Surgical Considerations in hEDS

See below for Anesthesia

Surgical management of patients with hypermobile Ehlers-Danlos syndrome (hEDS) presents complex challenges across all stages of care, from preoperative planning to postoperative recovery. These considerations apply to surgeries directly related to hEDS, such as joint stabilization or decompression, as well as to common procedures like appendectomies. Understanding the unique risks associated with hEDS can significantly improve outcomes.

General Perioperative Risks and Considerations

People with hEDS often experience reduced effectiveness of local anesthetics. This means that medications like lidocaine may not work as well or may wear off too quickly, requiring higher doses or different techniques to achieve adequate numbness. This resistance is more common in hEDS than in the general population and may be related to differences in connective tissue or nerve sensitivity. As a result, standard anesthetic protocols may need to be adjusted to ensure proper pain control.

Bleeding Risk

• Increased due to capillary fragility and occasional platelet dysfunction

• Common signs: easy bruising, mucocutaneous bleeding, prolonged bleeding after surgery

• Consider antifibrinolytics (e.g., tranexamic acid) or desmopressin when indicated

• Preoperative hematology consultation recommended if bleeding history is concerning

Wound Healing and Tissue Fragility

• Delayed wound healing and atrophic scarring are more common

• Risk of wound dehiscence and infection higher, especially in orthopedic or abdominal surgeries

• Use meticulous surgical technique and possibly reinforced or mesh closures

• A recent matched cohort study in plastic surgery found no significant difference in wound complication rates between EDS (including hEDS) and controls

hEDS Specific vs. General Population Differences

The following chart outlines how hEDS-specific surgical and anesthetic considerations differ from the general population—such as increased bleeding, joint instability, anesthetic resistance, and need for multidisciplinary care.

Anesthesia and Intraoperative Concerns

• Joint dislocation during positioning or intubation is a risk

• Skin fragility complicates IV access and adhesive use

• Cervical instability may require modified airway management

• Regional/local anesthesia may be less effective

• Multimodal, opioid-sparing analgesia is preferred

Systemic and Comorbid Issues

• Dysautonomia (e.g., POTS) increases risk of hypotension or syncope

• GI dysmotility may delay recovery

• Chronic pain and fatigue affect pain management and rehabilitation

• Mast cell activation syndrome (MCAS) may lead to allergic-type reactions

• Sleep apnea and other systemic issues can increase postoperative complications

Subgroups at Highest Surgical Risk

Not all patients with hEDS face the same degree of surgical risk. Research shows certain groups have especially poor outcomes after surgery, and people in these groups should be carefully considered in preoperative planning.

• People with multiple health problems: Conditions like mast cell activation syndrome (allergic-type reactions), postural orthostatic tachycardia syndrome (POTS, a form of fainting/dizziness), anxiety, depression, or other autonomic issues (problems with heart rate and blood pressure control). These make complications and recovery more difficult.

• People with significant mental health challenges: Daily anxiety or depression is very common in hEDS and is linked to worse pain and slower recovery after surgery.

• Women: Most surgical studies include a majority of female patients, and this group reports higher rates of ongoing symptoms and complications.

• Patients with neurological problems: Conditions such as Chiari malformation (a problem where brain tissue extends into the spinal canal), craniocervical instability (instability where the skull meets the spine), severe headaches, or cerebrospinal fluid (CSF) leaks are associated with higher complication and revision rates.

• Children and adolescents: Ongoing growth, very flexible joints, and tissue fragility make them more prone to recurrent instability and surgical failure.

• Patients with fragile skin and poor wound healing: Classic features of hEDS — such as easy bruising, delayed healing, or wounds reopening (dehiscence) — make infection and hardware problems more likely.

