Demographics, Practice Analysis, and Geographic Distribution of Neuro-Ophthalmologists in the United States in 2023.

PURPOSE: To assess the demographic characteristics and geographic distribution of neuro-ophthalmologists practicing in the United States. DESIGN: A cross-sectional study. PARTICIPANTS: Neuro-ophthalmologists across the United States. METHODS: In this cross-sectional study, public databases from the American Academy of Ophthalmology, North American Neuro-ophthalmology Society, American Neurological Association, and American Academy of Neurology were used to identify neuro-ophthalmologists in the United States as of April 2023. Providers' office locations were geocoded using ArcGIS pro, version 2.9 (Esri). Data on age, sex, and residency and fellowship training were collected. Analysis was performed using SPSS 28.0 (IBM Corp.). MAIN OUTCOME MEASURES: Neuro-ophthalmologists' demographics, and information about their medical education, postgraduate education, residency training, fellowship training, years in practice, practice environment, and geographic distribution of neuro-ophthalmologists across the United States. RESULTS: A total of 635 neuro-ophthalmologists (436 male, 68.7%) were identified. The majority (599, 94.3%) graduated from an allopathic medical school. Most of the 85 physicians who held a secondary graduate degree had a PhD (54, 63.5%). Although approximately three-quarters (429, 67.6%) completed their residency in ophthalmology, 159 (25%) had residency positions in neurology and 47 (7.4%) had residency positions in both. Approximately one-third (191, 30.0%) were trained in more than 1 fellowship, including oculoplastics (78, 12.3%) or pediatric ophthalmology (53, 8.3%). The average post-fellowship years of experience was 23.7±13.7 years, with 134 (21.1%) in their early career (< 10 years), 120 (18.9%) in their mid-careers (10-19 years), and 381 (60.0%) in their late careers (> 20 years). Male neuro-ophthalmologists had 10.5±1.1 more years of experience than female neuro-ophthalmologists (P < 0.001). Three states (Maine, South Dakota, Wyoming) and 2897 counties (93.2%) had no neuro-ophthalmologists. Counties without a neuro-ophthalmologist had lower median income (P < 0.001), lower access to a vehicle (P = 0.024), and lower rates of health insurance (P = 0.012). CONCLUSIONS: Practicing neuro-ophthalmologists are mostly male and often are trained in more than 1 subspecialty. More than half of the practicing neuro-ophthalmologists are in their late careers, which may further exacerbate the existing geographic and socioeconomic disparities in access to neuro-ophthalmology. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

The Risk of Sympathetic Ophthalmia Associated with Open-Globe Injury Management Strategies: A Meta-analysis.

