Airway Management in Patients with Ehlers-Danlos Syndrome

Ehlers Danlos Syndrome (EDS) is a group of collagen-related connective tissue disorders that is thought to affect 1 in 5000 individuals. EDS is divided into six main subtypes based on clinical picture, severity, pattern of inheritance, and specific genetic defect. The most common subtypes [type I, II (classical), and III (hypermobile)] make up about 90% of all cases of EDS while type IV (vascular) makes up about 3-10%. The remainder of subtypes [type VI (kyphoscoliosis), type VIIA/VIIB (arthrochalasis), type VIIC (dermatosparaxis)] are quite rare. Each subtype of EDS involves a specific mutation in genes coding for collagens or collagen-related proteins (collagen modifying enzymes, tenascin) that lead to connective tissue instability resulting in defects in cell attachment, platelet aggregation, organogenesis, and tensile strength in skin, bones, muscles, ligaments and tendons. These defects then lead to presentations that often go undetected with unknown clinical implications.(1) The purpose of this article is to review the current literature on the airway of EDS patients and discuss the challenges presented to the anesthesiologist. Although the manifestations of this disease are variable, there are several clinical features that are important for perioperative airway management.

Keywords: Ehlers-Danlos Syndrome, Airway management, Hypermobility, Obstructive Sleep apnea