Objective: To provide the collected evidence from all literature reports. Background: Vascular Ehlers-Danlos syndrome (EDS) is a rare connective tissue disorder with serious hemorrhagic consequences. Most experience on treatment is based on case reports and small case series. Method: A systematic literature review was performed. PubMed and reference lists were scrutinized. Results: A total of 231 patients were identified with no gender preponderance. Aneurysms were present in 40%, often multiple. In 33%, there was an arterial rupture without an underlying aneurysm. Carotidocavernous fistula was seen in 18%. After open surgery the mortality was 30%; after endovascular procedures, it was 24%; in a group of miscellaneous cases, it was 60%; and the overall mortality was 39%. The median age of patients at death was 31 years. The median follow-up time was 12 months (5 days-7 years), but in 20% cases, it was not reported. In only 29 of the 119 recent patients (24%) the mutation was verified with molecular genetic testing. Conclusions: Vascular EDS is a serious disorder with high mortality, which does not seem to have been influenced by new treatment methods. Invasive methods should be used only when necessary, primarily to save the patients' life. Whenever possible, the genetic molecular defect should be identified. The results of this review may be affected by publications bias. Ideally, a prospective registry should be created.
