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Cases of Familial Exudative Vitreoretinopathy (FEVR)

SCIENTIFIC POSTER 2016_Cases of Familial Exudative Vitreoretinopathy (FEVR) Young Joon Jo Purpose: To report the sporadic cases of Familial exudative vitreoretinopathy (FEVR) and to emphasize the importance of peripheral fundus examination Methods and results: The number of FEVR patients must be greater than one would expect. I will introduce my sporadic cases series of FEVR and how to exam the peripheral fundus with usual fluorescein...

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X Linked Retinoschisis and RD Pre- and Post-operatively

              Color image of an X linked retinoschisis and detachment pre and postoperatively. Contact Details: Email: parolinibarbara@gmail.com Cell Phone:...

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RD After PDT for VHL Syndrome

The power point and attached video presentation shows the development and management of the retinal detachment as a complication of PDT for retinal capillary hemangiomas in a case of VHL syndrome. Contact Details: Email: drkaderli@yahoo.com Cell Phone:...

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Macular Surgery in Retinitis Pigmentosa Agnieszka Nowosielska

Retinitis pigmentosa is devastating disorder which leads to poor vision. Usually central vision is relativelly spared for longer time. However RP patients may suffer from macular problems (CMO, MH, ERM, pseudoholes) which additionally destroy the island of vision which is left. In disorders mentioned above, in otherwise healthy eyes, there would be not doubts if one should perform surgery. But what should be done RP case with in macular...

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Relationship Between Color Vision Deficiency & Foveal Anatomical Changes in Retinitis Pigmentosa

Pamela Campos Figueroa_SCIENTIFIC POSTER 2015 Background: Retinitis pigmentosa (RP) is an inherited disease characterized by photoreceptors and retinal pigment epithelium degeneration. The peripheral vision in patients with RP is highly compromised and the remaining central vision is associated with functional vision, and is commonly used to monitor disease development. As the disease progresses, patients undergo loss of visual acuity and...

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Severe Retinitis Pigmentosa of Early Onset in a Previously Diagnosed Amblyopia: Case Report

SCIENTIFIC POSTER_Carolina Vale_2 Background: Retinitis pigmentosa (RP) is a group of hereditary disorders that diffusely involve photoreceptors and pigmental epithelial function with a prevalence of about 1:5000. All inheritance patterns are described and more than 45 genes causing RP have been reported that might explain the heterogenicity of clinical presentation. The majority of RP cases occurs isolated with disease process confined to...

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Ocular Changes in Familial Amyloidotic Polyneuropathy Type I

Familial amyloidotic polyneuropathy (FAP) type I is an inherited disorder with autosomal dominant transmission, related to mutant transthyretin (TTR) and characterized by systemic extracellular accumulation of amyloid. TTR is mainly synthesized in the liver, but there is also production in the retinal pigment epithelium. One point mutation in which a methionine residue replaces the valine residue at position 30 (TTR Val30Met) is the most...

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Mutation in the Exon 6 of Gene RDH12 Associated to Severe Early-onset Macular Degeneration

Poster 4 Joaquin Castro Navarro Advantages: To show the clinical evolution of a child with a novel mutation in exon 6 of gene RDH12 (gene typically associated with Leber Congenital Amaurosis: LCA) and severe early-onset macular degeneration. Methods: An 8-year-old male with unremarkable ocular and systemic history who came to our department with a complaint of progressive decline in vision in his both eyes. Full clinical ophthalmological...

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Can Retinitis Pigmentosa Influence MH Surgical Results?

Poster 2 Joaquin Castro Navarro Advantages: To report a case of unilateral recurrent macular hole in a patient affected by retinitis pigmentosa (RP). Methods: A 64-year-old woman, with the previous diagnosis of RP, was referred to our department with a complaint of central visual loss in her left eye (LE) for 12 months. A complete ocular exploration, including determination of Best Corrected Visual Acuity (BCVA) (decimal notation),...

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ERM Removal in a Case of Von Hippel Lindau Angioma Post-brachytherapy

We present an unusual case of Von Hipple Lindau angiomas managed by brachytherapy. During post-operative follow up, an epiretinal membrane developed causing dragging of the macula and diminution of visual acuity. A 23 gauge pars plana vitrectomy was performed with removal of ERM and endolaser to the angiomas residuals. ERM came as one sheet and was found to extend to the retinal periphery. Visual acuity and OCT thickness improved...

