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

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.