Ophthalmology Commons^™

Coats Disease In 351 Eyes: Analysis Of Features And Outcomes Over 45 Years (By Decade) At A Single Center., Carol L. Shields, Sanika Udyaver, Lauren A. Dalvin, Li-Anne S. Lim, Hatice T Atalay, Chloe T.L. Khoo, Mehdi Mazloumi, Jerry A. Shields

Wills Eye Hospital Papers

Purpose: To assess features and outcomes of Coats disease over 5-decades.

Methods: Retrospective review of Coats disease patients at a single center. Features and outcomes were compared based on decade of presentation.

Results: There were 351 patients with Coats disease. The presenting median age (6 years), male sex (84%), and unilaterality (100%) did not change per decade. Coats disease classification did not change per decade with Stage 1 (1%), Stage 2 (21%), Stage 3 (68%), Stage 4 (6%), and Stage 5 (1%). Clinical features that changed per decade (1970s vs. 1980s vs. 1990s vs. 2000s vs. 2010s) included 1980s features …

Changes In Ultraviolet Radiation Exposure To The Ocular Region: A Population-Based Study., Ezekiel Weis, Sebastian Q. Vrouwe, David B. Lebaron, Matthew B. Parliament, Jerry A Shields, Carol L. Shields

Wills Eye Hospital Papers

In contrast to the well-established association between ultraviolet radiation (UVR) exposure and skin cancers, the relationship between UVR and uveal malignant melanoma (UM) remains controversial. To address this controversy, we evaluated the incidence rates of cutaneous malignancies in the eyelids as a proxy for UVR exposure in the ocular region using a population-based cancer registry. Overall, 74,053 cases of eyelid basal cell carcinoma (BCC) and 7890 cases of melanoma over a 26-year period (1982–2007) were analyzed. The incidence of eyelid basal cell carcinoma and uveal melanoma remained stable, whereas other cutaneous areas demonstrated an increase in the rates. A comparability …

Failure Of Methotrexate Monotherapy And Subsequent Response To Tumor Necrosis Factor Inhibitors In Pediatric Non-Infectious Uveitis, Basak Can Ermerak Md

Research Days

No abstract provided.

Levels Of Human Tear Lacritin Isoforms In Healthy Adults, Brooke Justis

Senior Honors Projects, 2010-2019

Current diagnostics of dry eye disease are unable to identify the multiple complex causes of dry eye, thereby limiting the proper treatments of the disease. The tear glycoprotein lacritin has been reported to be decreased in dry eye patients, specifically those with Sjӧgren’s syndrome associated dry eye. Therefore, the use of lacritin in a diagnostic test for dry eye could improve the current diagnostics available. Western blot assays have revealed the presence of an active lacritin monomer as well as an inactive tissue transglutaminase cross-linked polymer in human tears. A third isoform of lacritin, the splice variant lacritin-c, has also …

For Your Eyes Only, Herpes Zoster Ophthalmicus Case Study, Alexander Fishman, Jared Ward, Richard Pescatore

Rowan-Virtua Research Day

Herpes Zoster Ophthalmicus is a reactivation of a latent Varicella Zoster Virus that specifically involves the eye. The reactivation often occurs during immunocompromised states which allows latent virus in the dorsal root ganglia of sensory nerves to begin viral replication an d s pread via peripheral axons. Diagnosis is based on two characteristic findings; herpetic lesions on the eyelids, forehead, or elsewhere within the V1 dermatome, and ocular findings. Symptoms can vary but are often quite painful. Some patients experience a prodrome of tingling sensation prior to the appearance of a zoster dermatitis . HZO can also present initially as …

Depletion Of Myo/Nog Cells In The Lens Mitigates Posterior Capsule Opacification In Rabbits., Jacquelyn Gerhart, Liliana Werner, Nick Mamalis, Joseph Infanti, Colleen Withers, Fathma Abdalla, Colby Gerhart, Arturo Bravo Nuevo, Olivia Gerhart, Lori Getts, Kelly Rhodes, Jessica Bowers, Robert Getts, Mindy George-Weinstein

PCOM Scholarly Works

Purpose: Posterior capsule opacification (PCO) is a vision-impairing disease that occurs in some adults and most children after cataract surgery. Contractile myofibroblasts contribute to PCO by producing wrinkles in the lens capsule that scatter light. Myofibroblasts in the lens originate from Myo/Nog cells named for their expression of the MyoD transcription factor and bone morphogenetic protein inhibitor noggin. In this study we tested the effects of depleting Myo/Nog cells on development of PCO.

