PMID: 16533486Mar 15, 2006Paper

The vascular Ehlers-Danlos syndrome

Current Treatment Options in Cardiovascular Medicine
Dominique P Germain

Abstract

Vascular Ehlers-Danlos syndrome (EDS) is a life-threatening inherited disorder of connective tissue causing severe arterial and gastrointestinal fragility and rupture, as well as complications of surgical and radiologic interventions. The diagnosis should be considered in patients under the age of 45 years who present with arterial tearing or dissection, colonic perforation, or visceral rupture. As for many orphan diseases, delayed diagnosis can lead to inaccurate care. Therefore, vascular EDS is particularly important to surgeons, radiologists, and obstetricians because knowledge of the diagnosis may help in the management of visceral complications. There are currently no specific treatments for this genetic condition, and medical intervention is limited to symptomatic treatment, precautionary measures, genetic counseling, and prenatal diagnosis. A clinical trial is ongoing to study the effectiveness of beta blockers with vasodilating properties in vascular EDS. Complications require hospitalization, observation in an intensive care unit, and sometimes emergency surgical intervention. CT, echography, and MRI are the most useful imaging modalities. Arteriography is contraindicated. Whenever possible, a wait-and-see attitude wit...Continue Reading

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