Medicine and Health Sciences Commons^™

Does The Novel Coronavirus Use The Ocular Surface As An Entrance Into The Body Or As An Infection Site?, Remzi Karadag, Alp Kayiran, Christopher J Rapuano

Wills Eye Hospital Papers

This study attempts to review whether the coronavirus disease-2019 (COVID-19) is transmitted through the ocular surface and examine the symptoms and signs of ocular disease. Considering that COVID-19 is transmitted by airborne droplets and close contact with infected individuals, we will also review the conditions to which eye clinics and ophthalmologists should pay attention to prevent the transmission of the disease. Although some researchers have argued that COVID-19 transmission cannot occur through the ocular surface, most of them are of the opinion that the ocular surface is a potential pathway of transmission. Until date, ocular signs and symptoms have been …

Conditional Analysis On New Tumor Formation With Solitary Unilateral Retinoblastoma In 482 Consecutive Patients, Carol L Shields, Philip W Dockery, Megan Ruben, Madalyne A Sunday, Martin Calotti, Antonio Yaghy

Wills Eye Hospital Papers

PURPOSE: The objective of the study was to understand dynamic risk (conditional analysis based on patient age) for new tumor development in patients with solitary unilateral retinoblastoma.

METHODS: This was a retrospective analysis.

RESULTS: Of 482 patients with solitary unilateral retinoblastoma, 55 new tumors developed in 20 patients (4%). Comparison (new tumor vs. no new tumor development) revealed those with new tumor demonstrated younger mean age at presentation (10 vs. 36 months,

CONCLUSION: Children (≤24 months) with solitary unilateral retinoblastoma showed decreasing risk for new tumors up to 24 months of life. Later onset of new tumor was more likely …

Photodynamic Therapy In Ocular Oncology., Mehdi Mazloumi, Lauren A Dalvin, Seyed-Hossein Abtahi, Negin Yavari, Antonio Yaghy, Arman Mashayekhi, Jerry A Shields, Carol L Shields

Wills Eye Hospital Papers

Over the past two decades, we have witnessed the increasing use of photodynamic therapy (PDT) in the field of ocular oncology. Based on a review of the literature and our own experience, we herein review the role of PDT for the management of intraocular tumors. The discussion includes two main topics. First, we discuss the application of PDT for benign tumors, including circumscribed choroidal hemangioma, choroidal osteoma, retinal astrocytoma, retinal capillary hemangioma (retinal hemangioblastoma), and retinal vasoproliferative tumor. Second, we assess the role of PDT for malignant tumors, including choroidal melanoma and choroidal metastasis.

Ophthalmic Manifestations Of Rosai-Dorfman Disease In Five Patients, Babak Masoomian, Sara E. Lally, Jerry A Shields, Carol L Shields

Wills Eye Hospital Papers

Purpose: To report clinical features, pathology characteristics, and treatment outcomes of five patients with Rosai-Dorfman disease (RDD).

Methods: A retrospective case series of patients with RDD from the Ocular Oncology Service of Wills Eye Hospital between 1974 and 2018.

Results: There were six eyes of five patients (3 males, 2 females) with ophthalmic manifestations of RDD. The mean age at the initial presentation was 33 years (median 35, range 10-52 years). Before referral, the tumor was initially suspected to be lymphoma (n = 3), idiopathic orbital inflammation (n = 2), or pterygium (n = 1). The disease was unilateral (n …

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 …

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 …

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.

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 …

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 …

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 …

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 …

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 …

Choroidal Metastasis From Leiomyosarcoma In Two Cases, Eric Feinstein, Swathi Kaliki, Carol L Shields, Hormoz Ehya, Jerry A Shields

Wills Eye Hospital Papers

Leiomyosarcoma is a malignant tumor of mesenchymal cells and is the most common soft-tissue sarcoma. Leiomyosarcoma is a notably rare tumor in the ophthalmic region and can be of primary, secondary or metastatic origin. To the best of our knowledge, there has only been one published case of leiomyosarcoma metastasis to the choroid. In this case study, we report two cases of primary leiomyosarcoma with metastasis to the choroid of the eye. Both cases displayed systemic metastasis and showed response to high dose plaque radiotherapy. Despite its prevalence as the leading form of sarcoma, leiomyosarcoma rarely metastasizes to the ocular …

Genetics For The Ophthalmologist., Karthikeyan A Sadagopan, Jenina Capasso, Alex V Levin

Wills Eye Hospital Papers

The eye has played a major role in human genomics including gene therapy. It is the fourth most common organ system after integument (skin, hair and nails), nervous system, and musculoskeletal system to be involved in genetic disorders. The eye is involved in single gene disorders and those caused by multifactorial etiology. Retinoblastoma was the first human cancer gene to be cloned. Leber hereditary optic neuropathy was the first mitochondrial disorder described. X-Linked red-green color deficiency was the first X-linked disorder described. The eye, unlike any other body organ, allows directly visualization of genetic phenomena such as skewed X-inactivation in …