Journal de gynécologie, obstétrique et biologie de la reproduction 2012-11-01

[Vascular Ehlers-Danlos syndrome and pregnancy: an obstetrical specific support].

A Jamard, C Le Hello, T Simonet, M Dreyfus

Index: J. Gynecol. Obstet. Biol. Reprod. (Paris.) 41(7) , 676-8, (2012)

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Abstract

The Ehlers-Danlos syndrome (EDS) is a rare inheritable disease, characterised by a defect in collagen synthesis. Various types have been described and the type IV or vascular type is the most severe characterised by vascular, gastrointestinal and gynaecologic complications. We describe in a case report the specific obstetrical support we applied to avoid the most frequent complications such as early spontaneous abortions, pre-term delivery, tearing of perineum, uterine and vascular rupture and hard healing. Pregnancy is very risky in women with vascular EDS. Combination of multidisciplinary support and advice of the rare vascular disease national reference centre may reduce the morbi-mortality rate, including celiprolol long-term treatment.Copyright © 2012 Elsevier Masson SAS. All rights reserved.