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.

Clinical and Optic Disc Characteristics of Patients Showing Visual Recovery in Leber Hereditary Optic Neuropathy.

BACKGROUND: The visual prognosis in Leber hereditary optic neuropathy (LHON) is generally poor. However, some individuals can have spontaneous visual recovery (VR) in one or both eyes by a mechanism that is not yet clearly understood. The purpose of this study was to determine whether certain clinical and optic disc features are associated with VR in patients with LHON. METHODS: We retrospectively examined 80 eyes of 40 patients with LHON using clinical databases, fundus photographs, and high-definition spectral-domain optical coherence tomography (OCT) images. VR was defined as a gain of 3 or more lines of logarithm of the minimum angle of resolution (logMAR)-scaled visual acuity from nadir; this represents a doubling of the visual angle. Patients were divided into VR and nonrecovery (NR) groups. Using fundus photographs, we measured optic disc size and evaluated for the presence of optic disc features, including peripapillary telangiectasia, disc hyperemia, and swelling. We also measured the disc area, cup-to-disc ratio, and rim area of the optic disc using OCT. RESULTS: Twenty-one of 80 eyes (26%) had a VR. The VR occurred within 2 years after onset in 81% of cases. The VR group showed younger age at onset (21 vs 29 years, P = 0.017) and better visual acuity at the nadir (1.39 vs 2.16 logMAR, P < 0.001) compared with the NR group. Optic disc features, particularly peripapillary telangiectasia (P = 0.027) and disc hyperemia (P = 0.006), were more prominent in the NR group. The cup-to-disc ratio was significantly smaller (0.64 vs 0.71, P = 0.004) and the rim area was significantly greater (1.17 vs 0.85 mm, P < 0.001) in the VR group compared with the NR group. CONCLUSIONS: A younger age at onset and a less severe reduction of visual acuity at the nadir were associated with a higher probability of VR. Presence of peripapillary telangiectasia and optic disc hyperemia may serve as predictive factors for poor visual prognosis in patients with LHON.

Comparison between aniridia with and without PAX6 mutations: clinical and molecular analysis in 14 Korean patients with aniridia.

PURPOSE: To describe clinical and molecular characteristics of Korean patients with aniridia and to compare the clinical phenotype between those having an identifiable PAX6 mutation and those not. DESIGN: Comparative case series. PARTICIPANTS: A total of 14 Korean patients from 10 families with aniridia. METHODS: Complete ophthalmologic examinations were performed for all patients. PAX6 analysis included direct sequencing of all coding regions and multiplex ligation-dependent probe amplification (MLPA) to detect large deletions when the sequencing was negative. If the PAX6 analysis failed to reveal any identifiable mutations, genomic copy number variation analysis via array comparative genomic hybridization (CGH) and candidate gene PITX3 and FOXE3 sequencing were then performed. MAIN OUTCOME MEASURES: Severity of ocular abnormalities and genetic findings. RESULTS: Sequencing of PAX6 exhibited 5 different heterozygous mutations in 8 patients from 5 families; 2 (p.Ser43Phe, IVS8-9C>G) were novel, and 3 (p.Arg208Trp, p.Arg317X, and p.X423L) have been previously reported. Among the remaining 6 patients in whom the PAX6 sequencing was negative, MLPA identified large deletions in 2 sporadic patients. However, the array CGH and candidate gene sequencing found no genomic or genetic abnormalities. The mutation detection rate was therefore 70%. Patients harboring an identifiable mutation in PAX6 had either a severe or a mild variant phenotype depending on the type of mutations. Likewise, among patients without an identifiable PAX6 mutation, their phenotypes varied widely from severe to very mild. CONCLUSIONS: This study adds 2 novel PAX6 mutations to those previously reported, providing further evidence for genetic and phenotypic heterogeneity in aniridic ocular malformation. There was no difference in the clinical phenotype between patients with and without detectable mutations in the PAX6 gene. The wide variability of ocular phenotype regardless of the presence or absence of PAX6 mutations calls for a further appreciation of the complexity in the molecular diagnosis of aniridia and suggests that this ocular malformation may be better regarded as a group of heterogeneous disorders, rather than a single disease entity, associated with mutations in PAX6 and/or other genes located elsewhere in the human genome. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any of the materials discussed in this article.

