Endovascular treatment of the patient with vascular type of Ehlers–Danlos syndrome with bilateral dissection stenoses and aneurysms in V3- andV4- segments of vertebral arteries

Abstract

A case of treatment of a patient with type 4 of Ehlers–Danlos syndrome and bilateral dissection stenoses and aneurysms in V3and V4-segments of vertebral arteries is described. Also another vascular anomaly was verified: the aberrant right subclavian artery (arteria lusoria). Сerebral angiography with dynamic rotation of the neck revealed a bow hunter's syndrom, which consists in the restriction of blood flow in the right vertebral artery when the head is turned to the left. Stage-by-stage endovascular reconstruction of both vertebral arteries was performed. Firstly, LVIS stent was implanted in the left vertebral artery on the level of the dissection lesion. Then, the aneurysm of the left vertebral artery was embolized by detachable coils. Balloon angioplasty was performed in the stented segment with the compliance balloon-catheter Scepter. On the series of angiograms: the aneurysm is totally excluded, the stenosis of the artery is eliminated. After 1 month, the second stage of endovascular treatment in the same volume in the right vertebral artery was performed. At attempts of a hemostasis with the vascular closure device Аngioseal were unsuccessful in both operations. Hemostasis was successfully performed by manual compression of the puncture site. A mutation in the gene COL3A1, responsible for the synthesis of procollagen III type, was confirmed by Ehlers–Danlos syndrome of type 4 (vascular type) at the subsequent genotyping of the patient. The patient's neurological symptoms regressed to a great extent. Endovascular methods can be effective in the treatment of vertebral arteries dissection lesions in patients with vascular type of Ehlers–Danlos syndrome.