Sammanfattning
Vascular Ehlers-Danlos’ Syndrome is a rare genetic disorder affecting procollagen type III, affecting approximately 50 individuals in Sweden. In this article recent advances in the treatment of the disease are summarized. Prophylaxis against vascular catastrophies can be performed with the specifically well designed betablocker celiprolol, and preliminary experiences of this treatment are reported. When arterial dissections and aneurysms develop, both open and endovascular surgery can be considered, but serious complications are common, and conservative management is the first option. It is important that the first line of treatment is decentralized, but in close collaboration with a dedicated academic unit.