Special Considerations for Surgery & Anesthesia in EDS
The essentials everyone needs to know
Surgical and anesthetic complications in Ehlers-Danlos Syndromes (EDS) are more common than many realize, and understanding the unique challenges of EDS patients can make a significant difference in outcomes. Whether you're a patient preparing for a procedure, a caregiver advocating for a loved one, or a provider seeking to improve care, this information is crucial. Both EDS and HSD (Hypermobility Spectrum Disorders) affect multiple body systems, which means that standard surgical and anesthesia protocols may not be effective—or could even be harmful. Knowing what to expect, what to discuss with a medical team, and how to prepare can help ensure safer, smoother procedures. Share this Bendy Bulletin with your medical team to help reduce the risk of perioperative complications.
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Whether you're a patient or a provider, this 4-day virtual event, New Integrative Ways to Manage Pain, offers a powerful lineup of lectures focused on pain medicine, including:
Healing Pelvic Pain
Regenerative Pain Medicine: Platelet Rich Plasma
Cannabis, Cannabinoids, and Pain
Neuroplastic Pain: When Pain Hijacks Normal Brain Function
...and much more
Dr. Linda Bluestein, MD will be presenting: Flexible Solutions: Integrative Pain Medicine for Hypermobility Disorders — a must-see session for anyone managing or treating complex pain conditions.
Event Details:
Dates: June 3–6, 2025
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Live ticket: $99
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Why Anesthesia Considerations Matter
EDS is more than just joint hypermobility—its impact on connective tissue means that patients experience complications that can be difficult to predict and manage. Without proper awareness and preparation, they may face increased risks of surgical complications, prolonged recovery times, and ineffective pain management. By recognizing these unique challenges, medical teams can improve surgical safety, reduce complications, and provide better care. For patients and caregivers, understanding these risks can empower them to advocate for themselves or their loved ones, ensuring that proper precautions are taken.
Preoperative Considerations
A thorough preoperative history and physical examination are essential. Identifying the specific EDS subtype whenever possible helps surgical planning. For example, while classical EDS (cEDS) is distinct from vascular EDS (vEDS), some cEDS patients may present with vascular anomalies typically associated with vEDS, such as aortic root dilation.
Joint Stability and Positioning: Many patients with EDS and HSD experience unstable joints. Whether joints fully dislocate or sublux (partial dislocation), identifying joint instability allows for proper intraoperative and postoperative positioning to reduce the risk of injury.
Craniocervical and Spinal Considerations: Many patients have craniocervical instability (CCI) or atlantoaxial instability (AAI) which means that the upper cervical spine (neck) is unstable. The presentation can be subtle so point this out to your anesthesiologist even if it is suspected and not yet diagnosed. Chiari malformation, idiopathic intracranial hypertension or hypotension, and scoliosis also require careful positioning and anesthesia planning.
Airway Management: Temporomandibular joint dysfunction (TMD) is common in people with symptomatic joint hypermobility. Younger patients often experience frequent subluxations or dislocations, while older patients may develop restricted motion due to arthritis.CCI and AAI also impact airway management. Fiberoptic intubation or video laryngoscopy help minimize cervical spine manipulation and strain on fragile joints.
Pulmonary Concerns: Obstructive sleep apnea and tracheomalacia (collapse of the trachea or windpipe) can be seen in EDS and HSD patients. A history of spontaneous pneumothorax (collapsed lung) should be elicited, and preoperative pulmonary (lung) function testing may be necessary in those with significant scoliosis or kyphosis. Lower airway pressures during positive pressure ventilation (being on a ventilator) help reduce the risk of pulmonary injury.
Intraoperative Considerations
Anesthetic Sensitivity: EDS patients often exhibit resistance to local anesthetics or experience atypical responses, including prolonged or diminished effects. Mepivacaine may be a better alternative to lidocaine. Be sure to share your prior local anesthetic history with your anesthesiologist.
