Evaluation
Evaluation at the time of emergency referral depends on the signs and symptoms. In general, non-invasive evaluation of the abdomen, chest, and head are preferred using MRI, CT, and venous angiographic approaches to understand blood loss.
Use of arteriography with high pressure injection is generally avoided because of the risk of further vascular injury. Some vascular events can be dealt with effectively with embolization. Although covered stents are being placed in life-threatening situations to forestall active bleeding, it is not known if and how arteries will withstand the pressure of the stents over the long course.
Aneurysmal dilation may occur in some while others may require open surgical intervention. Bowel rupture almost always requires surgical intervention and usually leads to isolation of the distal bowel, removal of the ruptured segment, and creation of a colostomy. Repair of colostomy has become more commonplace and is frequently successful.
Recurrent surgery may be associated with ilio-colic fistula formation and subtotal colectomy may prevent further colonic ruptures. This possibility could be discussed at the time of the first colonic rupture if the diagnosis of vascular Ehlers–Danlos syndrome has been established.
Source: Byers PH, Belmont J, Black J, De Backer J, Frank M, Jeunemaitre X, Johnson D, Pepin M, Robert L, Sanders L, Wheeldon N. 2017. Diagnosis, natural history, and management in vascular Ehlers–Danlos syndrome. Am J Med Genet Part C Semin Med Genet 175C:40–47. Images are generic.