Ophthalmology Commons^™

Retinitis Pigmentosa Genes Implicated In South Asian Populations: A Systematic Review, Sidra Zafar, Khabir Ahmed, Azam Ali, Rashid Baig

Section of Ophthalmology

Retinitis pigmentosa is one of the most prevalent causes of inherited retinal dystrophies worldwide. The widespread custom of consanguineous marriages in South Asian countries puts the population at risk for autosomal recessive disorders including retinitis pigmentosa. This systematic review was done between May and December 2015.A comprehensive literature search was carried out using MEDLINE and CINAHL databases and all relevant articles on causative mutations for non-syndromic Retinitis pigmentosa from 1999 till 2015 were included. Overall, 41 articles were identified involving 66 families; 28(68%)from Pakistan, 12(29%) from India and 1(2.4%) from Bangladesh. No data was available from the rest of countries …

A Comparison Of Two Conjunctival Rotation Autograft Techniques In Primary Pterygium Surgery., Remzi Karadag, Neslihan Sevimli, Seydi Okumus, Isilay Ozsoy, Huseyin Bayramlar, Ela Durucu, Umit Aksoy, Christopher J. Rapuano

Wills Eye Hospital Papers

PURPOSE: To compare the effects of 90° and 180° conjunctival rotational autograft (CRA) techniques used in primary pterygium surgery.

METHODS: Forty-five patients were included in this retrospective study. Visual acuity (VA), corneal topography, and auto-refractometer measurements, as well as detailed biomicroscopic examinations, were performed preoperatively and postoperatively. During surgery, the pterygium tissue was excised then rotated 90° in Group 1 and180° in Group 2, after which it was sutured to the bare sclera. Pterygium recurrence was defined as corneal invasion ≥1 mm.

RESULTS: Group 1 consisted of 21 patients with a mean age of 45.1 ± 11.8 years, while Group …

Uveal Effusion Syndrome In 104 Eyes: Response To Corticosteroids - The 2017 Axel C. Hansen Lecture., Carol L. Shields, Kelsey Roelofs, Maura Di Nicola, Kareem Sioufi, Arman Mashayekhi, Jerry A. Shields

Wills Eye Hospital Papers

PURPOSE: The purpose of the study was to investigate the corticosteroids for uveal effusion syndrome (UES).

METHODS: Retrospective series of 104 eyes with UES treated with oral corticosteroids (OCS), periocular corticosteroids (PCS), topical corticosteroids (TCS), or observation (OBS). Main outcome measure was UES resolution.

RESULTS: Of 104 eyes, treatment included OCS (n = 27), PCS (n = 12), TCS (n = 11), and OBS (n = 54). A comparison of the four groups (OCS vs. PCS vs. TCS vs. OBS) revealed differences in those managed with OCS versus OBS as younger (66 vs. 72 years, P = 0.049), PCS versus …

Metastatic Deaths In Retinoblastoma Patients Treated With Intraarterial Chemotherapy (Ophthalmic Artery Chemosurgery) Worldwide., David H. Abramson, Carol L. Shields, Pascal Jabbour, Luiz Fernando Teixeira, José Roberto Falco Fonseca, Marcio Chaves Pedro Marques, Francis L. Munier, Francesco Puccinelli, Theodora Hadjistilianou, Sandra Bracco, Guillermo Chantada, Alejandro Ceciliano, Y. Pierre Gobin

Department of Neurosurgery Faculty Papers

BACKGROUND: Ophthalmic artery chemosurgery [OAC, intra-arterial chemotherapy (IAC)] was introduced in 2006 as treatment modality for intraocular retinoblastoma. The purpose of this commentary is to retrospectively review the incidence of metastatic deaths in retinoblastoma patients treated with OAC worldwide over a 10 year period. Retrospective data regarding metastatic deaths was collected from six international retinoblastoma centers (New York City USA, Philadelphia USA, Sao Paulo Brazil, Siena Italy, Lausanne Switzerland and Buenos Aires Argentina). All retinoblastoma patients from these centers (naive and recurrent, unilateral and bilateral) treated with OAC/IAC since 2006 have been included in this study. Data regarding number of …

Association Of Disease Location And Treatment With Survival In Diffuse Large B-Cell Lymphoma Of The Eye And Ocular Adnexal Region., Aseef H H. Ahmed, C. Stephen Foster, Carol L. Shields

Wills Eye Hospital Papers

Importance: Primary diffuse large B-cell lymphoma (DLBCL) of the ocular region is rare, and the utility of surgery and radiation therapy remains unresolved.

