Ophthalmology Commons^™

Pilomatrix Carcinoma Of The Lacrimal Caruncle: A Case Report., Ibrahim Inan Harbiyeli, Altan A Ozcan, Arbil Acıkalin, Emine Ciloglu, Carol L Shields

Wills Eye Hospital Papers

A 45-year-old man presented with a 3-month history of a mass located in the caruncle of his right eye. An incisional biopsy had been performed one month prior by another specialist, and the histopathology report showed basal cell carcinoma. The mass was completely excised with a 2 mm safety margin, and the large conjunctival defect was reconstructed with one sheet of amniotic membrane allograft. A histological diagnosis of pilomatrix carcinoma was established. To prevent recurrence after surgery, we added bevacizumab (25 mg/mL, 1.25 mg/mL per drop) eye drops four times per day for three months. At the one-year follow-up, the …

Comparison Of Three Different Techniques Of Inverted Internal Limiting Membrane Flap In Treatment Of Large Idiopathic Full-Thickness Macular Hole., Fariba Ghassemi, Hassan Khojasteh, Alireza Khodabande, Lauren A. Dalvin, Mehdi Mazloumi, Hamid Riazi-Esfahani, Masoud Mirghorbani

Wills Eye Hospital Papers

Purpose: To evaluate and compare three different techniques of inverted internal limiting membrane (ILM) flap in the treatment of large idiopathic full-thickness macular hole.

Methods: In a comparative interventional case series, 72 eyes from 72 patients with large (> 400 µm) full-thickness macular hole were randomly enrolled into three different groups: group A - hemicircular ILM peel with temporally hinged inverted flap; group B - circular ILM peel with temporally hinged inverted flap; and group C - circular ILM peel with superior inverted flap. Best-corrected visual acuity (BCVA), anatomical closure rate, and ellipsoid zone (EZ) or external limiting membrane (ELM) …

Neurofibromatosis Type-1 With Retinal Microvascular Corkscrew Tortuosity., Adrian J Battiston, Lauren A Dalvin, Carol L Shields

Wills Eye Hospital Papers

No abstract available.

Melanocytoma Of The Optic Disk: A Review., Jerry A. Shields, Hakan Demirci, Arman Mashayekhi, Ralph C. Eagle Jr., Carol L. Shields

Wills Eye Hospital Papers

Melanocytoma is a deeply pigmented variant of melanocytic nevus that classically occurs in the optic disk, sometimes with contiguous involvement of the adjacent retina or choroid. Historically, this tumor was often confused with malignant melanoma both clinically and histopathologically. Today, however, it is generally recognized by its typical clinical features that differ from most melanomas and erroneous enucleation is rarely done. Histopathologically, melanocytoma is composed of intensely pigmented round to oval nevus cells with benign features. Although traditionally believed to be a relatively stationary lesion, it is now known to exhibit minor enlargement in 10--15% of cases and can cause …

Conjunctival Melanoma With Orbital Invasion And Liver Metastasis Managed With Systemic Immune Checkpoint Inhibitor Therapy., Michael Chang, Sara E Lally, Lauren A Dalvin, Marlana Orloff, Carol L Shields

Wills Eye Hospital Papers

A 60-year-old Caucasian female was referred for biopsy-proven amelanotic orbito-conjunctival melanoma. Map biopsies revealed residual invasive melanoma on the deep tarsal margin at the site of previous surgery. Repeat excisions were required after recurrence was detected following 3 months and 7 months. Positron emission tomography scan detected liver metastasis and additional orbito-conjunctival melanoma recurrence. Biomarker testing showed NRAS mutation without BRAF or c-KIT mutations and without PD-L1 expression. Systemic checkpoint inhibitor therapy was initiated with regression of both the orbito-conjunctival melanoma and liver metastasis. Invasive, non resectable orbito-conjunctival melanoma with liver metastasis can demonstrate a response to systemic checkpoint inhibitor …

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

Wills Eye Hospital Papers

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

Methods: A retrospective analysis of all patients older than 5 years treated with IAC for RB from 2008-2018. Comparisons were made to 26 active RB cases in older children treated in the prechemotherapy era and to 12 active RB cases treated in the IVC era.

