Ocular ischemic syndrome secondary to cerebral aneurysms

Abstract

Purpose: To describe the clinical findings in an 11-year-old male with a history of hemifacial microsomia presenting with ocular ischemic syndrome secondary to large cerebral aneurysms.

Observations: An 11-year-old male with a history of hemifacial microsomia presented to the Bascom Palmer Eye Institute Emergency Department complaining of nausea, diarrhea, headache, and decreased vision in the left eye. Visual acuity was light perception in the left eye and intraocular pressure was within normal limits. Gonioscopy revealed the presence of diffuse neovascularization of the angle. Posterior segment examination revealed mild vitreous hemorrhage, optic disc pallor, preretinal hemorrhage, generalized arteriolar narrowing, retinal microaneurysms, and abnormal arteriovenous communications with branching retinal vessels. Fluorescein angiography demonstrated patchy and delayed choroidal filling, a prolonged venous filling time, arteriolar attenuation, and vascular staining consistent with ocular ischemic syndrome. Magnetic resonance angiography was obtained which revealed large left internal carotid and anterior cerebral artery aneurysms. The patient underwent successful cerebral revascularization via bypass, ligation, clipping, and coiling procedures. At postoperative year 1, there was no evidence of ocular neovascularization and visual acuity remained light perception.

Conclusion and importance: Ocular ischemic syndrome is uncommon in children but may occur with any cause of ocular hypoperfusion. Hemifacial microsomia is a rare congenital disorder of craniofacial development caused by a vascular event in utero affecting the first and second branchial arches. This case demonstrates a rare cause of ocular ischemic syndrome and illustrates the potential for the development of clinically significant vascular abnormalities in patients with disorders of craniofacial development.

Keywords: Cerebral aneurysm; Hemifacial microsomia; Neovascularization; Ocular ischemic syndrome.

Fig. 1

Magnetic resonance angiography at diagnosis Magnetic resonance angiography of the brain of an 11-year-old male with a history of hemifacial microsomia and poor visual acuity in the left eye with ocular neovascularization demonstrating large left internal carotid and anterior cerebral artery aneurysms (arrows).

Fig. 2

Ophthalmic imaging of the left eye An 11-year-old male with ocular ischemic syndrome due to cerebral aneurysms. Fundus photography (A) demonstrates resolving vitreous hemorrhage, optic disc pallor with a small optic cup, generalized arteriolar narrowing, a fibrotic aneurysm in the supratemporal periphery (asterisk) with adjacent dot-blot hemorrhages, microaneurysms in the macula, and arteriovenous communications with abnormal branching vessels (arrows). Early phase fluorescein angiography at 29 seconds (B) demonstrates patchy and delayed choroidal filling, few small microaneurysms in the macula, arteriolar narrowing, and delayed venous filling (arrows). Late phase fluorescein angiography at 11 minutes (C) demonstrates peripheral branching vessels with abnormal arteriovenous communications (arrows) and associated staining. Optical coherence tomography (D) demonstrates moderate diffuse retinal atrophy, severe ganglion cell layer thinning, and mild vitreous opacities.

Fig. 3

Fundus photography of the left eye at postoperative year 1 Fundus photography 1 year after cerebral revascularization demonstrating resolution of vitreous hemorrhage with persistence of optic disc pallor and central and peripheral vascular abnormalities.

Fig. 4

Cerebral angiogram at postoperative year 1 Cerebral angiogram at postoperative year 1 demonstrating bypass patency and no residual filling of the left internal carotid or anterior cerebral artery aneurysms. Coiling can be seen in the A1 segment of the anterior cerebral artery aneurysm (arrow).