Shoulder replacement complications in the Ehlers-Danlos Syndrome Patient

Ross Hauser, MD

If you suffer from chronic subluxations, dislocations, and pain from ligament laxity due to Ehlers-Danlos Syndrome, you know firsthand that shoulder problems may only be one of the many challenges you face. You also know that your shoulder problems are “widespread” throughout your shoulder joint, you are beyond describing a simple tear or a bone spur. You may have a significant and developing shoulder osteoarthritis, small rotator cuff tears are gradually getting larger, and your shoulder which has an extensive history of subluxation or dislocating has taken it upon itself to grow more bone in an attempt to fuse your shoulder and prevent it from slipping out of place and stop the dislocations that may now be occurring with any arm movements.

Your medical history, just for your shoulder may include posture education, physical therapy, and specific exercises to produce muscle stability in your shoulder. If these treatments had helped or worked, you are probably not reading this article. You may have been given information or read the information on compression garments or compression jackets that will help keep your shoulder in place although research suggests mixed results and that a compression garment to hold your shoulder together is best effective if you never take it off. (1) You may have been told that before doctors will consider a shoulder replacement for you, you will have to “fail” at this extensive program. Doctor after doctor has told you to have a shoulder replacement. But you may not be quite there yet.

Shoulder Replacement in the Ehlers-Danlos Syndrome Patient

A March 2021 study in the Journal of Shoulder and Elbow Surgery (2) from doctors at the Mayo Clinic. Here the doctors examined the outcomes for Ehlers-Danlos Syndrome patients who were given shoulder arthroplasty (replacement) to help them with their issues of shoulder instability. Here are the learning points of this research:

Background: Patients with Ehlers-Danlos Syndrome (EDS) have high rates of shoulder instability, which places them at increased risk for instability-related (degenerative shoulder disease).
While there are many studies assessing the outcomes for both primary and revision (repair) shoulder instability procedures in this patient population there is little in the research regarding the outcome of shoulder replacement in EDS patients.
The doctors of this study then sought to evaluate the results and complications of shoulder replacement performed in a group of patients with EDS.

How the research was conducted:

Over an 11-year period, 10 patients with EDS had a primary shoulder replacement (6 anatomic total shoulder replacements, 4 reverse shoulder replacements.
The patient’s shoulders were evaluated at an average follow-up of 60 months (range 25-97 months).
The EDS patients were an average age of 55 years, a mean BMI of 26.1, (slightly to moderately overweight), and were all female.
The primary outcome measures were postoperative pain, range of motion, complications, and reoperations.
The results of this group were then measured against a similar group who had shoulder replacement but were not diagnosed with Ehlers-Danlos Syndrome

Outcomes:

Shoulder replacement produced similar postoperative pain, range of motion, complications, and reoperations in patients with Ehlers-Danlos Syndrome vs. the patients who did not have Ehlers-Danlos Syndrome
EDS patients improved pre- to postoperative pain scores (VAS score 0 (no pain to 10 severe pain) 6.5 to 1.7, and have much better arm elevation and external rotation.
Three EDS patients sustained postoperative complications (2 instability and 1 acromial fracture); however, no shoulder underwent reoperation.

Conclusions: “Ehlers-Danlos Syndrome patients undergoing shoulder replacement can expect comparable outcomes compared to patients with primary osteoarthritis or cuff tear disease, with clinically meaningful improvements in pain and range of motion. Although EDS patients had no statistically significant increase in complications when compared to controls, their absolute rate of overall complications (3/10 patients; 30%) and postoperative instability (2/10 patients; 20%) in this small case series was relatively high and should be considered when performing shoulder replacement.”

What the doctors are suggesting is basically what you read. People with Ehlers-Danlos Syndrome can have good benefits from shoulder replacement. However, surgeons should be aware that in this study, three out of ten patients had overall complications, and two out of ten patients continued to have post-operative instability. The people who had very successful surgeries are typically the people who do not reach out to our office, we tend to see the people with continued instability after the surgery.

Follow-up research on shoulder replacement in shoulder hypermobility patients

In April 2023 doctors at the Steadman Philippon Research Institute wrote in the Current Reviews in Musculoskeletal Medicine (3) that “if joint preserving stabilization procedures are unsuccessful, a conversion to a reverse total shoulder replacement may be considered. Especially in the setting of long-standing instability, instability arthropathy (degenerative shoulder disease) may ensue, requiring a joint replacing treatment option.” The researchers cited the above study suggesting that reverse and total shoulder replacement can be used successfully in patients with hEDS, with improvements in pain and no reoperations in the group of ten patients. However, the cohort demonstrated a relatively high complication rate of three in ten of the patients, with 2 patients demonstrating postoperative instability treated conservatively. The researchers added: “As a last resort, arthrodesis (shoulder fusion) may be used to achieve stabilization of the glenohumeral joint, with adequate postoperative range of motion permitted by scapulothoracic (joint) motion.

The next shoulder replacement and the one after that and the one after that

We do see Ehlers-Danlos Syndrome patients who have had a shoulder replacement, revision shoulder replacement, and in some instances revision of the shoulder replacement. You may be a person yourself who may be able to count out 3-4-5 surgeries or more.

