Faculty Bibliography

PURPOSE/OBJECTIVE:To develop consensus nomenclature amongst international retinal specialists for the distinctive optical coherence tomography (OCT) finding of a lesion originating from the retinal capillary bed, measuring ≥100 µm in size, and characterized by a hyperreflective wall with a hyporeflective lumen. METHODS:A comprehensive literature search was performed from inception to January 2024 on three databases to elicit publications reporting on relevant vascular abnormalities and corresponding nomenclature. A panel of retinal specialists with expertise in this topic reviewed the list of candidate terms and proposed other names for the lesion of interest. A refined list was then incorporated into a Delphi survey, which was distributed to the general membership of the International Retinal Imaging Society (IntRIS). Consensus was defined as at least 70% agreement amongst participants. RESULTS:An expert panel (n=11) reviewed candidate names for the lesion, with poor agreement noted amongst panel members regarding the relevant nomenclature. In the first Delphi survey, (n=70 IntRIS members), the need for a unified nomenclature was highlighted and two leading candidate names were established: large retinal capillary aneurysm (LRCA, n=38, 54.3%) and retinal capillary macroaneurysm (n=14, 20.0%). A second follow-up survey (n=54 IntRIS members) established LRCA (n=44, 81.5%) as the consensus term to identify the OCT vascular abnormality. CONCLUSION/CONCLUSIONS:This Delphi project reached consensus on a unifying term, large retinal capillary aneurysm, for a specific and signature OCT lesion. Identification of this characteristic OCT finding and adoption of this term may facilitate diagnosis, guide therapeutic decisions, and improve clinical and scientific communication.

Neuropathic corneal pain (NCP) is an underdiagnosed ocular disorder caused by aberrant nociception and hypersensitivity of corneal nerves, often resulting in chronic pain and discomfort even in the absence of noxious stimuli. Recently, microneuromas (aberrant growth and swelling of the corneal nerve endings) detected using in vivo confocal microscopy (IVCM) have emerged as a promising biomarker for NCP. However, this process is time-intensive and error-prone, limiting its clinical use and availability. In this work, we present a new NCP screening system based on a deep learning model trained to detect microneuromas using a multisite dataset with a combined total of 103,168 IVCM images. Our model showed excellent discriminative ability detecting microneuromas (AuROC: 0.97) and the ability to generalize to data from a new institution (AuROC: 0.90). Additionally, our pipeline provides an uncertainty quantification mechanism that allows it to communicate when its predictions are reliable, further increasing its clinical relevance.

BACKGROUND:The number of pediatric ophthalmology and strabismus (PO&S) fellowship positions filled each year remains consistently lower compared with other subspecialities. It is unclear where along the recruitment pathway trainees interested in pediatrics decide to select other subspecialities. This study assesses for differences in the competitiveness of ophthalmology residency applicants based on interest in PO&S, which may impact their ability to matriculate into residency. METHODS:and Mann-Whitney U tests were used to compare categorical and continuous variables, respectively. RESULTS:Of the 631 applications reviewed, 125 (19.8%) of applicants stated a subspeciality interest. Among those who stated an interest, 34 (27.2%) were interested in PO&S. When compared to all other applicants (with or without a stated subspecialty interest), applicants interested in PO&S were more likely to be female (P < 0.001). No significant difference was found in academic, research, or extracurricular variables based on subspeciality interest in PO&S. The same was true when applicants interested in PO&S were compared to those interested in competitive subspecialities (cornea, glaucoma, retina, or oculoplastics). CONCLUSIONS:Applicants interested in PO&S were observed to be as competitive as other applicants. Among students who expressed a fellowship interest, there was a high proportion interested in pediatrics. Recruitment efforts can be targeted toward encouraging medical students interested in pediatrics to apply into ophthalmology.