TOPIC: Sympathetic ophthalmia (SO) is a sight-threatening granulomatous panuveitis caused by a sensitizing event. Primary enucleation or primary evisceration, versus primary repair, as a risk management strategy after open-globe injury (OGI) remains controversial. CLINICAL RELEVANCE: This systematic review was conducted to report the incidence of SO after primary repair compared with that of after primary enucleation or primary evisceration. This enabled the reporting of an estimated number needed to treat. METHODS: Five journal databases were searched. This review was registered with International Prospective Register of Systematic Reviews (identifier, CRD42021262616). Searches were carried out on June 29, 2021, and were updated on December 10, 2022. Prospective or retrospective studies that reported outcomes (including SO or lack of SO) in a patient population who underwent either primary repair and primary enucleation or primary evisceration were included. A systematic review and meta-analysis were carried out in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Random effects modelling was used to estimate pooled SO rates and absolute risk reduction (ARR). RESULTS: Eight studies reporting SO as an outcome were included in total. The included studies contained 7500 patients and 7635 OGIs. In total, 7620 OGIs met the criteria for inclusion in this analysis; SO developed in 21 patients with OGI. When all included studies were pooled, the estimated SO rate was 0.12% (95% confidence interval [CI], 0.00%-0.25%) after OGI. Of 779 patients who underwent primary enucleation or primary evisceration, no SO cases were reported, resulting in a pooled SO estimate of 0.05% (95% CI, 0.00%-0.21%). For primary repair, the pooled estimate of SO rate was 0.15% (95% CI, 0.00%-0.33%). The ARR using a random effects model was -0.0010 (in favour of eye removal; 95% CI, -0.0031 [in favor of eye removal] to 0.0011 [in favor of primary repair]). Grading of Recommendations, Assessment, Development, and Evaluations analysis highlighted a low certainty of evidence because the included studies were observational, and a risk of bias resulted from missing data. DISCUSSION: Based on the available data, no evidence exists that primary enucleation or primary evisceration reduce the risk of secondary SO. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Surgical Treatments to Improve Visual Acuity in Infantile Nystagmus Syndrome: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the literature on the efficacy of surgical procedures to improve visual acuity (VA) in patients with infantile nystagmus syndrome (INS). METHODS: Literature searches were last conducted in January 2022 in the PubMed database for English-language studies with no date restrictions. The combined searches yielded 354 abstracts, of which 46 were reviewed in full text. Twenty-three of these were considered appropriate for inclusion in this assessment and were assigned a level of evidence rating by the panel methodologist. RESULTS: One included study was a randomized trial; the remaining 22 were case series. The 23 studies included children and adults with INS and a variable proportion with anomalous head position (AHP), strabismus, and sensory diagnoses. The surgical interventions evaluated included large recessions, tenotomy and reattachment (TAR), myectomy with or without pulley fixation, and anterior extirpation of the 4 horizontal rectus muscles, as well as various procedures to correct an AHP in which VA was reported as a secondary outcome. The data were mixed, with improvements in binocular best-corrected visual acuity (BCVA) ranging from no improvement to 0.3 logarithm of the minimum angle of resolution (logMAR), or 3 lines. (Most studies were in the range of 0.05-0.2 logMAR.) Statistically significant improvement in VA was noted in 12 of 16 studies (75%) that performed statistical analyses, with no clear advantage of any single procedure. Complications and reoperations were lowest in patients who underwent TAR and highest in those who underwent myectomy or anterior extirpation. CONCLUSIONS: The best available evidence suggests that eye muscle surgery in patients with INS results in a modest improvement in VA. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Levodopa/Carbidopa to Augment the Treatment of Amblyopia: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the published literature on the use of levodopa/carbidopa to augment the treatment of amblyopia. METHODS: Literature searches for English language studies were last conducted in October 2022 in the PubMed database with no date restrictions. The combined searches yielded 55 articles, of which 23 were reviewed in full text. Twelve of these were considered appropriate for inclusion in this assessment and were assigned a level of evidence rating by the panel methodologist. Nine studies were rated level I, and 3 studies were rated level II; there were no level III studies. RESULTS: The duration of treatment was limited to 3 to 16 weeks because of concern about long-term adverse effects such as tardive dyskinesia. This complication was not reported in any of the study participants. The dose of levodopa ranged from 1.5 to 8.3 mg/kg/day, generally divided into 3 daily doses. The carbidopa dose was approximately 25% of the levodopa dose in all treatments. Evidence from these studies indicates that augmenting traditional patch occlusion therapy with the oral administration of levodopa/carbidopa can improve the vision of amblyopic children, but the effect was small (0.17-0.3 logarithm of the minimum angle of resolution [logMAR] units) and only statistically significant when compared with patching alone in 2 of the 12 studies cited. Regression of vision was reported in the majority of studies (9 of 12 reported; range, 0-0.17 logMAR unit regression) after discontinuation of therapy. Short-term side effects of the medications were not consistently reported but were most frequently mild and included headache and nausea. CONCLUSIONS: The best available evidence is currently insufficient to show that augmenting amblyopia therapy using up to 16 weeks of levodopa/carbidopa will result in meaningful improvement in visual acuity. Given the potential for significant side effects such as tardive dyskinesia with long-term therapy, levodopa/carbidopa does not appear to be a viable option for amblyopia therapy FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Effectiveness of Laser Refractive Surgery to Address Anisometropic Amblyogenic Refractive Error in Children: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the published literature assessing the safety and effectiveness of laser refractive surgery to treat anisometropic amblyogenic refractive error in children aged ≤ 18 years. METHODS: A literature search of the PubMed database was conducted in October 2021 with no date limitations and restricted to publications in English. The search yielded 137 articles, 69 of which were reviewed in full text. Eleven articles met the criteria for inclusion and were assigned a level of evidence rating. RESULTS: The 11 included articles were all level III evidence and consisted of 1 case-control study and 10 case series. Six studies used laser-assisted in situ keratomileusis (LASIK), 1 used photorefractive keratectomy (PRK), 1 used refractive lenticule extraction/small incision lenticule extraction, and the rest used a combination of LASIK, PRK, laser epithelial keratomileusis (LASEK), or refractive lenticule extraction/small incision lenticule extraction. Five studies enrolled patients with anisometropic myopia, 2 studies enrolled patients with anisometropic hyperopia, and the remainder were mixed. Although all studies demonstrated an improvement in best-corrected visual acuity (BCVA), the magnitude of improvement varied widely. As study parameters varied, a successful outcome was defined as residual refractive error of 1 diopter (D) or less of the target refraction because this was the most commonly used metric. Successful outcomes ranged between 38% and 87%, with a mean follow-up ranging from 4 months to 7 years. Despite this wide range, all studies demonstrated an improvement in the magnitude of anisometropia. Regression in refractive error occurred more frequently and to a greater degree in myopic eyes and eyes with longer follow-up, and in younger patients. Although one study reported 2 free flaps, most studies reported no serious adverse events. The most common complications were corneal haze and striae. CONCLUSIONS: Findings from included studies suggest that laser refractive surgery may address amblyogenic refractive error in children and that it appears to decrease anisometropia. However, the evidence for improvement in amblyopia is unclear and long-term safety data are lacking. Long-term data and well-designed clinical studies that use newer refractive technologies in standardized patient populations would help address the role of refractive surgery in children and its potential impact on amblyopia.

