Searched for: department:Ophthalmology
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school:SOM
Clinical and Multimodal Imaging of Acute Outer Retinopathy Expanding the Spectrum of Acute Annular Outer Retinopathy
Ramtohul, Prithvi; Cicinelli, Maria Vittoria; Chen, Fred K; Oh, Daniel J; Freilich, Benjamin D; Singer, Michael A; Hartley, Matthew J; Biswas, Jyotirmay; Boulanger, Etienne; Bae, Kunho; Lim, Hun Young; Sujirakul, Tharikarn; Gascon, Pierre; Blinder, Kevin J; Fardeau, Christine; Pockar, Sasa; Androudi, Sofia; Nakashizuka, Hiroyuki; Kitagawa, Yorihisa; Shinojima, Ari; Miserocchi, Elisabetta; Freund, K Bailey
PURPOSE/OBJECTIVE:To describe the clinical features, multimodal imaging findings, natural history, and treatment outcomes of acute outer retinopathy (AOR), which represents an expanded spectrum of acute annular outer retinopathy (AAOR). DESIGN/METHODS:Retrospective, observational, longitudinal, multicenter case series. PARTICIPANTS/METHODS:Twenty-three patients (15 female, 8 male) with a mean age of 41.8 ± 18.6 years (range: 14-86 years) and a mean follow-up duration of 3.7 ± 1.5 years (range: 1-12 years). METHODS:Clinical characteristics, multimodal imaging findings, laboratory evaluations, genetic testing, natural history, therapeutic management, and outcomes were reviewed and analyzed. MAIN OUTCOMES MEASURES/METHODS:Specific multimodal imaging signatures of AOR were identified, including findings from ophthalmoscopy, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). Humphrey visual field testing, full-field electroretinography (ERG), and multifocal ERG were analyzed. Baseline features and the natural course of the disease were delineated. RESULTS:Thirty-eight eyes from 23 patients were analyzed. Presenting symptoms included photopsia (87%), blurred vision (57%), and scotoma (57%). On ophthalmoscopy, AOR was acutely characterized by yellow-greyish outer retinal lesions corresponding to hyperautofluorescent changes on FAF and the angular sign of Henle fiber layer hyperreflectivity (ASHH) on OCT. FAF imaging revealed ring-like hyperautofluorescent lesions surrounding the optic disc in 18% of eyes. Additional lesion patterns on FAF included perivenular (53%), sectoral (16%), and spot-like distributions (13%). FA and ICGA findings were mostly unremarkable. Lesion progression primarily occurred within the initial weeks following presentation and stabilized in size beyond this period in the majority of eyes. Over time, affected areas progressed to outer retinal atrophy with pigmentary changes. Foveal sparing was observed in 68% of the eyes. None of the therapeutic interventions appeared effective in halting the progression to complete outer retinal atrophy or preventing lesion enlargement. CONCLUSIONS:AOR is characterized by early photoreceptor disruption, evidenced by ASHH on OCT, leading to rapid outer retinal atrophy and subsequent degeneration of the retinal pigment epithelium within the damaged zones. Although distinct patterns of lesion distribution were observed, their consistent features on multimodal imaging support their inclusion within a unified disease spectrum termed acute outer retinopathy.
PMID: 40436146
ISSN: 2468-6530
CID: 5854872
Health-Related Quality of Life With Odronextamab Monotherapy for Relapsed/Refractory Follicular Lymphoma in the ELM-2 Study
Tessoulin, Benoit; Harnett, James; Cho, Seok-Goo; Taszner, Michał; Kim, Tae Min; Novelli, Silvana; Villasboas, Jose C; Merli, Michele; Jiménez-Ubieto, Ana; Poon, Michelle; Tucker, David; Walewski, Jan; Yi, Shuhua; Song, Yuqin; Chong, Geoffrey; Bachy, Emmanuel; Guidez, Stephanie; Alonso, Aranzazu; Jagadeesh, Deepa; Zhang, Wei; Magnano Mayer, Laura; Iskierka-Jażdżewska, Elżbieta; Tani, Monica; Cai, Jingxian; Ivanescu, Cristina; Reaney, Matthew; Chaudhry, Aafia; Mohamed, Hesham; Ambati, Srikanth; Chi, Lei; Kamat, Siddhesh; Luminari, Stefano; ,
BACKGROUND:In ELM-2, the human CD20 × CD3 bispecific antibody odronextamab was associated with deep, durable responses and a generally manageable safety profile in patients with relapsed/refractory follicular lymphoma (r/r FL). PATIENTS AND METHODS/METHODS:Prespecified analyses reported herein examined patient-reported outcomes in ELM-2 among 140 patients with r/r FL. RESULTS:Using the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30 (EORTC QLQ-C30), patients reported good global health status/quality of life (GHS/QoL), functioning, and low symptom burden at baseline, which were generally maintained. Emotional functioning improved significantly overall, with a clinically meaningful change at week 42. Patients were more likely to report clinically meaningful improvement or maintenance than worsening on QLQ-C30 scales. Median time to definitive deterioration (TTDD) was 22.4 months for GHS/QoL, 22.6 months for role functioning, 19.8 months for social functioning, 16.4 months for cognitive functioning, and not reached for physical functioning or emotional functioning. For each QLQ-C30 symptom scale, median TTDD was > 15 months or not reached. On the Functional Assessment of Cancer Therapy-Lymphoma (FACT-Lym) questionnaire, Lymphoma Subscale (LymS) scores improved significantly overall, with clinically meaningful improvement at weeks 26 and 42, and FACT-G, Trial Outcome Index, and Total Score were maintained. Most patients reported either limited or no bother related to side effects of treatment. Visual analog scale scores of the EQ-5D-3L questionnaire improved significantly overall. CONCLUSION/CONCLUSIONS:In this heavily pretreated, highly refractory population, maintenance or improvement of health-related quality of life, functioning, and symptoms support odronextamab as a potential treatment option in R/R FL.
