1/3. Preterm premature rupture of membranes in a patient with the hypermobility type of the ehlers-danlos syndrome. A case report.OBJECTIVES: This report wants to focus on the risk of severe prematurity in patients with the hypermobility type of the ehlers-danlos syndrome (EDS), a heritable disorder of connective tissue. Although various obstetrical complications have been reported in patients with EDS, most reports specifically comment on the severe complications in patients with the vascular type of EDS, including uterine and arterial rupture. pregnancy outcome in patients presenting the hypermobility type of EDS is poorly documented. CASE: A 33-year-old nullipara was referred for preconceptual genetic counseling with a history of easy bruising, generalized joint hypermobility and chronic arthralgia and myalgia. The diagnosis of the hypermobility type of EDS was confirmed on clinical examination. During her first pregnancy, she underwent a prophylactic McDonald cerclage at 14 weeks' gestation. Premature rupture of membranes occurred at 23 weeks' gestation. A female infant was delivered at 26 weeks and died 3 h after birth. Electron-microscopic examination showed collagen fibre abnormalities in the fetus' skin, which were compatible with the diagnosis of EDS. CONCLUSIONS: patients with the hypermobility type of EDS can have an increased risk for pregnancy complications, including prematurity due to cervical incompetence and to premature rupture of membranes. We therefore demand the clinician's alertness for possible signs of this underdiagnosed type of EDS and recommend the collaboration between the obstetrician and the medical geneticist in the obstetrical management of these patients.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
2/3. Successful treatment with the Smith-Hodge pessary of cervical incompetence due to defective connective tissue in ehlers-danlos syndrome.ehlers-danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders with at least ten distinct types. We report a case of EDS complicated by cervical incompetence. Treatment was undertaken after confirmation that the patient's EDS was not type IV, hence not associated with potentially serious vascular complications. Because of concern about stretching or tearing of the hyperextensible connective tissue associated with EDS, cerclage was not performed. The cervical incompetence due to defective connective tissue was treated with a Smith-Hodge pessary.- - - - - - - - - - ranking = 7keywords = connective (Clic here for more details about this article) |
3/3. ehlers-danlos syndrome type II in pregnancy.ehlers-danlos syndrome type II is a rare connective tissue disorder with unknown pregnancy-related maternal and fetal morbidity. The course and outcome of pregnancy in a primigravid woman with ehlers-danlos syndrome type II is described. At 17 weeks' gestation a prophylactic Shirodkar cerclage was performed without complications. From the 29th week on, decreased blood flow of the umbilical artery was measured, and fetal growth retardation was evident from 34 weeks' gestation on. Pregnancy ended at 41 weeks by spontaneous vaginal delivery of a healthy 2900-g female infant. Previous reports of 16 women with ehlers-danlos syndrome type II, who had a total of 24 pregnancies, are reviewed.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |