Although Vascular Ehlers Danlos Syndrome EDS (Type IV) is considered rare, the possibility of complications with all types of EDS should be considered in a surgery or a trauma situation. Do not assume that your patient has been typed correctly, as clinical diagnosis is often difficult. Take necessary precautions during procedures with any type of EDS. Thank You!
Ehlers-Danlos Syndrome (EDS) is a heterogeneous group of heritable connective tissue disorders characterized by articular hypermobility, skin extensibility and tissue fragility. There are six major types of EDS. The different types of EDS are classified according distinct features.
Reprinted with Permission from the Canadian Ehlers-Danlos Association (CEDA)
“However, examples of unclassified variants and ‘overlap phenotypes’ are becoming more common. Mutations in genes encoding fibrillar collagens or collagen-modifying enzymes have been identified in most forms of EDS, including the classic and vascular subtypes (collagen type V and III, respectively), and the rare arthrochalasis, kyphoscoliosis and dermatosparaxis variants (type I collagen defects). To date, the genetic background of the hypermobility type of EDS remains unclear, although some new insights have been gained recently.” *