Objective: To explore the clinical characteristics and determine factors associated with overall survival in primary vitreoretinal lymphoma (PVRL) and ocular adnexal (OA)-uveal DLBCL.

Design, Setting, and Participants: This retrospective analysis included 396 patients with ophthalmic DLBCL from January 1, 1973, through December 31, 2014, using the Surveillance, Epidemiology, and End Results database. The median follow-up was 39.0 months (interquartile range, 5.1-72.9 months). All patients diagnosed with primary DLBCL of the eye or retina (PVRL) or the eyelid, …

Optical Coherence Tomography Angiography Features Of Iris Racemose Hemangioma In 4 Cases., Jason L. Chien, Kareem Sioufi, Sandor Ferenczy, Emil Anthony T. Say, Carol L. Shields

Wills Eye Hospital Papers

Importance: Optical coherence tomography angiography (OCTA) allows visualization of iris racemose hemangioma course and its relation to the normal iris microvasculature.

Objective: To describe OCTA features of iris racemose hemangioma.

Design, Setting, and Participants: Descriptive, noncomparative case series at a tertiary referral center (Ocular Oncology Service of Wills Eye Hospital). Patients diagnosed with unilateral iris racemose hemangioma were included in the study.

Main Outcomes and Measures: Features of iris racemose hemangioma on OCTA.

Results: Four eyes of 4 patients with unilateral iris racemose hemangioma were included in the study. Mean patient age was 50 years, all patients were white, and …

An Investigation Of Human Beta-Defensins And Cathelicidin Expression In Patients With Pterygium., Remzi Karadag, Nurettin Bayram, Serpil Oguztuzun, Huseyin Bayramlar, Busra Bozer, Gulcin Simsek, Christopher J Rapuano

Wills Eye Hospital Papers

PURPOSE: To investigate human beta-defensins (HBDs) and cathelicidin LL-37 (LL-37) expressions in patients with pterygium.

METHODS: In this retrospective consecutive case series, 26 pterygium specimens and 15 normal conjunctival specimens of 15 control subjects were in vestigated. Expressions of HBD-1, HBD-2, HBD-3, and LL-37 were assessed using immuno histochemical staining. A brown color in the cytoplasm and/or nuclei of epithelial cells indicated positive staining for HBDs and LL-37. For each antibody, the intensity of the reaction (negative [-], weak [1+], moderate [2+], or strong [3+]) was determined to describe the immunoreactions.

RESULTS: The median age was 52 years in both …

Incontinentia Pigmenti In A Child With Suspected Retinoblastoma., Stephanie J. Weiss, Archana Srinivasan, Michael A. Klufas, Carol L. Shields

Wills Eye Hospital Papers

BACKGROUND: Incontinentia pigmenti is a rare X-linked dominant syndrome caused by mutation in the NEMO/IKKgamma gene, and characterized by a spectrum of cutaneous, ocular, neurologic and dental abnormalities. In the eye, findings include retinal vascular non-perfusion, occasionally with traction retinal detachment, retinal fibrosis, and retinal pigment epithelium defects. These findings can resemble retinoblastoma, especially when vitreoretinal fibrosis produces leukocoria.

CASE REPORT: A 2-month-old girl born full-term presented with leukocoria, suspicious for retinoblastoma. She was found to have an ischemic retrolental fibrovascular retinal detachment. In addition, there was linear cutaneous hyperpigmentation, diagnostic of incontinentia pigmenti.

