• Fatemah MamdaniEmail author
  • Suzanne K. W. Mankowitz


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.


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 


  1. 1.
    Eagleton MJ. Arterial complications of vascular Ehlers-Danlos syndrome. J Vasc Surg. 2016;64(6):1869–80.CrossRefGoogle Scholar
  2. 2.
    Malfait F, De Paepe A. The Ehlers-Danlos syndrome. Adv Exp Med Biol. 2014;802:129–43.CrossRefGoogle Scholar
  3. 3.
    Gazit Y, Jacob G, Grahame R. Ehlers–Danlos syndrome—hypermobility type: a much neglected multisystemic disorder. Rambam Maimonides Med J. 2016;7(4):e0034.CrossRefGoogle Scholar
  4. 4.
    Sobey G. Ehlers-Danlos syndrome: how to diagnose and when to perform genetic tests. Arch Dis Child. 2015;100(1):57–61.CrossRefGoogle Scholar
  5. 5.
    Christophersen C, Adams JE. Ehlers-Danlos syndrome. J Hand Surg Am. 2014;39(12):2542–4.CrossRefGoogle Scholar
  6. 6.
    Castori M, Morlino S, Ghibellini G, Celletti C, Camerota F, Grammatico P. Connective tissue, Ehlers-Danlos syndrome(s), and head and cervical pain. Am J Med Genet C Semin Med Genet. 2015;169(1):84–96.CrossRefGoogle Scholar
  7. 7.
    Volkov N, Nisenblat V, Ohel G, Gonen R. Ehlers-Danlos syndrome: insights on obstetric aspects. Obstet Gynecol Surv. 2007;62(1):51–7.CrossRefGoogle Scholar
  8. 8.
    Kulas Søborg M-L, Leganger J, Rosenberg J, Burcharth J. Increased need for gastrointestinal surgery and increased risk of surgery-related complications in patients with Ehlers-Danlos syndrome: a systematic review. Dig Surg. 2016;34(2):161–70.CrossRefGoogle Scholar
  9. 9.
    Cheng JL, JS A, Guzman JC, Morillo CA, MacDonald MJ. Cardiovascular profile in postural orthostatic tachycardia syndrome and Ehlers–Danlos syndrome type III. Clin Auton Res. 2016;27(2):113–6.CrossRefGoogle Scholar
  10. 10.
    Germain DP. Clinical and genetic features of vascular Ehlers-Danlos syndrome. Ann Vasc Surg. 2002;16(3):391–7.CrossRefGoogle Scholar
  11. 11.
    Hurst BS, Lange SS, Kullstam SM, Usadi RS, Matthews ML, Marshburn PB, et al. Obstetric and gynecologic challenges in women with Ehlers-Danlos syndrome. Obstet Gynecol. 2014;123(3):506–13.CrossRefGoogle Scholar
  12. 12.
    Castori M, Morlino S, Dordoni C, Celletti C, Camerota F, Ritelli M, et al. Gynecologic and obstetric implications of the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome hypermobility type) in 82 Italian patients. Am J Med Genet A. 2012;158A(9):2176–82.CrossRefGoogle Scholar
  13. 13.
    Sorokin Y, Johnson MP, Rogowski N, Richardson DA, Evans MI. Obstetric and gynecologic dysfunction in the Ehlers-Danlos syndrome. J Reprod Med. 1994;39(4):281–4.PubMedGoogle Scholar
  14. 14.
    Lind J, Wallenburg HCS. Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population. Acta Obstet Gynecol Scand. 2002;81(4):293–300.CrossRefGoogle Scholar
  15. 15.
    Sundelin HEK, Stephansson O, Johansson K, Ludvigsson JF. Pregnancy outcome in joint hypermobility syndrome and Ehlers-Danlos syndrome. Acta Obstet Gynecol Scand. 2016;96(1):114–9.CrossRefGoogle Scholar
  16. 16.
    Roop KA, Brost BC. Abnormal presentation in labor and fetal growth of affected infants with type III Ehlers-Danlos syndrome. Am J Obstet Gynecol. 1999;181(3):752–3.CrossRefGoogle Scholar
  17. 17.
    Jones TL, Ng C. Anaesthesia for caesarean section in a patient with Ehlers-Danlos syndrome associated with postural orthostatic tachycardia syndrome. Int J Obstet Anesth. 2008;17(4):365–9.CrossRefGoogle Scholar