Methods: Myo/Nog cells were eliminated by injecting the G8 antibody conjugated to 3DNA nanocarriers for the cytotoxin doxorubicin (G8:3DNA:Dox) during cataract surgery in rabbits. The severity of …

Trust And Glycemic Control In Black Patients With Diabetic Retinopathy: A Pilot Study, Barry W. Rovner, Robin J. Casten

Department of Psychiatry and Human Behavior Faculty Papers

Diabetic retinopathy (DR) is more prevalent in blacks than whites because, compared to whites, blacks on average have worse glycemic control. Both of these racial disparities reflect differences in sociocultural determinants of health, including physician mistrust. This randomized, controlled 6-month pilot trial compared the efficacy of a culturally tailored behavioral health/ophthalmologic intervention called Collaborative Care for Depression and Diabetic Retinopathy (CC-DDR) to enhanced usual care (EUC) for improving glycemic control in black patients with DR (n = 33). The mean age of participants was 68 years (SD 6.1 years), 76% were women, and the mean A1C was 8.7% (SD 1.5%). …

Commentary: Ganglion Cell Complex Of Retinal Layer Thickness By Optical Coherence Tomography In Cases Of Multiple Sclerosis Without Optic Neuritis Compared To Healthy Eyes, Padmaja Sudhakar

Neurology Faculty Publications

No abstract provided.

The Proliferation Of Porcine Retinal Ganglion Cells Using Pnu 282987, Rebecca Karl, David Linn

Honors Projects

The retinal ganglion cells (RGCs) from porcine eyes were extracted to work towards a potential therapy for glaucoma. There is not a known cause of glaucoma or the apoptosis of RGCs that cause the eventual blindness associated with glaucoma. The goal of this protocol is to protect the RGCs from dying by adding a nicotinic acetylcholine receptor activator to induce neuronal proliferation and protect against glutamate-induced excitotoxicity. PNU 282987 was used as the nicotinic acetylcholine receptor activator due to its neuroprotective properties. We hypothesized that we would observe the proliferation of RGCs (associated with increased cell numbers) with varying levels …

Papilledema Or Pseudopapilledema?, Lin Liu, Michael D. Yu, Carol L. Shields

Wills Eye Hospital Papers

No abstract provided.

Empiric Antibiotic Use For Infectious Conjunctivitis Provides Little Clinical Benefit, Matthew M. Rolain

Clinical Research in Practice: The Journal of Team Hippocrates

A critical appraisal and clinical application of Rose PW, Harnden A, Brueggemann AB, et al. Chloramphenicol treatment for acute infective conjunctivitis in children in primary care: a randomised double-blind placebo-controlled trial. Lancet. 2005;366(9479):37-43. doi: 10.1016/S0140-6736(05)66709-8.

Mucormycosis - Dual Therapy With Prolonged Survival, Maydelin Pecchio, Yariela Osabio, Yariela Morales, Forest W. Arnold

Journal of Refugee & Global Health

Mucorales fungal infection is a fungal disease with potentially fatal outcomes. The most frequent involvement in humans comes from the orders known as Mucorales and Entomophthorales. Mucorales is more acute and has a predilection for immunocompromised patients. Mucorales are associated with an affinity for vessels, which leads to invasion and infarction of tissue. Mucormycosis is a devastating complication that can be a life threatening fungal invasion in many patients in an immunocompromised state.

