Pregnancy Complications in Type Iv Ehlers-Danlos Syndrome

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

“…Serious complications and maternal mortality are reported in the literature associated with pregnancy and delivery in women with EDS type IV, such as bowel ruptures, ruptures of major arteries, and ruptures of the uterus and birth canal (2,5,6,10). Recently, Pepin et al reviewed the clinical and family histories, and medical and surgical complications in 220 index patients with biochemically confirmed EDS type IV and 199 of their affected relatives (2).…”

“…As a result of the rare occurrence of EDS it is difficult to estimate the true incidence of the complications. Most articles on EDS and pregnancy present or review case reports, which are notorious for publication bias, as a dramatic complication or outcome often formed the reason for publication (5)(6)(7). In a survey of clinical aspects of EDS patients Ainsworth and Aulicino (8) report the occurrence of preterm rupture of membranes in 26-75% of cases, depending on the type of EDS, and excessive bleeding antepartum was 'often' observed.…”

Ehlers-Danlos Syndrome Type IV Associated with Arterial and Bowel Perforation

“…Maternal mortality of up to 25% has been reported due to rupture of the artery, bowel and uterus. 1 The precise incidence of pregnancy complications in women with Ehlers-Danlos syndrome type IV is not really known, because usually only patients with complications are likely to be reported in the medical, genetic and obstetric literature. 12 The complications of pregnancy include rupture of the bowel, aorta, vena cava, or uterus, vaginal laceration, postpartum uterine hemorrhage, varicose veins, uterine and/or bladder prolapse, joint laxity, abdominal herniation, and wound dehiscence.…”

“…12 The complications of pregnancy include rupture of the bowel, aorta, vena cava, or uterus, vaginal laceration, postpartum uterine hemorrhage, varicose veins, uterine and/or bladder prolapse, joint laxity, abdominal herniation, and wound dehiscence. 1,2,12 This has led some authors to recommend that patients with Ehlers-Danlos syndrome type IV should be advised against pregnancy, 2 or be counseled for early pregnancy termination. 1,13 If the patient opts for pregnancy, both she and her physician should at least be aware of the potential serious complications.…”

“…1,2,12 This has led some authors to recommend that patients with Ehlers-Danlos syndrome type IV should be advised against pregnancy, 2 or be counseled for early pregnancy termination. 1,13 If the patient opts for pregnancy, both she and her physician should at least be aware of the potential serious complications. Moreover, the patient should be given genetic counseling with regard to the risk of her offspring having the same severe form of the disease (there is a 50% chance of inheriting the syndrome).…”