Results: There were …

From Lepidoptera To Uveal Melanoma: Finding My Career In Ocular Oncology., Jerry A Shields

Wills Eye Hospital Papers

No abstract provided.

Tumors Of The Conjunctiva And Cornea., Carol L. Shields, Jerry A. Shields

Wills Eye Hospital Papers

Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired lesions. The acquired lesions are further subdivided based on origin of the mass into surface epithelial, melanocytic, vascular, fibrous, neural, histiocytic, myxoid, myogenic, lipomatous, lymphoid, leukemic, metastatic and secondary tumors. Melanocytic lesions include nevus, racial melanosis, primary acquired melanosis, melanoma, and other ocular surface conditions like ocular melanocytosis and secondary pigmentary deposition. The most frequent nonmelanocytic neoplastic lesions include squamous cell carcinoma and lymphoma, both of which have typical features appreciated on clinical examination. …

Prognostication Of Uveal Melanoma Is Simple And Highly Predictive Using The Cancer Genome Atlas (Tcga) Classification: A Review., Carol L. Shields, Lauren A. Dalvin, Pornpattana Vichitvejpaisal, Mehdi Mazloumi, Arupa Ganguly, Jerry A. Shields

Wills Eye Hospital Papers

Purpose: The cancer genome atlas (TCGA) is a comprehensive project supported by the National Cancer Institute (NCI) in the United States to explore molecular alterations in cancer, including uveal melanoma (UM). This led to TCGA classification for UM. In this report, we review the American Joint Committee on Cancer (AJCC) classification and TCGA classification for UM from the NCI's Center for Cancer Genomics (NCI CCG) (based on enucleation specimens [n = 80 eyes]) and from Wills Eye Hospital (WEH) (based on fine needle aspiration biopsy [FNAB] specimens [n = 658 eyes]). We then compare accuracy and predictability of AJCC versus …

Intraocular Amyloidosis With Multifocal Iris And Anterior Chamber Translucent Spherules., Sima Das, Sweety Tiple, Julie Pegu, Suneeta Dubey, Umang Mathur, Kaustabh Mulay, Carol L Shields

Wills Eye Hospital Papers

Ocular amylodosis, although a rare entity, is known to affect the conjunctiva, extraocular muscles, orbit, lacrimal gland, and skin around the eyes. Intraocular deposition of amyloid mainly confines to the vitreous and cornea. In this report, we describe two cases of intraocular amyloidosis presenting as multiple iris and anterior chamber cysts. Histopathological examination with special stain like Congo Red and Transmission Electron Microscopy confirmed the diagnosis of amyloidosis. Systemic investigations ruled out systemic association confirming the diagnosis of primary ocular amyloidosis.

Trifocal Choroidal Melanoma In An Eye With Oculodermal Melanocytosis: A Case Report., Samuel J Fallon, Charlotte N Shields, Maura Di Nicola

Wills Eye Hospital Papers

We report a case of trifocal choroidal melanoma (three separate tumors) in a 48-year-old Caucasian female who had been followed for oculodermal melanocytosis since childhood. At presentation, no tumor was present and annual examination was advised. Seventeen years later, three choroidal melanocytic lesions were detected in the right eye. Growth of each was documented, enucleation was performed, and histopathology revealed three independent choroidal melanomas. The patient developed extensive liver and bone metastases and subsequently died. Oculodermal melanocytosis is a risk factor for the development of uveal melanoma and a potential marker for worse prognosis. Careful long-term follow-up is required.

