Ehlers-Danlos Syndrome Type II in Pregnancy

Ploeckinger, Barbara; Ulm, Martin; Chalubinski, Kinga

doi:10.1055/s-2007-994106

Cited by 18 publications

(8 citation statements)

References 5 publications

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“…cervical hypoplasia [51][52][53][54], exposure to diethylstilbestrol (DES) in utero [55][56][57][58][59][60][61], etc. ), genetic syndromes (such as Ehlers-Danlos syndrome) [62], subclinical intra-amniotic infection [63][64][65] or intra-amniotic inflammation [66,67], or the elusive condition referred to as cervical insufficiency [68][69][70]. A decrease in progesterone action has been implicated in untimely cervical ripening [71][72][73][74][75][76][77][78], as this hormone plays a key role in maintaining the cervix as long and closed during pregnancy [77][78][79][80][81][82][83][84][85][86][87][88].…”

Section: Introductionmentioning

confidence: 99%

Sterile intra-amniotic inflammation in asymptomatic patients with a sonographic short cervix: prevalence and clinical significance

Romero

Miranda

Chaiworapongsa

et al. 2014

The Journal of Maternal-Fetal & Neonatal Medicine

157

122

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Objective To determine the frequency and clinical significance of sterile- and microbial-associated intra-amniotic inflammation in asymptomatic patients with a sonographic short cervix. Methods Amniotic fluid (AF) samples obtained by transabdominal amniocentesis from 231 asymptomatic women with a sonographic short cervix [cervical length (CL) ≤25 mm] were analyzed using cultivation techniques (for aerobic and anaerobic as well as genital mycoplasmas) and broad-range polymerase chain reaction (PCR) coupled with electrospray ionization mass spectrometry (PCR/ESI-MS). The frequency and magnitude of intra-amniotic inflammation [defined as an AF interleukin (IL)-6 concentration ≥2.6 ng/mL], acute histologic placental inflammation, spontaneous preterm delivery, and the amniocentesis-to-delivery interval were examined according to the results of AF cultures, PCR/ESI-MS and AF IL-6 concentrations. Results Ten percent (24/231) of patients with a sonographic short cervix had sterile intra-amniotic inflammation (an elevated AF IL-6 concentration without evidence of microorganisms using cultivation and molecular methods). Sterile intra-amniotic inflammation was significantly more frequent than microbial-associated intra-amniotic inflammation [10.4% (24/231) vs. 2.2% (5/231); p<0.001]. Patients with sterile intra-amniotic inflammation had a significantly higher rate of spontaneous preterm delivery <34 weeks of gestation [70.8% (17/24) vs. 31.6% (55/174); p<0.001] and a significantly shorter amniocentesis-to-delivery interval than patients without intra-amniotic inflammation [median 35, (IQR: 10 – 70) vs. median 71, (IQR: 47 – 98) days, (p<0.0001)]. Conclusion Sterile intra-amniotic inflammation is more common than microbial-associated intra-amniotic inflammation in asymptomatic women with a sonographic short cervix, and is associated with increased risk of spontaneous preterm delivery (<34 weeks). Further investigation is required to determine the causes of sterile intra-amniotic inflammation and the mechanisms whereby this condition is associated with a short cervix and spontaneous preterm delivery.

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Section: Introductionmentioning

confidence: 99%

Sterile intra-amniotic inflammation in asymptomatic patients with a sonographic short cervix: prevalence and clinical significance

Romero

Miranda

Chaiworapongsa

et al. 2014

The Journal of Maternal-Fetal & Neonatal Medicine

157

122

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“…Therefore, it is questionable whether a prophylactic cerclage is indicated in patients with EDS, since these patients are already predisposed to PPROM in case the fetus is also affected with EDS. Literature data con-cerning cervical cerclage in EDS patients are scarce: we found only one case of a pregnancy in an EDS type II patient (recently comprised within the classical type of EDS) who underwent a prophylactic cervical cerclage [5]. This patient had an uneventful pregnancy and delivered at 41 weeks' gestation.…”