Pain Management: Opioid resistance is frequently reported, necessitating multimodal pain strategies. Certain opioids, like morphine and meperidine, cause histamine release whereas others are much less likely to do so (e.g.: fentanyl). Neuropathic and chronic pain are common which influence the ability to obtain good pain control. Low doses of ketamine (a dissociative anesthetic) may be beneficial in reducing opioid requirements and mitigating gastroparesis (slow gastric emptying) while minimizing histamine release.
Dysautonomia and Circulatory Support: Postural orthostatic tachycardia syndrome (POTS) and other forms of dysautonomia require careful intraoperative monitoring. These patients may need early IV placement, fluid resuscitation with electrolyte-rich solutions, vasopressors (medications that raise blood pressure), and/or beta blockers (medications that block the effects of epinephrine and norepinephrine) to maintain hemodynamic stability.
Mast Cell Activation Syndrome (MCAS): Patients with MCAS may react unpredictably to anesthetic agents. Common triggers of mast cell degranulation include opioids, nonsteroidal anti-inflammatory drugs (NSAIDs), neuromuscular blocking agents, and radiologic contrast media. Preoperative and intraoperative management should include H1 and H2 antihistamines, mast cell stabilizers, and corticosteroids if necessary. Slow IV administration of medications like diphenhydramine can help prevent adverse reactions.
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Postoperative Considerations
Pain Control: Due to variable responses to opioids, a multimodal approach including neuropathic pain medications and ketamine may be more effective.
Gastrointestinal Management: Gastroparesis is common and can increase the risk of postoperative nausea and vomiting. Liberalizing NPO guidelines (eating and drinking restrictions before surgery or procedures) for clear liquids (e.g.: water and plain jello) should be considered to reduce hypovolemia (inadequate fluid in the body) risk.
Orthostatic Intolerance: POTS symptoms may worsen postoperatively. Patients should be monitored for tachycardia (fast heart rate), hypotension (low blood pressure), and electrolyte imbalances.
Risk of Post-Dural Puncture Headache (PDPH): EDS patients are more prone to PDPH following neuraxial (spinal or epidural) anesthesia. They can experience spontaneous CSF leaks as well. Careful selection of needle size, tip shape, and technique is essential to minimize this risk.
Final Thoughts
Surgical and anesthetic management of EDS and HSD patients requires a multidisciplinary approach. Recognizing the unique challenges posed by connective tissue fragility, joint instability, dysautonomia, MCAS, and pain variability allows for better perioperative planning and improved patient outcomes.
Perioperative Care in Patients with Ehlers Danlos Syndromes
Read the peer-reviewed journal article by Drs. Bluestein and Chopra that inspired today’s newsletter:
Check out our Instagram graphic about anesthesia and Ehlers-Danlos Syndromes (EDS) inspired by their research.
LAST THURSDAY, April 17th 2025: Episode 141 with Melissa Dickinson and Tom Query
We were thrilled to host Melissa Dickinson, Licensed Professional Counselor and Chronic Illness Specialist, and Tom Query, Psychotherapist, Change Agent, and Coach, on the podcast. Together, they bring both professional expertise and lived experience to a thoughtful conversation about marriage, Ehlers-Danlos Syndrome (EDS), and neurodivergence. In this powerful episode, they explore the emotional landscape of relationships shaped by chronic illness—and share compassionate, practical strategies for building connection, resilience, and understanding.
THIS THURSDAY, April 24th 2025: Episode 142 with Dr. Linda Bluestein
In this solo episode, I’m answering your most pressing questions, including how to approach cosmetic treatments like microdermabrasion, fillers, Botox, and face lifts when living with EDS. I’ll also explore some fascinating studies on the connection between hypermobile EDS and transgender or gender-diverse populations, offering new insights into this emerging area of research.
Well that’s all for this week; thank you so much for reading and supporting this newsletter. Talk to you soon, Bendy Buddies!
Yours in Health,
Dr. Bluestein
Excellent post. I was just diagnosed with hEDS after yet another post surgical complication, and I finally understand what's going on inside of my body.