Etiological distribution of isolated oculomotor nerve palsy: analysis of 633 patients and literature review.

BACKGROUND AND PURPOSE: The etiological distribution of oculomotor nerve palsy has varied amongst the studies. This study aimed to define the clinical features and underlying etiologies of isolated oculomotor nerve palsy by recruiting patients from all departments in a referral-based university hospital. METHODS: The medical records of 672 patients who had a confirmed diagnosis of isolated oculomotor nerve palsy at all departments of Seoul National University Bundang Hospital, Seongnam, South Korea, from 2003 to 2020 were reviewed. A proportion of the etiology of isolated oculomotor nerve palsy was also compared with that of patients pooled from the previous studies that were searched on PubMed in May 2022. RESULTS: The most common etiology was microvascular (n = 168, 26.5%), followed by vascular anomalies (n = 110, 17.4%), neoplastic (n = 86, 13.6%), inflammatory (n = 79, 12.5%), idiopathic (n = 60, 9.5%) and traumatic (n = 53, 8.4%). Neurologists were mainly involved in the management of microvascular and inflammatory oculomotor nerve palsies whilst ophthalmologists mainly participated in the care of idiopathic, neoplastic and traumatic palsies. Neurosurgeons mostly took care of oculomotor nerve palsy due to vascular anomalies. CONCLUSIONS: The proportion of etiologies of isolated oculomotor nerve palsy may differ according to the specialties involved in the management. The results of previous studies on the etiological distribution of isolated oculomotor nerve palsy should be interpreted with this consideration.

Risk of central nervous system demyelinating attack or optic neuritis recurrence after pediatric optic neuritis in Korea.

PURPOSE: To investigate the rate of development of symptomatic central nervous system (CNS) demyelinating attacks or recurrent optic neuritis (ON) after the first episode of ON and its risk factors for Korean pediatric patients. METHODS: This multicenter retrospective cohort study included the patients under 18 years of age (n=132) diagnosed with ON without previous or simultaneous CNS demyelinating diseases. We obtained the clinical data including the results of neuro-ophthalmological examinations, magnetic resonance images (MRIs), antibody assays, and laboratory tests. We investigated the chronological course of demyelinating disease with respect to the occurrence of neurological symptoms and/or signs, and calculated the 5-year cumulative probability of CNS demyelinating disease or ON recurrence.  RESULTS: During the follow-up period (63.1±46.7 months), 18 patients had experienced other CNS demyelinating attacks, and the 5-year cumulative probability was 14.0±3.6%. Involvement of the extraorbital optic nerve or optic chiasm and asymptomatic lesions on the brain or spinal MRI at initial presentation were significant predictors for CNS demyelinating attack after the first ON. The 5-year cumulative probability of CNS demyelinating attack was 44.4 ± 24.8% in the AQP4-IgG group, 26.2±11.4% in the MOG-IgG group, and 8.7±5.9% in the double-negative group (P=0.416). Thirty-two patients had experienced a recurrence of ON, and the 5-year cumulative probability was 24.6±4.0%. In the AQP4-IgG group, the 5-year cumulative probability was 83.3±15.2%, which was significantly higher than in the other groups (P<0.001). CONCLUSIONS: A careful and multidisciplinary approach including brain/spinal imaging and antibody assay can help predict further demyelinating attacks in pediatric ON patients.

Etiologic distribution of isolated abducens nerve palsy: Analysis of 807 patients and literature review.