Section: Discussionmentioning

confidence: 90%

“…Patients with EDS are at risk for PPROM due to cervical incompetence, secondary to the connective tissue weakness [5,7,8]. However, the exact incidence of this complication in EDS is unknown.…”

Section: Discussionmentioning

confidence: 99%

Preterm Premature Rupture of Membranes in a Patient with the Hypermobility Type of the Ehlers-Danlos Syndrome

Vos

Nuytinck²,

Verellen³

et al. 1999

Fetal Diagn Ther

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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.

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“…Given the low incidence of EDS, no population studies of pregnancy outcomes have been published. Thus, even the significantly more serious, and thus reportable, complications such as atonic uterus at Cesarean section [35] and uterine rupture during labor [33], [34] have only been discussed as case reports and series of case reports. However, our data indicating that biglycan modulates TGF-β expression support the observation that uterine rupture during pregnancy also occurs in patients with aneurysm syndromes other than EDS that are caused by mutations in the TGF-β receptor [47].…”

Section: Discussionmentioning

confidence: 99%

“…Women with EDS type IV are at increased risk of uterine rupture [33], [34] and atonic uterus at Cesarean section [35], while infants born with type III Ehlers-Danlos syndrome may be at increased risk of malpresentation during labor [36].…”

Section: Introductionmentioning

confidence: 99%

Uterine Dysfunction in Biglycan and Decorin Deficient Mice Leads to Dystocia during Parturition

Aron

Macksoud

et al. 2012

PLoS ONE

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Cesarean birth rates are rising. Uterine dysfunction, the exact mechanism of which is unknown, is a common indication for Cesarean delivery. Biglycan and decorin are two small leucine-rich proteoglycans expressed in the extracellular matrix of reproductive tissues and muscle. Mice deficient in biglycan display a mild muscular dystrophy, and, along with mice deficient in decorin, are models of Ehlers-Danlos Syndrome, a connective tissue anomaly associated with uterine rupture. As a variant of Ehlers-Danlos Syndrome is caused by a genetic mutation resulting in abnormal biglycan and decorin secretion, we hypothesized that biglycan and decorin play a role in uterine function. Thus, we assessed wild-type, biglycan, decorin and double knockout pregnancies for timing of birth and uterine function. Uteri were harvested at embryonic days 12, 15 and 18. Nonpregnant uterine samples of the same genotypes were assessed for tissue failure rate and spontaneous and oxytocin-induced contractility. We discovered that biglycan/decorin mixed double-knockout dams displayed dystocia, were at increased risk of delayed labor onset, and showed increased tissue failure in a predominantly decorin-dependent manner. In vitro spontaneous uterine contractile amplitude and oxytocin-induced contractile force were decreased in all biglycan and decorin knockout genotypes compared to wild-type. Notably, we found no significant compensation between biglycan and decorin using quantitative real time PCR or immunohistochemistry. We conclude that the biglycan/decorin mixed double knockout mouse is a model of dystocia and delayed labor onset. Moreover, decorin is necessary for uterine function in a dose-dependent manner, while biglycan exhibits partial compensatory mechanisms in vivo. Thus, this model is poised for use as a model for testing novel targets for preventive or therapeutic manipulation of uterine dysfunction.

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Ehlers-Danlos Syndrome Type II in Pregnancy

Cited by 18 publications

References 5 publications

Sterile intra-amniotic inflammation in asymptomatic patients with a sonographic short cervix: prevalence and clinical significance

Sterile intra-amniotic inflammation in asymptomatic patients with a sonographic short cervix: prevalence and clinical significance

Preterm Premature Rupture of Membranes in a Patient with the Hypermobility Type of the Ehlers-Danlos Syndrome

Uterine Dysfunction in Biglycan and Decorin Deficient Mice Leads to Dystocia during Parturition

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