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https://www.readbyqxmd.com/read/28198786/optical-coherence-tomography-angiography-in-adult-onset-foveomacular-vitelliform-dystrophy
#1
Komal M Joshi, Peter L Nesper, Amani A Fawzi, Rukhsana G Mirza
PURPOSE: To determine the ability of optical coherence tomography angiography (OCTA) to detect choroidal neovascularization (CNV) in the pseudohypopyon stage of adult-onset foveomacular vitelliform dystrophy. METHODS: Prospective case series of eight consecutive patients with adult-onset foveomacular vitelliform dystrophy with at least one eye in the pseudohypopyon stage (a total of 14 eyes). Patients were assessed with spectral domain OCT, flourescein angiography, and OCTA...
February 14, 2017: Retina
https://www.readbyqxmd.com/read/28187978/optical-coherence-tomography-examination-of-the-retinal-pigment-epithelium-in-best-vitelliform-macular-dystrophy
#2
Cynthia X Qian, Dionisio Charran, Cameron R Strong, Timothy J Steffens, Thiran Jayasundera, John R Heckenlively
PURPOSE: To describe the anatomic changes and natural history of vitelliform lesions in Best vitelliform macular dystrophy (BVMD) using spectral-domain optical coherence tomography (OCT). DESIGN: Prospective comparative case series. PARTICIPANTS: Twenty patients (40 eyes) with molecular confirmation of mutation in the BEST1 gene and 20 age-matched controls were included. METHODS: Color fundus photographs, fundus autofluorescence, and spectral-domain OCT were obtained, and these findings were compared between the 2 groups...
February 7, 2017: Ophthalmology
https://www.readbyqxmd.com/read/28153808/bestrophin-1-and-retinal-disease
#3
REVIEW
Adiv A Johnson, Karina E Guziewicz, C Justin Lee, Ravi C Kalathur, Jose S Pulido, Lihua Y Marmorstein, Alan D Marmorstein
Mutations in the gene BEST1 are causally associated with as many as five clinically distinct retinal degenerative diseases, which are collectively referred to as the "bestrophinopathies". These five associated diseases are: Best vitelliform macular dystrophy, autosomal recessive bestrophinopathy, adult-onset vitelliform macular dystrophy, autosomal dominant vitreoretinochoroidopathy, and retinitis pigmentosa. The most common of these is Best vitelliform macular dystrophy. Bestrophin 1 (Best1), the protein encoded by the gene BEST1, has been the subject of a great deal of research since it was first identified nearly two decades ago...
January 30, 2017: Progress in Retinal and Eye Research
https://www.readbyqxmd.com/read/28153442/histologic-and-optical-coherence-tomographic-correlates-in-drusenoid-pigment-epithelium-detachment-in-age-related-macular-degeneration
#4
Chandrakumar Balaratnasingam, Jeffrey D Messinger, Kenneth R Sloan, Lawrence A Yannuzzi, K Bailey Freund, Christine A Curcio
PURPOSE: Drusenoid pigment epithelium detachment (DPED) is a known precursor to geographic atrophy in age-related macular degeneration (AMD). We sought histologic correlates for spectral-domain (SD) optical coherence tomography (OCT) signatures in DPED and determined the frequency and origin of these OCT signatures in a clinical cohort of DPED eyes. DESIGN: Laboratory imaging and histologic comparison, and retrospective, observational cohort study. PARTICIPANTS: Four donor eyes with histopathologic diagnosis of AMD (2 with nonneovascular DPED and 2 with neovascular pigment epithelium detachment [PED]) and 49 eyes of 33 clinic patients with nonneovascular DPED more than 2 mm in diameter...
