Ehlers-Danlos Syndromes (Continued)

Management of EDS disorders is directed at preventative care and supportive therapy. In general, individuals with EDS should not participate in contact sports or heavy exercise. Non–weight-bearing exercise is encouraged, and physical therapy is recommended for persons with motor delays or significant hypotonia. Persons with marked skin fragility should cover particularly at-risk areas with protective pads. Individuals with mitral valve prolapse and regurgitation require antibiotic prophylaxis for bacterial endocarditis. Cardiovascular surveillance with echocardiograms is indicated for persons with any type of EDS for which cardiovascular involvement is common, as well as for persons with clinical signs or symptoms suggestive of cardiac pathology, although explicit guidelines are not yet available. Persons with vascular types of EDS require numerous precautions, including avoidance of contact sports and isometric exercise, avoidance of anticoagulant medications and antiplatelet agents, and avoidance of arteriography and other vascular procedures unless necessary. Because of vascular and skin fragility, surgeries should be done with extreme caution. Careful follow-up is recommended throughout pregnancy and postpartum, especially for pregnant women with vascular type EDS who should be considered as high-risk patients. Psychosocial support for patients and their families is important and patient support groups are available and can be beneficial. Genetic counseling should be provided for pertinent family members who seek information about EDS, including information regarding reproductive risk and prenatal diagnosis. Overall, care for persons with EDS is best done by a multidisciplinary team with expertise in genetic connective tissue disorders.

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Jul 3, 2016 | Posted by in MUSCULOSKELETAL MEDICINE | Comments Off on Ehlers-Danlos Syndromes (Continued)
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