BACKGROUND AND PURPOSE: The etiologies of abducens nerve palsy have shown a large variability among studies. This study aimed to establish the clinical features and underlying etiologies of isolated abducens nerve palsy by recruiting patients from all departments in a referral-based university hospital. METHODS: We reviewed the medical records of 807 patients with a confirmed diagnosis of isolated abducens nerve palsy at all departments of Seoul National University Bundang Hospital, Seongnam, Republic of Korea, from 2003 to 2020. We also compared the proportion of etiology with that of the patients pooled from the previous studies. RESULTS: The most common etiology was microvascular (n = 296, 36.7%), followed by idiopathic (n = 143, 17.7%), neoplastic (n = 115, 14.3%), vascular anomalies (n = 82, 10.2%), inflammatory (n = 76, 9.4%), and traumatic (n = 35, 4.3%). Patients were mostly managed by ophthalmologists (n = 576, 71.4%), followed by neurologists (n = 479, 59.4%), emergency physicians (n = 278, 34.4%), neurosurgeons (n = 191, 23.7%), and others (n = 72, 8.9%). The proportion of etiology significantly differed according to the age and sex of the patients and the specialties involved in the management (p < 0.001). Compared to the pooled data from the previous reports, the current study showed a higher prevalence of microvascular cause but a lower occurrence of traumatic and neoplastic causes. CONCLUSIONS: The results of previous studies on etiologic distribution of isolated abducens nerve palsy should be interpreted with consideration of the demographic features of patients recruited and the specialties involved.

Etiologic distribution of isolated trochlear palsy: Analysis of 1020 patients and literature review.

BACKGROUND AND PURPOSE: Trochlear palsy is the most common cause of vertical diplopia. The etiologies of trochlear palsy have shown a large discrepancy among studies. This study aimed to establish the clinical features and underlying etiologies of isolated trochlear palsy by recruiting the patients from all departments in a referral-based university hospital. METHODS: We reviewed the medical records of 1258 patients who had a confirmed diagnosis of isolated trochlear palsy at all departments of Seoul National University Bundang Hospital, Seongnam, South Korea, from 2003 to 2020. We also compared the proportion of etiologies with that of the patients pooled from previous studies. RESULTS: The most common etiology was congenital (n = 330, 32.4%), followed by idiopathic (n = 256, 25.1%), microvascular (n = 212, 20.8%), and traumatic (n = 145, 14.2%). These four etiologies explained 92.5% of isolated trochlear palsy. Patients were mostly managed by ophthalmologists (n = 841, 82.5%), followed by neurologists (n = 380, 37.3%), emergency physicians (n = 197, 19.3%), neurosurgeons (n = 75, 7.4%), and others (n = 18, 1.8%). The etiologic distribution of isolated trochlear palsy in the current study did not differ from that of 2664 patients pooled from the previous studies. CONCLUSIONS: The proportion of etiologies of isolated trochlear palsy differs according to the age ranges of the patients and specialties involved in the management. The etiologic distribution of isolated trochlear palsy in the current study was comparable to the pooled result of previous reports.

Optic Disc Classification by Deep Learning versus Expert Neuro-Ophthalmologists.

OBJECTIVE: To compare the diagnostic performance of an artificial intelligence deep learning system with that of expert neuro-ophthalmologists in classifying optic disc appearance. METHODS: The deep learning system was previously trained and validated on 14,341 ocular fundus photographs from 19 international centers. The performance of the system was evaluated on 800 new fundus photographs (400 normal optic discs, 201 papilledema [disc edema from elevated intracranial pressure], 199 other optic disc abnormalities) and compared with that of 2 expert neuro-ophthalmologists who independently reviewed the same randomly presented images without clinical information. Area under the receiver operating characteristic curve, accuracy, sensitivity, and specificity were calculated. RESULTS: The system correctly classified 678 of 800 (84.7%) photographs, compared with 675 of 800 (84.4%) for Expert 1 and 641 of 800 (80.1%) for Expert 2. The system yielded areas under the receiver operating characteristic curve of 0.97 (95% confidence interval [CI] = 0.96-0.98), 0.96 (95% CI = 0.94-0.97), and 0.89 (95% CI = 0.87-0.92) for the detection of normal discs, papilledema, and other disc abnormalities, respectively. The accuracy, sensitivity, and specificity of the system's classification of optic discs were similar to or better than the 2 experts. Intergrader agreement at the eye level was 0.71 (95% CI = 0.67-0.76) between Expert 1 and Expert 2, 0.72 (95% CI = 0.68-0.76) between the system and Expert 1, and 0.65 (95% CI = 0.61-0.70) between the system and Expert 2. INTERPRETATION: The performance of this deep learning system at classifying optic disc abnormalities was at least as good as 2 expert neuro-ophthalmologists. Future prospective studies are needed to validate this system as a diagnostic aid in relevant clinical settings. ANN NEUROL 2020;88:785-795.