Site-Specific Surgical Considerations

• Orthopedic Surgery

  • High complication and reoperation rates

  • Persistent pain, subluxations, and hardware-related issues common

  • Conservative treatment preferred before surgery

  • Arthroscopy may help but has variable results

• Abdominal and GI Surgery

  • Risk of wound dehiscence, anastomotic failure, and hernia recurrence

  • May require mesh reinforcement or diversion procedures (e.g., ileostomy)

  • hEDS linked to vascular compression syndromes like MALS and Nutcracker

• Oral and Maxillofacial Surgery

  • Risks: mucosal fragility, poor local anesthesia response, TMJ dysfunction

  • Bleeding and poor healing are common but not contraindications with proper technique

• Cervical Spine and Airway Surgery

  • Risk of cervical instability, Chiari malformation, and craniocervical abnormalities

  • Intubation may cause trauma (tracheal/esophageal rupture)

  • Techniques like myofascial flap closure help reduce CSF leaks

• Urogenital and Pelvic Surgery

  • Higher rates of prolapse recurrence, incontinence, and tissue tearing

  • Wound healing concerns may require surgical modification

Quantitative Outcomes Compared to General Population

Research studies confirm that people with hEDS face much higher surgical risks than the general population. Key findings include the following.

• High complication rates

  • Around 1 in 3 people who had joint surgery developed complications.

  • For spine surgery, the rate was closer to 2 in 5.

  • In one review of orthopedic surgeries, 9 out of 10 patients had at least one complication such as ongoing pain, joint instability, infection, or problems with surgical hardware.

• Frequent reoperations

  • The need for repeat surgery is very common.

  • Rates are especially high for the knee (about 60%) but are also elevated for the ankle, shoulder, elbow, and wrist (roughly one-third to one-half of patients).

  • By contrast, in the general population, only about 1 in 5 patients usually need another surgery.

• Joint replacements

  • After hip or knee replacement, people with hEDS are two to three times more likely to experience problems such as joint dislocation, loosening of the implant, wound issues, or the need for revision.

  • Even though some patients report pain relief and improved function, their artificial joints typically do not last as long as in people without hEDS.

• GI and abdominal surgery

  • Higher rates of complications such as bleeding, wound breakdown, or organ injury are reported.

  • For hernia repairs, special mesh techniques can lower the chance of recurrence, but wound healing problems are still frequent.

• Overall takeaway

  • Surgery in hEDS carries much higher risks than for people without the condition.

  • While some patients do feel better afterwards, complications and repeat surgeries are common, and non-surgical options remain the safer first approach whenever possible.

    
    

Pediatric Surgical Considerations

• Growth-Aware Surgical Planning

  • Account for skeletal growth and joint development

  • Use growth-friendly implants and avoid rigid fixation across open growth plates

  • Multidisciplinary input essential

  • Conservative management preferred when possible

• Caregiver Education

  • Joint protection strategies, adaptive devices, pain management education

  • Psychosocial and pacing strategies for both children and caregivers

• Age-Specific Rehabilitation

  • Play-based motor therapy (young children)

  • Ergonomic accommodations (school-aged)

  • Exercise education and safe activity plans (adolescents)

Importance of Surgeon Experience

Outcomes are improved when surgery is performed by experienced providers in connective tissue disorders. The following chart highlights leading physicians with hEDS surgical experience across orthopedics, neurosurgery, rheumatology, and nerve repair. It may or may not be accurate or complete. It is provided as a starting point for you to do your own research. Please do not rely on it but do your own due diligence. If you know of other surgeons, or would like to be included, please see the Contact page.

Notable Surgeons with hEDS Experience

Multidisciplinary Surgical Centers

These institutions offer coordinated care and experienced multidisciplinary surgical teams for hEDS. The following chart highlights leading surgical centers with hEDS surgical experience. It may or may not be accurate or complete. It is provided as a starting point for you to do your own research. Please do not rely on it but do your own due diligence. If you know of other surgical centers, or would like your surgical center to be included, please see the Contact page.

North American Surgery Centers for hEDS

These institutions offer coordinated care and experienced multidisciplinary surgical teams for hEDS. The following chart highlights leading surgical centers with hEDS surgical experience. It may or may not be accurate or complete. It is provided as a starting point for you to do your own research. Please do not rely on it but do your own due diligence. If you know of other surgical centers, or would like your surgical center to be included, please see the Contact page.