PMID: 40571446
ISSN: 2152-2669
CID: 5881542
Assessing the competitiveness of applicants in the ophthalmology match based on interest in pediatric ophthalmology
Cubells, Caroline; Martinez, Philip; Heilenbach, Noah; Lee, Ting-Fang; Elkin, Zachary
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.
PMID: 40355073
ISSN: 1528-3933
CID: 5844002
Boucher-Neuhauser Syndrome: Chorioretinal Changes in a Single Case Over Time
Adeghate, Jennifer O; Sherman, Jerome; Bass, Sherry; Liu, James; Hufnagel, Robert B; Yannuzzi, Lawrence A
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.
PMCID:11377864
PMID: 38447053
ISSN: 1937-1578
CID: 5723142
Myopic Tractional Maculopathy and Retinoschisis with Telangiectasia
Faes, Livia; Freund, K Bailey
PMID: 39503686
ISSN: 2468-6530
CID: 5803632
Optic disc pit: A trigger for secondary multiple evanescent white dot syndrome
Varma, Shivesh; Chen, Royce W S; Liebmann, Jeffrey M; Yannuzzi, Lawrence A
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.
PMID: 40279536
ISSN: 1937-1578
CID: 5830732
VITELLIFORM LESIONS ASSOCIATED WITH ANGIOID STREAKS: Long-Term Follow-Up of a Rarely Described Phenotype
Feo, Alessandro; Bousquet, Elodie; Faes, Livia; Ramtohul, Prithvi; Sacconi, Riccardo; Rissotto, Federico; Boscia, Giacomo; Abraham, Néda; Lior, Tal Eshkoly; Faghihi, Shahin; Popovic, Marko M; Chan, Hiok Hong; Gemmy Cheung, Chui Ming; Fouad, Yousef; Cabral, Diogo; Govetto, Andrea; Romano, Mario R; Querques, Giuseppe; Farvo, SriniVas R Sadda; Freund, K Bailey; Sarraf, David
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.
PMID: 40239167
ISSN: 1539-2864
CID: 5828322
Low-Dose Valacyclovir for Postherpetic Neuralgia in the Zoster Eye Disease Study: A Randomized Clinical Trial [Comment]
Warner, David B; Jeng, Bennie H; Kim, Jiyu; Liu, Mengling; Troxel, Andrea B; Hochman, Judith S; Baratz, Keith H; Mian, Shahzad I; Choulakian, Mazen Y; Meyer, Jay J; Lu, Ying; Twi-Yeboah, Alberta; Lee, Ting-Fang; Lopez-Jimenez, Carlos; Laury, Sarah C; Cohen, Elisabeth J; ,
IMPORTANCE/UNASSIGNED:Evidence regarding suppressive valacyclovir treatment on postherpetic neuralgia is necessary to guide care. OBJECTIVE/UNASSIGNED:To test the hypothesis that suppressive treatment with 1000 mg/d of oral valacyclovir for 12 months reduces the prevalence, severity, and duration of postherpetic neuralgia compared with placebo at 12 and 18 months in participants with herpes zoster ophthalmicus (HZO). DESIGN, SETTING, AND PARTICIPANTS/UNASSIGNED:Multicenter, placebo-controlled randomized clinical trial including 527 immunocompetent, nonpregnant adults with history of HZO rash, documented keratitis, or iritis within 1 year and an estimated glomerular filtration rate of 45 mL/min/1.73 m2 or greater. The study was conducted at 95 participating sites (in Canada, New Zealand, and the US) from November 2017 to June 2024 and participant visits occurred every 3 months. INTERVENTION/UNASSIGNED:Treatment with 1000 mg/d of valacyclovir or placebo for 12 months. MAIN OUTCOMES AND MEASURES/UNASSIGNED:Prevalence of postherpetic neuralgia, severity as determined by pain score (a score of ≥3 on a scale of 1-10), pain duration (≥3 months after HZO onset), and total daily dose of pain medication. RESULTS/UNASSIGNED:Of the 527 participants (490 completed 12 months of treatment and 460 completed 18 months), 73 (14%) had postherpetic neuralgia and were analyzed by age at HZO onset (<60 years or ≥60 years) and disease duration (recent [<6 months] or chronic [≥6 months]). Of the 73 participants with postherpetic neuralgia (34 in the valacyclovir group and 39 in the placebo group), the mean age was 62.4 years (SD, 13.6 years), 59% were female, 5% were Black or African American, and 10% were Hispanic. The prevalence of postherpetic neuralgia at 12 months was not reduced by valacyclovir (12/32 [38%]) compared with placebo (14/35 [40%]) (between-group difference, 2.5% [95% CI, -20.8% to 25.8%]; P>.99). The participants who were younger than 60 years at HZO onset and had a chronic disease duration had