Proposing a new scoring system in intermittent exotropia: towards a better assessment of control.

OBJECTIVE: There is a clinical need for a valid and reliable assessment system to quantify the severity and measure the progression of intermittent exotropia. We have developed a new scoring system (Look And Cover, then Ten seconds of Observation Scale for Exotropia [LACTOSE]) based on the examinee's potential to regain the phoria for both distance and near fixation. The test takes only 1 minute to complete. The aim of this study was to evaluate the reliability and validity of LACTOSE. METHODS: The LACTOSE was created by incorporating both distance and near score (0-4 each) into a total score ranging from 0 to 8. A total of 235 consecutive patients with intermittent exotropia were prospectively evaluated using this new scoring system. Inter-rater and test-retest reliability was assessed from a cohort of 10 patients who were video recorded while undergoing the scoring testing and then scored by 10 trained ophthalmologists independently. Construct validity was studied by comparison with clinical variables indicating disease severity. RESULTS: Inter-rater reliability was good for both distance and near (intraclass correlation coefficient [ICC] = 0.729 and 0.818). Test-retest reliability was excellent for both distance and near (ICC = 0.849 and 0.727). Median scores for distance, near, and total were 3, 1, and 4, respectively. A broad representation of the total scores was significantly linked to the age of onset and the amount of exotropia (r2 = 0.194, p = 0.003 and r2 = 0.159, p < 0.001). CONCLUSIONS: We found that our newly developed scoring system is a reliable and valid tool for assessing the severity of intermittent exotropia. This new measure can be implemented easily and efficiently across diverse clinical settings.

New concept in cavernous sinus dural arteriovenous fistula: correlation with presenting symptom and venous drainage patterns.

BACKGROUND AND PURPOSE: An extradurally located cavernous sinus dural arteriovenous fistula (CSDAVF) exhibits different clinical behavior from other dural arteriovenous fistulas (DAVFs) located between 2 dural leaves. The aim of this study is to define angiographic types of CSDAVF associated with presenting symptom (Sx) and venous drainage patterns. METHODS: CSDAVFs during a mean of 23-month follow-up period of 58 patients (17 to 73 years, male:female ratio=8:50) were retrospectively analyzed. The 3 types of CSDAF, ie, proliferative (PT), restrictive (RT), and late restrictive (LRT) types, were categorized by the degrees and patterns of prominent arteriovenous shunt as well as venous flow. The status of the venous connection with CS and the presenting Sx patterns classified as orbital (OrbSxP), ocular (OcuSxP), cavernous (CavSxP), and cerebral (CerSxP) were associated with angiographic types as well as symptom onset, age, and gender. Correlations of discrete and categorical variables were statistically analyzed using the chi2 or Fisher exact test. RESULTS: PT (n=23) and RT (n=23) of CSDAVF were more common than LRT (n=12) (P=0.016) in patients with younger than 65 years and were related to OrbSxP (P=0.015) and CavSxP (P=0.038) in contrast to LRT to OcuSxP (P=0.004). Early onset of Sxs was related to the OrbSxP (P=0.08) and CavSxP (P<0.001). CerSxP (5%) was noted in RT or LRT. OrbSxP was related to the superior ophthalmic venous drainage (P=0.026) and CavSxP to the inferior petrosal sinus (P=0.046) and posterior fossa venous drainages (P=0.014). Seven patients revealed chronological progression of CSDAVF from PT to LRT and even to complete healing. CONCLUSIONS: CSDAVF presents as 3 distinctive angiographic types and is associated with presenting Sxs and venous drainage patterns.

Isolated superior rectus palsy due to contralateral midbrain infarction.

BACKGROUND: Isolated superior rectus palsy due to a contralateral midbrain lesion has not been reported. CASE DESCRIPTION: A 71-year-old woman suddenly developed diplopia. Examination showed that she had isolated superior rectus paresis. Magnetic resonance imaging showed a tiny infarct at the area of the oculomotor nucleus on the contralateral side. CONCLUSION: Isolated superior rectus palsy may be caused by a contralateral midbrain lesion that selectively involves crossing superior rectus nerve fibers.

