Arterial/intestinal/uterine fragility or rupture commonly arise in Vascular Type EDS. Spontaneous arterial rupture has a peak incidence in the third or fourth decade of life, but may occur earlier. Midsize arteries are commonly involved.

Arterial rupture is the most common cause of sudden death. Acute diffuse or localized abdominal or flank pain is a common presentation of arterial or intestinal rupture.

(For more information about treating complications of Vascular Type EDS in an emergency, please see the Emergency Physician's Reference Guide.)