Skip to main content
Top
Published in: Archives of Gynecology and Obstetrics 1/2012

01-01-2012 | Materno-Fetal Medicine

Ehlers Danlos Syndrome Type IV and pregnancy

Authors: Rebecca Hammond, Nicholas Oligbo

Published in: Archives of Gynecology and Obstetrics | Issue 1/2012

Login to get access

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.
Literature
1.
go back to reference Germain DP (2002) Clinical and genetic features of vascular Ehlers-Danlos syndrome. Int J Vasc Surg 16:391–397CrossRef Germain DP (2002) Clinical and genetic features of vascular Ehlers-Danlos syndrome. Int J Vasc Surg 16:391–397CrossRef
2.
go back to reference 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 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
3.
go back to reference Peaceman AM, Cruikshank DP (1987) Ehlers-Danlos syndrome and pregnancy: association of type IV disease with maternal death. Obstet Gynaecol 69:428–431 Peaceman AM, Cruikshank DP (1987) Ehlers-Danlos syndrome and pregnancy: association of type IV disease with maternal death. Obstet Gynaecol 69:428–431
4.
go back to reference Erez Y, Ezra Y, Rojansky N (2007) Ehlers-Danlos type IV in pregnancy. A case report and a literature review. Fetal Diag Ther 23:7–9CrossRef Erez Y, Ezra Y, Rojansky N (2007) Ehlers-Danlos type IV in pregnancy. A case report and a literature review. Fetal Diag Ther 23:7–9CrossRef
5.
go back to reference 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 18:245–248PubMedCrossRef 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 18:245–248PubMedCrossRef
6.
go back to reference Rudd NL, Nimrod C, Holbrook KA, Byers PH (1983) Pregnancy complications in type IV Ehlers-Danlos syndrome. Lancet 1:50–53PubMedCrossRef Rudd NL, Nimrod C, Holbrook KA, Byers PH (1983) Pregnancy complications in type IV Ehlers-Danlos syndrome. Lancet 1:50–53PubMedCrossRef
7.
go back to reference 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 342:673–680PubMedCrossRef 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 342:673–680PubMedCrossRef
9.
go back to reference Volkov N, Nisenblat V, Ohel G, Gonen R (2006) Ehlers-Danlos syndrome: insights on obstetric aspects. Obstet Gynaecol Surv 62:51–57CrossRef Volkov N, Nisenblat V, Ohel G, Gonen R (2006) Ehlers-Danlos syndrome: insights on obstetric aspects. Obstet Gynaecol Surv 62:51–57CrossRef
10.
go back to reference Beighton P, DaPaepe A, Steinmann B, Tsipouras P, Westrup RJ (1998) Ehlers-Danlos syndromes: revised nosology, Villefranche 1977. Am J Med Genet 77:31–37PubMedCrossRef Beighton P, DaPaepe A, Steinmann B, Tsipouras P, Westrup RJ (1998) Ehlers-Danlos syndromes: revised nosology, Villefranche 1977. Am J Med Genet 77:31–37PubMedCrossRef
11.
go back to reference 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 73:290–293PubMedCrossRef 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 73:290–293PubMedCrossRef
12.
go back to reference Lind J, Wallenburg HCS (2002) Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population. Acta Obstet Gynecol Scand 81:293–300PubMedCrossRef Lind J, Wallenburg HCS (2002) Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population. Acta Obstet Gynecol Scand 81:293–300PubMedCrossRef
13.
go back to reference 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 63:173–175PubMedCrossRef 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 63:173–175PubMedCrossRef
15.
go back to reference 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 376:1476–1484PubMedCrossRef 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 376:1476–1484PubMedCrossRef
Metadata
Title
Ehlers Danlos Syndrome Type IV and pregnancy
Authors
Rebecca Hammond
Nicholas Oligbo
Publication date
01-01-2012
Publisher
Springer-Verlag
Published in
Archives of Gynecology and Obstetrics / Issue 1/2012
Print ISSN: 0932-0067
Electronic ISSN: 1432-0711
DOI
https://doi.org/10.1007/s00404-011-1899-5

Other articles of this Issue 1/2012

Archives of Gynecology and Obstetrics 1/2012 Go to the issue