To describe chorioretinal changes in a single case of Boucher-Neuhauser Syndrome (BNHS) over 45 years of follow-up. Methods: Retrospective chart review was performed. Color fundus photography from 1977 to 2003 was obtained and digitized. Current fundus photography was obtained with widefield imaging. High-resolution spectral-domain optical coherence tomography (OCT) was performed. Genetic analysis was performed using an inherited retinal disorders panel. Results: Fundus examination demonstrated central chorioretinal atrophy with sclerotic choroidal vessels. Short posterior ciliary arteries became more prominent and tortuous over time. Mid-peripheral atrophy extends to the equator and demonstrates a scalloped pattern with islands of atrophy intervening with areas of normal retina. The far periphery remained minimally affected. High-resolution OCT demonstrated outer retinal atrophy and choriocapillaris loss. Genetic testing showed a homozygous variant for PNPLA6 and a heterozygous variant for TYRP1. Conclusion: Chorioretinal changes in BNHS vary in onset and severity. It is important to diagnose this condition in order to begin timely management of visual and systemic sequelae.

PURPOSE/OBJECTIVE:Thyroid eye disease-related retraction and strabismus treatment is complicated by the activity level of the disease. Botulinum toxin injection can provide relief of symptoms in lieu of, or while waiting for surgery, radiation, or alternative medications. This study reviews techniques, outcomes, and effectiveness of botulinum toxin usage in thyroid eye disease. METHODS:A systematic review was conducted using PubMed, Embase, Web of Science, and Cochrane to identify research investigating botulinum toxin treatment of thyroid eye disease through May 2024. A meta-analysis was performed on change in marginal reflex distance in retraction patients, resolution of diplopia in strabismus patients, necessity of further strabismus surgery, and side effects. RESULTS:Of 157 studies screened by 2 reviewers, 30 underwent analysis. In 19 upper eyelid retraction studies (299 patients), 1.5 to 15 U Botox (onabotulinum toxin A) or 10 to 40 U Dysport (abobotulinum toxin A) was administered to the superior tarsal border, with an 84% success rate and an average decrease in marginal reflex distance of 2.42 mm lasting 1 to 6 months. In 10 strabismus studies (205 patients), 5 to 20 U Botox or 25 U Dysport was administered in extraocular muscles; 24% achieved resolution of diplopia lasting 2 to 6 months, while 58% required further surgical management. In upper eyelid retraction studies, side effects included ptosis (13%) and diplopia (2%). In strabismus studies, side effects included ptosis (2%). CONCLUSIONS:This systematic review and meta-analysis confirmed that botulinum toxin is an effective treatment for thyroid eye disease-related lid retraction and strabismus. Randomized controlled studies are warranted to optimize botulinum toxin administration.

PURPOSE/OBJECTIVE:We describe a case of secondary multiple evanescent white dot syndrome (MEWDS) with optic disc pit as the underlying triggering pathology. METHODS:Observational case report. RESULTS:A 41-year-old well man with a background of right optic disc pit presented with right eye loss of vision and photopsia. Visual acuity at presentation was 20/100. He was found to have clinical and multimodal imaging features consistent with right unilateral MEWDS, and the spatial distribution of lesions made it likely that the optic disc pit was the trigger. Fundus autofluorescence revealed hyperautofluorescent 'spots' that gradually faded over serial imaging, with the complete absence of hyperautofluorescent 'dots'. Over three months of follow-up visual acuity recovered to 20/25. CONCLUSION/CONCLUSIONS:Optic disc pit may act as a trigger for secondary MEWDS, which is an increasingly recognized epiphenomenon. Secondary MEWDS may present as 'spots without dots', and we hypothesize that this feature may differentiate it from primary MEWDS.