Association of lateral rectus muscle volume and ocular motility with the abducens nerve in Duane's retraction syndrome.

PURPOSE: To investigate the relationship between ocular motility and lateral rectus (LR) muscle volume according to the presence or absence of the abducens nerve in patients with Duane's retraction syndrome (DRS) using high-resolution magnetic resonance imaging (MRI). METHODS: A total of 54 unilateral DRS patients were divided into two groups according to high-resolution MRI findings: DRS without an abducens nerve on the affected side (absent CN6 group, n = 45) and DRS with symmetric abducens nerves on both sides (present CN6 group, n = 9). Ocular motility was measured by image analysis based on nine gaze photographs. LR volume was measured on T2-weighted coronal MRI of the orbit, and the ratio of paretic/normal side (P/N) LR volume was investigated. Association of the abducens nerve with various parameters including ocular motility, LR volume, and ratios of P/N LR volume were determined. RESULTS: LR volume was smaller in the affected eye than the non-affected eye in both groups. In the present CN6 group, abducens nerve diameter and the ratio of P/N LR volume showed a positive correlation. A smaller LR volume and more limitation of abduction in the affected eye were predictive of an absent abducens nerve in DRS. CONCLUSIONS: LR muscle hypoplasia was apparent in the affected eye of DRS patients. Abducens nerve diameter positively correlated with the ratio of P/N LR volume in the present CN6 group. Graphical abstract.

Glaucoma-like Parapapillary Choroidal Microvasculature Dropout in Patients with Compressive Optic Neuropathy.

PURPOSE: To characterize peripapillary choroidal microvasculature dropout (MvD) in patients with compressive optic neuropathy (CON) as compared with those with open-angle glaucoma (OAG) using OCT angiography (OCTA). DESIGN: Cross-sectional, observational study. PARTICIPANTS: Eighty-eight eyes of 44 patients with CON; 88 eyes of 88 patients with OAG matched by age, spherical error, and OCT-determined retinal nerve fiber layer thickness (RNFLT); and 88 eyes of 44 control participants matched by age and spherical error. METHODS: Peripapillary microvasculature was evaluated, and peripapillary vessel density was measured in en face images segmented into inner-retinal and choroidal layers using swept-source OCTA. An MvD was defined as a focal sectoral capillary dropout with no visible microvascular network in the choroidal layer. MAIN OUTCOME MEASURES: Comparative characteristics of MvD in eyes with CON and OAG. RESULTS: Microvasculature dropout was observed in 30 eyes (34.1%) of 22 patients (50.0%) with CON, and in 48 eyes of 48 patients (54.5%) with OAG (P = 0.011). All MvDs in the CON group were located in the temporal parapapillary sector, whereas MvDs in the OAG group were located in the temporal-inferior (n = 36) and temporal-superior (n = 4) sectors. At their locations, MvDs in the CON group were accompanied by significant reductions in retinal vessel density and RNFLT, but this was not observed in the OAG group. The presence of MvD was associated significantly with female gender (P = 0.020) and thinner global retinal nerve fiber layer (P = 0.006) in the CON group, but not in the OAG group. CONCLUSIONS: OCT angiography of the peripapillary area showed retinal and choroidal microvasculature impairment in patients with both CON and OAG. However, the features and associated characteristics of MvD differed between these groups, suggesting that the pathogenesis of peripapillary microvascular impairment may be diverse.

Efficacy of bevacizumab injection after pterygium excision and limbal conjunctival autograft with limbal fixation suture.

