Ehlers Danlos
Background
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A type of connective tissue disorder
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Characterized by skin hyperextensibility, joint hypermobility, & tissue fragility
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Classified into 6 types: classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis
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Vascular type associated with ↑ risk of death
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Considerations
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Multisystem disease:
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High risk of bleeding & friable tissue → affects ETT placement, regional techniques, invasive lines
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Desmopressin useful if bleeding
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Cardiovascular: mitral regurgitation, proximal aortic dilatation, conduction abnormalities
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Spontaneous ruptures: bowel, uterus, or major arteries
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Skin laxity/fragility & joint hypermotility
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↑ risk pneumothorax: keep low airway pressures
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Regional anesthesia is relatively contraindicated due to hematoma risk
Pregnancy Considerations
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Very difficult management, requires multidisciplinary effort
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High risk of preterm labor, uterine rupture, & hemorrhage
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Neuraxial relatively contraindicated, may need GA
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If vascular type, recommendations are either termination of pregnancy or cesarean section before 32 weeks