Ehlers-Danlos Syndrome Treatment & Management

Updated: Mar 31, 2023
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Surgical Care

Extreme caution is mandatory in any surgical maneuver. Type IV Ehlers-Danlos syndrome (EDS) patients should be carefully monitored because they are at high risk for spontaneous rupture of a large artery (eg, splenic artery, aorta) or perforation of internal organs. These patients should be educated that surgery can pose life-threatening risks.

Plastic re-excision of scars sometimes provides acceptable cosmetic results.

Anesthetic implications, although rare, are very important in Ehlers-Danlos syndrome, especially in the type IV (or vascular) patients. Risk of complications is higher, spontaneous vascular rupture can occur, and cervical spine and airway trauma must be kept in mind. Bleeding is also reported. [52] Lethal arterial events may occur. [53]  Fernandez-Alcantud reviewed management of anesthesia in vascular type IV Ehlers-Danlos syndrome patients. [54]  Jones reports the use of local anesthesia for elective cesarian delivery in a type III Ehlers-Danlos syndrome woman. [55]

Recombinant factor VIIa has been successful in the treatment of intractable bleeding in vascular-type Ehlers-Danlos syndrome. [56]



Patients with Ehlers-Danlos syndrome (EDS) types IV or VI should avoid participating in dangerous contact sports. Some authors mention risks with activities that can increase intracranial pressure as a result of the Valsalva effect. An example of one such activity is playing the trumpet.