FOLLOW-UP ADHERENCE IN PATIENTS WITH NONPROLIFERATIVE DIABETIC RETINOPATHY PRESENTING TO AN OPHTHALMIC EMERGENCY DEPARTMENT.

PURPOSE: To determine the rate of follow-up after emergent encounters for nonproliferative diabetic retinopathy and to identify patient or visit characteristics associated with follow-up adherence. METHODS: A retrospective cohort study of patients presenting to an ophthalmic emergency department with nonproliferative diabetic retinopathy between May 2014 and December 2018 was conducted. Demographic and encounter data were gathered. Adherence to follow-up was defined as a completed encounter within 5 weeks of the recommended follow-up. RESULTS: A total of 1,248 patients were included. The overall follow-up rate was 53%. Significantly decreased odds of follow-up adherence were associated with longer physician recommended follow-up intervals (odds ratio: 0.81, P < 0.001), longer interval to scheduled appointment (OR: 0.98, P < 0.001), commercial insurance (OR: 0.76, P = 0.01), and lack of any insurance (OR: 0.57, P < 0.01). Significantly increased odds were associated with a longer emergency department visit duration (OR: 1.002, P = 0.001), farther home distance (1.02, P < 0.01), increased likelihood of living in a higher income area (OR: 1.07, P = 0.04), greater NPDR severity (OR: 1.23, P < 0.01), Medicare (OR: 1.38, P = 0.04), presence of macular edema (OR: 1.66, P < 0.001), and worse vision (OR: 1.73, P < 0.001). CONCLUSION: Patients presenting emergently with non-proliferative diabetic retinopathy are at high risk for follow-up nonadherence. Several patient and encounter characteristics were associated with follow-up adherence.