January 30, 2017: Ophthalmology
https://www.readbyqxmd.com/read/28145974/quantitative-analysis-of-optical-coherence-tomography-angiography-in-adult-onset-foveomacular-vitelliform-dystrophy
#5
Maurizio Battaglia Parodi, Alessandro Rabiolo, Maria Vittoria Cicinelli, Pierluigi Iacono, Francesco Romano, Francesco Bandello
PURPOSE: To quantify the foveal avascular zone area at superficial capillary plexus (SCP) and deep capillary plexus (DCP) and the global, parafoveal, and perifoveal vessel densities at SCP, DCP, and choriocapillaris using optical coherence tomography angiography in patients affected by adult-onset foveomacular vitelliform dystrophy (AOFVD). METHODS: Twenty eyes of 20 consecutive patients (10 females, 50%) with AOFVD and 20 eyes of 20 healthy controls presenting at the Department of Ophthalmology of San Raffaele Hospital, Milan, Italy were enrolled...
January 31, 2017: Retina
https://www.readbyqxmd.com/read/28111324/bestrophinopathy-an-rpe-photoreceptor-interface-disease
#6
REVIEW
Karina E Guziewicz, Divya Sinha, Néstor M Gómez, Kathryn Zorych, Emily V Dutrow, Anuradha Dhingra, Robert F Mullins, Edwin M Stone, David M Gamm, Kathleen Boesze-Battaglia, Gustavo D Aguirre
Bestrophinopathies, one of the most common forms of inherited macular degenerations, are caused by mutations in the BEST1 gene expressed in the retinal pigment epithelium (RPE). Both human and canine BEST1-linked maculopathies are characterized by abnormal accumulation of autofluorescent material within RPE cells and bilateral macular or multifocal lesions; however, the specific mechanism leading to the formation of these lesions remains unclear. We now provide an overview of the current state of knowledge on the molecular pathology of bestrophinopathies, and explore factors promoting formation of RPE-neuroretinal separations, using the first spontaneous animal model of BEST1-associated retinopathies, canine Best (cBest)...
January 19, 2017: Progress in Retinal and Eye Research
https://www.readbyqxmd.com/read/28005720/fundus-autofluorescence-in-rubella-retinopathy-correlation-with-photoreceptor-structure-and-function
#7
Danuta M Bukowska, Sue Ling Wan, Avenell L Chew, Enid Chelva, Ivy Tang, David A Mackey, Fred K Chen
PURPOSE: To illustrate altered fundus autofluorescence in rubella retinopathy and to investigate their relationships with photoreceptor structure and function using multimodal imaging. METHODS: The authors report four cases of rubella retinopathy aged 8, 33, 42, and 50 years. All patients had dilated clinical fundus examination; wide-field color photography; blue, green, and near-infrared autofluorescence imaging and spectral domain optical coherence tomography...
January 2017: Retina
https://www.readbyqxmd.com/read/27984836/-do-we-still-need-electrophysiology-in-ophthalmology
#8
H Tegetmeyer
Electrophysiological methods in clinical ophthalmology include the full-field electroretinogram (ERG) for assessment of outer and middle retinal layers, pattern ERG (PERG) for assessment of ganglion cell function, the electrooculogram (EOG) for assessment of retinal pigment epithelium function, as well as visual evoked potentials (VEP) for assessment of the visual pathway, including the optic nerve and visual cortex. Multifocal recording techniques for ERG and VEP are used for tests within selected areas of the visual field...
December 2016: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/27977838/multimodal-imaging-features-in-acute-exudative-paraneoplastic-polymorphous-vitelliform-maculopathy
#9
Daniel Q Li, John Golding, Carl Glittenberg, Netan Choudhry
An 85-year-old woman with stage IV breast cancer was referred for gradually progressive blurred vision. Dilated fundus examination revealed unifocal, yellow, round vitelliform lesions in the macular region of both eyes. The diagnosis of acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM) was confirmed with swept-source optical coherence tomography (SS-OCT), fundus autofluorescence, and fluorescein angiography. SS-OCT angiography revealed normal vascular findings in both eyes. Multimodal imaging is useful in the diagnosis and monitoring of AEPPVM and may further the understanding of its pathophysiology...
