Gynecologic and obstetric impact of the ehlers-danlos syndrome : clues from scrutinizing dermal ultrastructural alterations

Hermanns‐Lê, Trinh; Pierard, Gérald; Piérard-Franchimont, C; Delvenne, Philippe

doi:10.7243/2052-6210-2-1

Cited by 5 publications

(3 citation statements)

References 46 publications

(55 reference statements)

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“…Midwives are required to practise in line with the best available evidence (NMC, 2015); yet there are no uniform management guidelines for childbearing women with hEDS, and the evidence available in relation to prevalence is sometimes highly conflicting . For example, in contrast to the majority of evidence presented in this paper, some studies reported that the incidences of adverse outcomes in those with some subtypes of EDS were no different from those in the general obstetrical population (Castori et al, 2012;Khalil et al, 2013;Hermanns-Lê et al, 2014;Sundelin et al, 2017). This presents additional challenges to educating and making the best evidence-based decisions in partnership with women.…”

Section: The Role Of Midwiferymentioning

confidence: 61%

Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth and beyond

Pezaro

Pearce

Reinhold³

2018

British Journal of Midwifery

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Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth and beyond T he Ehlers-Danlos Syndromes (EDS) are a group of multisystemic, inherited conditions that affect connective tissue (Malfait et al, 2017). The various subtypes of EDS can share symptoms such as joint hypermobility and hyperextensible and/or fragile skin (Bloom et al, 2017), yet many EDS subtypes also include clinical characteristics relating to pain, extreme fatigue, irritable bowel, sleep disturbance, depression, anxiety and other cardiovascular, gastrointestinal, orthopaedic, oromandibular, neurological, allergic/immunological, and psychological aspects of health (Tinkle et al, 2017). The prevalence of EDS was historically estimated to be 1 in 5000 for all subtypes (Beighton et al, 1998), although other work suggests a prevalence of 0.75-2% (Hakim and Sahota, 2006). There has been no high-quality prevalence study carried out since EDS received a major reclassification in 2017 (Tinkle et al, 2017). Despite these estimated prevalence rates, EDS is considered to remain largely underdiagnosed (Castori, 2012; Gazit et al, 2016). This is concerning for those receiving maternity care, as it is also associated with a number of complications relating to pregnancy and birth. Such complications can include precipitate labour, preterm rupture of membranes, scoliosis (problems with anaesthesia), atonic uterus, bleeding, vaginal and/ or perineal tears during birth, wound dehiscence and tissue fragility (Lawrence, 2005; Castori et al, 2012). This presents a unique opportunity for midwives and other members of the multidisciplinary team to understand, raise awareness of and more effectively support undiagnosed pregnant women, and those suspected of having or diagnosed with EDS. Those diagnosed with EDS perceive a lack of awareness among health professionals and describe delays in access to appropriate healthcare services (Terry et al, 2015). If EDS remains poorly understood by the multidisciplinary team, this may significantly compromise maternity care (Ross and Grahame, 2011). Consequently, this paper draws from wider literature and a number of key contemporary reviews to present evidence-based care considerations for both the mother and the neonate

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Section: The Role Of Midwiferymentioning

confidence: 61%

Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth and beyond

Pezaro

Pearce

Reinhold³

2018

British Journal of Midwifery

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“…Severe dorsal and pelvic pains and bleeding are frequent complaints in pregnant EDS women. Major post-partum complications such as intestinal and vascular ruptures are present in vascular, kyphoscoliotic and dermatosparaxis EDS [45][46][47][48] . In contrast, pregnancy outcome remains often unaffected in EDS hypermobile type [49] .…”

Section: P R E G N a N C Y A N D P O S T -P A R T U M Complicationmentioning

confidence: 99%

Skin Ultrastructural Clues on the Impact of Ehlers-Danlos Syndrome in Women

Pi閞ard

2016

Journal of Dermatological Research

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Ehlers-Danlos syndrome is heterogeneous in its genetic origins, molecular defects, and ultrastructure of connective tissues (CT). It frequently represents underdiagnosed heritable conditions. The altered mechanical functions of the involved tissues lead to variable combinations of increased skin distensibility and elasticity, joint laxity and CT fragility. In addition, EDS women commonly suffer from some gynecologic and obstetric disorders. In most instances, the diagnosis is based on selected clinical criteria. Dermal ultrastructural changes show representative patterns suggesting some EDS types: the collagen and elastic fibres are altered in the dermis, ligaments, vessel walls and internal organs. Although EDS is presumed to be a rare condition, physicians should view the disease outside the limits of conventional medical practices. Women are at risk of adverse pregnancy outcomes and various menstrual abnormalities. A multidisciplinary approach is usually welcome.

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“…The latter condition includes, for example, some cases of fibromyalgia (3), spontaneous preterm premature rupture of fetal membranes (4,5), spontaneous cervical artery dissections (SCAD) (6)(7)(8)(9)(10)(11), and spontaneous dissection of medium-size arteries (12,13). Any of these disorders is characterized by specific alterations in the morphologic presentation and association of fibrillar collagen and elastic fibers.…”

Section: Introductionmentioning

confidence: 99%

Familial Expression of Spontaneous Cervical Artery Dissections and Ehlers-Danlos Syndrome Hypermobile Type

Hermanns‐Lê¹,

Manicourt²,

Pierard³

2014

J Skin Stem Cell

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Introduction: Spontaneous cervical artery dissection (SCAD) and Ehlers-Danlos syndrome hypermobile type (EDSH) represent two heritable clinical connective tissue disorders, which show some abnormal ultrastructural features in the dermis. Genetic testing for most of those patients is not available at this time. Case Presentation: We present a woman with SCAD and her five close relatives showing obvious or suggestive signs of EDSH. In this family, the ultrastructural aspects of both diseases were closely related. Conclusions: Alterations were present in both the aspect and spacing of collagen fibrils and in the shape of elastic fibers. The present familial combination of SCAD and EDSH, with almost similar ultrastructural features suggests a possible connection between both conditions.

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Gynecologic and obstetric impact of the ehlers-danlos syndrome : clues from scrutinizing dermal ultrastructural alterations

Cited by 5 publications

References 46 publications

Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth and beyond

Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth and beyond

Skin Ultrastructural Clues on the Impact of Ehlers-Danlos Syndrome in Women

Familial Expression of Spontaneous Cervical Artery Dissections and Ehlers-Danlos Syndrome Hypermobile Type

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