Gut microbial dysbiosis in individuals with Sjögren's syndrome.

BACKGROUND: Autoimmune diseases have been associated with changes in the gut microbiome. In this study, the gut microbiome was evaluated in individuals with dry eye and bacterial compositions were correlated to dry eye (DE) measures. We prospectively included 13 individuals with who met full criteria for Sjögren's (SDE) and 8 individuals with features of Sjögren's but who did not meet full criteria (NDE) for a total of 21 cases as compared to 21 healthy controls. Stool was analyzed by 16S pyrosequencing, and associations between bacterial classes and DE symptoms and signs were examined. RESULTS: Results showed that Firmicutes was the dominant phylum in the gut, comprising 40-60% of all phyla. On a phyla level, subjects with DE (SDE and NDE) had depletion of Firmicutes (1.1-fold) and an expansion of Proteobacteria (3.0-fold), Actinobacteria (1.7-fold), and Bacteroidetes (1.3-fold) compared to controls. Shannon's diversity index showed no differences between groups with respect to the numbers of different operational taxonomic units (OTUs) encountered (diversity) and the instances these unique OTUs were sampled (evenness). On the other hand, Faith's phylogenetic diversity showed increased diversity in cases vs controls, which reached significance when comparing SDE and controls (13.57 ± 0.89 and 10.96 ± 0.76, p = 0.02). Using Principle Co-ordinate Analysis, qualitative differences in microbial composition were noted with differential clustering of cases and controls. Dimensionality reduction and clustering of complex microbial data further showed differences between the three groups, with regard to microbial composition, association and clustering. Finally, differences in certain classes of bacteria were associated with DE symptoms and signs. CONCLUSIONS: In conclusion, individuals with DE had gut microbiome alterations as compared to healthy controls. Certain classes of bacteria were associated with DE measures.

Correlation between age and corneal edema in pediatric patients with Peters anomaly.

PURPOSE: To evaluate corneal edema in different-aged pediatric patients with Peters anomaly and to correlate in vivo with ex vivo histopathologic findings. METHODS: A retrospective cross-sectional study was performed. The medical records of patients diagnosed with Peters anomaly who underwent examination under anesthesia (EUA) between 2011 and 2015 were reviewed. Eyes in which central corneal thickness (CCT) measurements were taken were included. The thickest point in the CCT pachymetric map was used to objectively quantify corneal edema. Correlation between CCT and age was calculated. Additionally, a retrospective review of histopathologic studies of excised corneal buttons from pediatric eyes with Peters anomaly between 2011 and 2015 was performed. RESULTS: Eighteen eyes of 12 children were included. Mean age was 14 ± 15 months, and mean CCT was 842 ± 304 µm. A significant inverse correlation was noted between the CCT and the age of the patients, with lower CCT values in older children (r = 0.6; P = 0.01). Seven excised corneal buttons that underwent penetrating keratoplasty were reviewed. All corneal buttons showed absence of Descemet membrane and localized absence of endothelium. However, three specimens showed presence of corneal endothelium in areas of absent or attenuated Descemet membrane. CONCLUSIONS: In Peters anomaly, the CCT decreases with age, possibly due to a decrease in corneal edema. Histopathologic studies show cases of endothelial expansion in areas of absent or attenuated Descemet membrane. This may contribute to improved endothelial function and decreased edema with age.

Outcome of optical iridectomy in Peters anomaly.