Latent Sensitization In A Mouse Model Of Ocular Neuropathic Pain, Jooyoung Cho, Nicholas Bell, Gregory Botzet, Paras Vora, Benjamin J. Fowler, Renee R. Donahue, Heather M. Bush, Bradley K. Taylor, Romulo J. C. Albuquerque

Ophthalmology and Visual Science Faculty Publications

Purpose: Chronic ocular pain is poorly understood and difficult to manage. We developed a murine model of corneal surface injury (CSI)–induced chronic ocular neuropathic pain. The study focuses on changes in corneal nerve morphology and associated short- and long-term pain-like behavior after CSI.

Methods: CSI was induced in mice by local application of an alkali solution (0.75 N NaOH). Corneal nerve architecture, morphology, density, and length were studied. Eye-wiping was evaluated before and after CSI in response to hypertonic saline (2 M NaCl). Naltrexone (NTX) or Naloxone-methiodide (NLX-me), opioid receptor antagonists, were given subcutaneously (s.c., 3 mg/kg) or …

Is Retinal Metabolic Dysfunction At The Center Of The Pathogenesis Of Age-Related Macular Degeneration?, Thierry Léveillard, Nancy J. Philp, Florian Sennlaub

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

The retinal pigment epithelium (RPE) forms the outer blood⁻retina barrier and facilitates the transepithelial transport of glucose into the outer retina via GLUT1. Glucose is metabolized in photoreceptors via the tricarboxylic acid cycle (TCA) and oxidative phosphorylation (OXPHOS) but also by aerobic glycolysis to generate glycerol for the synthesis of phospholipids for the renewal of their outer segments. Aerobic glycolysis in the photoreceptors also leads to a high rate of production of lactate which is transported out of the subretinal space to the choroidal circulation by the RPE. Lactate taken up by the RPE is converted to pyruvate and metabolized …

Management Of Retinoblastoma In Older Children (>5 Years) Using Intra-Arterial Chemotherapy: Comparison Of Outcomes To Pre-Chemotherapy And Intravenous Chemotherapy Eras, Evan Selzer, Ms, R. Joel Welch, Md, Pascal Jabbour, Md, Ann Leahey, Md, Carol L. Shields, Md

Phase 1

Introduction: Intra-arterial chemotherapy (IAC) has emerged as an effective treatment for retinoblastoma (RB), but little information exists regarding its use in older patients (>5 years). We evaluate the use of IAC (2008-2018) for RB in older patients and compare outcomes to those in the pre-chemotherapy (<1994) and intravenous chemotherapy (IVC) (1994-2007) eras.

Objective: To evaluate the hypothesis that IAC is effective in managing RB in older patients.

Methods: We performed a retrospective analysis of all patients older than 5 years who were treated with IAC for RB from 2008-2018 on the Ocular Oncology Service at the Wills Eye Hospital. Comparisons were made to historic, …

Just Another Chalazion?, Jonathan Mclaughlin, Sara E. Lally, Carol L. Shields

Wills Eye Hospital Papers

No abstract provided.

Bilateral Acute Ocular Ischemic Syndrome Following Head And Neck Radiation, Dipali Nemade, Paul Ferguson, Vikram Shivkumar, Dennis Cole

Marshall Journal of Medicine

Previous literature has established a clear correlation between radiation therapy (RT) to the head and neck leading to aggressive atherosclerosis of the carotid arteries and subsequent increased risk for ischemic stroke. Stenosis of carotid arteries can lead to Ocular Ischemic syndrome (OIS). We present a case of acute bilateral OIS following head and neck RT for left tonsillar squamous cell carcinoma (SCC) in a 64 year-old Caucasian male. Routine large vessel cerebrovascular screening following head and neck radiotherapy should be considered as a means of potentially mitigating future stroke risk through initiation of medical therapy and interventional modalities as a …

Hyperautofluorescent Dots Are Characteristic In Ceramide Kinase Like-Associated Retinal Degeneration., Jesse D Sengillo, Galaxy Y Cho, Maarjaliis Paavo, Winston Lee, Eugenia White, Ruben Jauregui, Janet R Sparrow, Rando Allikmets, Stephen H Tsang

