Ehlers-Danlos syndromes

Ehlers-Danlos syndromes

Anesthetic relevance
Anesthetic management	{{{anesthetic_management}}}
Specialty
Signs and symptoms
Diagnosis
Treatment
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Ehlers-Danlos (EDS) is a set of heritable heterogenous connective tissue disorders characterized by defects in collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organ. EDS patients often have vascular fragility syndromes with arterial aneurysms and dissections. Patients also have kyphoscoliosis and due to the connective tissue abnormalities, these patients often present for orthopedic surgery. Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.

Anesthetic implications

Preoperative optimization

Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency

Intraoperative management

Positioning:

• Eyes should be protected to present retinal detachment and globe rupture
• Careful padding of extremities

Skin Fragility

• Minor forces can result in skin injuries such as tape, stickers, and other monitors that attach to the patient
• Bruising and hematoma formation occurs with non-invasive BP monitoring