December 1, 2016: Ophthalmic Surgery, Lasers & Imaging Retina
https://www.readbyqxmd.com/read/27902541/central-serous-chorioretinopathylike-mimicking-multifocal-vitelliform-macular-dystrophy-an-ocular-side-effect-of-mitogen-extracellular-signal-regulated-kinase-inhibitors
#10
Chiara Giuffrè, Elisabetta Miserocchi, Giulio Modorati, Adriano Carnevali, Alessandro Marchese, Lea Querques, Giuseppe Querques, Francesco Bandello
PURPOSE: To describe a case of multiple detachments of the neurosensory retina mimicking multifocal vitelliform macular dystrophy after chemotherapy with mitogen/extracellular signal-regulated kinase inhibitor for metastatic ovarian cancer. METHODS: Case report. RESULTS: A 38-year-old woman presented to our clinic for eye examination before the initiation of chemotherapy with trametinib. One month after starting treatment, the patient complained of vision loss and metamorphopsia in both eyes...
November 29, 2016: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/27867022/choroidal-neovascularization-secondary-to-best-vitelliform-macular-dystrophy-detected-by-optical-coherence-tomography-angiography
#11
Rida Shahzad, Muhammad Abdul Rehman Siddiqui
Best vitelliform macular dystrophy (VMD) is a hereditary condition that causes childhood-onset macular dystrophy. In rare cases, VMD is accompanied by formation of a choroidal neovascular membrane (CNVM). We report a case of CNVM secondary to Best VMD in a 10-year-old boy that was detected using optical coherence tomography angiography, a new noninvasive diagnostic modality that detects motion contrast in repeated B-scans to identify blood flow within vessels.
November 17, 2016: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/27847623/choroidal-thickness-using-edi-oct-in-adult-onset-vitelliform-macular-dystrophy
#12
Renato Menezes Palácios, Thaís Sousa Mendes, Ronaldo Yuiti Sano, Davi Chen Wu, Teruo Aihara, Roberta Pereira de Almeida Manzano
BACKGROUND: To compare choroidal thickness in patients with adult-onset foveomacular vitelliform dystrophy (AOFVD) with healthy subjects and to correlate choroidal thickness with age, gender and spherical equivalent. METHODS: A prospective, observational study of 37 eyes (15 eyes in AOFVD group and 22 eyes in control group) was conducted. Images were acquired by enhanced depth imaging optical coherence tomography (EDI-OCT). Choroidal thickness measurements were performed in the subfoveal region and at 500, 1000 and 1500 µm intervals from the foveal center to nasal and to temporal regions for subsequent averaging of values...
2016: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/27775230/clinical-and-genetic-heterogeneity-in-slovenian-patients-with-best-disease
#13
Damjan Glavač, Martina Jarc-Vidmar, Katarina Vrabec, Metka Ravnik-Glavač, Ana Fakin, Marko Hawlina
PURPOSE: To determine the spectrum of BEST1 mutations and to study the phenotype in Slovenian families with Best vitelliform macular dystrophy (BVMD) to identify genotype-phenotype correlations. METHODS: Twenty patients from five families underwent the ophthalmological examination including electrooculogram (EOG; N = 17), fundus autofluorescence imaging (N = 16) and optical coherence tomography (N = 14). Mutational screening was performed by direct DNA sequencing of the BEST1 gene...
December 2016: Acta Ophthalmologica
https://www.readbyqxmd.com/read/27760262/associations-between-retinal-pigment-epithelium-and-drusen-volume-changes-during-the-lifecycle-of-large-drusenoid-pigment-epithelial-detachments
#14
Chandrakumar Balaratnasingam, Lawrence A Yannuzzi, Christine A Curcio, William H Morgan, Giuseppe Querques, Vittorio Capuano, Eric Souied, Jesse Jung, K Bailey Freund
Purpose: Drusenoid pigment epithelial detachments (PEDs) are a defined path to atrophy in age-related macular degeneration (AMD). We analyzed the relationships between retinal pigment epithelium (RPE) and drusen volume changes during the PED lifecycle, using spectral-domain optical coherence tomography (SD-OCT). Methods: Twenty-one cases of drusenoid PED tracked using SD-OCT through periods of growth and collapse were evaluated. Volumetric calculations and piece-wise linear regression analysis were used to determine the breakpoint between growth and collapse...
