title: [Pregnancy and Ehlers-Danlos vascular syndrome: patients' care and complications].
abstract: BACKGROUND
  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.
  
  
  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.
  
  
  CONCLUSIONS
  Pregnancy in patient with EDS4 needs obstetrical cares in a special unit's motivated medical team with intensive care and surgical disponibilities.
authors.affiliation: Service de génétique, hôpital Louis-Pradel, hospices civils de Lyon, 28, avenue Doyen-Lépine, 69500 Bron, France. estelle.dubruc@free.fr
authors.forename: E
authors.initials: E
authors.lastname: Dubruc
authors.forename: S
authors.initials: S
authors.lastname: Dupuis-Girod
authors.forename: P
authors.initials: P
authors.lastname: Khau Van Kien
authors.forename: E
authors.initials: E
authors.lastname: Denis-Belicard
authors.forename: C
authors.initials: C
authors.lastname: Chirossel
authors.forename: S
authors.initials: S
authors.lastname: Fokstuen
authors.forename: R
authors.initials: R
authors.lastname: Touraine
authors.forename: H
authors.initials: H
authors.lastname: Plauchu
mesh_terms: D000328:Adult; D002429:Cecal Diseases / Q000209:etiology; D002585:Cesarean Section; D036861:Delivery, Obstetric; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000628:therapy*; D005260:Female; D006349:Heart Valve Diseases / Q000209:etiology; D006801:Humans; D008943:Mitral Valve; D010502:Perineum / Q000293:injuries; D011247:Pregnancy; D011248:Pregnancy Complications / Q000628:therapy*; D011256:Pregnancy Outcome; D012422:Rupture, Spontaneous