Visual field loss in patients with refractory partial epilepsy treated with vigabatrin: final results from an open-label, observational, multicentre study.

BACKGROUND: Use of the antiepileptic drug vigabatrin is associated with an elevated risk of visual field loss. OBJECTIVE: To determine the frequency of, and risk factors for, vigabatrin-attributed visual field loss (VAVFL) in the setting of a large-scale, multinational, prospective, observational study. STUDY DESIGN: A comparative, open-label, parallel-group, multicentre study. SETTING: Hospital outpatient clinics at 46 centres in five countries. PATIENTS: 734 patients with refractory partial epilepsy, divided into three groups and stratified by age (8-12 years; >12 years) and exposure to vigabatrin. Group I comprised patients treated with vigabatrin for > or =6 months. Group II comprised patients previously treated with vigabatrin for > or =6 months who had withdrawn from the drug for > or =6 months. Group III comprised patients never treated with vigabatrin. Patients underwent perimetry at either 4- or 6-month intervals, for up to 36 months. Visual field outcome was evaluated masked to drug exposure. INTERVENTION: Perimetry. MAIN OUTCOME MEASURE: The visual field outcome at each of four analysis points: (i) at enrolment (i.e. baseline, all patients); (ii) for patients exhibiting a conclusive outcome at the initial visual field examination; (iii) for patients exhibiting at least one conclusive outcome to the visual field examinations; and (iv) at the last conclusive outcome to the visual field examinations. RESULTS: Of the 734 patients, 524 yielded one or more conclusive visual field examinations. For Group I, the frequency of VAVFL at the last conclusive examination was 10/38 (26.3%) for those aged 8-12 years and 65/150 (43.3%) for those aged >12 years. For Group II, the respective frequencies were 7/47 (14.9%) and 37/151 (24.5%). One case resembling VAVFL was present amongst the 186 patients in Group III at the last conclusive examination. The frequency of VAVFL in Groups I and II combined was 20.0% for those aged 8-12 years and 33.9% for those aged >12 years. VAVFL was associated with duration of vigabatrin therapy (odds ratio [OR] up to 15.2; 95% CI 4.4, 51.7), mean daily dose of vigabatrin (OR up to 26.4; 95% CI 2.4, 291.7) and male gender (OR 2.51; 95% CI 1.5, 4.1). VAVFL was more frequently detected with static than with kinetic perimetry (OR up to 0.43; 95% CI 0.24, 0.75). CONCLUSIONS: Since the probability of VAVFL is positively associated with treatment duration, careful assessment of the risk-benefit ratio of continuing treatment with vigabatrin is recommended in patients currently receiving this drug. All patients continuing to receive vigabatrin should undergo visual field examination at least every 6 months for the duration of treatment. We recommend two-level (three-zone), gradient-adapted, suprathreshold static perimetry of the peripheral field together with threshold perimetry of the central field out to 30 degrees from fixation. The frequency of ophthalmological and perimetric examinations should be increased in the presence of VAVFL.

Vigabatrin and epilepsy: lessons learned.

PURPOSE: The risk factors for visual field loss attributable to vigabatrin (VAVFL) are equivocal. This multinational, prospective, observational study aimed to clarify the principal/major factors for VAVFL. METHODS: Interim analysis of three groups with refractory partial epilepsy, stratified by age (8-12 years; >12 years) and exposure to vigabatrin (VGB). Group I comprised participants treated with VGB for >or=6 months, Group II participants previously treated with VGB for >or=6 months who had discontinued the drug for >or=6 months and Group III those never treated with VGB. Perimetry was undertaken at least every six months, for up to 36 months; results were evaluated masked to drug exposure. RESULTS: Based upon 563 participants in the locked data set, 432 yielded one or more Conclusive visual field examinations. For Group I, the frequency of VAVFL at the last Conclusive examination was 10/32 (31.2%) for those aged 8-12 years and 52/125 (41.6%) for those aged >12 years. For Group II, the proportions were 4/39 (10.3%) and 31/129 (24.0%). No cases resembling VAVFL manifested in Group III. VAVFL was associated with duration of VGB therapy (Odds ratio [OR] 14.2; 95% CI 5.0 to 40.5); mean dose of VGB (OR 8.5; 95% CI 2.2 to 33.2); and male gender (OR 2.1; 95% CI 1.2 to 3.7). VAVFL was more common with static than kinetic perimetry (OR 2.3, 95% CI 1.3 to 4.2). CONCLUSIONS: The therapeutic benefit of VGB is counteracted by the progressive accrual of the risk of VAVFL with continued exposure and with increase in mean dose.

