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Inherited Disorders and Birth Defects

Surgery Risks for Ehlers-Danlos Patients?

01/30/2009

Question:

I have suspected EDS, mild, because I have the hyper-mobile joints, problem with past surgery healing, pelvic prolapse despite having no or very few risk factors such as childbirth or age I am awaiting now my June appointment for evaluation by an expert here at Washington U. I have herniated discs in my neck, and have been told that discectomy and fusion with rods and screws is the definitive treatment.

I have had other treatments and am right on the line between "bearable, but with reduced quality of life" vs. "can`t stand it anymore". I am wondering if people with milder EDS are poor candidates for orthopedic surgeries or surgeries affecting ligaments (such as the pelvic reconstruction w/ mesh that is already sagging 2 yrs later). Are they also at risk for much greater (or easier) injury after surgery, say in a low-speed whiplash-type accident? That really scares me--the idea that the hardware itself could be displaced more easily and cause even more severe injury due to lack of support in that area. If I am at higher risk for failure or complications, I will wait until there are less invasive options that my insurance will cover, as it only covers the tried-and-true radical options. I`m also wondering if there is considered any genetic relationship to connective tissue autoimmune disease, as my mother has "Mixed Connective Tissue Disease" and autoimmune diseases run rampant through my family history. Thanks! PM in St. Louis

Answer:

As you know, Ehler-Danlos syndromes (EDS) are a group of genetic disorders that usually have hypermobile joints, can have hyperextensible (stretchy) skin and poor wound healing. There are a number of different types of Ehler-Danlos - some of them mild, as you mention, others that can be more severe (such as the vascular type).

Because there are problems with wound healing, having surgery needs to be carefully considered with your doctor. However, that is a question that would be best answered by an orthopedic surgeon.

To answer your question about EDS being associated with autoimmune disorders, researchers do not know if there is a specific link between EDS and autoimmune diseases. However, there are chronic autoimmune diseases which affect connective tissue, so it is possible that because you have both diseases in your family they could be related. Also, it could be a coincidence.

Genetic testing is available for EDS. Because it is easy to mis-diagnose the type of EDS a person has, if you have not had genetic testing, you may want to talk to a geneticist or genetic counselor about testing. Knowing specifically whether you have EDS type I, II, III, etc may give you more information and help in discussing surgery and other problems running through your family.

You can locate a genetics center near you at the National Society of Genetic Counselors Resource Center website below.

Related Resources:

National Society of Genetic Counselors Resource Center

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Response by:

Anne   Matthews, RN, PhD Anne Matthews, RN, PhD
Associate Professor of Genetics
School of Medicine
Case Western Reserve University