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 multidisciplinary 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