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Ehler-Danlos

  • Fatemah MamdaniEmail author
  • Suzanne K. W. Mankowitz
Chapter

Abstract

Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders caused by mutations in genes that are involved in the production of collagen or that interact with collagen in the extracellular matrix. The syndrome primarily affects the skin, joints, and blood vessels. However, many other organs have also been affected. The most common EDS subtypes are classical, hypermobility, and vascular. The unifying characteristics of all EDS types are joint hypermobility, skin hyper-elasticity, tissue fragility, easy bruising and bleeding, and poor wound healing. The effects are varied, ranging from minor to major complications such as organ and arterial vascular rupture. Maternal and neonatal complications are described as well as anesthetic management of pregnant women with EDS.

Keywords

Ehlers-Danlos syndrome Collagen Pregnancy Connective tissue disorder Joint hypermobility Tissue fragility Easy bruising and bleeding Poor wound healing Uterine rupture Arterial dissection and rupture Scoliosis Tarlov cyst 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of AnesthesiologyColumbia University Medical CenterNew YorkUSA

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