VASCULAR COMPLICATIONS IN EHLERS-DANLOS SYNDROME

Ehlers-Danlos syndrome (EDS) type IV results in a high incidence of va scular lesions. The extreme fragility of arteries is associated with m ultiple aneurysm formation, spontaneous rupture, and dissection. Surgi cal management of patients with this disorder is hazardous and often u nrewarding. In this report we describe the difficulties encountered in the management of three patients with EDS-related vascular lesions. T hree patients presented with pain and exhibited characteristic feature s of EDS. Diagnostic modalities included computerized tomography, tran sesophageal echocardiography, and magnetic resonance. Aortography was performed only in specific situations. One patient with pericardial an d mediastinal hemorrhage was stabilized and treated conservatively, wi th a good outcome. An adolescent with a ruptured aortic pseudoaneurysm died at surgery. The third patient underwent successful surgical corr ection of multiple aortic and renal aneurysms. In view of the increase d risk of fatal vascular complications, surgeons should identify patie nts with EDS before performing invasive procedures. Arteriography shou ld be used only when necessary. Although operative mortality remains a t a high level due to the tendency of vessels to tear with even minima l manipulation, mortality from hemorrhage without surgical interventio n is even greater. The key to favorable outcomes lies in identificatio n of the syndrome preoperatively, surgical intervention only in life- or limb-threatening situations, and appropriate modification of surgic al technique.