The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: A 30-year experience

Abstract

Operative mortality in patients with vascular complications of EDS-IV was not excessively high, but the incidence of postoperative bleeding complications and late graft-related problems was significant. Despite successful repair of vascular complications, survival was shortened because of secondary vascular or graft-related complications.

Spontaneous Haemomediastinum and Fatal Haemoperitoneum in woman with Vascular Ehlers-Danlos Syndrome

“…Embolization of aortic branch vessels and other medium sized-arteries has been successful in EDS patients presenting with mayor acute bleeding [2,3]. Current devices achieve complete obliteration of pathologic vessels with few complications.…”

False Aneurysms

“…There are some systemic factors which are thought to contribute to anastamotic aneurysm formation: smoking, hypertension, hyperlipidemia, anticoagulation, systemic vasculitides and generalized arterial weakness [72,73].…”

Liver transplantation in a patient with Ehlers-Danlos syndrome

“…Retractors should be placed carefully to prevent damage to the adjacent bowel or mesentery, and the anastomosis should be tensionless. 8 In our case, a graft for the arterial anastomosis was first sewn to the recipient's hepatic artery to prevent excessive traction. The anastomosis may be reinforced with Teflon felt strips or pledgeted sutures.…”

“…[2][3][4] Because of the difficulty in managing fragile tissues, surgical procedures in patients with EDS type IV are associated with high rates of complications, including severe postoperative bleeding (37%), re-exploration (20%), and late graft anastomotic complications (40%). 8 Although EDS type IV is associated with the most serious complications and a significantly increased risk of death, vascular or visceral disease can present in different types of EDS patients. 2 On the basis of the available literature, one could infer that the risk for surgery is high in patients with EDS type IV, intermediate in patients with type I or II, and low in patients with type III.…”

“…2 Ideally, the surgeon should be aware of the diagnosis of EDS before any procedure, although the patient is aware of the diagnosis in <30% of cases. 2,8 In surgery, gentle, atraumatic, and delicate handling of tissues is mandatory to prevent vascular tears, intimal dissection, and peri-adventitial hematomas. The use of soft, protected arterial clamps or balloon occlusion is preferable to the use of standard vascular clamps.…”