Ehlers-Danlos Syndrome Presenting as Severe Headache in a Young Adult

A 27-year-old male with a positive family history of abdominal aortic aneurysm presented to his primary care physician and ultimately to the emergency department with the worst headache of his life and hypertension. An aneurysm of the right internal carotid artery was noted on CT and MRI. Upon further imaging and analysis, multiple vascular abnormalities were found, including possible dissection or pseudoaneurysm and multifocal narrowing and dilatation of the arteries of the head and neck. The patient underwent genetic testing revealing Ehlers-Danlos, type IV.