Ehlers-Danlos syndromes
Anesthetic relevance
Anesthetic management

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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.[1][2]

Anesthetic implications

Preoperative optimization

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

Intraoperative management

Postoperative management

Related surgical procedures

Pathophysiology

Signs and symptoms

Diagnosis

Treatment

Medication

Surgery

Prognosis

Epidemiology

References

  1. Parapia, Liakat A.; Jackson, Carolyn (2008-04). "Ehlers-Danlos syndrome--a historical review". British Journal of Haematology. 141 (1): 32–35. doi:10.1111/j.1365-2141.2008.06994.x. ISSN 1365-2141. PMID 18324963. Check date values in: |date= (help)
  2. Wiesmann, Thomas; Castori, Marco; Malfait, Fransiska; Wulf, Hinnerk (2014-07-23). "Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s)". Orphanet Journal of Rare Diseases. 9 (1): 109. doi:10.1186/s13023-014-0109-5. ISSN 1750-1172. PMC 4223622. PMID 25053156.CS1 maint: PMC format (link)