PURPOSE: To determine the efficacy of subconjunctival bevacizumab injection after pterygium excision with limbal conjunctival autograft and limbal fixation suture. METHODS: This retrospective study included a total of 150 eyes of 150 patients with primary pterygium who received three different procedures after pterygium excision, i.e., 49 eyes with limbal conjunctival graft (group A), 48 eyes with limbal conjunctival autograft with limbal fixation suture (group B), and 53 eyes with limbal conjunctival autograft with limbal fixation suture followed by bevacizumab injection (group C). Image analysis was performed using preoperative anterior segment photographs to measure parameters including relative length, relative width, relative area, and vascularity index of pterygium. Recurrence of pterygium was determined at 1 year after surgery, and outcomes were compared between the 3 groups. Risk factors related to recurrence were evaluated using univariate and multivariate analyses. RESULTS: Recurrence rates after 1 year were 18.4% (9/49), 8.3% (4/48), and 1.9% (1/53) in groups A, B, and C, respectively (P = 0.004). Multivariate analysis showed that patients in group C had significantly reduced risk of recurrence compared with those in group A (P = 0.009), whereas the risk of recurrence was not significantly different between groups A and B (P = 0.227) and groups B and C (P = 0.068), respectively. Among various parameters, higher vascularity index had significant correlation with increased risk of recurrence (P = 0.008). CONCLUSIONS: Bevacizumab injection after limbal conjunctival autograft and limbal fixation suture may effectively reduce recurrence after pterygium excision. The vascularity of pterygium was associated with a higher risk of recurrence.

Ocular coloboma combined with cleft lip and palate: a case report.

BACKGROUND: Ocular coloboma is an excavation of ocular structures that occurs due to abnormal fusion of the embryonic optic fissure. Further, cleft lip/palate (CL/P), a congenital midline abnormality, is caused by a defect in the fusion of the frontonasal, maxillary, and mandibular prominences. No study has reported the association between these two phenotypes in the absence of other systemic abnormalities. We present a case of ocular coloboma along with CL/P and without other neurological abnormalities. CASE PRESENTATION: A 5-year-old Asian boy presented with decreased visual acuity in his right eye. Physical examination revealed no abnormal findings except CL/P, which was surgically corrected at the age of 9 months. Best-corrected visual acuity was 20/60 in the right eye and 20/25 in the left eye. Anterior segment examination revealed iris coloboma in the inferior quadrant of his right eye as well as a large inferonasal optic disc and chorioretinal coloboma in the same eye. He was prescribed glasses based on his cycloplegic refractive errors and part-time occlusion of the left eye was recommended. After 3 months, best-corrected visual acuity improved to 20/30 in the right eye. CONCLUSION: The association of ocular coloboma should be kept in mind when encountering a patient with CL/P without other neurological or systemic abnormalities.

Clinical Perspectives of Parkinson's Disease for Ophthalmologists, Otorhinolaryngologists, Cardiologists, Dentists, Gastroenterologists, Urologists, Physiatrists, and Psychiatrists.

Parkinson's disease (PD) is a multisystemic disorder characterized by various non-motor symptoms (NMS) in addition to motor dysfunction. NMS include sleep, ocular, olfactory, throat, cardiovascular, gastrointestinal, genitourinary, or musculoskeletal disorders. A range of NMS, particularly hyposmia, sleep disturbances, constipation, and depression, can even appear prior to the motor symptoms of PD. Because NMS can affect multiple organs and result in major disabilities, the recognition and multidisciplinary and collaborative management of NMS by physicians is essential for patients with PD. Therefore, the aim of this review article is to provide an overview of the organs that are affected by NMS in PD together with a brief review of pathophysiology and treatment options.

Infrared photographs with a selective wavelength filter to diagnose small-angle esotropia in young children.

PURPOSE: To validate the effectiveness of the selective wavelength filter analysis with infrared photographs for diagnosing small-angle esotropia in children under age 4. METHODS: This is a retrospective, observational, case-control study. A total of 83 esotropes with an esodeviation of ≤ 16 prism diopters (PD) and 75 orthotropic controls under 4 years of age were included. Full-face infrared photographs were taken with a selective wavelength filter in front of either eye. The angles of esodeviation on photographs were measured with the three-dimensional Strabismus Photo Analyzer. The alternate prism and cover test or the Krimsky test were repeatedly performed to measure ocular alignment. RESULTS: The testability of infrared photographs using selective wavelength filters in children under 4 years of age was 85.6%. The mean angle of esodeviation was 11.3 ± 4.0 PD by manual measurements and 11.5 ± 4.4 PD by the infrared photograph analysis. Manual measurements and the infrared photograph analysis showed a strong positive correlation (R = 0.815, P < 0.001). The sensitivity and specificity of the infrared photograph analysis for detecting small-angle esotropia were 95.2% and 77.9%, respectively, with a cutoff value of 4.0 PD. CONCLUSIONS: The automated infrared photograph analysis was simple and effective for diagnosing small-angle esotropia in young children.