Shoulder replacement for many Ehlers-Danlos Syndrome patients is considered a success if they have the results like those in the above Mayo Clinic study. This may be you as well. Initial stability, less pain, better function. But over time less than a year out, more than a few years out, the laxity of the shoulder and the shoulder ligaments progressed. Your shoulder situation may now be worse than it was before the first shoulder replacement. More complex surgeries may now be necessary. If the shoulder continues to dislocate your shoulder may have to be fused.

Prolotherapy for Hypermobile Ehlers-Danlos Syndrome

In this section, we will lay the foundation for the treatment of patients suffering from Ehlers-Danlos Syndrome, specifically Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders with Prolotherapy injections. The treatment is designed to stimulate connective tissue growth and strengthen the ligaments of the shoulder complex.

Our research: Prolotherapy and Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (Hypermobility Spectrum Disorders (HSD)).

In our 2013 paper in the Journal of Prolotherapy, the full research can be downloaded as a PDF file – Treatment of Joint Hypermobility Syndrome, Including Ehlers-Danlos Syndrome, with Hackett-Hemwall Prolotherapy. we give an outline of Prolotherapy treatments.

Here are our learning points:

While traditional medical treatments including education and lifestyle advice, behavior modification, physiotherapy, taping and bracing, exercise prescription, functional rehabilitation, and pain medications offer some symptomatic control, they do little in regard to curbing the progressive debilitating nature of the diseases.
Excessive joint mobility with its subsequent joint degeneration and multiple joint dislocations can then lead the individual to seek out surgical intervention, which has suboptimal results in the hypermobile patient population versus the normal population. As such, some patients with Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (HSD) are seeking alternative treatments for their pain, including Prolotherapy.
Prolotherapy offers great hope for those with symptoms from generalized hypermobility because it is designed to successfully treat the ligament and tissue laxity that accompanies Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome.
Prolotherapy works by initiating a brief inflammatory response, which causes a reparative cascade to generate new collagen and extracellular matrix giving connective tissue strength and the ability to handle strain and force.
Prolotherapy has a long history of success in treating ligament injuries, including patients with joint hypermobility.
Studies on Prolotherapy have shown that it eliminates chronic pain even in those patients who have been told by their medical doctor(s) that surgery was the only treatment option for their pain.

Some of the rationales for using Prolotherapy for patients with Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome include that it has:

A high safety record is comprehensive (all or most joints can be treated at each visit),
is an outpatient procedure,
is cost-effective (compared to surgery),
Pain relief is often quick,
and it provides joint stabilization.

Perhaps its greatest asset is the fact that this one treatment modality can handle most of the painful musculoskeletal conditions that occur in individuals with Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome.

Platelet Rich Plasma Therapy (PRP) and shoulder instability

Danielle R. Steilen-Matias, MMS, PA-C narrates the video and is the practitioner giving the treatment:

The patient in this video does not have Ehlers-Danlos Syndrome but has a history of repeated dislocations. The video is offered to demonstrate the treatment.

PRP or Platelet Rich Plasma treatment takes your blood, like going for a blood test, and re-introduces the concentrated blood platelets and growth and healing factors from your blood into the shoulder. The treatment is explained further below.
In the shoulder treatment, I treat all aspects of the shoulder including the ligament and tendon injections to cover the whole shoulder.
The patient in this video is not sedated in any way. Most patients tolerate the injections very well. The treatment goes quickly. However, we do make all patients comfortable including sedation if needed.
This patient in particular comes to us for a history of repeated shoulder dislocations. His MRI findings showed multiple labral tears and rotator cuff problems.
The patient complained of shoulder instability typical of the ligament and tendon damage multiple dislocations can do.
With the patient lying down, treatment continues to the anterior or front of the shoulder. The rotator cuff insertions, the anterior joint capsule, and the glenohumeral ligaments are treated.
PRP is introduced into the treatment and injected into the front of the shoulder. PRP is a form of Prolotherapy where we take concentrated cells and platelets from the patient’s blood and inject that back into the joint. It is a more aggressive form of Prolotherapy and we typically use it for someone who has had a labral tear, shoulder osteoarthritis, and cartilage lesions.
PRP is injected into the shoulder joint and the remaining solution is injected into the surrounding ligaments in this case it was in his anterior shoulder attachments to address the chronic dislocations.

Questions about our treatments?

If you have questions about your pain and how we may be able to help you, please contact us and get help and information from our Caring Medical staff.

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Ehlers-Danlos Syndrome and knee replacement complications

References:

1 Chaléat-Valayer E, Denis A, Zelmar A, Pujol A, Bernadou A, Bard-Pondarré R, Touzet S. VETCOSED study: efficacy of compressive garments for patients with hypermobile Ehlers–Danlos syndrome on shoulder stability and muscles strength. Disability and Rehabilitation. 2020 Dec 5:1-8. [Google Scholar]
2 Rogers T, Werthel JD, Crowe M, Ortiguera C, Elhassan B, Sperling J, Sanchez-Sotelo J, Schoch BS. Shoulder Arthroplasty is a Viable Option in Patients with Ehlers-Danlos Syndrome. Journal of Shoulder and Elbow Surgery. 2021 Apr 1. [Google Scholar]
3 Rupp MC, Rutledge JC, Quinn PM, Millett PJ. Management of shoulder instability in patients with underlying hyperlaxity. Current Reviews in Musculoskeletal Medicine. 2023 Apr;16(4):123-44. [Google Scholar]

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