Bilateral Vertebral Artery Dissection and Unilateral Carotid Artery Dissection in Case of Ehlers-Danlos Syndrome Type IV

Abstract

Ehlers-Danlos syndrome (EDS) is a rare genetic connective tissue disorders, but the vascular type (type IV) typically poses the greatest risk to patients. We report a case of multiple cranial artery dissection, which was successfully treated with carotid artery stenting. A 50-year-old woman presented with recurrent severe headaches caused by bilateral vertebral artery dissections that were treated conservatively at our hospital. However, she developed right cervical pain and dizziness at 3 days after admission, and a magnetic resonance angiogram revealed dissection of the right internal carotid artery. Because the dissected portion of the artery had narrowed, a stent was placed. The pearl and string formations in the bilateral vertebral arteries then improved spontaneously. Subsequently, the patient was diagnosed with EDS type IV via a skin biopsy, and review of her family history revealed that multiple family members had suffered from subarachnoid hemorrhages. No neurologic deficits were observed, and the patient was discharged without further events at 30 days after admission. It is extremely rare for multiple artery dissections to occur at the same time. EDS type IV should be considered as an important differential diagnosis in similar cases, even in adult patients without a known history of connective tissue disease or vascular complications.