Anesthesia Considerations 

Ehlers Danlos 

 

 

Background 

 

• A type of connective tissue disorder 

• Characterized by skin hyperextensibility, joint hypermobility, & tissue fragility

• Classified into 6 types: classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis

  • ​Vascular type associated with ↑ risk of death 

 

 

Considerations 

 

• Multisystem disease:

  • High risk of bleeding & friable tissue → affects ETT placement, regional techniques, invasive lines 

    • ​Desmopressin useful if bleeding 

  • Cardiovascular: mitral regurgitation, proximal aortic dilatation, conduction abnormalities,  

  • Spontaneous ruptures: bowel, uterus, or major arteries 

  • Skin laxity/fragility & joint hypermotility

• ↑ risk pneumothorax: keep low airway pressures 

• Regional anesthesia is relatively contraindicated due to hematoma risk 

 

 

Pregnancy Considerations

 

• Very difficult management, requires multidiscplinary effort 

• High risk of preterm labor, uterine rupture, & hemorrhage 

• Neuraxial relatively contraindicated, may need GA 

• If vascular type, recommendations are either termination of pregnancy or cesarean section before 32 weeks