Healing and Long-Term Outcomes

• Healing is often slower with increased risk of recurrence

• Persistent pain, dissatisfaction, and poor scar formation are more common

• Psychological distress from prior care experiences may impair recovery

Pain Management and Opioid-Sparing Strategies

• Chronic pain is often centrally sensitized and opioid-resistant

• Opioid-induced hyperalgesia is a risk

• Recommended agents: lidocaine, ketamine, dexmedetomidine

• Non-pharmacologic therapies (PT, dry needling, CBT) are beneficial

Rare and Under-Recognized Complications

• Autonomic crises

• Anaphylaxis from MCAS

• Poor GI healing

• Immune vulnerability and fatigue flares

Preoperative Planning

• No hEDS-specific risk calculators exist

• Use detailed history and general tools (e.g., ASA-PS)

• Involve a multidisciplinary team early in the process

Perioperative Protocols and Technical Modifications

• Avoid overstretching joints

• Use tension-free closures

• Implement early mobilization

• Avoid opioids when possible

Postoperative Rehabilitation

• Requires hEDS-informed therapists

• Must address proprioception, fatigue, and pacing

• Pediatric rehabilitation must align with school and developmental needs

Emerging Therapies and Techniques

• Prolotherapy under study

• Advanced mesh techniques for hernias

• Minimally invasive procedures

• Extra substances used to help stop bleeding—such as the drug tranexamic acid or clot‑promoting powders and sponges applied directly to the surgical area.

Summary

Surgery in patients with hEDS requires tailored strategies to prevent complications from tissue fragility, autonomic dysfunction, chronic pain, and delayed healing. Multidisciplinary collaboration, surgeon expertise, and patient-centered rehabilitation are essential for safe and effective outcomes.

Recent studies confirm that complication rates are much higher than in the general population. For example, about 1 in 3 people experience complications after joint surgery, and up to 9 in 10 patients in some orthopedic series report problems such as pain, instability, or infection. The chance of needing repeat surgery can be as high as 60% for the knee and is also elevated for the ankle, shoulder, elbow, and wrist. Even after joint replacements, hEDS patients are two to three times more likely to face problems like dislocation or loosening compared to people without hEDS.

Certain subgroups are at especially high risk, including patients with multiple comorbidities (like POTS or MCAS), significant anxiety or depression, women, children and adolescents with ongoing growth, those with neurological conditions such as Chiari malformation or craniocervical instability, and individuals with fragile skin or delayed wound healing.

Because of these risks, conservative management — such as physical therapy, bracing, pain management, and multidisciplinary care — remains the first-line treatment. Surgery should be reserved for cases where non-surgical options have been exhausted and the expected benefits outweigh the substantial risks.

Anesthetic Considerations with hEDS

Anesthesia in individuals with hEDS presents unique challenges due to features such as resistance to local anesthetics, airway instability, dysautonomia (autonomic nervous system dysfunction), and chronic pain. This article outlines current knowledge on anesthetic efficacy, safety, perioperative complications, and management strategies for patients with hEDS.

hEDS Patients vs. General Population

People with hEDS often experience reduced effectiveness of local anesthetics. This means that medications like lidocaine may not work as well or may wear off too quickly, requiring higher doses or different techniques to achieve adequate numbness. This resistance is more common in hEDS than in the general population and may be related to differences in connective tissue or nerve sensitivity. As a result, standard anesthetic protocols may need to be adjusted to ensure proper pain control.

Efficacy (effectiveness) of Anesthetic Agents in hEDS

• Local Anesthetics

  • Many individuals with hEDS report poor response to standard doses of local anesthetics (e.g., lidocaine), particularly for dental and minor procedures

  • Resistance to local anesthetics is more common in hEDS. Patients frequently report incomplete numbing despite adequate technique. Some may benefit from higher doses or alternative agents

  • May require higher doses or alternative medications

  • Likely related to altered connective tissue, increased tissue permeability, or nerve differences

  • No evidence-based dosing guidelines exist

• Regional Anesthesia (e.g., peripheral nerve blocks)

