Adrian Au1, Kirk Hou1, Caroline R Baumal2, David Sarraf1,3. 1. Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California, Los Angeles. 2. New England Eye Center, Tufts University School of Medicine, Boston, Massachusetts. 3. Greater Los Angeles VA Healthcare Center, Los Angeles, California.
Abstract
Importance: Radial hemorrhage in the outer plexiform layer of Henle may be a complication of macular telangiectasia type 2 (MacTel 2) and may occur because of microvascular abnormalities of the deep retinal capillary plexus in the absence of subretinal neovascularization. Objective: To describe the multimodal imaging findings, including cross-sectional and en face optical coherence tomography (OCT), of radial hemorrhage in the outer plexiform layer of Henle, which may be a complication of MacTel 2. Design, Setting, and Participants: This retrospective case series from 2 tertiary referral centers (Stein Eye Institute, Los Angeles, California; New England Eye Center, Boston, Massachusetts) between January 1, 2012, and December 31, 2017, describes 3 patients with MacTel 2 complicated by characteristic radial hemorrhage in the outer plexiform layer of Henle. Main Outcomes and Measures: Color fundus photography, cross-sectional and en face OCT, OCT angiography (OCTA), fundus autofluorescence, and fluorescein angiography. Results: Three male patients presented with sudden vision loss in the right eye. A characteristic radial pattern of hemorrhage was noted with color fundus photography. Cross-sectional and en face OCT and OCTA localized the hemorrhage to the outer plexiform layer of Henle in the absence of subretinal neovascularization. Optical coherence tomography findings consistent with MacTel 2 were identified in the fellow eye in each patient. At the follow-up visit 1 to 2 months after presentation, spontaneous resolution of the hemorrhage was noted in all 3 patients, and OCTA illustrated underlying microvascular abnormalities in the deep retinal capillary plexus in 2 patients. Conclusions and Relevance: This report describes 3 patients with MacTel 2 complicated by characteristic radial hemorrhage in the outer plexiform layer of Henle, which may represent a characteristic finding in MacTel 2 that may develop as a result of microvascular abnormalities of the deep retinal capillary plexus in the absence of subretinal neovascularization.
Importance: Radial hemorrhage in the outer plexiform layer of Henle may be a complication of macular telangiectasia type 2 (MacTel 2) and may occur because of microvascular abnormalities of the deep retinal capillary plexus in the absence of subretinal neovascularization. Objective: To describe the multimodal imaging findings, including cross-sectional and en face optical coherence tomography (OCT), of radial hemorrhage in the outer plexiform layer of Henle, which may be a complication of MacTel 2. Design, Setting, and Participants: This retrospective case series from 2 tertiary referral centers (Stein Eye Institute, Los Angeles, California; New England Eye Center, Boston, Massachusetts) between January 1, 2012, and December 31, 2017, describes 3 patients with MacTel 2 complicated by characteristic radial hemorrhage in the outer plexiform layer of Henle. Main Outcomes and Measures: Color fundus photography, cross-sectional and en face OCT, OCT angiography (OCTA), fundus autofluorescence, and fluorescein angiography. Results: Three male patients presented with sudden vision loss in the right eye. A characteristic radial pattern of hemorrhage was noted with color fundus photography. Cross-sectional and en face OCT and OCTA localized the hemorrhage to the outer plexiform layer of Henle in the absence of subretinal neovascularization. Optical coherence tomography findings consistent with MacTel 2 were identified in the fellow eye in each patient. At the follow-up visit 1 to 2 months after presentation, spontaneous resolution of the hemorrhage was noted in all 3 patients, and OCTA illustrated underlying microvascular abnormalities in the deep retinal capillary plexus in 2 patients. Conclusions and Relevance: This report describes 3 patients with MacTel 2 complicated by characteristic radial hemorrhage in the outer plexiform layer of Henle, which may represent a characteristic finding in MacTel 2 that may develop as a result of microvascular abnormalities of the deep retinal capillary plexus in the absence of subretinal neovascularization.
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