Ocular ischemic syndrome secondary to cerebral aneurysms

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.