Reading Hospital Internal Medicine Residency

There is a lack of studies which seek to discern disease expression in patients with mutations that alter retinal ceramide metabolism, specifically in the ceramide kinase like (CERKL) gene. This cross-sectional case series reports a novel phenotypic manifestation of CERKL-associated retinopathy. Four unrelated patients with homozygous CERKL mutations underwent a complete ocular exam, spectral-domain optical coherence tomography, short-wavelength fundus autofluorescence (SW-AF), quantitative autofluorescence (qAF), and full-field electroretinogram (ffERG). Decreased visual acuity and early-onset maculopathy were present in all patients. All four patients had extensive hyperautofluorescent foci surrounding an area of central atrophy on SW-AF imaging, which has not been previously …

An Artificial Intelligence Approach To Detect Visual Field Progression In Glaucoma Based On Spatial Pattern Analysis., Mengyu Wang, Lucy Q. Shen, Louis R. Pasquale, Paul Petrakos, Sydney Formica, Michael V. Boland, Sarah R. Wellik, Carlos Gustavo De Moraes, Jonathan S. Myers, Osamah Saeedi, Hui Wang, Neda Baniasadi, Dian Li, Jorryt Tichelaar, Peter J. Bex, Tobias Elze

Wills Eye Hospital Papers

Purpose: To detect visual field (VF) progression by analyzing spatial pattern changes.

Methods: We selected 12,217 eyes from 7360 patients with at least five reliable 24-2 VFs and 5 years of follow-up with an interval of at least 6 months. VFs were decomposed into 16 archetype patterns previously derived by artificial intelligence techniques. Linear regressions were applied to the 16 archetype weights of VF series over time. We defined progression as the decrease rate of the normal archetype or any increase rate of the 15 VF defect archetypes to be outside normal limits. The archetype method was compared with mean …

Ischemic Necrosis Caused By Retroanastomotic Hernia After Subtotal Gastrectomy, S. H. Lee, S. C. Park, S. J. Lee, C. D. Kang, S. J. Nam, S. Y. Lee, S. K. Hong, S. K. Lee

Journal Articles

After gastrojejunostomy, a small space can occur between the jejunum at the anastomosis site, the transverse mesocolon, and retroperitoneum, which may cause an intestinal hernia. This report presents a rare case of intestinal ischemic necrosis caused by retroanastomotic hernia after subtotal gastrectomy. A 56-year-old male was admitted to Kangwon National University Hospital with melena, abdominal pain, and nausea. His only relevant medical history was gastrectomy due to stomach cancer. Endoscopic findings revealed subtotal gastrectomy with Billroth-II reconstruction and a bluish edematous mucosal change with necrotic tissue in afferent and efferent loops including the anastomosis site. Abdominopelvic CT showed strangulation of …

Influence Of Bacterial Burden On Meibomian Gland Dysfunction And Ocular Surface Disease, A. Nattis, H. D. Perry, E. D. Rosenberg, E. D. Donnenfeld

Journal Articles

© 2019 Nattis et al. Purpose: Bacterial burden on the eyelid margin and within meibomian glands was evaluated for influence on specific ocular surface disease (OSD) markers across the meibomian gland dysfunction (MGD) spectrum. Methods: In this prospective, observational, single-center study, 40 patients were divided into 4 equal groups of 10 that encompassed increasingly worse MGD/OSD categories. All patients answered the standard Ocular Surface Disease Index questionnaire, and underwent tear osmolarity testing (TOT), Schirmer 1, matrix metalloproteinase 9 (MMP-9) testing, meibography, and lissamine green staining. Cultures of eyelid margins and meibomian gland secretions were directly plated on blood, chocolate, and …

Utility Of En-Face Imaging In Diagnosis Of Occult Macular Dystrophy With Rp1l1 Mutation: A Case Series, M. Z. Ruan, S. A. Hussnain, A. Thomas, M. Mansukhani, S. Tsang, L. Yannuzzi

