Any pregnancy puts the cardiovascular system (heart and blood vessels) under exceptional pressure. There are additional risks women with vascular EDS in pregnancy, due to blood vessel fragility. It is essential that the obstetrician and midwives are aware of the diagnosis of vascular EDS as soon as the pregnancy is confirmed Women with Vascular Ehlers-Danlos Syndrome (VEDS) experiencing pregnancy have a risk of uterine rupture during late pregnancy and delivery, are more likely to have vaginal and cervical tears than unaﬀected women, and have a small risk of arterial rupture late in pregnancy and in the peripartum period (after the baby is born) Pregnancy for women with vascular EDS can be life-threatening. Mortality rates are high due to the increased risk for uterine and arterial rupture in the peripartum period. Case: We describe the counseling, multidisciplinary management, protocol, and successful pregnancy outcome of a 32-year-old woman with vascular EDS Vascular Type EDS and Pregnancy • 167/183 delivered at term • 12/81 maternal mortalities • 5 uterine ruptures • 2 intrapartum vessel ruptures • 5 postpartum • 2 of mortalities occurred in the 6th pregnancy Pepin, et al. NEJM 2000;342:67 Women with vascular Ehlers-Danlos syndrome should be engaged in a shared decision-making process when contemplating pregnancy and pregnancy management. Genet Med 16 12, 874-880
EDS subtypes. While the much rarer vascular EDS holds the most significant risks for childbearing women, including arterial dissection/rupture, uterine rupture and haemorrhage (Murray et al, 2014); this article will primarily focus on hypermobility EDS (hEDS), as it is the most common form of EDS (Volkov et al, 2007) The most important thing is to find an ob/gyn who has treated patients with vEDS before. Because what many doctors do not know is that an abortion, whether surgical or with medication, can be just as dangerous to women with vEDS as continuing the pregnancy might be. I have hEDS, but unusually severe bleeding issues Ehlers-Danlos syndromes (EDS) are hereditary collagen disorders. EDS may affect the skin, ligaments, muscle, blood vessels, and organs since collagen is a major component of connective tissue and is found throughout the body. The hypermobile, classical, and vascular forms are the most common, with hypermobile EDS (hEDS) being the most common Ehlers-Danlos Syndrome In a story from Medical Xpress, the authors of a recent study declared that maternity care for pregnant women with Ehlers-Danlos syndrome (EDS) is in dire need of improvement I've seen a lot of posts created about the hypermobility type and pregnancy but I haven't seen anything for the vascular type. I'm the first woman in 3 generations in my family to have EDS type 4. I'm 29 and I wasn't planning on starting a family for several more years
If you have Ehlers-Danlos syndrome (EDS), you likely know that the laxity of your tissues, and other symptoms, could present challenges during pregnancy.If you do decide to conceive, careful planning and management are key. What is EDS? EDS is a group of genetic disorders that affect the connective tissue that provides structure to joints, skin, blood vessels, and other tissues and organs Unlike the vascular form of the Ehlers-Danlos Syndrome (EDS), formerly EDS Type IV, the Joint Hypermobility Syndrome (JHS), and EDS-Hypermobility Type (formerly EDS III) (EDS-HT) are not associated with heart disease or major hazards during pregnancy and labour. In 2016 an important paper was published from Sweden (Sunderlin et al)
. They can occur in the legs (varicose veins), anus (haemorrhoids or piles) and the vulva/vaginal area (vulval varicosities) Pregnancy is considered contraindicated in women with vascular EDS due to the high risk of mortality, but shared decision making is essential, and women who choose to pursue pregnancy should be followed by a multidisciplinary team at a specialized center [20,21] As pregnancy progresses pregnant women with vascular EDS have a higher chance of blood vessel or uterine rupture. The timing of delivery of the baby will be decided by the obstetrician. A planned Caesarean delivery, in a hospital with access to specialist vascular surgery is recommended
