Ehlers-Danlos Syndrome

  • Etiology: vasculopathy due to connective tissue disorder of collagen, Type 4 is most commonly associated with vascular complications
  • Imaging: affects medial + large arteries in any location
  • Complications: vascular – vessel aneurysm / dissection / rupture / fistulae, intestinal rupture, uterine rupture
  • Clinical: hypermotility, skin extensibility, tissue fragility

Cases of Ehlers-Danlos Syndrome

CT of pseudoaneurysm in Ehlers-Danlos Syndrome
Axial CT with contrast of the abdomen shows an intimal flap in the superior mesenteric artery and a large retroperitoneal hematoma.