Journal Articles

© 2019 The Authors Purpose: To report en-face imaging findings at the level of ellipsoid zone (EZ) in two cases of occult macular dystrophy (OMD) with retinitis pigmentosa 1-like 1 (RP1L1) p.Arg45Trp mutation. Observations: In both patients who presented with decreased vision, pupillary examination, intraocular pressure, and anterior examination were normal. Ophthalmoscopic examination showed prominent choroidal marking whereas fundus autofluorescence was unremarkable. Spectral domain optical coherence tomography (SD-OCT) showed subtle gaps between EZ and retinal pigment epithelium (RPE). The photoreceptor disruption became more evident with en-face imaging at the EZ plane. Conclusions and importance: This is a report of two …

The Fate And Prognostic Implications Of Hyperreflective Crystalline Deposits In Nonneovascular Age-Related Macular Degeneration, S. Fragiotta, P. Fernáandez-Avellaneda, M. P. Breazzano, C. A. Curcio, B. C. Leong, K. Kato, L. A. Yannuzzi, K. B. Freund

Journal Articles

© 2019 The Authors. All rights reserved. PURPOSE. To explore patterns of disease progression in nonneovascular age-related macular degeneration (AMD) associated with hyperreflective crystalline deposits (HCDs) in the subretinal pigment epithelium–basal laminar space. METHODS. Retrospective review of medical records, multimodal imaging, and longitudinal eyetracked near-infrared reflectance (NIR) and optical coherence tomography (OCT) spanning years. NIR/OCT images were analyzed with ImageJ software to identify HCD morphology and location. Associated macular complications were reviewed from the time of HCD detection to the most recent follow-up, using NIR/OCT. RESULTS. Thirty-three eyes with HCDs from 33 patients (mean age: 72 ± 7.5 years) had …

Mutations In Arl2bp, A Protein Required For Ciliary Microtubule Structure, Cause Syndromic Male Infertility In Humans And Mice, Abigal R. Moye, Nicola Bedoni, Jessica G. Cunningham, Urikhan Sanzhaeva, Eric S. Tucker, Peter Mathers, Virginie G. Peter, Mathieu Quinodoz, Liliana P. Paris, Luisa Coutinho-Santos, Pedro Camacho, Madeleine G. Purcell, Abbie C. Winkelmann, James A. Foster, Elena N. Pugacheva, Carlo Rivolta, Visvanathan Ramamurthy

Faculty & Staff Scholarship

Cilia are evolutionarily conserved hair-like structures with a wide spectrum of key biological roles, and their dysfunction has been linked to a growing class of genetic disorders, known collectively as ciliopathies. Many strides have been made towards deciphering the molecular causes for these diseases, which have in turn expanded the understanding of cilia and their functional roles. One recently-identified ciliary gene is ARL2BP, encoding the ADP-Ribosylation Factor Like 2 Binding Protein. In this study, we have identified multiple ciliopathy phenotypes associated with mutations in ARL2BP in human patients and in a mouse knockout model. Our research demonstrates that spermiogenesis …

Metabolic Deregulation Of The Blood-Outer Retinal Barrier In Retinitis Pigmentosa, Wei Wang, Ashwini Kini, Yekai Wang, Tingting Liu, Yao Chen, Eric Vukmanic, Douglas Emery, Yongqing Liu, Xiaoqin Lu, Lei Jin, San Joon Lee, Patrick Scott, Xiao Liu, Kevin Dean, Qingxian Lu, Enzo Fortuny, Robert James, Henry J. Kaplan, Jianhai Du, Douglas C. Dean

Faculty & Staff Scholarship

Retinitis pigmentosa (RP) initiates with diminished rod photoreceptor function, causing peripheral and nighttime vision loss. However, subsequent loss of cone function and high-resolution daylight and color vision is most debilitating. Visual pigment-rich photoreceptor outer segments (OS) undergo phagocytosis by the retinal pigment epithelium (RPE), and the RPE also acts as a blood-outer retinal barrier transporting nutrients, including glucose, to photoreceptors. We provide evidence that contact between externalized phosphatidylserine (PS) on OS tips and apical RPE receptors activates Akt, linking phagocytosis with glucose transport to photoreceptors for new OS synthesis. As abundant mutant rod OS tips shorten in RP, Akt activation …

