[Pregnancy and Ehlers-Danlos vascular syndrome: patients' care and complications]. / Grossesse et syndrome d'Ehlers-Danlos vasculaire : prise en charge et complications.

ABSTRACT

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.