428 Emory Eye Center

Atlanta, GA, United States

428 Emory Eye Center

Atlanta, GA, United States
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Zhang Q.,Emory University | Zhang Q.,Central South University | Small K.W.,Molecular Insight LLC | Grossniklaus H.E.,Emory University | Grossniklaus H.E.,428 Emory Eye Center
Graefe's Archive for Clinical and Experimental Ophthalmology | Year: 2011

Purpose: To correlate the clinical and histopathologic features of Best vitelliform macular dystrophy (BVMD). Methods: Two eyes were obtained postmortem from a patient with BVMD. The patient's clinical information was reviewed. Series sections of the globes were performed and sequentially stained with hematoxylin-eosin, periodic acid-Schiff or Masson trichrome. A section of the left macula was submitted for electron microscopic processing. Histopathologic findings were reconstructed in a scaled two-dimensional map and compared with fundus photography, fundus autofluorescence (FAF), fundus fluorescein angiography (FFA) and optical coherence tomography (OCT) images. Results: The macular lesion of the right eye was identified as a well-demarcated region with pigment, elevated submacular yellow material and subretinal fluid. This corresponded histopathologically to a well-circumscribed area of RPE hyperplasia, accumulation of lipofuscin in the RPE, deposition of granular material in the photoreceptors, macrophages and drusen. The left eye displayed a 1 disc diameter chorioretinal scar with surrounding shallow fluid and submacular pigment. This corresponded to RPE changes and a fibrocellular proliferation in the choriocapillaris. Conclusion: Histopathologic mapping revealed retinal edema, RPE abnormalities, drusen and a chorioretinal scar in BVMD that correlated with the fundus, FFA, FAF and OCT findings. © 2010 Springer-Verlag.


Grossniklaus H.E.,428 Emory Eye Center
American Journal of Ophthalmology | Year: 2014

Purpose: To review the progress made in understanding the genetic basis, molecular pathology, and treatment of retinoblastoma since the previous Jackson lecture on the topic was published 50 years ago.Design: Perspective based on personal experience and the literature.Methods: The literature regarding retinoblastoma was reviewed since 1963. Advances in understanding the biology and treatment of retinoblastoma provided context through the authors clinical, pathologic, and research experiences.Results: Retinoblastoma was first identified in the 1500s and defined as a unique clinicopathologic entity in 1809. Until the mid-1900s, knowledge advanced sporadically, with technological developments of ophthalmoscopy and light microscopy, and with the introduction of surgical enucleation, chemotherapy, and radiation therapy. During the last 50 years, research and treatment have progressed at an unprecedented rate owing to innovations in molecular biology and the development of targeted therapies. During this time period, the retinoblastoma gene was discovered; techniques for genetic testing for retinoblastoma were developed; and plaque brachytherapy, chemoreduction, intra-arterial chemotherapy, and intraocular injections of chemotherapeutic agents were successfully introduced.Conclusion: Nearly all patients with retinoblastoma in developed countries can now be cured of their primary cancer-a remarkable achievement for a childhood cancer that once was uniformly fatal. Much of this success is owed to deciphering the role of the Rb gene, and the benefits of targeted therapies, such as chemoreduction with consolidation as well as intra-arterial and intravitreal chemotherapies. Going forward, the main challenge will be ensuring that access to care is available for all children, particularly those in developing countries. © 2014 by Elsevier Inc. All rights reserved.

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