vascular ehlers-danlos syndrome page 3 • Intestinal rupture in the absence of known diverticular disease or other bowel issues • Uterine rupture during the third trimester of pregnancy in the absence of previous c-sectio Pregnancy and postnatal complications are type specific (Table 1). Careful screening and follow-up can help decrease the potential complications of a surgical delivery, anesthesia, and postpartum.. Manage pregnancy appropriately. Pregnant women with VEDS should be followed in a high-risk obstetric program. Prenatal testing is available for pregnancies that are at an increased risk of passing the COL3A1 mutation to oﬀspring because of a known disease-causing mutation in one of the parents
Vascular EDS presents the most potential problems for pregnancy. Because of the highly delicate state of the body, several complications can occur before and during birth. The uterus could hemorrhage or rupture before delivery, which can be fatal for the mother and would almost certainly require a hysterectomy immediately after the birth, if. . This gene controls the production and assembly of type III collagen. Collagen is the most abundant protein found throughout the entire body. This is what gives connective tissues its. A more severe form of the disorder, called vascular Ehlers-Danlos syndrome, can cause the walls of your blood vessels, intestines or uterus to rupture. Because vascular Ehlers-Danlos syndrome can have serious potential complications in pregnancy, you may want to talk to a genetic counselor before starting a family
. Equally, there is a 50% (1 in 2) chance of a child getting the unaltered copy of the gene and not inheriting the condition Pregnancy for women with vascular EDS can be life-threatening. Mortality rates are high due to the increased risk for uterine and arterial rupture in the peripartum period A case report is presented of a 23-year-old patient who was diagnosed with Ehlers Danlos syndrome (EDS) Type IV (vascular type) in the 23rd week of her second pregnancy. EDS Type IV has one of the highest mortality rates for pregnant women of any condition with significant morbidity if the mother survives
Key words: Ehlers-Danlos syndrome, Carotid artery, Dissection, Pregnancy. CASE PRESENTATION INTRODUCTION Ehlers-Danlos syndrome is a hereditary connective tissue disorder. The type most frequently encountered by surgeons is vascular type, also named type IV. The diagnosis is often made afte Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders that can result in a range of complications during pregnancy. Pregnant EDS patients generally have a favourable outcome, but those with vascular EDS are more likely to su er from severe maternal complications vascular Ehlers-Danlos syndrome; therefore, doctors recommend surgery only when there is a risk of life-threatening bleeding. • Pregnancy management: Pregnant women with vascular Ehlers-Danlos syndrome should b
Vascular EDS and pregnancy: statistics. A study published in 2014 found that pregnancy-related deaths in women with Vascular EDS occurred in 30 of 565 deliveries (5.3%). Interviews with 39 women indicated that 46% had uncomplicated pregnancies, while the most common pregnancy-related complications were third-/fourth-degree lacerations (20%) and. well i've heard and red stories about such things happening. an my ob recommended i dont get pregnant because i have vascular. of course i cant get preg any ways because of the PCOS but there is good news, i had an older friend who had EDS as well, she was 45. she was about to have 3 kids but all three children were diagnosed with eds. we have a 50/50 chance of passing it to our children if we. Among the respondents, spontaneous abortion, premature birth, and ectopic pregnancies occurred more frequently among women with Ehlers-Danlos syndrome than are reported in the general population . 8-10 It is reasonable to monitor pregnant women with classical, hypermobility, or vascular Ehlers-Danlos syndrome with early ultrasound scanning to. INTRODUCTION. Vascular Ehlers-Danlos syndrome (vascular EDS, vEDS, or VEDS, previously known as Ehlers-Danlos type IV) is a dominantly inherited disorder that results from mutations in COL3A1, the gene that encodes the chains of type III collagen [Pope et al., 1975; Pepin et al., 2014; Frank et al., 2015a].The initial diagnosis is usually suspected on the basis of family history, or a.