Surgical outcomes of strabismus after iatrogenic ophthalmic artery occlusion caused by cosmetic filler injections.

BACKGROUND: To investigate the surgical outcomes of strabismus related to iatrogenic occlusion of the ophthalmic artery and its branches from cosmetic facial filler injection. METHODS: A retrospective study was performed on 6 patients who underwent strabismus surgery among 23 patients who had suffered occlusion of the ophthalmic artery and its branches after cosmetic facial filler injection. Initial, preoperative and final ocular motility examinations, the type of surgery and surgical outcomes were evaluated. RESULTS: At initial presentation, visual acuity was no light perception in 5 patients and hand motion in one patient. Five out of 6 patients showed initial ophthalmoplegia. Among these 5 patients, eye motility fully recovered in 3 patients although sensory strabismus developed during follow-up, while the remaining 2 patients had persistent ocular motility limitations. Strabismus surgery was performed at 2.2 ± 1.5 years after iatrogenic ophthalmic artery occlusion. Preoperatively, 5 of the 6 patients showed exotropia, and one patient had esotropia. Vertical deviation was found in 3 out of 6 patients in addition to the horizontal deviation. Successful outcome was achieved only in the 4 patients without persistent ophthalmoplegia after 1.4 ± 1.0 years from surgery. The other two patients with persistent ocular motility limitations failed to achieve successful alignment after surgery, and one patient eventually underwent evisceration due to phthisis bulbi. CONCLUSIONS: In our study, surgical outcomes of strabismus caused by cosmetic facial filler injection were successful only in patients without persistent ophthalmoplegia at the time of surgery.

Dry Eye Symptoms May Have Association With Psychological Stress in Medical Students.

PURPOSE: To evaluate the prevalence and risk factors of symptomatic dry eye disease (DED) among medical students. METHODS: This cross-sectional study included 209 students at a medical school in Korea. Dry eye symptoms were assessed using a 9-item questionnaire, and DED was defined as having one or more dry eye symptoms often or all the time. The ocular surface disease index (OSDI) and visual analog scale (VAS) questionnaires were also administered to quantify the dry eye symptoms. A survey including demographic data, potential risk factors for DED, personal habits, and psychological stress was also performed. RESULTS: Of the 209 students, 188 (93 men and 95 women) completed the survey. The average age was 28.0±2.5 (mean±SD; range, 23-37) years. Prevalence of DED was 27.1% (51/188). Participants with DED had significantly higher VAS and OSDI than those without DED (P<0.001 for both). Univariate analysis revealed that female sex (P=0.001), contact lens (CL) wear (P=0.034), prolonged computer use (P=0.001), and higher psychological stress score (P<0.001) had significant association with DED. Multivariate analysis also showed the significant association between DED and female sex (P=0.026), CL wear (P=0.042), prolonged computer use (P=0.004), and higher stress score (P=0.014). CONCLUSIONS: Symptomatic DED was prevalent among medical students. Increased psychological stress was associated with higher risk of DED. Its risk also increased among women, CL wearers, and prolonged computer users.

IgG4-related ophthalmic disease involving extraocular muscles: case series.

BACKGROUND: To elucidate the clinical features of strabismus associated with IgG4-related ophthalmic disease (IgG4-ROD). All of the four patients with IgG4-ROD showed marked enlargement of the extraocular muscles, however, two patients showed orthotropia with full ductions and versions. One patient showed a small angle of exotropia and hypertropia of less than 5 prism diopters. One remaining patient showed orthotropia, full ductions and versions despite marked enlargement of the extraocular muscles, then developed hypertropia up to 35 prism diopters with activation of inflammation, which promptly improved after treatment with oral steroids. CONCLUSIONS: IgG4-ROD usually shows normal ocular motility despite extraocular muscle enlargement, which is the key distinguishing feature from other orbital inflammatory diseases. Active flare-up with increased serum IgG4 levels may produce a large angle of eye deviation, but mostly respond well to steroid treatment.

