Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population.
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.


CONCLUSIONS
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.
Lind
Jan
J
Department of Obstetrics and Gynecology, Medisch Centrum Haaglanden, Westeinde Hospital, Den Haag, The Netherlands. jan@lind.demon.nl
Wallenburg
Henk C S
HC
D000328:Adult; D003376:Counseling; D004535:Ehlers-Danlos Syndrome / Q000453:epidemiology*; D005260:Female; D017408:Guidelines as Topic; D006801:Humans; D009426:Netherlands / Q000453:epidemiology; D007752:Obstetric Labor, Premature / Q000453:epidemiology; D006473:Postpartum Hemorrhage / Q000453:epidemiology; D011247:Pregnancy; D011248:Pregnancy Complications / Q000453:epidemiology*; D011256:Pregnancy Outcome / Q000453:epidemiology*; D012189:Retrospective Studies; D018570:Risk Assessment