Vascular Ehlers-Danlos syndrome is the most severe form of EDS with symptoms that can be life-threatening. These include vascular events, described in detail below, and rupture of hollow organs such as bowel and the uterus (in pregnant women) Ehlers-Danlos syndrome (EDS) is the name given to a group of related genetic diseases characterized by weakness in the connective tissue that makes up joints and holds tissues together. The symptoms of the different types of EDS overlap. Vascular EDS (vEDS) is characterized by thin, translucent skin that is easily bruised, and fragile arteries, muscles, and internal organs
Vascular Ehlers-Danlos Syndrome is a rare, inherited, connective tissue disorder caused by abnormalities in the formation of collagen, an important and the most abundant protein in the body. Connective tissue is made up of structural proteins known as collagens and fibroblast cells, which are arranged in rows Request PDF | Ehlers-Danlos Syndrome in Pregnancy: A Review | Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders that can result in a range of complications during pregnancy. The vascular type of Ehlers-Danlos syndrome is characterized by the major complications of arterial and bowel rupture, uterine rupture during pregnancy, and the clinical features of easy bruising, thin skin with visible veins, and characteristic facial features (summary by Leistritz et al., 2011) Ehlers-Danlos type IV Type IV or vascular EDS is the most dangerous type of EDS and may result in death related to arterial, intestinal and uterine fragility or rupture. The heredity pattern is autosomal dominant. Overall life expectancy is estimated to be 40-50 years, and most deaths are secondary to arterial rupture
Ehlers-Danlos syndrome type IV (EDS IV or Vascular EDS, OMIM# 130050) is characterized by thin, translucent skin; easy bruising; characteristic facial appearance; and arterial, intestinal, or uterine fragility (Pepin & Byers. GeneReviews. 2011). Vascular dissection or rupture, gastrointestinal perforation, or organ rupture are the presenting signs in the majority of adults identified to have. Ed's & GI problems - @Madame-de-Jardins - Ehlers-Danlos Syndromes - 20210724 - Inspire. Inspire. Ehlers-Danlos Syndromes and Hypermobility Spectrum Disorders. Hypermobile EDS (hEDS A case report is presented of a 23-year-old patient who was diagnosed with Ehlers Danlos syndrome (EDS) Type IV (vascular type) in the 23rd week of her second pregnancy. EDS Type IV has one of the highest mortality rates for pregnant women of any condition with significant morbidity if the mother survives. Current literature is presented and supports the necessity for close monitoring of. Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders that can result in a range of complications during pregnancy. Pregnant EDS patients generally have a favourable outcome, but those with vascular EDS are more likely to suffer from severe maternal complications. Early diagnosis of EDS and subtype characterization can aid in pre-pregnancy counselling, planning of antenatal.
Vascular Ehlers-Danlos syndrome is a rare, life-threatening, autosomal dominant variant of EDS, resulting from mutations in COL3A1 gene. Affected individuals are prone to serious and potentially fatal complications, especially vascular, intestinal, and uterine ruptures. Delay in diagnosis is common, even when the clinical presentation is typical Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome. Genet Med 2014; 16:874. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J, et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy More information: Sally Pezaro et al. Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth and beyond, British Journal of Midwifery (2018). DOI: 10.12968/bjom.2018.26.4.217 Provided by. premature rupture of membranes during pregnancy, and; rupturing of the eyeball. Symptoms of Vascular Ehlers-Danlos. Vascular EDS has separate symptoms and long term-effects. Those with vascular EDS have distinct facial features, including thin noses, thin upper lips, small earlobes, and prominent eyes
Some complications of Ehlers-Danlos syndrome include: Chronic joint pain, early-onset arthritis, failure of surgical wounds to close (or stitches tear out), premature rupture of membranes during pregnancy, rupture of major vessels, including a ruptured aortic aneurysm (only in vascular EDS), rupture of a hollow organ such as the uterus or bowel. EDS classical type, EDS hypermobility type, EDS vascular type, and EDS arthrochalasia type follow autosomal dominant inheritance. Sometimes there is no family history of EDS and the change (mutation) in the gene that causes EDS occurs randomly for unknown reasons and is not inherited Pregnancy may also be very dangerous for women with vascular Ehlers Danlos syndrome. Obstetric complications include risk of uterine rupture during labor, damage to the vagina and perineum, bleeding, and rupture of blood vessels and the colon. If you are diagnosed with Loeys-Dietz syndrome, please visit the Loeys-Dietz Syndrome Foundation website The Ehlers-Danlos syndromes (EDS) are a group of 13 heritable connective tissue disorders generally character-ized by joint hypermobility, skin hyperextensibility, and tissue fragility.1 Vascular EDS (vEDS), previously called EDS type IV, is one of the subtypes with an estimated frequency of 1 in 50,000 and accounts for 5% of all EDS Vertebral artery dissection a few years ago, feeling scared about possibility of vascular EDS. I've always wondered if it was linked to pregnancy and was looking it up today. I have a lot of the symptoms or signs of vascular EDS. I feel petrified with fear. Not only the life expectancy, but for my children's possibility of having it as well.
