Ehlers-Danlos syndrome: complications and solutions concerning anesthetic management

Abstract

Ehlers-Danlos syndrome is an inherited disorder that results in dysfunctional collagen bundles. These dysfunctional collagen bundles are most noticeable in tissues rich with collagen fibers--skin, vessels, GI, and ligaments. Until gene therapy advancements can correct the underlying gene mutations causing faulty collagen, the mainstay of treatment is prevention of traumatic injury. The success of anesthetic management in patients with EDS requires and understanding of the role of collagen in the various tissues of the body. Collagen-rich tissue fragility, skin hyperextensibility, joint hypermobility, hematoma formation and cardiovascular disease are just some of the complications that need to be accounted for before every anesthetic procedure involving EDS patients. Anesthesiologists should be keenly that any physical manipulation of EDS patients incurs risks of trauma.