Vigabatrin and visual field defects in pediatric epilepsy patients.

We studied the prevalence, type and severity of vigabatrin (VGB)-attributed visual field defects (VFDs), and used these data to assess the associated risk factors in pediatric patients. Medical records were retrospectively reviewed for 67 pediatric patients who received VGB alone or in combination with other antiepileptic drugs, and who had undergone visual field examinations using a Humphrey visual field analyzer. Of the 67 patients, 15 had VGB-attributed VFDs: 13 had nasal arcuate type, 1 had nasal and temporal constricted type and 1 had nasal constricted type. In terms of severity, 7 patients had Grade I VGB-attributed VFDs, 5 had Grade II, 2 had Grade III, and 1 had Grade IV. Although there were no significant differences between the VFD and non-VFD groups with regards to all tested parameters, there were no cases of VGB-attributed VFDs in patients with total treatment durations <2 yr and cumulative doses <10 g/kg. In conclusion, the prevalence of VGB-attributed VFDs in VGB-treated pediatric epilepsy patients was 22%. The high frequency of VGB-attributed VFDs indicates that physicians should inform all patients of this risk prior to VGB treatment and perform periodic visual field examinations.

Wall-Eyed Monocular Internuclear Ophthalmoplegia (WEMINO) with Contraversive Ocular Tilt Reaction.

Wall-eyed monocular internuclear ophthalmoplegia (WEMINO) with contraversive ocular tilt reaction has not been previously reported. A 71-year-old woman suddenly developed blurred vision. Examination revealed left internuclear ophthalmoplegia, left exotropia, right hypotropia, and rightward head tilt. Magnetic resonance imaging showed a tiny infarction at the area of the left medial longitudinal fasciculus in the upper pons. WEMINO with contraversive ocular tilt reaction may be caused by a paramedian pontine tegmental infarction that selectively involves the medial longitudinal fasciculus.

Nonsurgically placed nasolacrimal stents for epiphora: long-term results and factors favoring stent patency.

PURPOSE: To evaluate long-term effectiveness of a polyurethane stent in lacrimal system treatment. MATERIALS AND METHODS: Fluoroscopically guided placement of a polyurethane lacrimal stent was evaluated in 727 obstructed lacrimal systems in 588 patients with a mean follow-up period of 77 weeks (range, 1-277 wk), from which the technical and initial success rates and long-term patency were calculated. The impact of factors affecting long-term patency, including symptom duration, previous probing, presence of pus or eye discharge, lesion multiplicity, and site of obstruction were evaluated. Changes in 295 patients' lacrimal sac configuration after stent removal were analyzed and classified into three categories: contraction, widening, and no change. RESULTS: The overall technical success rate in this study was 95.9%, with a 90.8% initial clinical success rate. The median primary patency duration was 504.33 days plus minus 23.17; secondary median patency duration was 642.87 days plus minus 24.68. Most favorable factors such as symptom duration, previous probing, presence of pus, or eye discharge showed no significant difference; however, site of obstruction and lesion multiplicity influenced mean patency. Lesions below the junction and single lesions showed longer patency periods. Common canalicular obstruction after stent removal occurred in 50 (17%) lacrimal systems. Irregular sac configuration appeared in 171 (58%) lacrimal systems after stent removal. Sac configuration was the same in 185 (62.7%) lacrimal systems, contracted in 105 (35.6%), and widened in five (1.7%). In 151 eyes of 295, epiphora recurred 121.6 days (range, 1-1,182 d) after stent removal. CONCLUSION: Fluoroscopic lacrimal stent placement is a simple and safe outpatient procedure, but high recurrence and sac irregularities after stent removal are not encouraging.