  • Reduced effectiveness in some patients with hEDS

  • Regional blocks have a higher failure rate in hEDS and do not consistently reduce postoperative pain

  • Cases of block failure and persistent postoperative pain reported despite technically successful catheter placement

  • Reassuringly, no increased rate of complications such as hematoma or nerve damage

• General Anesthesia (e.g., intravenous or inhaled agents)

  • No known pharmacologic resistance to agents like propofol or sevoflurane

  • Safety can be affected by

    • Craniocervical instability (instability at the skull and spine junction which is common in hEDS) can cause neurological complications during airway manipulation. Refer to “hEDS vs. General Population” chart: airway management is a notable difference.

    • Airway abnormalities

    • Dysautonomia (e.g., orthostatic intolerance, erratic blood pressure) leads to greater perioperative instability, necessitating hemodynamic monitoring and vasopressor support

Patient-Reported Outcomes

• Patients with hEDS often report

  • Local and regional anesthesia as less effective

  • Better results with opioids, surgery, bracing, and heat therapy

  • Greater dependence on systemic pain medications and non-drug therapies

Safety Concerns and Perioperative (the period around an operation) Complications

• Airway Management

  • Temporomandibular joint (TMJ) dysfunction may limit mouth opening

  • Laryngeal inflammation, swallowing difficulty (dysphagia), and voice changes (dysphonia) more common

  • Craniocervical instability increases risk during intubation—use in-line stabilization

• Vascular Fragility and Bleeding Risk

  • Easy bruising and soft tissue bleeding are common

  • Surgical bleeding, hematomas, and prolonged wound drainage occur more often

  • Some may benefit from preoperative evaluation and medications such as desmopressin (DDAVP®, used to promote clotting)

• Joint Dislocation and Positioning Injury

  • Joint dislocations or subluxations (partial dislocations) may occur during:

    • Transfers

    • Positioning

    • Traction

  • Use extra padding and avoid stretching or extreme joint positions

• Autonomic Nervous System Dysfunction (Dysautonomia)

  • Symptoms include

    • Postural orthostatic tachycardia syndrome (POTS)

    • Neurally mediated hypotension (fainting due to nervous system reflexes)

    • Labile (unstable) blood pressure and heart rate

  • Requires careful monitoring and possible use of IV fluids and vasopressors

• Chronic Pain and Opioid Sensitivity

  • Pain often results from central sensitization (increased sensitivity in the brain and spinal cord)

  • May respond poorly to opioids or develop opioid-induced hyperalgesia (worsening pain from opioids)

  • Multimodal strategies using medications like ketamine, lidocaine, and dexmedetomidine are often more effective

• Additional Complications

  • Fragile skin may lead to pressure sores and wound complications

  • Mast cell activation syndrome (MCAS) may cause allergic-type reactions during surgery

  • Sleep apnea may interfere with postoperative breathing

Demographic and Population Considerations

• Pediatric vs. Adult Patients

  • No significant differences in anesthetic risks between children and adults with hEDS

  • Pediatric spine surgery complication rates are not higher in patients with Ehlers-Danlos syndrome compared to others

  • Standard pediatric dosing adjustments should still apply

• Sex and Comorbidities

  • hEDS is more frequently diagnosed in females

  • Common comorbidities include

    • Postural orthostatic tachycardia syndrome (POTS)

    • Mast cell activation syndrome (MCAS)

    • Anxiety and depression

    • Gastroesophageal reflux disease (GERD)

    • Temporomandibular joint dysfunction (TMJ)

  • No data yet on racial or ethnic variation due to lack of studies

Guidelines and Expert Consensus

• No formal guidelines from anesthesia societies exist

• GeneReviews (a medical reference for genetic disorders) provides the most authoritative summary

• Key recommendations include

  • Expect resistance to local anesthesia

  • Prepare for airway and joint-related challenges

  • Use multidisciplinary teams

hEDS Specific vs. General Population Differences

Rare and Emerging Complications

• Opioid-induced hyperalgesia

• Central sensitization-related pain

• Mast cell-related allergic reactions

• Nerve injury from unstable neck joints (craniocervical instability)

