OSLI Retina

May 2019

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322 Ophthalmic Surgery, Lasers & Imaging Retina | Healio.com/OSLIRetina Macular Microvascular Findings in Familial Exudative Vitreoretinopathy on Optical Coherence Tomography Angiography S. Tammy Hsu, MD; Avni P. Finn, MD, MBA; Xi Chen, MD, PhD; Hoan T. Ngo; Robert J. House, MD; Cynthia A. Toth, MD; Lejla Vajzovic, MD BACKGROUND AND OBJECTIVE: To describe depth- resolved macular microvasculature abnormalities in patients with familial exudative vitreoretinopa- thy (FEVR) using optical coherence tomography angiography (OCTA). PATIENTS AND METHODS: Twenty-two eyes (11 eyes of six patients with FEVR and 11 control eyes) were imaged with OCTA. Graders qualitatively analyzed the OCTA images of the superficial and deep vas- cular complexes for abnormal vascular features and compared to fluorescein angiography (FA). RESULTS: Seven of 11 eyes with FEVR displayed ab- normal macular vascular findings. Abnormalities in the superficial vascular complex included dilation, disorganization, straightening, heterogeneous vessel density, and curls/loops. In the deep vascular com- plex, abnormalities included areas of decreased den- sity, disorganization, curls/loops, and "end bulbs." Except for dragging and straightening of the vessels, none of these macular features were visible on FA. CONCLUSION: OCTA revealed marked macular ab- normalities in eyes with FEVR that have not been previously observed with FA alone, suggesting this is more than a disease of the retinal periphery and involves macular and deep retinal vasculature ab- normalities. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:322-329.] INTRODUCTION Familial exudative vitreoretinopathy (FEVR) is a rare, inherited disorder of retinal vascular devel- opment leading to incomplete and anomalous vas- cularization of the peripheral retina. 1,2 The disease is thought to be caused by genetic mutations in the Wnt-signaling pathway that is necessary for retinal angiogenesis. 3 Genetic mutations in Wnt pathway genes NDP, 4 FZD4, 5,6 LRP5, 7 TSPAN12, 8 ZNF408, 9 CTNNB1, 10 and KIF11 11 have been implicated in the From the Department of Ophthalmology, Duke University Medical Center, Durham, North Carolina (STH, APF, XC, RJH, CAT, LV); the Department of Biomedical Engineering, Duke University, Durham, North Carolina (CAT); the Biomedical Engineering Department, International University, Vietnam National University – Ho Chi Minh City (VNU-HCMC), Ho Chi Minh City, Vietnam (HTN); and the Department of Ophthalmology, Medical Univer- sity of South Carolina, Charleston, South Carolina (RJH). Originally submitted June 15, 2018. Revision received October 7, 2018. Accepted for publication January 3, 2018. Presented at the American Academy of Ophthalmology 2018 Annual Meeting, October 27-30, 2018, Chicago. Supported by the International Association of Government Officials Fund (LV), Heidelberg Engineering (LV, STH), a Research to Prevent Blindness un- restricted grant to Duke Eye Center, Research to Prevent Blindness Career Development Award and NEI K23EY028227 (XC), a Lions Duke Pediatric Eye Research Endowment (RH), NIH grant No. R01EY25009 (CAT), and NIH grant No. P30EY005722. The funding organizations had no role in study design, collection, analysis and interpretation of data, writing the report, or the decision to submit the report for publication. Research equipment (Spectralis tabletop and Flex module) was provided by Heidelberg Engineering. Aside from agreement to submit the report for publication and corrections regarding terminology, the manufacturer had no role in study design, collection, analysis and interpretation of data, or writing the report. Dr. Toth has received royalties for patents from Alcon outside the submit- ted work. Dr. Chen has received personal fees from Allergan outside the submitted work. Dr. House has received grants from Knights Templar Eye Foundation. Dr. Vajzovic has received grants and personal fees from Alcon; personal fees from DORC, Genentech, Janssen Pharmaceuticals, and Alimera Sciences; and grants from Roche and Second Sight outside the submitted work. The remaining authors report no relevant financial disclosures. The authors would like to thank their pediatric ophthalmology colleagues, Sharon F. Freedman, MD, and Nathan Cheung, DO, for their help in subject recruitment, as well as their ophthalmic photographer, Michael P. Kelly, for assisting with image capturing. Address correspondence to Lejla Vajzovic, MD, 2351 Erwin Road, Durham, NC 27705; email: Lejla.Vajzovic@duke.edu. doi: 10.3928/23258160-20190503-11 ■ B R I E F R E P O R T ■

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