PURPOSE: Optical iridectomy creates a defect in the iris to allow an alternative clear visual axis in cases of central corneal opacities occluding the pupillary axis. The purpose of this study is to evaluate the outcomes of optical sector iridectomy in children with Peters anomaly. METHODS: Retrospective case series. The medical records of all patients diagnosed with Peters anomaly who underwent optical iridectomy during the years 2002-2014 were reviewed. Data collection included surgical and visual acuity outcomes. RESULTS: Twenty-nine eyes (22 patients) were included in the study. Mean age at the time of surgery was 15.6 ± 26.3 months. Eighteen (81.8%) patients had bilateral disease. No intraoperative complications occurred. A red reflex was obtained in 28 (96.6%) eyes after surgery. Mean visual acuity improved from 2.5 ± 0.3 to 1.8 ± 0.6 in logMAR (p < 0.001). Vision improved in 21 (72.4%) eyes, remained stable in 5 (17.2%) eyes, and deteriorated in 3 (10.3%) eyes. Postoperatively visual acuity improved significantly in the patients with the bilateral disease (p < 0.05), but not in the unilateral group (p = 0.056). Mean follow-up time was 41.6 ± 43.8 months. During the follow-up period, five (17.2%) eyes were diagnosed with glaucoma, two (6.9%) eyes underwent PK, one (3.4%) eye underwent an additional sector iridectomy, and one (3.4%) eye underwent keratoprosthesis. CONCLUSIONS: In this largest series published of optical iridectomy for Peters anomaly, it was found to be a safe procedure. Improvement in visual acuity is expected, particularly in bilateral cases. The utility of optical iridectomy in unilateral cases necessitates further studies.

Systemic Amyloidosis and Extraocular Muscle Deposition.

Isolated amyloid deposition in an extraocular muscle is a rare event but can be a presenting feature of systemic amyloidosis. A 67-year-old woman with an acquired exotropia and hypertropia was found to have unilateral diffuse extraocular muscle enlargement on magnetic resonance imaging. Owing to the progressive nature of her strabismus and the negative laboratory testing for thyroid disease, she underwent an extraocular muscle biopsy that revealed amyloid deposition. Further workup demonstrated a monoclonal gammopathy consistent with systemic amyloidosis. This case demonstrates the need to consider amyloidosis in the differential diagnosis of patients presenting with an atypical acquired strabismus. We review other reports of isolated amyloid deposition in extraocular muscles and its association with systemic amyloidosis, emphasizing the importance of the ophthalmologist in the early recognition of this disease to prevent irreversible, life-threatening end organ damage.

Idiopathic Unilateral Enlargement of the Extraocular Muscles in an Infant.

A 6-month-old boy presented with a unilateral motility deficit of the right eye in all fields of gaze. Neuroimaging revealed unilateral enlargement of the medial, lateral, and inferior rectus muscles with sparing of the tendons. An evaluation for thyroid eye disease, idiopathic orbital inflammation, myositis, inflammatory and neoplastic infiltration of the muscle, vascular anomalies, and metastatic neuroblastoma was unrevealing. Biopsy of the muscle revealed normal architecture with an absence of inflammation, infiltration, or fibrosis. A review of the literature reveals the exceptionally rare nature of this finding. While the authors cannot rule out an atypical case of congenital euthyroid eye disease, this constellation of findings is not consistent with thyroid eye disease and may represent previously described cases of idiopathic enlargement of the extraocular muscles.

A novel characterization of posterior keratoconus using anterior segment optical coherence tomography in an infant: a case report.

BACKGROUND: Posterior keratoconus is a rare cause of a corneal opacity in an infant. It is characterized by thinning of the posterior cornea without ectasia of the anterior cornea. Imaging of this condition with anterior segment optical coherence tomography (AS-OCT) has not been reported in the literature. CASE PRESENTATION: A six week old African-American male presented with a congenital corneal opacity of the right eye. He underwent an examination under anesthesia in which photography and AS-OCT were performed. AS-OCT confirmed the diagnosis of posterior keratoconus. The patient subsequently underwent an optical iridectomy for visual development. CONCLUSION: AS-OCT is a useful tool in cases when a child presents with a corneal opacity of unknown or unclear etiology. In our patient, AS-OCT showed the classic description of central corneal thinning seen in this condition. Additionally, it revealed an associated detached Descemet membrane, a feature which has not been previously described in posterior keratoconus.