October 1, 2016: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/27716122/atypical-presentation-of-primary-intraocular-lymphoma
#15
Koji Komatsu, Tsutomu Sakai, Toshikatsu Kaburaki, Hideki Tsuji, Hiroshi Tsuneoka
BACKGROUND: In 2014, Pang et al. reported three cases with vitelliform submaculopathy as a preceding lesion of primary intraocular lymphoma (PIOL). Here, we report a case with an atypical presentation of PIOL who initially presented with vitelliform submaculopathy, vitreous haze and preripheral retinal focus. CASE PRESENTATION: A 73-year-old female initially visited another hospital with a chief complaint of acute reduced vision in the right eye. Funduscopic examination of the right eye showed a yellowish retinal lesion at the fovea with vitreous haze and retinal foci scattered in the peripheral region...
October 3, 2016: BMC Ophthalmology
https://www.readbyqxmd.com/read/27685498/a-case-of-cone-dystrophy-associated-with-choroidal-neovascularization
#16
Gokhan Gulkilik, Sevil Karaman Erdur, Mustafa Eliacik, Mahmut Odabasi, Mustafa Ozsutcu, Goktug Demirci, Mehmet Selim Kocabora
PURPOSE: To report a case of choroidal neovascularization (CNV) in a patient with cone dystrophy (CD). METHODS: Case report. RESULTS: A 20-year-old woman presented with diminished vision in her right eye. Fundus examination showed perifoveal retinal pigment epithelial changes and retinal hemorrhage consistent with subretinal CNV in the right eye, and mild retinal pigment epithelial changes with a dull foveal reflex in the left eye. Optical coherence tomography analysis and fundus fluorescein angiography also confirmed the subfoveal CNV in the right eye...
September 28, 2016: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/27668931/torpedo-maculopathy-presenting-with-a-vitelliform-lesion
#17
Rosa Dolz-Marco, Norman A Saffra, K Bailey Freund
No abstract text is available yet for this article.
February 2017: Retina
https://www.readbyqxmd.com/read/27640006/clinical-characteristics-choroidal-neovascularization-and-predictors-of-visual-outcomes-in-acquired-vitelliform-lesions
#18
Chandrakumar Balaratnasingam, Quan V Hoang, Maiko Inoue, Christine A Curcio, Rosa Dolz-Marco, Nicolas A Yannuzzi, Elona Dhrami-Gavazi, Lawrence A Yannuzzi, K Bailey Freund
PURPOSE: To quantify the temporal properties of the acquired vitelliform lesion (AVL) lifecycle, define the clinical characteristics of choroidal neovascularization (NV) in this setting and determine the predictors of long-term visual outcomes. DESIGN: Retrospective cohort study METHODS: Clinical and imaging data from 199 eyes of 124 consecutive patients with AVLs associated with age-related macular degeneration (AMD) and adult-onset foveomacular vitelliform dystrophy (AOFVD) were analyzed...
September 14, 2016: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/27558081/bilateral-choroidal-neovascularisation-associated-with-adult-onset-foveomacular-vitelliform-dystrophy
#19
Cheryl P Au, Vivek B Pandya, Paul Mitchell
No abstract text is available yet for this article.
January 2017: Clinical & Experimental Optometry: Journal of the Australian Optometrical Association
https://www.readbyqxmd.com/read/27533784/focal-choroidal-excavation-in-retinal-dystrophies
#20
Imoro Zeba Braimah, Shruthi Rapole, Sunila Dumpala, Jay Chhablani
AIM: To investigate the presence of focal choroidal excavation (FCE) in patients with retinitis pigmentosa (RP), Stargardt's disease (STGD), and Best disease in the Indian population. METHODS: This retrospective consecutive case series included 309 eyes of 157 patients with RP (183 eyes), STGD (93 eyes), and Best disease (33 eyes) with good-quality, enhanced-depth spectral domain optical coherence tomography scans. Comprehensive ophthalmic examination data were collected...
August 17, 2016: Seminars in Ophthalmology
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