CONCLUSIONS: Retinoblastoma can be a challenge …

Bilateral Retinal Problem In A Patient With Alport Syndrome., Sundeep K. Kasi, Murtaza K Adam, David S Ehmann

Wills Eye Hospital Papers

No abstract provided.

Integrated Results From The Copernicus And Galileo Studies., Amelie Pielen, W. Lloyd Clark, David S. Boyer, Yuichiro Ogura, Frank G. Holz, Jean-Francois Korobelnik, Brigitte Stemper, Friedrich Asmus, Kay D. Rittenhouse, Christiane Ahlers, Robert Vitti, Namrata Saroj, Oliver Zeitz, Julia A. Haller

Wills Eye Hospital Papers

OBJECTIVES: To report on the efficacy and safety of intravitreal aflibercept in patients with macular edema secondary to central retinal vein occlusion (CRVO) in an integrated analysis of COPERNICUS and GALILEO.

PATIENTS AND METHODS: Patients were randomized to receive intravitreal aflibercept 2 mg every 4 weeks or sham injections until week 24. From week 24 to week 52, all intravitreal aflibercept-treated patients in both studies and sham-treated patients in COPERNICUS were eligible to receive intravitreal aflibercept based on prespecified criteria. In GALILEO, sham-treated patients continued to receive sham treatment through week 52.

RESULTS: At week 24, mean gain in best-corrected …

Combining Magnetic Resonance Spectroscopy And Magnetic Resonance Imaging In Diagnosing Focal Brain Lesions In Children, Farah Naz Farid, Waseem Mirza, Nauman Hashmani

Section of Ophthalmology

Introduction: We attempted to find the sensitivity and specificity of various pediatric brain masses in the Pakistani population while keeping histopathology or clinical diagnosis as the gold standard.
Methods: This was a retrospective study that was conducted from January 2007 to January 2016. We reviewed the records of 204 patients that presented to the radiology department of Aga Khan University Hospital (AKUH). Out of the 204, 135 pediatric patients in the 0–18 age group with focal brain lesions who underwent magnetic resonance spectroscopy (MRS) and a biopsy or clinical diagnosis were included. If histopathology was available, it was taken as …

Unilateral Isolated Foveal Hypoplasia, A Jacobson, Thaddeus Wandel, Alan Springer

NYMC Faculty Publications

No abstract provided.

Protection Of Cells From Degeneration And Treatment Of Geographic Atrophy, Jayakrishna Ambati

Ophthalmology and Visual Science Faculty Patents

Therapeutic uses P2X₇ inhibition and inhibition of IRAK1 and/or IRAK4, methods protecting a cell, and screening methods for identifying inhibitors are described herein.

Synaptic Ribbon Active Zones In Cone Photoreceptors Operate Independently From One Another., Justin J. Grassmeyer, Wallace B. Thoreson

Journal Articles: Ophthalmology

Cone photoreceptors depolarize in darkness to release glutamate-laden synaptic vesicles. Essential to release is the synaptic ribbon, a structure that helps organize active zones by clustering vesicles near proteins that mediate exocytosis, including voltage-gated Ca(2+) channels. Cone terminals have many ribbon-style active zones at which second-order neurons receive input. We asked whether there are functionally significant differences in local Ca(2+) influx among ribbons in individual cones. We combined confocal Ca(2+) imaging to measure Ca(2+) influx at individual ribbons and patch clamp recordings to record whole-cell ICa in salamander cones. We found that the voltage for half-maximal activation (V50) of whole …

Intravitreal Melphalan For Treatment Of Primary Vitreoretinal Lymphoma: A New Indication For An Old Drug., Carol L. Shields, Kareem Sioufi, Arman Mashayekhi, Jerry A. Shields

Wills Eye Hospital Papers

No abstract provided.

