Vision-Threatening Complications of Juxtapapillary Retinal Capillary Hemangioma: A Case of Retinal Artery Occlusion

Abstract

BACKGROUND Juxtapapillary retinal capillary hemangioma (JRCH) is a rare vascular hamartoma that, when present, can cause many complications. We report a case of branch retinal artery occlusion (BRAO) in the setting of progressive exophytic JRCH, which, to the best of our knowledge, has not been reported until now. CASE REPORT The patient was 34-year-old woman who presented with visual blurring and visual field defects of the left eye and with no significant medical history. Fundoscopy and other auxiliary examinations revealed a red, elevated mass at the optic disc with surrounding hemorrhage and vascular abnormalities. JRCH was then diagnosed, and she underwent conservative treatment. However, 3 days later, during the follow-up, she developed a worsened best corrected visual acuity (BCVA) and visual field defect. The optical coherence tomography angiography found swelling of the tumor, macular edema, and vascular occlusion of the inferior macula; therefore, secondary BRAO was diagnosed. With emergency treatments, the tumor size reduced during the follow-up; however, the vision field, BCVA, and vascular occlusion were not improved. CONCLUSIONS JRCH are rare benign tumors associated with progressive vision loss due to complications such as macular exudate and retinal detachment. Various treatments, including laser, radiotherapy, and anti-angiogenic intravitreal (anti-VEGF) injections, have been suggested; however, the optimal approach is unclear. JRCH complicated with BRAO has not been reported before. Our report suggests that the tumor was closely related to the retinal arteriovenous system, and BRAO could be a vision-threatening complication of JRCH that deserves more attention.

Figure 1.

(A) Fundoscopy images of the left eye at admission showing trace vitreous opacity and a red, well-circumscribed, elevated mass about 1 disc diameter in size on the inferior part of the disc with a small amount of surrounding intraretinal hemorrhage and overlying dilated and tortuous blood vessels, indicated by white circle. (B) B-scan ultrasound shows scattered points in the vitreous body of the left eye and hyperechoic protuberance on the optic disc surface, indicated by green arrow. (C) Fluorescein angiogram shows filling of the tumor during the arterial phase with late diffuse leakage. In the early phase, the infratemporal retinal artery straddles the tumor and wraps around the infratemporal retinal vein.

Figure 2.

(A) The optical coherence tomography scan shows the lesion to be located within the retina and protruded into the vitreous cavity as well as vitreous hemorrhage. Exudation in the outer nuclear layer from the vascular lesion is extending toward but not involving the fovea, indicated by a yellow star. (B) The sub-structure of the tumor is not clearly displayed on optical coherence tomography angiography. (C) Visual field examination shows that the physiological blind spot is enlarged in the left eye.

Figure 3.

(A) Fundoscopy images of the left eye. Branch retinal artery occlusion is in the setting of progressive exophytic juxtapapillary retinal capillary hemangioma, vitreous hemorrhage in front of the optic disc, swelling of the tumor, and macular edema inferiorly with a cherry red spot. (B) Repeat visual field testing revealed a new defect superiorly. (C, D) Macular edema on optical coherence tomography, tumor body increase in the length, and vascular occlusion of the inferior macula on optical coherence tomography angiography corresponds to the visual field defect, and vitreous hemorrhage had been absorbed, compared with before.

Figure 4.

(A) Fundoscopy images of the left eye. After 10 days of treatment, the tumor body shrank, and retinal and intravitreal hemorrhage were almost absorbed. (B) The superior visual field defect and the vascular occlusion of the inferior macula were not improved. (C, D) The shrinking of the tumor body also could be seen on optical coherence tomography and optical coherence tomography angiography.

Figure 5.

A brief timeline of the case.