Ophthalmic Surgery, Lasers and Imaging Retina

Images in Ophthalmology 

Retinal Artery Macroaneurysm Associated With Retinal Hemangioblastoma

Nora C. Elson, MS; Ferdinand Rodriguez, MD; Maura Di Nicola, MD; Basil K. Williams Jr., MD

Abstract

A 34-year-old Caucasian woman with a past medical history of hypertension presented with acute floaters in the right eye (OD) for 1 week. Best-corrected visual acuity (VA) was 20/20 in both eyes.

Fundus examination OD (Figure 1) revealed a retinal hemangioblastoma (RH) in the temporal midperiphery with a prominent feeding artery and draining vein. A retinal arterial microaneurysm (RAM) was noted within the feeding artery, and subretinal hemorrhage with a cuff of fluid was present. Fundus autofluorescence (Figure 2) showed hypoautofluorescence in the area of the RH, RAM, and subretinal hemorrhage. A hyperautofluorescent ring surrounding the hemorrhage suggested shallow subretinal fluid. Early phase fluorescein angiography (Figure 3) demonstrated appropriate arterial and venous filling and hyperfluorescence of the RH.

Figure 1.

Ultra-widefield fundus photograph of the right eye revealing a midperipheral retinal hemangioblastoma with a prominent feeding artery and draining vein. A retinal arterial microaneurysm arises from the feeding artery, producing subretinal hemorrhage with a surrounding cuff of subretinal fluid, and a small amount of intraretinal and vitreous hemorrhage along the inferior arcade.

Figure 2.

Fundus autofluorescence demonstrates hypoautofluorescence corresponding to the retinal hemangioblastoma, retinal arterial microaneurysm, and intraretinal and subretinal hemorrhage with a ring of hyperautofluorescence around hemorrhage, suggestive of subretinal fluid.

Figure 3.

Early phase fluorescein angiography shows hyperfluorescence of the retinal hemangioblastoma and retinal arterial microaneurysm in the dilated feeding artery with blockage from the underlying hemorrhage.

The RAM appeared hyperfluorescent, whereas the subretinal hemorrhage blocked. There was no evidence of leakage on late images. Optical coherence tomography (OCT) (Figure 4) through the RAM revealed an inner retinal structure with a central lumen and hyperreflective border. Mild outer retinal exudation was also noted. Fovea was normal on OCT. Fundus examination of the left eye was normal.

Figure 4.

Optical coherence tomography at the level of the microaneurysm demonstrates the dilated vascular lumen with hyperreflective borders (white arrow), posterior shadowing, adjacent subretinal hemorrhage (asterisk), and outer retinal exudation (arrowhead).

Genetic work-up was negative for von Hippel-Lindau disease. Treatment of the hemangioblastoma was offered, but the patient preferred observation. VA remained stable, and better systemic hypertension control was recommended.

[Ophthalmic Surg Lasers Imaging Retina. 2020;51:365–366.]…

A 34-year-old Caucasian woman with a past medical history of hypertension presented with acute floaters in the right eye (OD) for 1 week. Best-corrected visual acuity (VA) was 20/20 in both eyes.

Fundus examination OD (Figure 1) revealed a retinal hemangioblastoma (RH) in the temporal midperiphery with a prominent feeding artery and draining vein. A retinal arterial microaneurysm (RAM) was noted within the feeding artery, and subretinal hemorrhage with a cuff of fluid was present. Fundus autofluorescence (Figure 2) showed hypoautofluorescence in the area of the RH, RAM, and subretinal hemorrhage. A hyperautofluorescent ring surrounding the hemorrhage suggested shallow subretinal fluid. Early phase fluorescein angiography (Figure 3) demonstrated appropriate arterial and venous filling and hyperfluorescence of the RH.

Ultra-widefield fundus photograph of the right eye revealing a midperipheral retinal hemangioblastoma with a prominent feeding artery and draining vein. A retinal arterial microaneurysm arises from the feeding artery, producing subretinal hemorrhage with a surrounding cuff of subretinal fluid, and a  small amount of intraretinal and vitreous hemorrhage along the inferior arcade.

Figure 1.

Ultra-widefield fundus photograph of the right eye revealing a midperipheral retinal hemangioblastoma with a prominent feeding artery and draining vein. A retinal arterial microaneurysm arises from the feeding artery, producing subretinal hemorrhage with a surrounding cuff of subretinal fluid, and a small amount of intraretinal and vitreous hemorrhage along the inferior arcade.

Fundus autofluorescence demonstrates hypoautofluorescence corresponding to the retinal hemangioblastoma, retinal arterial microaneurysm, and intraretinal and subretinal hemorrhage with a ring of hyperautofluorescence around hemorrhage, suggestive of subretinal fluid.

Figure 2.

Fundus autofluorescence demonstrates hypoautofluorescence corresponding to the retinal hemangioblastoma, retinal arterial microaneurysm, and intraretinal and subretinal hemorrhage with a ring of hyperautofluorescence around hemorrhage, suggestive of subretinal fluid.

Early phase fluorescein angiography shows hyperfluorescence of the retinal hemangioblastoma and retinal arterial microaneurysm in the dilated feeding artery with blockage from the underlying hemorrhage.

Figure 3.

Early phase fluorescein angiography shows hyperfluorescence of the retinal hemangioblastoma and retinal arterial microaneurysm in the dilated feeding artery with blockage from the underlying hemorrhage.

The RAM appeared hyperfluorescent, whereas the subretinal hemorrhage blocked. There was no evidence of leakage on late images. Optical coherence tomography (OCT) (Figure 4) through the RAM revealed an inner retinal structure with a central lumen and hyperreflective border. Mild outer retinal exudation was also noted. Fovea was normal on OCT. Fundus examination of the left eye was normal.

Optical coherence tomography at the level of the microaneurysm demonstrates the dilated vascular lumen with hyperreflective
            borders (white arrow), posterior shadowing, adjacent subretinal hemorrhage (asterisk), and outer retinal exudation (arrowhead).

Figure 4.

Optical coherence tomography at the level of the microaneurysm demonstrates the dilated vascular lumen with hyperreflective borders (white arrow), posterior shadowing, adjacent subretinal hemorrhage (asterisk), and outer retinal exudation (arrowhead).

Genetic work-up was negative for von Hippel-Lindau disease. Treatment of the hemangioblastoma was offered, but the patient preferred observation. VA remained stable, and better systemic hypertension control was recommended.

[Ophthalmic Surg Lasers Imaging Retina. 2020;51:365–366.]

Authors

From Cincinnati Eye Institute, Cincinnati, Ohio (NCE, FR, BKW); and Ocular Oncology Service, Department of Ophthalmology, University of Cincinnati College of Medicine, Cincinnati, Ohio (NCE, MDN, BKW).

The authors report no relevant financial disclosures.

Address correspondence to Basil K. Williams Jr., MD, Ocular Oncology Service, University of Cincinnati College of Medicine, 231 Albert Sabin Way, Suite 5414, Cincinnati, OH 45267; email: basilkwilliams@gmail.com.

Received: February 19, 2020
Accepted: March 30, 2020

10.3928/23258160-20200603-07

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