lower pain scores in the valacyclovir group (mean score, 0.3 [SD, 0.9]) vs the placebo group (mean score, 0.8 [SD, 1.9]) at 12 months (P = .045) and at 18 months (mean score, 0.2 [SD, 0.9] vs 1.0 [SD, 2.3], respectively; P = .02). There was a decrease in pain duration in the valacyclovir group at 18 months (mean, 13.6 [SD, 11.4] months) vs the placebo group (mean, 18.7 [SD, 29.5] months) (linear mixed-effects model between-group difference, -3.39 months [95% CI, -6.73 to -0.04 months]; P = .046). The total daily dose of neuropathic pain medication was lower in the valacyclovir group (mean, 271.4 [SD, 593.8] mg/d) vs the placebo group (mean, 363.4 [SD, 592.2] mg/d) at 12 months (linear mixed-effects model P = .006) and at 18 months (mean, 209.0 [SD, 412.8] mg/d vs 286.2 [SD, 577.9] mg/d, respectively; linear mixed-effects model P = .01). CONCLUSIONS AND RELEVANCE/UNASSIGNED:One year of suppressive treatment with valacyclovir was associated with a lower dosage of neuropathic pain medication. Participants in the valacyclovir group, who were younger at HZO onset and had a chronic disease duration, had lower pain scores. These secondary outcomes support consideration of 1 year of suppressive treatment with valacyclovir to reduce dosage of pain medications and pain due to HZO. TRIAL REGISTRATION/UNASSIGNED:ClinicalTrials.gov Identifier: NCT03134196.
PMID: 40048191
ISSN: 2168-6173
CID: 5827172
Low-Dose Valacyclovir in Herpes Zoster Ophthalmicus: The Zoster Eye Disease Randomized Clinical Trial [Comment]
Cohen, Elisabeth J; Troxel, Andrea B; Liu, Mengling; Hochman, Judith S; Baratz, Keith H; Mian, Shahzad I; Choulakian, Mazen Y; Warner, David B; Lu, Ying; Twi-Yeboah, Alberta; Lee, Ting-Fang; Kim, Jiyu; Lopez-Jimenez, Carlos; Laury, Sarah C; Jeng, Bennie H; ,
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.
PMID: 40048183
ISSN: 2168-6173
CID: 5827152
DREDge: robust motion correction for high-density extracellular recordings across species
Windolf, Charlie; Yu, Han; Paulk, Angelique C; Meszéna, Domokos; Muñoz, William; Boussard, Julien; Hardstone, Richard; Caprara, Irene; Jamali, Mohsen; Kfir, Yoav; Xu, Duo; Chung, Jason E; Sellers, Kristin K; Ye, Zhiwen; Shaker, Jordan; Lebedeva, Anna; Raghavan, R T; Trautmann, Eric; Melin, Max; Couto, João; Garcia, Samuel; Coughlin, Brian; Elmaleh, Margot; Christianson, David; Greenlee, Jeremy D W; Horváth, Csaba; Fiáth, Richárd; Ulbert, István; Long, Michael A; Movshon, J Anthony; Shadlen, Michael N; Churchland, Mark M; Churchland, Anne K; Steinmetz, Nicholas A; Chang, Edward F; Schweitzer, Jeffrey S; Williams, Ziv M; Cash, Sydney S; Paninski, Liam; Varol, Erdem
High-density microelectrode arrays have opened new possibilities for systems neuroscience, but brain motion relative to the array poses challenges for downstream analyses. We introduce DREDge (Decentralized Registration of Electrophysiology Data), a robust algorithm for the registration of noisy, nonstationary extracellular electrophysiology recordings. In addition to estimating motion from action potential data, DREDge enables automated, high-temporal-resolution motion tracking in local field potential data. In human intraoperative recordings, DREDge's local field potential-based tracking reliably recovered evoked potentials and single-unit spike sorting. In recordings of deep probe insertions in nonhuman primates, DREDge tracked motion across centimeters of tissue and several brain regions while mapping single-unit electrophysiological features. DREDge reliably improved motion correction in acute mouse recordings, especially in those made with a recent ultrahigh-density probe. Applying DREDge to recordings from chronic implantations in mice yielded stable motion tracking despite changes in neural activity between experimental sessions. These advances enable automated, scalable registration of electrophysiological data across species, probes and drift types, providing a foundation for downstream analyses of these rich datasets.
PMID: 40050699
ISSN: 1548-7105
CID: 5823502