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Keywords:

  • Ehlers–Danlos syndrome;
  • maternal complications;
  • neonatal outcome;
  • obstetric outcome;
  • pregnancy

Background.  The study was carried out to assess the course and outcome of pregnancies in women with the Ehlers–Danlos syndrome, with the aim of developing guidelines for assessment of risk and counseling, and for providing optimum medical and obstetric care.

Methods.  A retrospective study based on data collected from members of the Dutch Association of Ehlers–Danlos patients. Pregnancies and neonatal outcomes of the affected mothers were compared with those of the nonaffected mothers who delivered an infant with Ehlers–Danlos syndrome.

Results.  In a group of 46 women with Ehlers–Danlos syndrome, 128 pregnancies beyond 24 weeks gestation were evaluated and compared with 43 pregnancies of 33 nonaffected women. The main maternal complications consisted of pelvic pain and instability (26% vs. 7%). One bowel rupture was reported in a woman with Ehlers–Danlos syndrome type IV. Preterm delivery occurred in 21% of the affected mothers compared with 40% of the nonaffected women with an affected infant; the women with Ehlers–Danlos syndrome experienced postpartum hemorrhage (19% vs. 7%) and complicated perineal wounds (8% vs. 0%) more often than the unaffected women. The floppy infant syndrome was diagnosed in 13% of the affected infants and did not occur in the nonaffected neonates.

Conclusion.  Pregnancy is generally well-tolerated in women with Ehlers–Danlos syndrome, with favorable maternal and neonatal outcomes. In Ehlers–Danlos syndrome type IV it may be associated with severe maternal complications. Preconceptional counseling concerning specific possible complications and a multidisciplinary approach are recommended.