Hemorrhagic Retinopathy and Optic Nerve Sheath Hemorrhage Associated With Fatal Subarachnoid Hemorrhage from a Ruptured Intracranial Aneurysm Due to Segmental Fibromuscular Dysplasia

Abstract

Fibromuscular dysplasia is an idiopathic, nonatheromatous, and noninflammatory arterial disease that most commonly affects the renal and carotid arteries. We report a child with subarachnoid and ocular hemorrhage associated with an aneurysm due to fibromuscular dysplasia. Computed tomography following a witnessed collapse revealed diffuse subarachnoid hemorrhage and severe cerebral edema. An autopsy confirmed the radiographic findings and detected bilateral retinal hemorrhages, optic nerve sheath hemorrhages, and a ruptured saccular aneurysm due to focal fibromuscular dysplasia involving the intracranial right vertebral artery. This case documents a fatal subarachnoid hemorrhage in a child with an intracranial saccular aneurysm caused by fibromuscular dysplasia. The associated retinal hemorrhages are easily detected by postmortem monocular indirect ophthalmoscopy.

Ocular ischemic manifestations in fibromuscular dysplasia

“…Additionally, hemorrhagic complications such as optic nerve sheath and diffuse retinal hemorrhages can occur secondary to subarachnoid hemorrhage from a ruptured intracranial aneurysm. 18 When arterial input of the eye is compromised for a long period, chronic ischemic manifestations occur. This chronic ischemic retinopathy includes arteriovenous anastomoses and neovascularization, which can produce complications such as retinal hemorrhages, massive vitreous hemorrhage or tractional retinal detachment.…”

“…Additionally, hemorrhagic complications such as optic nerve sheath and diffuse retinal hemorrhages can occur secondary to subarachnoid hemorrhage from a ruptured intracranial aneurysm. 18…”