EDS Fact 13 - Vascular EDS

Vascular EDS is the most serious type of EDS and the most examined because it has more life threatening complications than the rest. Vascular EDS effects the organs, specifically the heart, more than with other types. You have an increased risk of heart problems and possible failure.

Unpredictable ruptures of arteries and organs are the most serious complications of the Vascular Type of Ehlers-Danlos Syndrome. A torn artery can cause internal bleeding, stroke, or shock, and is the most common cause of death in patients with this disorder. Rupture of the intestine is seen in 25 to 30 percent of affected individuals and tearing of the uterus (womb) during pregnancy affects 2 to 3 percent of women. Although serious problems are rare in childhood, more than 80 percent of patients experience severe complications by the age of 40. 

---

The combination of any two of the major diagnostic criteria should have a high specificity for the Vascular Type of EDS.  Biochemical testing is strongly recommended to confirm the diagnosis. The presence of one or more minor criteria supports the diagnosis of the vascular type of EDS but is not sufficient to establish the diagnosis. 
Major Diagnostic Criteria for the Vascular Type of EDS

• Arterial rupture 

• Intestinal rupture 

• Uterine rupture during pregnancy 

• Family history of the vascular type of EDS 

Minor Diagnostic Criteria for the Vascular Type of EDS

• Thin, translucent skin (especially noticeable on the chest/abdomen) 

• Easy bruising (spontaneous or with minimal trauma) 

• Characteristic facial appearance (thin lips and philtrum, small chin, thin nose, large eyes) 

• Acrogeria (an aged appearance to the extremities, particularly the hands) 

• Hypermobility of small joints 

• Tendon/muscle rupture 

• Early-onset varicose veins 

• Arteriovenous carotid-cavernous sinus fistula 

• Pneumothorax/pneumohemothorax 

• Chronic joint subluxations/dislocations 

• Congenital dislocation of the hips 

• Talipes equinovarus (clubfoot) 

• Gingival recession 

Source - http://www.ehlersdanlosnetwork.org/vascular.html