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

[Sheehan's syndrome: an erratic diagnosis in the early postpartum]. / Le syndrome de Sheehan: un diagnostic difficile en post-partum immédiat.

Post-partum pituitary necrosis (Sheehan's syndrome) is a rare complication of the post-partum haemorrhage. The diagnosis can be erratic and often delayed. We report the case of a patient who had headaches and meningitis signs few hours after a post-partum haemorrhage. Magnetic Resonance Imaging (MRI) performed at day 2 showed a specific hypophysitis. The onset of asthenia, loss of weight, polyuro-polydipsy, persistent amenorrhoea and absence of lactation led to hormonal investigation. This permitted to diagnose global antehypopituitarism associated with diabetes insipidus. Progressive pituitary atrophy due to necrosis was found using MRI follow-up over 1 year. Our report summarises pathophysiological features of Sheehan's syndrome and early clinical and biological signs are discussed. MRI of the sellar region may be useful to early suspect the diagnosis.