Ehlers-Danlos syndrome is an inherited disorder that affects your connective tissues— primarily your skin, joints, and blood vessel walls. These connective tissues provide strength and elasticity to the structures of the body. People with this disorder usually have over-flexible (loose) joints and stretchy skin. Flexible joints can result in joints that are unstable and may slip out of place (subluxation) or completely come out of place (dislocation). With recurrent episodes of the joint coming out of place, early onset of arthritis is also a possibility.
The two most affected joints include the knee joint, especially the patella (knee cap), and the shoulder. The hip, ankle, and joints around the clavicle can also be loose and uncomfortable. If the shoulder is dislocated, this can stretch or tear the capsule and damage other parts of the joint. Once the shoulder has dislocated, there is an increased probability of sustaining recurrent instability events (subluxation or dislocation). An important component of keeping the shoulder in place is the muscles and tendons of the rotator cuff and scapula.
The first effort at treatment this problem is a dedicated physical therapy program to teach good posture, improved positioning of the scapula during shoulder and arm movements, better activation of the rotator cuff to help compress the humeral head into the socket, and supportive biofeedback including the use of shirts or blouses designed to help the patient understand the overall position of the shoulder better. No less than 6 months, and preferably one year, of physical therapy treatment is required before this plan is abandoned for consideration of surgical options.
Surgery to treat an unstable shoulder in a patient with Ehlers-Danlos Syndrome has a high rate of failure, even in the hands of experts. It is not unusual for Dr. Romeo to see a patient with this problem who has already had two or more procedures for the symptomatic shoulder. The history is often that the surgeon felt the shoulder was tightened up more than usual, only to see the repair site stretch out over 9-18 months and the problem return to same level or worse than it was before surgery. More advanced and complex procedures may provide a longer lasting outcome, including the use of bone to provide more stability that cannot stretch out after the procedure, or the use of tendon tissues from a cadaver (allograft material) to reinforce the surgical repair. Unfortunately, for some patients, after multiple procedures the joint may be come arthritic, or when the shoulder remains subluxed or dislocated out the bottom (inferior), a shoulder fusion may be the only remaining treatment to provide some improvement in comfort and function despite the permanent loss of motion.
Ehlers-Danlos Syndrome FAQ
What causes Ehlers-Danlos syndrome?
Ehlers-Danlos Syndrome (EDS) is a group of inherited connective tissue disorders. In other words, it comes from the genes received from your parents. It is important for patients to understand if they fit a certain sub-type of this condition (there are now 13) as each subtype has unique features, including some subtypes that can affect the vascular system, intestinal system, the spine, and the heart. Although the overall condition cannot be cured, the various risks of the syndrome can be effectively treated. Dr. Romeo recommends an evaluation from a medical geneticist to understand your condition and the potential risks.
How can this condition affect a patient’s shoulder stability?
Due to the frequent association of elastic connective tissue associated with EDS, the most common problem with the shoulder is too much looseness that cannot be controlled by the muscles and tendons which results in the shoulder shifting partially or completely out of socket. When this happens repetitively over time, or if multiple surgeries are ineffective in controlling the loose shoulder, there is an increased risk of arthritis and increasing pain with dysfunction.
For more information about Ehlers-Danlos syndrome, please request an appointment with experienced Chicago orthopaedic surgeon Dr. Anthony Romeo. Call or email our office today to schedule your visit. Also, if you want to more about EDS, please visit their web site for more information from the EDS Society: https://www.ehlers-danlos.com/what-is-eds/