How is Ehlers-Danlos syndrome (EDS) relevant to surgery?

Ehlers-Danlos Syndrome Considerations in Surgery

Surgical interventions in patients with Ehlers-Danlos syndrome (EDS) carry significant risks due to tissue fragility, vascular vulnerability, and delayed wound healing, requiring meticulous surgical technique and specialized perioperative management to prevent complications. 1

Types of EDS with Surgical Implications

• Vascular EDS (Type IV) - Most concerning for surgical interventions due to:

  • Spontaneous aortic and arterial dissections, aneurysms, and rupture at young ages 1
  • Vascular fragility leading to increased bleeding complications 1, 2
  • Vessels may rupture or dissect even without significant dilation 1
  • Surgical repair carries increased risk due to tissue fragility 1, 3

• Classical and Hypermobile EDS - Present challenges including:

  • Tissue fragility causing wound dehiscence 4
  • Delayed wound healing after primary approximation 4
  • Higher rate of post-operative complications compared to general population 5

Preoperative Considerations

• Comprehensive vascular assessment is essential before any surgical intervention 3, 6

  • Baseline imaging from head to pelvis to evaluate entire aorta and branches 1
  • Non-invasive imaging (MRI/CT) strongly preferred over invasive procedures 3

• Genetic testing should be considered for definitive diagnosis, particularly for identification of causal variants in vascular EDS (COL3A1) 3, 6

• Multidisciplinary approach involving specialists in cardiology, vascular surgery, and anesthesiology 3, 6

Intraoperative Management

• Meticulous surgical technique is required to minimize tissue trauma 1, 4

  • Careful tissue handling to prevent wound dehiscence 4
  • Pledgeted sutures for anastomoses in vascular procedures 3

• Hemostatic management requires special attention 2

  • Increased bleeding tendency due to blood vessel fragility 4, 2
  • May require more aggressive hemostatic measures during surgery 2, 7

• Anesthesia considerations

  • Some patients may show resistance to local anesthetics 4
  • Careful blood pressure control is essential 6

Postoperative Complications to Anticipate

• Wound complications are common 4, 5

  • Delayed healing 4
  • Dehiscence due to decreased tissue strength 4
  • Aberrant scarring 4

• Vascular complications

  • Higher risk of hematoma formation 4
  • Potential for delayed bleeding 2

• Other common complications reported in orthopedic surgeries 5

  • Persistent pain/discomfort (most common) 5
  • Continued joint subluxation/dislocation 5
  • Instability 5
  • Pain from hardware 5
  • Infection 5

Special Surgical Scenarios

• Vascular interventions should be approached with extreme caution 1

  • Open surgery requires techniques to lessen vascular and tissue trauma 1
  • Interventional techniques may involve arterial embolization and endovascular therapy 1
  • Decision to intervene should involve a Multidisciplinary Aortic Team 1

• Orthopedic surgery has reported high complication rates 5

  • 91% of cases reported post-operative complications in one study 5
  • Careful pre-operative planning and full knowledge of risks is essential 5

• Breast reconstruction can be successful with proper precautions 7

  • Meticulous intraoperative hemostasis is critical 7
  • May experience more than average bleeding during procedures 7

• Spine surgery in pediatric EDS patients 8

  • Recent data suggests complication rates may not be significantly higher than non-EDS patients 8
  • Blood transfusions were required in 41% of EDS patients 8

Long-term Follow-up

• Regular follow-up with a multidisciplinary team is essential 3, 6
• Annual surveillance imaging for vascular EDS patients to monitor any dilated or dissected aortic or arterial segments 1
• Extended monitoring for delayed complications 5

Common Pitfalls to Avoid

• Invasive diagnostic procedures in vascular EDS patients should be avoided whenever possible 3, 6
• Underestimating tissue fragility can lead to surgical complications 4
• Inadequate hemostasis during surgery can result in significant bleeding 2, 7
• Insufficient postoperative monitoring may miss delayed complications 5