Surgery in Ehlers-Danlos Syndrome: Risks, Considerations, and Recovery

Navigating Orthopedic Surgery in Ehlers-Danlos Syndrome: Risks, Considerations, and Recovery

Orthopedic surgery can be a challenging decision for individuals with Ehlers-Danlos Syndrome (EDS), particularly those with hypermobile EDS (hEDS) or Hypermobility Spectrum Disorders (HSD). While surgery may sometimes be necessary to address joint instability or nerve compression, it is generally considered a last resort after all non-invasive treatment options, such as physiotherapy, have been exhausted. Here’s an in-depth look at the considerations, risks, and special precautions associated with orthopedic surgery for EDS patients.

The Role of Physiotherapy Before Surgery

For most individuals with hEDS or HSD, physiotherapy is the cornerstone of managing joint instability and pain. A skilled physiotherapist can help strengthen muscles and stabilize joints, teaching patients techniques to protect their joints by avoiding hyperextension, which can lead to long-term traumatic arthritis. The goal is to maintain movements within a normal range of motion, similar to those who are not hypermobile​.

When Surgery Becomes Necessary

Surgery might be considered when conservative treatments like physiotherapy fail to provide relief, or in cases of severe joint dislocations, fractures, or nerve compression. However, due to the nature of EDS, surgery on soft tissues alone often provides only temporary relief. The soft tissues may stretch again, causing the original problem to recur. Therefore, surgical interventions in EDS often combine soft tissue procedures with bone surgeries, such as joint stabilization or arthrodesis (joint stiffening), and in severe cases, joint replacement may be necessary​.

Preoperative Considerations

Before undergoing surgery, a comprehensive evaluation is crucial. This assessment should include a review of potential cardiac, pulmonary, and bleeding issues, as these are common concerns in EDS patients:

• Cardiac Concerns: Mitral valve prolapse, common in EDS, may require antibiotic prophylaxis to prevent infection.
• Pulmonary Issues: Patients with spinal curvatures might have restricted lung capacity, increasing the risk of lung collapse during surgery.
• Bleeding Disorders: The fragile blood vessels in EDS patients increase the risk of excessive bleeding, making careful planning essential​ .

Anesthesia and Perioperative Management

Anesthesia management in EDS patients requires special attention due to the condition’s impact on various body systems. For example:

• Anesthetic Challenges: Local anesthetics might be less effective in EDS patients, particularly those with hEDS. This can complicate pain management during and after surgery. It’s important to discuss your past experiences with local anesthetics with your anesthesiologist, particularly if dental nerve blocks have been ineffective for you.
• General Anesthesia Risks: General anesthesia is generally safe but requires careful monitoring. Mask ventilation and intubation should be performed with caution to avoid temporomandibular joint dislocation or cervical spine instability.
• Epidural and Spinal Anesthesia: Although there are concerns about increased bleeding risks with epidural anesthesia, it has been used successfully in EDS patients, particularly during childbirth. However, this should be discussed in detail with your anesthetist .

For more detailed guidance, refer to the recommendations on anesthesia management in EDS, which cover all EDS subtypes and emphasize the need for careful perioperative planning .

Intraoperative Care

During surgery, several precautions must be taken to accommodate the unique needs of EDS patients:

• Tissue Handling: The surgeon must be extremely careful when handling tissues, as EDS patients have fragile skin and connective tissues. A multilayered closure technique is recommended to reduce tension on the skin and improve wound healing. Wounds should be closed with subcuticular stitches and steristrips, which should be left in place longer than usual to allow for delayed healing.
• Joint Protection: Care should be taken when positioning the patient on the operating table to avoid dislocations or nerve injuries. Intramuscular injections should be avoided due to the high risk of bruising​.

Postoperative Recovery and Rehabilitation

Successful recovery from surgery in EDS patients depends heavily on a carefully tailored rehabilitation program. Unlike standard postoperative protocols, which often emphasize aggressive range-of-motion exercises, EDS patients should avoid stretching repaired tissues excessively. Instead, focus should be on maintaining joint stability without overextending the repaired areas:

• Pain Management: Postoperative pain management should be individualized, especially given the potential resistance to local anesthetics in some EDS patients.
• Rehabilitation: Rehabilitation should avoid exercises that place undue stress on the joints, such as open kinetic chain exercises where the limb moves freely without ground contact. Intensive physiotherapy may be required to ensure the full benefits of the surgery are realized​ .

Conclusion: Surgery as a Last Resort

Surgical intervention in EDS, especially in hypermobile EDS and HSD, is fraught with challenges and should be considered only after all conservative treatments have been exhausted. The decision to undergo surgery should be made in full consultation with a multidisciplinary team, ensuring that all potential risks and benefits are thoroughly discussed and understood. With proper planning, specialized care, and tailored rehabilitation, surgery can lead to positive outcomes, although it remains a complex and high-risk option .

References

1. Hashemi-Nejad, Aresh, and Ivor Vanhegan. "Orthopaedic Surgery in Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders." Royal National Orthopaedic Hospital, 1 Apr. 2016. Link
2. Yonko, Elizabeth A., et al. "Orthopedic Considerations and Surgical Outcomes in Ehlers-Danlos Syndromes." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 187, no. 4, 2021, pp. 458-465. doi:10.1002/ajmg.c.31958.
3. Wiesmann, Thomas, et al. "Recommendations for Anesthesia and Perioperative Management in Patients with Ehlers-Danlos Syndrome(s)." Orphanet Journal of Rare Diseases, vol. 9, 2014, p. 109, doi:10.1186/s13023-014-0109-5.

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