PURPOSE/OBJECTIVE:To describe the clinical and multimodal imaging features, and long-term outcomes, of acquired vitelliform lesions (AVLs) in angioid streaks (AS). METHODS:Retrospective case series including 14 patients (23 eyes) with AS-related AVLs. Clinical data, color fundus photography, fundus autofluorescence, spectral-domain optical coherence tomography (OCT), en face OCT, and OCT angiography were evaluated at baseline and final visits. Snellen visual acuity (VA), lesion dimensions, subfoveal choroidal thickness (SFCT), and outer retinal integrity were recorded. RESULTS:AS were secondary to pseudoxanthoma elasticum in 64.3% and idiopathic in 35.7%. Baseline VA was 0.18 ± 0.17 LogMAR (20/30) and declined to 0.43 ± 0.33 LogMAR (20/50) over a mean follow-up of 77 months (p<0.001). AVLs were often foveal (78.3%), multifocal (82.6%), and peripapillary (73.9%), with OCT detecting subretinal hyperreflective material in all eyes. Both lesion width and SFCT decreased over time. Complete retinal pigment epithelium (RPE) and outer retinal atrophy increased from 17.4% to 69.6%, and exudative choroidal neovascularization developed in 26.1%. CONCLUSION/CONCLUSIONS:AS-related AVLs represent a rare phenotype reflecting multifactorial pathogenesis involving Bruch's membrane alterations and RPE dysfunction. Over prolonged follow-up, lesion size decreased, yet progressive retinal atrophy led to significant vision loss. Further research is warranted to clarify disease progression and optimize treatment approaches.

IMPORTANCE/UNASSIGNED:High-quality evidence regarding suppressive valacyclovir treatment in herpes zoster ophthalmicus (HZO) is necessary to guide care. OBJECTIVE/UNASSIGNED:To determine whether suppressive valacyclovir compared with placebo delays the occurrence of new or worsening stromal keratitis (SK), endothelial keratitis (EK), iritis, or dendriform epithelial keratitis (DEK) during 12 months of treatment and if treatment benefit persisted at 18 months (secondary end point). DESIGN, SETTING, AND PARTICIPANTS/UNASSIGNED:The Zoster Eye Disease Study (ZEDS) was a randomized clinical trial conducted in 95 sites from November 2017 to June 2024. Immunocompetent, nonpregnant adults with a history of an HZO rash, documented active keratitis or iritis within 1 year, and an estimated glomerular filtration rate of 45 mL/min/1.73 m2 or greater were eligible. After determined to be eligible, participants were randomized in 4 strata: age at onset (<60 years vs ≥60 years) and disease duration (<6 months vs ≥6 months). INTERVENTIONS/UNASSIGNED:A total of 12 months of double-masked daily valacyclovir, 1000 mg, or placebo. MAIN OUTCOMES AND MEASURES/UNASSIGNED:The primary outcome was time to first occurrence within 12 months of new or worsening SK, EK, iritis, or DEK. RESULTS/UNASSIGNED:A total of 527 participants (median [IQR] age, 60 [50-68] years; 266 female [50.5%]; 266 in the valacyclovir group; 261 in the placebo group) were randomized in 4 strata; 481 completed 12 months, and 460 completed 18 months. Data were analyzed by intention to treat. At 12 months, primary end points occurred in 86 participants (33%) assigned to placebo and 74 (28%) assigned to valacyclovir, and at 18 months in 104 participants (40%) assigned to placebo and 86 (32%) assigned to valacyclovir. The hazard ratio (HR) of the primary end point at 12 months was 0.77 for participants taking valacyclovir vs placebo (HR, 0.77; adjusted 95% CI, 0.56-1.05; P = .09) and 0.73 at the secondary end point at 18 months (HR, 0.73; adjusted 95% CI, 0.55-0.97; P = .03). There was a reduction of multiple other secondary end points at 12 months (HR, 0.70; 95% CI, 0.52-0.95; P = .02) and 18 months (HR, 0.72; 95% CI, 0.55-0.95; P = .02). CONCLUSIONS AND RELEVANCE/UNASSIGNED:Although the primary outcome did not show a benefit of suppressive valacyclovir treatment, secondary study outcomes showed treatment superiority at the 18-month end point and reduced number of multiple episodes of keratitis or iritis at both 12 and 18 months. These results support consideration of 1 year of suppressive valacyclovir treatment for HZO. TRIAL REGISTRATION/UNASSIGNED:ClinicalTrials.gov Identifier: NCT03134196.