Central Serous Chorioretinopathy: Towards An Evidence-Based Treatment Guideline, T. J. Van Rijssen, E. H. Van Dijk, S. Yzer, K. Ohno-Matsui, J. E. Keunen, R. O. Schlingemann, S. Sivaprasad, G. Querques, L. A. Yannuzzi, C. J. F. Boon, +10 Additional Authors

Journal Articles

© 2019 The Authors Central serous chorioretinopathy (CSC) is a common cause of central vision loss, primarily affecting men 20–60 years of age. To date, no consensus has been reached regarding the classification of CSC, and a wide variety of interventions have been proposed, reflecting the controversy associated with treating this disease. The recent publication of appropriately powered randomised controlled trials such as the PLACE trial, as well as large retrospective, non-randomised treatment studies regarding the treatment of CSC suggest the feasibility of a more evidence-based approach when considering treatment options. The aim of this review is to provide a …

Differential Bacterial Colonization And Biofilm Formation On Punctal Occluders, M. Hadjiargyrou, E. D. Donnenfeld, L. M. Grillo, H. D. Perry

Journal Articles

© 2019 by the authors. Dry eye is a common condition that is treated primarily by topical lubricants, immunomodulation, and a variety of punctal and canalicular plugs (occluders). Biofilm formation has been reported as an ongoing problem with the clinical use of occluders. In order to explore the role of biofilm formation on occluders, we tested the bacteria strain, Staphylococcus aureus, with three different types of occluders, Delta R , Odyssey R , and Alphamed R . Scanning electron microscopy (SEM) of these occluders revealed a variation in surface appearance, with Odyssey R being the smoothest (but with grooves), followed …

Anti-Inflammatory Effects Of Apocynin On Dextran Sulfate Sodium-Induced Mouse Colitis Model, Y. J. Hwang, S. J. Nam, W. Chun, S. I. Kim, S. C. Park, C. D. Kang, S. J. Lee

Journal Articles

© 2019 Hwang et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Background and aim Various drugs have been developed for inflammatory bowel disease (IBD), but still there are limitations in the treatment due to the insufficient responses and significant adverse effects of immunosuppressant. Apocynin is an NADPH-oxidase inhibitor with established safety profiles. We aimed to investigate the protective efficacy of apocynin in IBD using chemicalinduced mouse colitis model. Method We induced experimental …

Imi – Defining And Classifying Myopia: A Proposed Set Of Standards For Clinical And Epidemiologic Studies, D. I. Flitcroft, M. He, J. B. Jonas, M. Jong, K. Naidoo, K. Ohno-Matsui, J. Rahi, S. Resnikoff, S. Vitale, L. Yannuzzi

Journal Articles

© 2019 The Authors. PURPOSE. We provide a standardized set of terminology, definitions, and thresholds of myopia and its main ocular complications. METHODS. Critical review of current terminology and choice of myopia thresholds was done to ensure that the proposed standards are appropriate for clinical research purposes, relevant to the underlying biology of myopia, acceptable to researchers in the field, and useful for developing health policy. RESULTS. We recommend that the many descriptive terms of myopia be consolidated into the following descriptive categories: myopia, secondary myopia, axial myopia, and refractive myopia. To provide a framework for research into myopia prevention, …

Colonic Diffuse Large B-Cell Lymphoma Hidden In Actinomycosis, S. H. Lee, S. J. Nam, S. J. Lee, S. C. Park, C. D. Kang, D. H. Choi, J. M. Park, S. K. Lee

Journal Articles

Actinomycosis can mask malignant diseases. This paper reports a case of colonic diffuse large B-cell lymphoma (DLBCL), which was misdiagnosed as abdominal actinomycosis. A 76-year-old woman presented with right flank pain and weight loss. Abdominal CT and colonoscopy revealed a huge ascending colon mass. Despite the initial impression of a malignancy, a colonoscopic biopsy revealed no malignant cells, but sulfur granules and a filamentous organism suggesting actinomycosis. Intravenous penicillin G was administered under the impression of abdominal actinomycosis but her condition deteriorated rapidly. Follow up CT showed markedly increased colon mass and new multiple nodular lesions around the ascending colon. …