• Acute autonomic crises (blood pressure and heart rate instability)

• Psychiatric complications (e.g., anxiety flare-ups)

• Rare vascular events (e.g., arterial dissections)

• Ocular and dental surgery complications

Perioperative Management Strategies

• Opioid-Minimization and Multimodal Analgesia

  • Avoid or reduce opioid use

  • Central sensitization contributes to poor opioid efficacy and heightened pain sensitivity (opioid-induced hyperalgesia)

  • Patient feedback suggests better relief from systemic therapies, bracing, and heat therapy than from local anesthetics alone

  • Use combinations of medications like

    • Ketamine (blocks pain and reduces opioid need)

    • Lidocaine (local anesthetic with systemic pain benefits)

    • Dexmedetomidine (sedative and pain reliever)

• Enhanced Recovery After Surgery (ERAS)

  • Includes short-acting anesthetics

  • Customized fluid and blood pressure management

  • Multidisciplinary planning and multimodal pain relief

• Preoperative Planning

  • Evaluate airway, bleeding history, joint stability

  • Identify autonomic or mast cell dysfunction

  • Team approach for optimal care

• Hemodynamic Monitoring

  • Watch for blood pressure instability during surgery

  • Use IV fluids and medications as needed

• Managing Bleeding Risk

  • Continue usual medications to avoid withdrawal

  • Consider tranexamic acid (to reduce bleeding) in high-risk cases

• Choosing Anesthetic Technique

  • Both inhaled and intravenous anesthetics acceptable (per American College of Cardiology 2024)

  • Must consider procedure type, joint status, and chronic pain profile

• Non-Drug Pain Therapies

  • Physical and occupational therapy

  • Dry needling

  • Cognitive behavioral therapy

  • Prolotherapy (injection therapy for tissue repair)

Risk Management Strategies

• Use enhanced recovery protocols customized to hEDS

• Expect resistance to local anesthetics and plan accordingly

• Carefully position joints and avoid hyperextension or overstretching

• Monitor for mast cell-related reactions and bleeding complications

• Ensure anesthesia teams are briefed on comorbidities (POTS, MCAS, GI dysmotility)

• Pediatric dosing and risk appear similar to adults but should be individualized

Surgical Outcomes

• Higher Risks

  • Implant failure and persistent joint problems more common

  • Reoperation rates are higher

• Variable Success

  • Some patients benefit from careful planning and experienced surgical teams

Patient-Reported Satisfaction

• Individuals with hEDS report

  • Lower satisfaction with anesthesia care

  • Frustration with inadequate pain control, provider misunderstanding, and delayed diagnoses

• General population reports greater satisfaction overall

Costs and Healthcare Use

• No direct cost-effectiveness studies comparing hEDS to general population

• Suggestive evidence of

  • Higher overall use of supportive care

  • Greater need for multidisciplinary interventions

Gaps and Limitations in the Literature

• Few controlled trials specific to hEDS

• Lack of

  • Standardized dosing studies

  • Complication rate data

  • Clinical guidelines

  • Diverse patient representation

• More research needed

  • Prospective studies

  • Registries and clinical trials

  • Pain management investigations

Summary

Anesthesia in people with hEDS presents unique challenges due to tissue fragility, autonomic dysfunction, and altered pain sensitivity. Local anesthetics are often less effective, likely due to differences in tissue structure or nerve response, and airway management can be risky because of TMJ dysfunction and craniocervical instability. Dysautonomia, such as POTS, may cause unstable blood pressure and heart rate during surgery, requiring careful monitoring.

Pain is often amplified by central sensitization, making opioids less effective and sometimes harmful. Multimodal, opioid-sparing strategies—using agents like ketamine, lidocaine, and dexmedetomidine—are preferred, along with non-drug therapies. Additional concerns include increased bleeding risk, skin fragility, and reactions from mast cell activation syndrome (MCAS). Though general anesthetics remain effective, individualized, multidisciplinary planning is essential for safe and effective care.