Worldwide Inequality In Access To Full Text Scientific Articles: The Example Of Ophthalmology., Christophe Boudry, Patricio Alvarez-Muñoz, Ricardo Arencibia-Jorge, Didier Ayena, Niels J Brouwer, Zia Chaudhuri, Brenda Chawner, Emilienne Epee, Khalil Erraïs, Akbar Fotouhi, Almutez M Gharaibeh, Dina H Hassanein, Martina C Herwig-Carl, Katherine Howard, Dieudonne Kaimbo Wa Kaimbo, Patricia-Ann Laughrea, Fernando A Lopez, Juan D Machin-Mastromatteo, Fernando K Malerbi, Papa Amadou Ndiaye, Nina A Noor, Josmel Pacheco-Mendoza, Vasilios P Papastefanou, Mufarriq Shah, Carol L Shields, Ya Xing Wang, Vasily Yartsev, Frederic Mouriaux

Wills Eye Hospital Papers

Background: The problem of access to medical information, particularly in low-income countries, has been under discussion for many years. Although a number of developments have occurred in the last decade (e.g., the open access (OA) movement and the website Sci-Hub), everyone agrees that these difficulties still persist very widely, mainly due to the fact that paywalls still limit access to approximately 75% of scholarly documents. In this study, we compare the accessibility of recent full text articles in the field of ophthalmology in 27 established institutions located worldwide.

Methods: A total of 200 references from articles were retrieved using the …

Eyelid Chalazion Or Not?, Jerry A. Shields, Sara E. Lally, Tatyana Milman, Carol L. Shields

Wills Eye Hospital Papers

No abstract provided.

Vascular Perfusion In Persistent Pupillary Membrane Of The Iris., Michael Chang, David Ancona-Lezama, Carol L. Shields

Wills Eye Hospital Papers

No abstract provided.

Evidence To Date: Ranibizumab And Its Potential In The Treatment Of Retinopathy Of Prematurity, Samir Patel, Michael A. Klufas

Wills Eye Hospital Papers

Retinopathy of prematurity (ROP) is a leading and preventable cause of childhood blindness worldwide. Although laser photocoagulation remains the gold standard for treatment, the off-label use of anti-vascular endothelial growth factor (anti-VEGF) therapy to treat ROP, particularly posterior zone I disease, is increasing. Although initial studies on anti-VEGF therapy for ROP have focused on bevacizumab, recent studies have proposed that ranibizumab may be a safer and more effective alternative for use in this population. This review updates recent evidence regarding the use of ranibizumab in the management of ROP.

Unilateral Enophthalmos., Grace L. Su, Sara E. Lally, Carol L. Shields

Wills Eye Hospital Papers

No abstract provided.

Intra-Arterial Chemotherapy In Retinoblastoma - A Paradigm Change., Fairooz P. Manjandavida, Christina Stathopoulos, Jing Zhang, Santhosh G. Honavar, Carol L. Shields

Wills Eye Hospital Papers

Intra-arterial chemotherapy (IAC), also known as superselective ophthalmic artery chemotherapy or chemosurgery, is currently widely accepted as one of the primary treatment modalities for intraocular retinoblastoma worldwide. Following the introduction of the technique in 1998, IAC has evolved over the past decades to be safer and more effective. Accumulated evidence shows that IAC is more effective in providing eye salvage in group D and E retinoblastoma as compared to conventional systemic intravenous chemotherapy (IVC). In contrast to IVC, IAC has the added benefits of reduced overall treatment duration and minimal systemic toxicity. This review provides a comprehensive update on the …

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 …

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

Wills Eye Hospital Papers

No abstract provided.

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

Wills Eye Hospital Papers

No abstract provided.

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 …

Bilateral Mastectomy, Choroidal Mass: A Curve Ball., Phillip Gordon, Carol L. Shields

Wills Eye Hospital Papers

No abstract provided.

Comparison Of Germline Versus Somatic Bap1 Mutations For Risk Of Metastasis In Uveal Melanoma., K. G. Ewens, E. Lalonde, J. Richards-Yutz, C. L. Shields, A. Ganguly

Wills Eye Hospital Papers

BACKGROUND: Germline mutations in BAP1 have been associated with BAP1-Tumor Predisposition Syndrome (BAP1-TPDS), a predisposition to multiple tumors within a family that includes uveal melanoma (UM), cutaneous melanoma, malignant mesothelioma and renal cell carcinoma. Alternatively, somatic mutations in BAP1 in UM have been associated with high risk for metastasis. In this study, we compare the risk of metastasis in UM that carry germline versus somatic BAP1 mutations and mutation-negative tumors.