Non-Adherence To Eye Care In People With Diabetes, Ann P. Murchison, Lisa A. Hark, Laura T. Pizzi, Yang Dai, Eileen Mayro, Philip P. Storey, Benjamin E. Leiby, Julia A. Haller

Wills Eye Hospital Papers

Objective Evaluate individual factors that impact adherence to eye care follow-up in patients with diabetes. Design and methods A 4-year retrospective chart review was conducted for 1968 patients with diabetes over age 40 from an urban academic center. Data collected included demographics, insurance, visual acuity, smoking status, medications, dates of dilated fundus examinations (DFE), and reported hemoglobin A1C and blood glucose levels. The primary outcome was timely DFE follow-up adherence following the initial eye exam visit. Results Overall, 41.6% of patients adhered to initial follow-up eye care recommendations. Multivariable analysis demonstrated that patients with severe diabetic retinopathy (DR) were more …

Autoimmune Retinopathy Associated With Systemic Lupus Erythematosus: A Diagnostic Dilemma., Wadakarn Wuthisiri, Yu-Hung Lai, Jenina Capasso, Martin Blidner, David Salz, Erik Kruger, Alex V. Levin

Wills Eye Hospital Papers

Visual loss in systemic lupus erythematosus (SLE) due to autoimmune retinopathy (AIR) is rare and easily misdiagnosed as hydroxychloroquine retinopathy. We report the rare clinical presentation of severe visual loss in a patient with SLE due to nonparaneoplastic AIR as differentiated from hydroxychloroquine toxicity. A 70-year-old female diagnosed and treated for lupus for 17 years and had been taking hydroxychloroquine for 15 years. Over the past 2 years, she developed progressive peripheral visual loss oculus uterque which rapidly advanced in the latter 6 months. Hydroxychloroquine toxicity was initially suspected, but diagnostic testing revealed a retinal degeneration. Antiretinal autoantibody testing using …

More Than Meets The Eye: The Contributions Of John Dalrymple (1803-1852)., Joy Mh Wang, Bryan Edwards, Gabrielle G Tardieu, Marios Loukas, Rod J Oskouian, R Shane Tubbs

Articles, Abstracts, and Reports

Having authored two major ophthalmology textbooks and honored with the eponym, the "Dalrymple sign," many are familiar with the works of Dr. John Dalrymple when it comes to the eye. However, few are aware of his other, numerous and wide-ranging contributions to the fields of science and medicine. In this article, we discuss the life and work of a man dedicated to the pursuit and advancement of knowledge and education.

Corneal Edema And Keratitis Following Selective Laser Trabeculoplasty, E T. Liu, L S. Seery, A Arosemena, Tania Lamba, C J. Chaya

Ophthalmology Faculty Publications

Purpose

To describe three cases of keratitis following Selective Laser Trabeculoplasty(SLT).

Observations

Three females with a history of glaucoma presented with corneal edema, keratitis (endothelial, epithelial) and decreased visual acuity shortly after SLT. There was variable resolution of symptoms after starting treatment with oral antiherpetics and topical steroids.

Conclusions and importance

With the increase in usage of SLT as a treatment for glaucoma and subsequent reports of keratitis, it is imperative for ophthalmic surgeons to be aware of herpes simplex as a possible risk factor. Prompt treatment with antivirals and steroids can potentially prevent scarring and permanent …

An Objective Assessment Of The Variability In Number Of Drops Per Bottle Of Glaucoma Medication, Daniel B. Moore, Judy Beck, Richard J. Kryscio

Ophthalmology and Visual Science Faculty Publications

Background: The purpose of this study is to evaluate the number of eyedrops available per bottle of a variety of commonly prescribed glaucoma medications.

Methods: Six bottles of each glaucoma medication were tested: three each in the vertical and horizontal orientations. Bottles were housed in a customized force gauge apparatus designed to mimic ballpoint fingertip contact with a bottle. At a standard rate, all drops were expressed from each bottle and counted with an automated drop counter. Simultaneously, bottle volume was measured and drop size and number were also estimated. The main outcome measures were: total number of drops, volume …