Medicine and Health Sciences Commons^™

Impact Of Natural Blind Spot Location On Perimetry., Mengyu Wang, Lucy Q. Shen, Michael V. Boland, Sarah R. Wellik, Carlos Gustavo De Moraes, Jonathan S. Myers, Peter J. Bex, Tobias Elze

Wills Eye Hospital Papers

We study the spatial distribution of natural blind spot location (NBSL) and its impact on perimetry. Pattern deviation (PD) values of 11,449 reliable visual fields (VFs) that are defined as clinically unaffected based on summary indices were extracted from 11,449 glaucoma patients. We modeled NBSL distribution using a two-dimensional non-linear regression approach and correlated NBSL with spherical equivalent (SE). Additionally, we compared PD values of groups with longer and shorter distances than median, and larger and smaller angles than median between NBSL and fixation. Mean and standard deviation of horizontal and vertical NBSL were 14.33° ± 1.37° and -2.06° ± …

Genetic And Genomic Characterization Of 462 Melanoma Patient-Derived Xenografts, Tumor Biopsies, And Cell Lines., Bradley Garman, Ioannis N. Anastopoulos, Clemens Krepler, Patricia Brafford, Katrin Sproesser, Yuchao Jiang, Bradley Wubbenhorst, Ravi Amaravadi, Joseph Bennett, Marilda Beqiri, David Elder, Keith T. Flaherty, Dennie T. Frederick, Tara C. Gangadhar, Michael Guarino, David Hoon, Giorgos Karakousis, Qin Liu, Nandita Mitra, Nicholas J. Petrelli, Lynn Schuchter, Batool Shannan, Carol L. Shields, Jennifer Wargo, Brandon Wenz, Melissa A. Wilson, Min Xiao, Wei Xu, Xaiowei Xu, Xiangfan Yin, Nancy R. Zhang, Michael A. Davies, Meenhard Herlyn, Katherine L. Nathanson

Wills Eye Hospital Papers

Tumor-sequencing studies have revealed the widespread genetic diversity of melanoma. Sequencing of 108 genes previously implicated in melanomagenesis was performed on 462 patient-derived xenografts (PDXs), cell lines, and tumors to identify mutational and copy number aberrations. Samples came from 371 unique individuals: 263 were naive to treatment, and 108 were previously treated with targeted therapy (34), immunotherapy (54), or both (20). Models of all previously reported major melanoma subtypes (BRAF, NRAS, NF1, KIT, and WT/WT/WT) were identified. Multiple minor melanoma subtypes were also recapitulated, including melanomas with multiple activating mutations in the MAPK-signaling pathway and chromatin-remodeling gene mutations. These well-characterized …

Parafoveolar Retinoblastoma Regression With Foveal Preservation Following Intra-Arterial Chemotherapy Documented On Hand-Held Optical Coherence Tomography In A Newborn., Vera Yarovaya, Kareem Sioufi, Carol L Shields

Wills Eye Hospital Papers

Background: Optical coherence tomography (OCT) has become an invaluable tool in retinoblastoma management, providing submillimeter visualization of tumor control following treatment. Herein, we document OCT-detection of a subtle tumor recurrence, allowing early intervention and achieving foveal microanatomy preservation.

Case presentation: A 3-week-old girl was diagnosed with bilateral familial retinoblastoma, classified as group D in the right eye (OD) and group B in the left eye (OS), and treated with intravenous chemoreduction. At 6-months follow-up, the right eye was under control, but the left eye revealed a subtle juxtafoveal tumor recurrence, documented on handheld OCT (HH-OCT) and measuring 2750 µm in …

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 …

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 …

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 …

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, …

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 …

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 …

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.

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 …

Phosphorylation Of Prb: Mechanism For Rb Pathway Inactivation In Mycn-Amplified Retinoblastoma., Kathryn G. Ewens, Tricia R. Bhatti, Kimberly A. Moran, Jennifer Richards-Yutz, Carol L. Shields, Ralph C. Eagle Jr., Arupa Ganguly

Wills Eye Hospital Papers

A small, but unique subgroup of retinoblastoma has been identified with no detectable mutation in the retinoblastoma gene (RB1) and with high levels of MYCN gene amplification. This manuscript investigated alternate pathways of inactivating pRb, the encoded protein in these tumors. We analyzed the mutation status of the RB1 gene and MYCN copy number in a series of 245 unilateral retinoblastomas, and the phosphorylation status of pRb in a subset of five tumors using immunohistochemistry. There were 203 tumors with two mutations in RB1 (RB1(-/-) , 83%), 29 with one (RB1(+/-) , 12%) and 13 with no detectable mutations (RB1(+/+) …

Stones, Bones, Groans, Thrones, And Psychiatric Overtones: Systemic Associations Of Sclerochoroidal Calcification., Jordan A Sugarman, Alexzandra M Douglass, Emil Anthony T. Say, Carol L. Shields

Wills Eye Hospital Papers

Sclerochoroidal calcification (SCC) is a frequent masquerader of choroidal melanoma with important systemic associations such as hyperparathyroidism and parathyroid adenoma. Herein, we describe a case of a 67-year-old male who presented with an amelanotic choroidal lesion in the right eye (OD) and a history of kidney stones. Ultrasonography showed the lesion to be flat and calcified OD. Incidentally, a subclinical calcified plaque was also found in the fellow eye. Optical coherence tomography showed an elevated suprachoroidal mass in a table mountain configuration OD and flat configuration left eye, consistent with type 4 and type 1 SCC. The patient was referred …

Serum Hypercoagulability States In Coats' Disease., Fariba Ghassemi, Carol L. Shields, Masoumeh Mohebbi, Mehdi Nili Ahmadabadi, Fatemeh Morsali, Siamak Sabour

Wills Eye Hospital Papers

PURPOSE: The purpose of this study was to investigate the serum hypercoagulability state and common viral and protozoan infections in Coats' disease versus a normal control group.

MATERIALS AND METHODS: In this comparative case series, 22 consecutive patients with Coats' disease and 19 non-Coats' patients undergoing lensectomy for congenital, traumatic, or senile cataract between January 2011 and June 2014 were included. Laboratory data for hypercoagulability states and common viral and protozoan infections were investigated.

RESULTS: The mean age for the Coats' group was 14.5 years (median 8 years, range: 2 months to 59 years), and for the control group it …