"Phacing" a New Cause of Carotid Artery Dissection

Cervical arterial dissection (CAD) is a frequent and preventable cause of ischemic stroke in young patients. Several arguments suggest that genetic and developmental disorders could play an important role as part of a multifactorial predisposition of sporadic CAD.We present 2 cases of young patients with CAD in association with cutaneous lesions and nonatherosclerotic multivessel arteriopathy.

Our first patient was a 17-year-old white girl with sudden onset of weakness in her right upper limb. A magnetic resonance angiogram showed a severe stenosis along the extracranial and intracranial segments of the left internal carotid artery and the left middle cerebral artery. A complete ultrasound study confirmed the stenoses with characteristics suggestive of dissection.Our second patient was a 7-year old white girl with a past history of left middle cerebral artery ischemic stroke. During the follow-up, an ultrasonographical examination discovered an asymptomatic dissection of the right internal carotid artery.Both patients reported a history of large unilateral hemangiomas; in the first case a coloboma of the left optic disc and an aortic aneurysm were also present. These findings were suggestive of PHACE-a neurocutaneous developmental syndrome associated with constitutional arteriopathy of the major cerebral vessels.

Noteworthy, among vascular abnormalities of PHACE, CADs have never been reported before. Our 2 cases suggest that CAD is an underecognized cerebrovascular manifestation of PHACE and it should be searched for in these patients. Ultrasound, being noninvasive and portable, is a useful tool for the assessment and follow-up of these patients.