Vascular complications in Ehlers-Danlos syndrome type IV - Case report

Abstract

Ehlers-Danlos syndrome is the most common inherited connective tissue disease and is a consequence of abnormal collagen synthesis. Type IV of this syndrome, called vascular, is considered to be the most severe. Patients with vascular type are prone to large arteries rupture and have higher likelihood of aneurysms development. We present a case of a patient with type IV Ehlers-Danlos syndrome, who suffered aortic rupture after surgical treatment of coeliac trunk compression syndrome. During the operation, the suturing the prosthesis to aorta was impossible due to the fragility of the aorta wall. Instead, a portion of a Dacron graft was used to envelop the aorta at the level of the rupture. Postoperative period was uneventful. The patient required delayed stent placement in common iliac artery due to its dissection. After 8 years of follow up patient remains in good condition.