METHODS: DNA extracted from 142 UM and matched blood samples was sequenced using Sanger or next generation sequencing to identify BAP1 gene mutations.

RESULTS: Eleven of 142 UM (8%) carried …

Unilateral Macular Drusen In A Young Female., Kareem Sioufi, Katherine E Talcott, Marc J Spirn

Wills Eye Hospital Papers

No abstract provided.

Pretty Peachy., Li-Anne Lim, Basil K. Williams, Carol L. Shields

Wills Eye Hospital Papers

No abstract provided.

Lacrimal Gland Tumors In Turkey: Types, Frequency, And Outcomes., Yağmur Seda Yeşiltaş, Ahmet Kaan Gündüz, Esra Erden, Carol L. Shields

Wills Eye Hospital Papers

AIM: To evaluate the clinical, radiological, and treatment features of lacrimal gland tumors.

METHODS: Retrospective review of 99 eyes of 92 patients with lacrimal gland tumors diagnosed and managed in a single institution between January 1999 and March 2017. Clinical and radiological features, histopathology, treatment methods, and prognosis were evaluated.

RESULTS: The mean patient age was 40.3 (range: 7-80)y. The diagnosis was made histopathologically in 91 (91.9%) tumors and on a clinical and radiological basis in 8 (8.1%) tumors. Final diagnoses included idiopathic orbital inflammation (pseudotumor) in 46 (46.5%) lesions, pleomorphic adenoma in 14 (14.1%), adenoid cystic carcinoma in 12 …

Ophthalmic Manifestations Of Heimler Syndrome Due To Pex6 Mutations., Nutsuchar Wangtiraumnuay, Waleed Abed Alnabi, Mai Tsukikawa, Avrey Thau, Jenina Capasso, Reuven Sharony, Chris F. Inglehearn, Alex V. Levin

Wills Eye Hospital Papers

BACKGROUND/AIMS: Pigmentary retinal dystrophy and macular dystrophy have been previously reported in Heimler syndrome due to mutations in PEX1. Here we reported the ocular manifestations in Heimler syndrome due to mutations in PEX6.

MATERIALS AND METHODS: Medical records were reviewed to identify patient demographics, ophthalmic and systemic findings, and results of diagnostic testing including whole genome sequencing.

RESULTS: Patient 1 is 12-year-old boy with a novel mutation c.275T>G (p.Val92Gly) and known mutation c.1802G>A (p.Arg601Gln) in PEX6. Patient 2 is a 7-year-old girl with the same known c.1802G>A (p.Arg601Gln) mutation and another novel missense mutation c.296G>T (p.Arg99Leu). …

Mud-Splattered Fundus, Roger Joel Welch, Xintong Li, Carol L. Shields

Wills Eye Hospital Papers

No abstract provided.

Submillimeter Retinoblastoma Monitoring Following Transpupillary Thermotherapy Using Hand-Held Optical Coherence Tomography, Mark Mcgarrey, Thamolwan Surakiatchanukul, Carol L. Shields

Wills Eye Hospital Papers

Background: Precise, submillimeter visualization of retinal microstructures is useful for treatment monitoring of retinoblastoma. Herein, we report the use of hand-held optical coherence tomography (HH-OCT) to document a nearly-invisible retinoblastoma and monitor tumor response to transpupillary thermotherapy (TTT). Case presentation: A 3-week-old boy was diagnosed with unilateral familial retinoblastoma in the left eye, classified as group B, and treated with intravenous chemoreduction. At 13-month follow-up, the tumor in the left eye was regressed, and evaluation of the right eye revealed a microscopic, nearly invisible tumor measuring 372 μm in thickness and 1.51 mm in basal dimension. The tumor was confirmed …