Radial Hemorrhage in Henle Layer in Macular Telangiectasia Type 2

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.