Spontaneous Extracranial Vertebral Artery Dissection in a Neurofibromatosis 1 Patient with Bilateral Intrathoracic Spinal Meningoceles around the Scoliosis: Report of an Autopsy Case

The annual incidence of cervical artery dissection is 2.6-3.0 per 100,000 population, and that of vertebral artery dissection, in particular, is 1-1.5 per 100,000 population. The mean age of patients with a cervical artery dissection is 46.3 years, and their male-to-female ratio is equivalent [1,2]. Cervical artery dissection may arise spontaneously or traumatically, even due to trivial events such as a sudden rotation of the neck. In addition, many studies have reported various pathophysiological risk factors for cervical artery dissection, such as connective tissue disorders [2]. Neurofibromatosis 1 (NF1) is a common autosomal dominant disorder (incidence of 1 per 3,000 population) that leads to systemic conditions including neurofibromas, gliomas of the optic nerve, hamartomatous lesions, mental retardation, skeletal defects, and cutaneous hyperpigmented macules (café au lait spots) [3]. Many studies have been reported that NF1 is associated with intrathoracic meningoceles and scoliosis [4,5]. Here, we repor t an autopsy case of massive intrathoracic hemorrhage due to spontaneous vertebral artery dissection in a patient with NF1, who had Korean J Leg Med 2016;40:14-18 http://dx.doi.org/10.7580/kjlm.2016.40.1.14