[Pregnancy and Ehlers-Danlos vascular syndrome: patients' care and complications].

INTRODUCTION

Elhers-Danlos vascular syndrome type IV (EDS4) is a hereditary pathology of the connective tissue responsible for an increased risk of lethal arterial, uterine and digestive complications during and after pregnancy.

PATIENTS AND

METHODS

We describe the obstetrical care, the nature and frequency of complications related to pregnancy of patients with EDS4 and their relatives.

RESULTS

Twenty-seven pregnancies were studied including 23 deliveries, 18 vaginal deliveries and five caesarean, no maternal death and two major life-threatening complications (8.7%) were recorded which could be directly linked to EDS4 (rupture of the biscupid valve pillar after vaginal delivery and a rupture of the caecum after a prophylactic caesarean). Ten deliveries underwent epidural anesthesia without complication. Six perineal injuries (33.3%) were observed.

CONCLUSION

Pregnancy in patient with EDS4 needs obstetrical cares in a special unit's motivated medical team with intensive care and surgical disponibilities.