High risk and low prevalence diseases: Spontaneous cervical artery dissection

Spontaneous cervical artery dissection (sCAD) is a serious condition that carries with it a high rate of morbidity and mortality. This review highlights the pearls and pitfalls of sCAD, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. sCAD is a condition affecting the carotid or vertebral arteries and occurs as a result of injury and compromise to the arterial wall layers. The dissection most commonly affects the extracranial vessels but may extend intracranially, resulting in subarachnoid hemorrhage. Patients typically present with symptoms due to compression of local structures, and the presentation depends on the vessel affected. The most common symptom is headache and/or neck pain. Signs and symptoms of ischemia may occur, including transient ischemic attack and stroke. There are a variety of risk factors for sCAD, including underlying connective tissue or vascular disorders, and there may be an inciting event involving minimal trauma to the head or neck. Diagnosis includes imaging, most commonly computed tomography angiography of the head and neck. Ultrasound can diagnose sCAD but should not be used to exclude the condition. Treatment includes specialist consultation (neurology and vascular specialist), consideration of thrombolysis in appropriate patients, symptomatic management, and administration of antithrombotic medications. An understanding of sCAD can assist emergency clinicians in diagnosing and managing this potentially deadly disease.