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Management of ME in Retinitis Pigmentosa with Topical Dorzolamide

Advantages: Retinitis pigmentosa may be associated with macular edema and cystoid spaces. Topical dorzolamide may be useful in improving macular edema. Methods: Prospective evaluation of the use of dorzolamite  (topical 3 times daily for 3-6 months) in ME cases associated with RP. Six cases with RP were identified by OCTas having macular edema. A course of dorzolamide drops was initiated 3 times/day for 3-6 months. Evaluation of...

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Macular Dilemas in Retinitis Pigmentosa

Advantages: Retinitis pigmentosa is inherited, progressive disease. There is no approved therapy for the disorder itself and there is no approved therapy for its complications as well. Although we are not able to treat RP itself, many secondary changes like cataract, macular edema, macular holes and ERM influencing patients’ vision could be addressed. Methods: Case series to present to diagnostic and treatment challenges in patients...

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Cystoid Macular Edema in Retinitis Pigmentosa

Advantages: To report on medical and surgical treatment for retinitis pigmentosa-associated cystoid macular edema (CME). Retinitis pigmentosa is a progressive inherited retinal disease and one of the main causes of acquired blindness in developed countries. There is no approved therapy able to stop the degeneration of the photorecptor cells and the retinal pigment epithelium. The current management using medical and surgical therapy...

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Vitreoretinal Surgery for Retinal Detachment in Retinal capillary Hemangioma (RCH)

Maurizio Mete*, Grazia Pertile (Negrar, Italy) Retinal capillary haemangioma (RCH) is one of the most common expressions of Von Hippel-Lindau (VHL) disease. In case of small- or medium-sized RCHs (up to 2 disk diameters), the standard treatment remains laser photocoagulation or cryotherapy. Various treatments have been proposed such as brachytherapy, external beam radiotherapy, transpupillary thermotherapy, photodynamic therapy and...

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Retinal Capillary Hemangioma and von Hippel-Lindau Disease: Diagnostic and Therapeutic Implications

C Gonzalez Castano, R Salazar Mendes, J Castro Navarro, M Fonolla Gil, A Garcia Alonso, P Rozas Reyes (Oviedo, Spain) Advantages: To confirm the efficacy of laser photocoagulation in retinal capillary hemangioma’s treatment, but also the retinal complications associated to this procedure and to reveal the implications of its diagnosis in patients with von Hippel-Lindau disease. Methods: A male carrier of VHL gene with large-evolution loss...

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Photodynamic Therapy and Argon Laser Photocoagulation in von Hippel Lindau Disease in Children: Our Experience

Michele Fortunato (Rome, Italy), Rossana Di Pietro (Pinerolo (TO) Italy) Advantages: Photodynamic therapy represents a safe and efficient procedure for the treatment of retinal angiomas in children, by the selective occlusion of vascular lesions, without damaging adjacent retinal structures. Methods: The authors describe their experience in the treatment of five cases of von Hippel Lindau disease; patients present the age between 5 and 9...

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Management of Retinal Vein Obstruction Secondary to Congenital Arteriovenous Communication in Children

http://www.evrs.eu/medias/2010/congress/Vascular-Pathologies-in-Young-Patients.flv Barbara Delas, Eva Villota, Alvaro Fernandez-Vega, Jeroni Nadal (Barcelona, Spain) Advantages: Racemose hemangiomas of the retina are rare developmental anomalies. Although visual prognosis is usually good because of their clinical stability, the development of retinal vascular complications should be taken into account when planning follow-up, especially in...

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Surgical Treatment of von Hippel Peripheral Capillary Hemangioma Complications

http://www.evrs.eu/medias/2010/congress/Vascular-Pathologies-in-Young-Patients.flv Andriy Sergiienko, Lyubomyr Lytvynchuk, Oleksandr Fillipchuk (Kiev, Ukraine) Advantages: Exudative maculopathy and tractional retinal detachment are common complication of peripheral capillary hemangioma. Surgical approach necessitates big challenges and careful treatment planning. Methods: 5 patients with exudative maculopathy and tractional retinal...

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Scleral Buckling, Vitrectomy and Lensectomy in a Marfan Syndrome Child with Retinal Detachment and Subluxation of Crystalline

http://www.evrs.eu/medias/2009/videos/ElenaPalaciosPozo_VP6_768K.flv Authors: Elena Palacios-Pozo, Maria Garcia-Pous, Amparo Navea, M. Carmen Desco, Jorge Mataix, Valencia, Spain Advantages: To evaluate the management of retinal detachment and subluxation of the lens in a child with Marfan Syndrome. Methods: Marfanʼs syndrome is an autosomic dominant genetic disorder of the elastic fibers of connective tissue. That syndrome affects many...

