Ehlers-Danlos syndromes

Ehlers-Danlos syndromes

Anesthetic relevance
Anesthetic management	{{{anesthetic_management}}}
Specialty
Signs and symptoms
Diagnosis
Treatment
Article quality
Editor rating	Unrated
User likes	0

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. Overall, there is very little evidence-based knowledge for the management of EDS. Much of the literature is based upon expert opinion.

Anesthetic implications

Preoperative optimization

• Preoperative evaluation should focus on bleeding history, cervical/TMJ hypermobility, skin fragility, scoliosis, signs of mitral/aortic insufficiency
• Aortic root dilation may exacerbate aortic insufficiency
• EDS patients can have both aortic and mitral insufficiency

Intraoperative management

Monitoring:

• Arterial lines run the risk of vascular wall dissection. Consider ultrasound placements for EDS patients
• EDS patients can often demonstrate orthostatic tachycardia syndrome (POTS)

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 repetitive non-invasive BP monitoring
• Special attention to removing tape from eyes and skin
• Discuss the risk/benefits of using a tourniquet in these patients with surgical team

Airway Management:

• Care should be taken for TMJ joint laxity during laryngoscopy
• EDS patients may present with occipitalatlantoaxial instability causing TMJ joint dislocation

Postoperative management

• Early mobilization is helpful to prevent muscle loss
• Inspect surgical sites for hematomas or bleeding
• Patients with POTS should be monitored early after surgery for bleeding and cardiovascular instability
• Treat PONV aggressively to avoid esophageal rupture from violent vomiting
• EDS patients can have pneumothorax after positive pressure ventilation
• EDS patients with epidurals have greater risk for PDPH and epidural hematoma formation

Pathophysiology

Mutations to genes that code for fibrillar collagen proteins in connective tissue

Their are six manor subtypes ranging phenotypically from very mild to life-threatening phenotypes.

Signs and symptoms

Joint hypermobility/instability

kyphoscoliosis

Skin fragility

Aortic root dilitation causing aortic insufficiency