Managing Shoulder Instability in EDS: Surgical and Non-surgical Options

Managing Shoulder Instability in Ehlers-Danlos Syndrome: Surgical Options and Outcomes

Ehlers-Danlos Syndrome (EDS), particularly its hypermobility type (hEDS), is a hereditary connective tissue disorder known for causing joint hypermobility, skin elasticity, and tissue fragility. One of the significant challenges for individuals with hEDS is shoulder instability. This instability can lead to recurrent dislocations, subluxations, and chronic pain, severely affecting the quality of life. While conservative treatments are often the first line of defense, surgery becomes necessary for many patients to restore stability and improve function.

Shoulder Instability in EDS

The shoulder joint is particularly vulnerable in individuals with EDS due to the ligamentous laxity caused by abnormal collagen production. This lack of structural integrity results in frequent dislocations or subluxations, where the joint partially slips out of place. These events often cause pain and limit physical activities, including everyday tasks like reaching, lifting, or dressing. Additionally, repeated dislocations can lead to further joint damage and chronic conditions like arthritis​​.

Non-Surgical Management: First-Line Approach

Non-surgical interventions are usually the initial approach for managing shoulder instability in EDS patients. Physical therapy plays a critical role in this, focusing on strengthening the rotator cuff and scapular muscles, which act as dynamic stabilizers of the shoulder. Exercises that emphasize low-impact, isometric, and eccentric movements can help improve joint stability while minimizing pain​. It is crucial that these rehabilitation programs avoid overstretching already lax joints, as improper exercises can exacerbate instability​​.

Additionally, joint braces or splints can help limit excessive motion in the shoulder, preventing further subluxations. Despite these efforts, many EDS patients find that conservative measures alone are not sufficient to control symptoms, leading them to explore surgical options.

Surgical Approaches for Shoulder Instability in EDS

Surgical treatment for shoulder instability in EDS patients is complex due to the fragility of the connective tissues. Various surgical techniques are employed to restore joint stability, and each carries unique risks and challenges for EDS patients.

Arthroscopic Stabilization is the most commonly performed procedure, particularly for younger patients. It involves tightening the joint capsule and repairing any damaged tissues. Arthroscopic procedures are minimally invasive and generally favored because they have lower complication rates compared to open surgeries. However, EDS patients may still face complications such as postoperative instability and difficulty with tissue healing​​.

Open Capsulolabral Repairs and Shifts are sometimes required when arthroscopic methods are insufficient. These open procedures involve repairing or shifting the labrum and capsule to stabilize the shoulder. While these surgeries can be effective, they are more invasive and carry a higher risk of complications, such as prolonged recovery times or recurrence of instability​.

Latarjet Procedure is a technique used for patients with significant bone loss or recurrent dislocations. It involves transferring a piece of the coracoid bone to the shoulder socket, which provides additional stability to the joint. Though highly effective in some cases, the procedure is more invasive and comes with a higher risk of complications, especially for patients with EDS​.

Shoulder Arthroplasty in EDS

For patients with advanced instability or shoulder arthritis, shoulder arthroplasty (joint replacement) may be considered. Studies have shown that patients with EDS who undergo shoulder arthroplasty experience similar improvements in pain relief and range of motion compared to non-EDS patients​. However, the rate of complications, such as postoperative instability, is higher in this population. For example, in one study, 20% of EDS patients experienced postoperative shoulder instability following arthroplasty​. Despite these risks, shoulder arthroplasty can provide significant functional improvements when performed carefully.

Complications in EDS Shoulder Surgery

EDS patients face unique challenges when undergoing surgery due to their connective tissue disorder. Complications include poor wound healing, a higher likelihood of infections, and a greater risk of joint instability post-surgery​​. Additionally, the fragility of tissues in EDS means that even successful surgical repairs may not be as durable as in patients without EDS. For this reason, EDS patients are more likely to require revision surgeries over time​.

Furthermore, local anesthetics can be less effective in patients with EDS, which complicates both the surgical procedure and postoperative pain management. These patients often require higher doses of anesthetics or alternative pain control strategies​.

Importance of a Multidisciplinary Approach

The management of shoulder instability in EDS requires a multidisciplinary approach, involving orthopedic surgeons, physical therapists, rheumatologists, and pain specialists​. Surgeons with experience in treating EDS are essential, as they understand the unique challenges posed by this connective tissue disorder. A well-rounded care team can better tailor rehabilitation programs, assess surgical risks, and manage postoperative complications.

Pre-surgical consultations should include detailed discussions of potential outcomes and the higher risk of complications. EDS patients must be informed about realistic expectations regarding the durability of surgical repairs and the possibility of needing further procedures in the future​​.

Postoperative Rehabilitation

Rehabilitation is critical to the success of surgical intervention in EDS patients. A carefully managed postoperative plan typically involves immobilization of the shoulder for several weeks, followed by gradual reintroduction of movement through controlled range-of-motion exercises. Strengthening the rotator cuff and scapular stabilizers is essential to regain shoulder stability, but exercises must be done cautiously to avoid overstressing the repaired tissues​.

Patients with EDS often face a longer recovery period compared to those without the condition, and long-term maintenance programs may be necessary to prevent future dislocations or instability​​.

Conclusion

Shoulder instability is a common and debilitating issue for individuals with EDS, particularly hypermobility type. While non-surgical management is often the first line of treatment, surgery may be required for those who experience recurrent dislocations and chronic pain. Arthroscopic stabilization remains the most common surgical approach, but open procedures and shoulder arthroplasty are options for more severe cases. Despite the challenges posed by EDS, advancements in surgical techniques and postoperative rehabilitation protocols have shown promising results in improving shoulder stability and function.

A multidisciplinary approach is crucial for addressing the complex needs of EDS patients, ensuring they receive comprehensive care from diagnosis through rehabilitation. By working closely with a knowledgeable care team, EDS patients can achieve better outcomes and improved quality of life.

References

Broida, Samuel E., et al. "Management of Shoulder Instability in Hypermobility-Type Ehlers-Danlos Syndrome." JSES Reviews, Reports, and Techniques, vol. 1, no. 3, 2021, pp. 155-164, https://doi.org/10.1016/j.xrrt.2021.03.002.

Ericson, William B. Jr., and Roger Wolman. "Orthopaedic Management of the Ehlers-Danlos Syndromes." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 175, no. 2, 2017, pp. 188-194, https://doi.org/10.1002/ajmg.c.31551.

Kishan, Arman, et al. "Trends in Surgical Procedures for Shoulder Instability Among Patients with Ehlers-Danlos Syndrome or Joint Hypermobility Syndrome." JSES International, 2024, https://doi.org/10.1016/j.jseint.2024.08.178.

Manske, Robert C., and Daniel Prohaska. "Postoperative Rehabilitation Following a Novel Surgical Procedure to Treat Glenohumeral Instability in a Patient with Ehlers-Danlos Syndrome: A Case Report." JOSPT Cases, vol. 1, no. 4, 2021, pp. 233-239, https://doi.org/10.2519/josptcases.2021.10110.

Rogers, Thomas, et al. "Shoulder Arthroplasty is a Viable Option in Patients with Ehlers-Danlos Syndrome." Journal of Shoulder and Elbow Surgery, vol. 30, no. 11, 2021, pp. 2484-2490, https://doi.org/10.1016/j.jse.2021.03.146.

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