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A Case of Oguchi Disease Associated with Diabetes Mellitus

S. Morteza Entezari, MD (Tehran, Iran), Alireza Ramezani, MD (Tehran, Iran), Hamid Reza Ghamari, MD (Tehran, Iran) PURPOSE: To report an Iranian patient with diagnosis of Oguchi disease associated with Diabetes mellitus. METHODS: A 50 year old woman with history of Diabetes mellitus was referred. She complained from mild blurred vision and night blindness. She was evaluated by complete eye examination, precise funduscopy, fluorescein...

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Chronic Retinal Chip Implant in Blind Patients with Retinitis Pigmentosa

http://www.evrs.eu/medias/2006/congress/Chronic-Retinal-Chip-Implant-in-Blind-Patients-with-Retinitis-Pigmentosa.swf Gisbert Richard, MD (Hamburg, Germany), Michaela Velikay Parel, MD (Graz, Austria), Ralf Hornig, MD (Bonn, Germany), Matthias Feucht, MD (Hamburg, Germany) PURPOSE: To evaluate the performance of an investigational Retinal Implant System in blind patients with retinitis pigmentosa. Objectives of the study are also to test the...

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Evaluation of the Artificial Silicon RetinaTM Device for the Treatment of Vision Loss from Retinitis Pigmentosa

http://www.evrs.eu/medias/2006/congress/Evaluation-of-the-Artificial-Silicon-RetinaTM-Device-for-the-Treatment-of-Vision-Loss-from-Retinitis-Pigmentosa.swf John S. Pollack, MD (Joliet, IL),* Kirk H. Packo, MD (Chicago, IL),* Gislin Dagnelie, PhD (Baltimore, MD), Ron A. Schuchard, PhD (Atlanta, GA), Julia A. Haller, MD (Baltimore, MD), Thomas M. Aaberg, MD (Atlanta, GA), Mathew W. MacCumber, MD, PhD (Chicago, IL), Pauline T. Merrill, MD (Oak...

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Neuroprotective Effects of Sustained-release Intravitreal Fluocinolone Acetonide in RCS Rats

Raymond Iezzi, MD (Detroit, MI), Inna Glybina, MD, PhD (Detroit, MI), Paul Ashton, PhD (Watertown, MA),* Gary W. Abrams, MD (Detroit, MI) PURPOSE: Corticosteroids and estrogens have been shown to exibit neuroprotective effects, mediated via the stabilization of lipid membranes and the inhibition of apoptosis. The purpose of this study was to determine if chronic intravitreal delivery of the synthetic corticosteroid, Fluocinolone Acetonide...

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Anti-VEGF Therapy for Pediatric Retinopathies: Which Case Is the Right Case?

http://www.evrs.eu/medias/2006/congress/Anti-VEGF-Therapy-for-Pediatric-Retinopathies:-Which-Case-Is-the-Right-Case.swf Kimberly A. Drenser, MD (Royal Oak, CA), Michael T. Trese, MD (Royal Oak, MI), Antonio Capone, MD (Royal Oak, MI) PURPOSE: To review the usefulness and the appropriate case selection of anti-VEGF agents for the treatment of vitreoretinopathies in the pediatric population. METHODS: Patients with either retinopathy of...

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Indocyanine Green (ICG) Mediated Phototrombosis of a Large Retinal Hemangioblastoma in von Hippel-Lindau Disease

Enrico Bertelli, (Bolzano, Italy) PURPOSE: To assess the efficacy of ICG mediated phototrombosis in the treatment of large vascular lesions, and in particular of a 6 mm large, actively exudating retinal hemangioblastoma in a case of von Hippel- Lindau disease. METHODS: A 31 year old female patient presented with history of decreased vision in her right eye dating back several months. Visual acuity had decreased to 20/200. Fundus examination...

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Screening for Retinopathy in the Pediatric Sickle Cell Patient Population

http://www.evrs.eu/medias/2006/congress/Screening-for-Retinopathy-in-the-Pediatric-Sickle-Cell-Patient-Population.swf Harmeet S. Gill, MD (North York, Canada), Melanie Kirby-Allen, MD, FRCP (Toronto, Canada), Wai-Ching Lam, MD, FRCS (Toronto, Canada) PURPOSE: Individuals with sickle cell disease (SCD) are at risk for developing proliferative retinopathy and visionthreatening complications. This study aims to determine the prevalence and age...

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