Ehlers-Danlos Syndrome Treatment & Management

Updated: Apr 12, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Treatment is unsatisfactory.

One isolated report showed that patients with type VI disease benefited from oral vitamin C at 4 g/d. Scars and bleeding time seemed to improve with this treatment.

Rombaut et al performed a cross-sectional study of self-reported medication use, surgery, and physiotherapy in 79 patients with hypermobility-type Ehlers-Danlos syndrome (EDS) (type III), finding that 73 patients (92.4%) were taking medication, 56 (70.9%) had undergone surgery, and 41 (51.9%) were currently receiving physical therapy. [43] The patients taking medication all took analgesics; many also reported using antidepressants. Severe pain was frequent; patients taking strong opiates made more complaints and experienced greater functional impairment than those using weaker analgesics.

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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. [44] Lethal arterial events may occur. [45]  Fernandez-Alcantud reviewed management of anesthesia in vascular type IV Ehlers-Danlos syndrome patients. [46]  Jones reports the use of local anesthesia for elective cesarian delivery in a type III Ehlers-Danlos syndrome woman. [47]

Recombinant factor VIIa has been successful in the treatment of intractable bleeding in vascular-type Ehlers-Danlos syndrome. [48]

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Activity

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.

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