  18. 18.
    Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers–Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342(10):673–80.CrossRefGoogle Scholar
  19. 19.
    Rudd NL, Nimrod C, Holbrook KA, Byers PH. Pregnancy complications in type IV Ehlers-Danlos syndrome. Lancet (London, England). 1983;1(8314–5):50–3.CrossRefGoogle Scholar
  20. 20.
    Murray ML, Pepin M, Peterson S, Byers PH. Pregnancy-related deaths and complications in women with vascular Ehlers–Danlos syndrome. Genet Med. 2014;16(12):874–80.CrossRefGoogle Scholar
  21. 21.
    Hammond R, Oligbo N, Hammond R. Ehlers Danlos syndrome type IV and pregnancy. Arch Gynecol Obs. 2012;285:51–4.CrossRefGoogle Scholar
  22. 22.
    Erez Y, Ezra Y, Rojansky N. Ehlers-Danlos type IV in pregnancy. A case report and a literature review. Fetal Diagn Ther. 2008;23(1):7–9.CrossRefGoogle Scholar
  23. 23.
    Pepin MG, Murray ML, Byers PH. Vascular Ehlers-Danlos syndrome. GeneReviews(®). Seattle: University of Washington; 1993.Google Scholar
  24. 24.
    Naing BT, Watanabe A, Tanigaki S, Ono M, Iwashita M, Shimada T. Presymptomatic genetic analysis during pregnancy for vascular type Ehlers-Danlos syndrome. Int Med Case Rep J. 2014;7:99–102.PubMedPubMedCentralGoogle Scholar
  25. 25.
    Palmquist M, Pappas JG, Petrikovsky B, Blakemore K, Roshan D. Successful pregnancy outcome in Ehlers-Danlos syndrome, vascular type. J Matern Fetal Neonatal Med. 2009;22(10):924–7.CrossRefGoogle Scholar
  26. 26.
    Levy HP. Ehlers-Danlos syndrome, hypermobility type. GeneReviews(®); 2016. 1–4 p.Google Scholar
  27. 27.
    Campbell N, Rosaeg OP. Anesthetic management of a parturient with Ehlers Danlos syndrome type IV. Can J Anesth. 2002;49(5):493–6.CrossRefGoogle Scholar
  28. 28.
    Weinbaum PJ, Cassidy SB, Campbell WA, Rickles FR, Vintzileos AM, Nochimson DJ, et al. Pregnancy management and successful outcome of Ehlers-Danlos syndrome type IV. Am J Perinatol. 1987;4(2):134–7.CrossRefGoogle Scholar
  29. 29.
    Lurie M, Manor ZJ, Hagay S, Manor M, Hagay ZJ. The threat of type IV Ehlers-Danlos syndrome on maternal well-being during pregnancy: early delivery may make the difference. J Obstet Gynaecol (Lahore). 1998;18(3):245–8.CrossRefGoogle Scholar
  30. 30.
    Dutta I, Wilson H, Oteri O. Pregnancy and delivery in Ehlers-Danlos syndrome (hypermobility type): review of the literature. Obstet Gynecol Int. 2011;2011:1–3.CrossRefGoogle Scholar
  31. 31.
    Wiesmann T, Castori M, Malfait F, Wulf H. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet J Rare Dis. 2014;9:109.CrossRefGoogle Scholar
  32. 32.
    Glynn JC, Yentis SM. Epidural analgesia in a parturient with classic type Ehlers-Danlos syndrome. Int J Obstet Anesth. 2005;14(1):78–9.CrossRefGoogle Scholar
  33. 33.
    Kuczkowski KM. Ehlers-Danlos syndrome in the parturient: an uncommon disorder—common dilemma in the delivery room. Arch Gynecol Obstet. 2005;273:60–2.CrossRefGoogle Scholar
  34. 34.
    Fedoruk K, Chong K, Sermer M, Carvalho JCA. Anesthetic management of a parturient with hypermobility phenotype but possible vascular genotype Ehlers-Danlos syndrome. Can J Anaesth. 2015;62(12):1308–12.CrossRefGoogle Scholar

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© 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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