Ehlers-Danlos syndrome

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Complications

By Mayo Clinic staff

Most people with Ehlers-Danlos syndrome live a relatively normal life, although there may be restrictions on physical activity. EDS doesn't affect your intelligence. Complications depend on your symptoms and type of EDS, but some common ones include:

  • Prominent scarring
  • Difficulty with surgical wounds — stitches may tear out, or healing may be incomplete
  • Chronic joint pain
  • Joint dislocation
  • Early onset arthritis
  • Premature aging with sun exposure

People with vascular type EDS are at risk of serious complications because of the fragility of blood vessels and organs. Serious complications of vascular type EDS include tearing (rupture) of major blood vessels, such as a ruptured or dissected artery or an aneurysm, or rupture of organs, such as the intestines or uterus. These complications can be fatal. About 1 in 4 people with vascular type EDS develop a significant health problem by age 20, and more than 80 percent develop complications by age 40. The median age of death is 48 years.

Some people with Ehlers-Danlos syndrome may develop osteoporosis. Osteoporosis is generally treated with prescription medications to increase bone density. Specific physical therapy exercises may help, too.

Pregnancy and EDS
Most women with EDS can have successful pregnancies. However, having EDS puts you at higher risk of premature delivery, postpartum hemorrhage and poor wound healing after delivery. Anesthesia during labor also can be difficult.

If you have vascular type EDS, pregnancy can be dangerous. The increase in blood volume and cardiac output puts more demand on your fragile blood vessels and organs, and there's a high risk of aortic or uterine rupture, both of which can be fatal.

If you have EDS and want to start a family, talk to your doctor first. Your doctor may suggest monitoring your pregnancy closely for any signs of complications. In some cases, your doctor may advise you against becoming pregnant because of the high risk of complications.

References
  1. Lawrence EJ. The clinical presentation of Ehlers-Danlos syndrome. Advances in Neonatal Care. 2005;5:301.
  2. Whitelaw SE. Ehlers-Danlos syndrome, classical type: Case management. Dermatology Nursing. 2004;16:433.
  3. Sheon RP. Clinical manifestations and treatment of the hypermobility syndrome. http://www.uptodate.com/home/index.html. Accessed Jan. 27, 2010.
  4. Tong I. Ehlers-Danlos syndrome. In: Ferri FF. Ferri's Clinical Advisor 2010. Philadelphia, Pa: Mosby Elsevier; 2009. http://www.mdconsult.com/das/book/body/188281291-3/965853179/2088/208.html#4-u1.0-B978-0-323-05609-0..00014-9--sc0025_4217. Accessed Feb. 10, 2010.
  5. Volkov N, et al. Ehlers-Danlos syndrome: Insights on obstetric aspects. Obstetrical & Gynecological Survey. 2006;62:51.
  6. Voermans NC, et al. Neuromuscular involvement in various types of Ehlers-Danlos syndrome. Annals of Neurology. 2009;65:687.
  7. Krakow D. Heritable diseases of connective tissue. In: Firestein GS, et al. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa.: W.B. Saunders; 2008. http://www.mdconsult.com/das/book/body/188281291-3/965853179/1807/728.html. Accessed March 10, 2010
DS00706 April 20, 2010

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