significantly affected by postoperative complications such as an epiretinal membranes, posterior capsule opacity, and cystoid macular edema. Therefore, further studies should examine methods to prevent these postoperative complications.

Clinical Characteristics and Course in Divergence Paralysis

PURPOSE: To improve the understanding of divergence paralysis by identifying its clinical characteristics. METHODS: We performed a retrospective chart review analysis of patients diagnosed with divergence paralysis that were followed up for at least 6 months. Clinical features, including disease onset, course, neurological examinations, and imaging studies were evaluated. RESULTS: Fifteen patients were included in the present study and the average age was 55.6 years. Thirteen patients had an acute onset and 2 had an insidious onset. The initial distance deviation ranged from 4 to 14 prism diopters (PD) of esotropia (mean, 8 PD esotropia) and near deviation ranged from 6 PD exophoria to 10 PD esophoria (mean, 1.2 PD esophoria). None of the patients developed additional neurological disorders associated with divergence paralysis during the follow-up period. Eleven of 13 patients with primary divergence paralysis continued to depend on the prism glasses with the same diopters. In the 2 patients with secondary divergence paralysis, distant diplopia disappeared as the underlying disease improved. CONCLUSIONS: In our study, the majority of divergence paralysis was not associated with neurological diseases and the patients had an acute onset. Primary divergence paralysis lasted for an extended period. However, secondary divergence paralysis was resolved quickly as the underlying disease improved.

Clinical Characteristics and Course in Divergence Paralysis

PURPOSE: To improve the understanding of divergence paralysis by identifying its clinical characteristics. METHODS: We performed a retrospective chart review analysis of patients diagnosed with divergence paralysis that were followed up for at least 6 months. Clinical features, including disease onset, course, neurological examinations, and imaging studies were evaluated. RESULTS: Fifteen patients were included in the present study and the average age was 55.6 years. Thirteen patients had an acute onset and 2 had an insidious onset. The initial distance deviation ranged from 4 to 14 prism diopters (PD) of esotropia (mean, 8 PD esotropia) and near deviation ranged from 6 PD exophoria to 10 PD esophoria (mean, 1.2 PD esophoria). None of the patients developed additional neurological disorders associated with divergence paralysis during the follow-up period. Eleven of 13 patients with primary divergence paralysis continued to depend on the prism glasses with the same diopters. In the 2 patients with secondary divergence paralysis, distant diplopia disappeared as the underlying disease improved. CONCLUSIONS: In our study, the majority of divergence paralysis was not associated with neurological diseases and the patients had an acute onset. Primary divergence paralysis lasted for an extended period. However, secondary divergence paralysis was resolved quickly as the underlying disease improved.

The Optical Coherence Tomography Findings of Optic Tract Syndrome

PURPOSE: To report a case of optic tract syndrome in which optical coherence tomography (OCT) demonstrated the specific findings of the retinal nerve fiber layer (RNFL). CASE SUMMARY: A 32-year-old male patient visited the hospital with right side visual field defect in both eyes that occurred immediately after a traffic accident 8 months prior. The visual acuity of both eyes was normal, and a relative afferent papillary defect was evident in the right eye. In addition, suspicious band atrophy of the right optic disc and thinning of the superior and inferior arcuate bundle were observed in the left eye. On visual field examination, homonymous hemianopia was present. Optic tract syndrome was suspected, although there was no abnormality of the visual tract on MRI. On fast RNFL thickness 3.4 scan, a thinning of RNFL in the nasal and temporal segments in the right eye and superior and inferior segments in the left eye were observed. Based on the findings, optic tract syndrome was diagnosed in the left eye. CONCLUSIONS: We report specific OCT findings which can be useful when making a diagnosis of optic tract syndrome.

A Case of Thiamine (Vitamin B1)-Deficient Optic Neuropathy Associated with Wernicke's Encephalopathy

PURPOSE: To report a case of subacute optic neuropathy caused by thiamine (vitamin B1) deficiency. CASE SUMMARY: A 44-year-old woman was referred to the ophthalmology department due to decreased vision which began 10 days prior to presentation. The patient history indicated that she had undergone chemotherapy for ovarian cancer and she had been dependent on total parenteral nutrition for 3 weeks due to nausea and vomiting. Her best corrected vision of the right and the left eyes were 0.15 and 0.2, respectively. Color vision was severely impaired in both eyes without retrobulbar pain. There was marginal blurring on the temporal side of the optic discs of both eyes. The optic nerves were unremarkable on orbital and brain magnetic resonance imaging (MRI). There was high signal intensities in both the mammillary body and periaqueductal gray matter on T2-weighted imaging. In addition, the patient exhibited ataxia along with short-term memory loss. She was diagnosed with Wernicke's encephalopathy. Thiamine was administrated based on the diagnosis, and after 2 days of administration, the patient's vision and neurologic symptoms began to improve. Two weeks later, the patient recovered her vision. CONCLUSIONS: Thiamine deficiency may cause optic neuropathy and can be recovered with early thiamine supplementation. This condition could occur due to deficient oral thiamine administration. We should keep this point in mind and try to prevent or diagnose early.

Usefulness of Exophthalmos Measurement on Upgaze

PURPOSE: To evaluate the usefulness of exophthalmos measurement on upgaze in patients who are not able to expose corneal vertex on primary gaze. METHODS: The present study included 39 patients with blow out fracture, pseudotumor, or thyroid associated ophthalmopathy and 21 people who don't have any ocular disease. In total, 60 people had exophthalmometry using Hertel exophthalmometer on the basis of corneal vertex, inferior limbus, and inferior sclera by three examiners. RESULTS: The relative exophthalmometry in normal group was 0.71 +/- 0.75 mm, 0.67 +/- 0.62 mm, and 0.69 +/- 0.60 mm on the basis of corneal vertex, inferior limbus, and inferior sclera, respectively. The relative exophthalmometry in exophthalmic group was 1.10 +/- 0.99 mm, 1.13 +/- 0.99 mm, 1.10 +/- 0.91 mm on the basis of corneal vertex, inferior limbus, and inferior sclera, respectively. The relative exophthalmometry in enophthalmic group was 0.79 +/- 0.90 mm, 0.74 +/- 0.92 mm, 0.74 +/- 0.87 mm on the basis of corneal vertex, inferior limbus, and inferior sclera, respectively. There was no statistically significant difference between different measuring points (p > 0.05). The inter-examiner reproducibility was shown to be highly reliable. CONCLUSIONS: The upgaze exophthalmometry on the basis of inferior limbus or inferior sclera would be useful in the patients who are not able to expose corneal vertex on primary gaze.