Abstract
Introduction
A case report is presented of a 23-year-old patient who was diagnosed with Ehlers Danlos syndrome (EDS) Type IV (vascular type) in the 23rd week of her second pregnancy. EDS Type IV has one of the highest mortality rates for pregnant women of any condition with significant morbidity if the mother survives.
Discussion
Current literature is presented and supports the necessity for close monitoring of mother and child in a specialist unit with involvement of a multi-disciplinary team. There is no agreed consensus on the mode or timing of delivery but recent literature is supportive of delivery by caesarean section at 32 weeks.
Conclusion
This case report demonstrates a successful outcome for both mother and child with a planned delivery at 34 weeks by caesarean section and emphasises the importance of strategic planning for complicated deliveries and the effectiveness of good communication networks. Patients with EDS Type IV should be counselled about the potential risks for both themselves and the child to enable them to make informed decisions about their obstetric care.
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References
Germain DP (2002) Clinical and genetic features of vascular Ehlers-Danlos syndrome. Int J Vasc Surg 16:391–397
Brees CK, Gall SA (1995) Rupture of the external iliac artery during pregnancy: a case of type IV Ehlers-Danlos syndrome. KMA J 93:553–555
Peaceman AM, Cruikshank DP (1987) Ehlers-Danlos syndrome and pregnancy: association of type IV disease with maternal death. Obstet Gynaecol 69:428–431
Erez Y, Ezra Y, Rojansky N (2007) Ehlers-Danlos type IV in pregnancy. A case report and a literature review. Fetal Diag Ther 23:7–9
Lurie S, Manor M, Hagay ZJ (1998) The threat of type IV Ehlers-Danlos syndrome on maternal well-being during pregnancy: early delivery may make the difference. J Obstet Gynaecol 18:245–248
Rudd NL, Nimrod C, Holbrook KA, Byers PH (1983) Pregnancy complications in type IV Ehlers-Danlos syndrome. Lancet 1:50–53
Pepin M, Schwarze U, Superti-Furga A, Byers PH (2000) Clincal and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med 342:673–680
Germain DP (2007) Ehlers-Danlos syndrome type IV. Orphanet J Rare Dis 2:32
Volkov N, Nisenblat V, Ohel G, Gonen R (2006) Ehlers-Danlos syndrome: insights on obstetric aspects. Obstet Gynaecol Surv 62:51–57
Beighton P, DaPaepe A, Steinmann B, Tsipouras P, Westrup RJ (1998) Ehlers-Danlos syndromes: revised nosology, Villefranche 1977. Am J Med Genet 77:31–37
Drera B, Tadini G, Barlati S, Colombi M (2008) Identification of a novel TGFBR1 mutation in a Loeys–Dietz syndrome type II patient with vascular Ehlers-Danlos syndrome phenotype. Clin Genet 73:290–293
Lind J, Wallenburg HCS (2002) Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population. Acta Obstet Gynecol Scand 81:293–300
Bjorck M, Pigg M, Kragsterman B, Bergqvist D (2007) Fatal bleeding following delivery: a manifestation of the vascular type of Ehlers Danlos syndrome. Gynecol Obstet Invest 63:173–175
Walsh CA, Foley ME (2008) Ehlers-Danlos syndrome in pregnancy. Fetal Diagn Ther 24:79
Ong K, Perdu J, De Backer J, Bozec E, Collignon P, Emmerich J, Fauret A, Fiessinger J, Germain D, Georgesco G, Hulot J, De Paepe A, Plauchu H, Jeunemaitre X, Laurent S, Boutouyrie P (2010) Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomized, open, blinded-endpoints trial. Lancet 376:1476–1484
Acknowledgments
We would like to thank the patient described in this case report for consenting for us to publish her story. No funding was required for this case report.
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We declare that we have no conflict of interest.
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Hammond, R., Oligbo, N. Ehlers Danlos Syndrome Type IV and pregnancy. Arch Gynecol Obstet 285, 51–54 (2012). https://doi.org/10.1007/s00404-011-1899-5
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DOI: https://doi.org/10.1007/s00404-011-1899-5