Case Report: Ocular Myasthenia Gravis Associated with In Vitro Fertilization Procedures.

SIGNIFICANCE: Ocular myasthenia gravis is a localized form of myasthenia gravis, which is a postsynaptic disorder of the neuromuscular junction that causes fluctuating weakness of extraocular muscles resulting from autoimmune mechanisms. In women with myasthenia, changes in sex hormone levels and administration of corticosteroids can trigger or worsen symptoms of myasthenia gravis. PURPOSE: To describe a case of seronegative ocular myasthenia gravis whose first symptom appeared a day after in vitro fertilization procedure. CASE REPORT: A 37-year-old woman suddenly developed mild ptosis and fluctuating diplopia that worsened in the evening. Before the development of symptoms, she had undergone in vitro fertilization procedure and had taken oral steroids. Ocular motility examination revealed an intermittent exotropia in primary gaze at both distance and near. The neostigmine test confirmed her diagnosis as ocular myasthenia gravis. CONCLUSIONS: When taking a history for young women with sudden onset of binocular diplopia, steroids and sex hormones should be taken into account, which may trigger or exacerbate symptoms of ocular myasthenia gravis.

Incidental branch retinal artery occlusion on optical coherence tomography angiography presenting as segmental optic atrophy in a child: a case report.

BACKGROUND: Retinal artery occlusion is extremely rare in the pediatric population and most patients have risk factors. We report a case of a healthy child with segmental optic atrophy, complicated by incidental branch retinal artery occlusion (BRAO). CASE PRESENTATION: A 10-year-old boy who had a history of his mother's gestational diabetes presented with an inferonasal visual field defect in the left eye. His best-corrected visual acuities were 20/20 in both eyes (OU). Fundoscopic examination revealed segmental pallor of the left optic disc, thinning of the superotemporal rim, a relative superior entrance of the central retinal artery and superior peripapillary scleral halo. Fluorescein angiography showed patchy filling delays in the corresponding disc area without retinal vascular abnormalities. Spectral domain optical coherence tomography (SD OCT) via automated segmentation analysis demonstrated sectoral absence of the ganglion cell layer and retinal nerve fiber layer with thinning of the inner plexiform layer, inner nuclear layer and outer plexiform layer in the corresponding retina. OCT angiography (OCTA) showed focal attenuation of superficial and intermediate/deep capillary plexuses in the corresponding areas. Systemic evaluation was unremarkable. The patient was diagnosed with segmental optic atrophy caused by incidental BRAO. CONCLUSIONS: Retinal vascular occlusions are rare in childhood, and may present as segmental optic atrophy mimicking congenital anomalies. OCTA allows the detection of previous microvascular abnormalities in the chronic phase. To the best of our knowledge, this is the first report of a child with segmental optic atrophy presumably caused by BRAO, which was documented by SD OCT and OCTA in detail.

Combined Brown syndrome and superior oblique palsy without a trochlear nerve: case report.

BACKGROUND: Congenital Brown syndrome is characterized by limited elevation particularly during adduction. The pathogenesis of congenital Brown syndrome is still controversial. CASE PRESENTATION: A 6-year-old boy had been tilting his head to the left since infancy. He showed right hypertropia (RHT) of 2 prism diopters (Δ) in the primary position. He showed RHT 6Δ in right gaze, RHT 2Δ in left gaze, RHT 12Δ in right head tilt, and orthotropia in left head tilt. The right eye showed limitation of elevation and depression on adduction, and the left eye showed overdepression on adduction. MR images showed an absent right trochlear nerve with a hypoplastic ipsilateral superior oblique muscle. CONCLUSIONS: Congenital Brown syndrome may be associated with an absent trochlear nerve and hypoplastic superior oblique muscle suggesting an etiologic mechanism of congenital cranial dysinnervation disorder.