Guidelines for Facial Plastic Surgery in Patients with Ehlers-Danlos Syndrome

Introduction:

‍Ehlers-Danlos Syndromes (EDS) are a group of hereditary connective tissue disorders that affect collagen and other structural components of the skin, joints, and blood vessels. Because collagen is a key structural protein, its dysfunction in EDS leads to increased skin fragility, joint hypermobility, and poor wound healing. These traits pose unique challenges in surgical procedures, especially facial plastic surgery, where cosmetic outcomes are closely tied to tissue healing. Surgeons must consider a range of factors from preoperative planning to postoperative care to minimize complications and maximize patient satisfaction. This guide provides a comprehensive overview of best practices for facial plastic surgery in EDS patients, with a focus on preventing complications like scarring, tissue breakdown, and poor wound healing.

Preoperative Considerations:

1. Comprehensive Medical and Genetic Evaluation:
   • Diagnosis Confirmation: Before proceeding with surgery, it’s critical to confirm whether the patient has EDS and, if so, determine which subtype. Classical and hypermobile EDS subtypes are most associated with skin fragility and joint hypermobility, while vascular EDS poses a high risk of severe complications due to vascular fragility. Genetic testing is often necessary, particularly for vascular EDS, as patients with this subtype are at significant risk for life-threatening hemorrhage during surgery​​.
   • Multidisciplinary Team Approach: In complex cases, collaboration with a geneticist, cardiologist, and rheumatologist may be necessary to fully assess the risks. A thorough cardiovascular assessment, including echocardiography, should be conducted for patients suspected of having vascular EDS, as they are prone to arterial rupture​​.
2. Patient Education and Expectations:
   • Managing Expectations: EDS patients should be informed about the inherent risks associated with surgery, particularly related to poor healing, widened scars, and the possibility of repeat surgeries. Given the high probability of delayed wound healing, patients may not achieve the same aesthetic outcomes as non-EDS individuals​.
   • Counseling for Realistic Outcomes: Surgeons should clearly communicate that results may be less predictable, and healing may take longer. Scars may remain more visible or wider, and patients should prepare for the potential need for future revisions​.
   • Emphasis on Non-Surgical Alternatives: Patients should be advised to explore non-surgical treatments or conservative approaches where possible, particularly if the goal is primarily aesthetic. Skin treatments like fillers or laser therapies may be preferable to surgical interventions​.
3. Detailed Medical History:
   • Joint and Cervical Stability: Patients with hypermobile EDS (hEDS) often experience joint instability, particularly in the cervical spine. Before surgery, surgeons should assess for any history of neck pain, dislocations, or spinal issues, as these can impact both surgical positioning and anesthetic management​.
   • Anesthetic Risks: Patients with EDS are more susceptible to complications from anesthesia, including joint dislocations during intubation or positioning. A specialized anesthesiology team familiar with EDS should be involved in the surgical planning​.

Intraoperative Considerations:

1. Tissue Handling and Surgical Technique:
   • Minimizing Trauma: Tissue fragility is a hallmark of EDS, so surgeons should use atraumatic surgical techniques. Instruments that exert excessive tension should be avoided, and retractors should be applied gently to prevent tearing of delicate tissues​.
   • Suturing Techniques: Given the fragility of EDS skin, wound closure should involve layered sutures to distribute tension evenly. Fine, non-reactive sutures like nylon or polypropylene may be preferred for skin closure. Deeper suturing techniques should be used to provide internal support and reduce tension on the skin surface​. Suture retention time should be extended to promote stronger healing, as removing sutures too early may increase the risk of wound dehiscence​.
2. Hemostasis and Avoiding Bleeding Complications:
   • Vascular Fragility: Patients with vascular EDS are at a particularly high risk for intraoperative bleeding. Surgeons should take extreme care in achieving hemostasis, using minimal pressure and avoiding sharp dissection near blood vessels. The use of electrocoagulation or bipolar cautery can reduce bleeding, but care must be taken to avoid damage to fragile tissues​​.
   • Conservative Dissection: Minimal dissection should be done in areas prone to bleeding, and incisions should follow natural skin creases to reduce the tension on the wound during healing​.
3. Careful Use of Anesthesia:
   • Cervical Spine and Joint Protection: Anesthesiologists should be aware of the potential for joint dislocations, particularly in the cervical spine and jaw. Careful positioning is essential to prevent overstretching of joints during surgery​.
   • Local Anesthetic Considerations: Some EDS patients exhibit resistance to local anesthetics, possibly due to abnormal collagen affecting nerve tissue. This may require adjustments in the type or dosage of anesthetic agents used​.

Postoperative Care:

1. Wound Monitoring and Extended Healing Time:
   • Prolonged Suture Retention: Sutures in EDS patients should be left in place longer than usual (often twice the standard duration) to ensure the wound has sufficient time to heal and minimize the risk of dehiscence​. Sutures should be removed gradually to prevent sudden reopening of the wound.
   • Regular Follow-Up: Frequent postoperative evaluations are necessary to monitor for complications like infection, wound breakdown, or excessive scarring. Early signs of wound failure should prompt intervention, including wound revision or reinforcement with additional sutures​.
2. Scar Management:
   • Topical Treatments: Once the wound has healed, scar-reducing therapies such as silicone sheets, steroid injections, or laser treatments may help improve the cosmetic appearance of scars, although their effectiveness can be limited in EDS patients​.
   • Non-Surgical Revision: In cases where scarring is severe, non-invasive options such as dermal fillers or microneedling may help reduce the appearance of scars without subjecting the patient to further surgery​.
3. Pain Management:
   • Chronic Pain Considerations: EDS patients are prone to chronic pain due to joint instability and tissue fragility. Postoperative pain management should be tailored to avoid exacerbating joint or muscle issues. Non-opioid pain relief options are preferable where possible, as these patients may require long-term pain management plans​.
4. Long-Term Surveillance:
   • Risk of Relapse and Revisions: Given the nature of EDS, the skin and connective tissue remain fragile long after surgery. Patients may require additional interventions over time, and the likelihood of revision surgery should be communicated clearly from the outset​​.

Conclusion:

Facial plastic surgery in EDS patients requires a careful, conservative approach. Surgeons must account for the unique challenges posed by fragile tissues, poor wound healing, and a high risk of complications such as bleeding or wound dehiscence. Thorough preoperative planning, meticulous intraoperative care, and close postoperative monitoring are essential to optimizing outcomes. Moreover, patient education and managing expectations are critical, as aesthetic results may not match those of individuals without EDS. By integrating multidisciplinary care and tailoring surgical techniques to the needs of EDS patients, it is possible to achieve improved outcomes while minimizing the risk of complications.

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Reference

Mitakides, John, and Brad T. Tinkle. "Oral and Mandibular Manifestations in the Ehlers-Danlos Syndromes." American Journal of Medical Genetics Part C: Seminars in Medical Genetics, vol. 175, no. 1, 2017, pp. 220-225.