Pregnancy: pregnant women should be managed by high-risk obstetricians; women with vascular EDS should be informed at the time of diagnosis of the vascular form that a pregnancy carries a high risk of uterine rupture with consequent maternal death The term Ehlers-Danlos syndrome (EDS) encompasses a group of inherited connective tissue disorders. The manifestations of EDS can be seen in skin, joints, blood vessels and internal organs and vary from mild to severe and life threatening. Each subtype is a separate and different condition. The genetic basis of many subtypes has now been elucidated, confirming heterogeneity A number of case reports and case series over several decades suggest that pregnancy may trigger aortic dissection or rupture. 5 Aortic complications in pregnancy have been described in Marfan syndrome, 6,7 Loeys-Dietz syndrome, 8 the vascular type (type 4) of Ehlers-Danlos syndrome, 9 Turner syndrome, 10 and congenital aortic malformations. Vascular Ehlers-Danlos syndrome. The vascular form of Ehlers-Danlos syndrome (vEDS) is a connective tissue disorder than can affect many different parts of the body, including the skin, arteries, muscles, and organs. Connective tissue is a type of tissue in the body that helps to hold everything together, like a glue for your body
People with vascular type EDS may have a shorter life expectancy because of elevated risk of sudden tearing of blood vessels, etc. Rupture of arteries typically occurs when patients are 20-30 years old, but it can also happen at any age. This type of EDS comes with an average life expectancy of around 48-50 years Peaceman AM and Cruikshank DP (1987) Ehlers-Danlos syndrome and pregnancy: association of type IV disease with maternal death. Obstet Gynecol 69:428-31 ; Pepin M, Schwarze U, Superti-Furga A, Byers PH (2000) Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med 342:673-8 Ehlers Danlos syndromes, of which there are 13 sub-types, are barely beginning to become known by doctors, if at all. 95% of patients don't even know they have it, according to EDS experts. And most doctors have a vague memory of it mentioned in med school Just because you have EDS doesn't mean you can't have a successful pregnancy. 13 If you're considering getting pregnant, talk to your doctor, because women with EDS may be at higher risk for certain obstetric complications like premature labor and premature rupture of membranes. 6 Sometimes your EDS symptoms may include connective tissue. The features of vascular EDS (EDS IV) include thin, fragile translucent skin, atrophic scars, easy bruisability, increased risk for pneumothorax, and spontaneous organ and vascular rupture. The features of kyphoscoliosis EDS (EDS VI) are significant hypotonia, progressive early-onset scoliosis, lax joints, poor wound healing, atrophic scars.
Ehlers-Danlos syndrome (EDS) is a group of inherited disorders marked by extremely loose joints, very stretchy (hyperelastic) skin that bruises easily, and easily damaged blood vessels. Causes There are six major types and at least five minor types of EDS pregnancy complications: variable: medium: Unlike vascular EDS, hypermobile EDS is not associated with heart disease or major hazards (e.g., bowel rupture) during pregnancy and labour. Lind J, Wallenburg HC. Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population The VASCERN Pregnancy and Family Planning Working Group (WG) is a transversal WG of VASCERN, coordinated by Professor Julie De Backer (Chair), Professor Guillaume Jondeau (Co-Chair) and Petra Borgards (ePAG representative), that deals with issues specifically related to pregnancy and the diseases of our five Rare Disease Working Groups.. The group meets every three months and liaises with the. Aortic Dissection in a Patient with Vascular Ehlers- Danlos Syndrome Kimberly Cheong, M.D. and Minisha Kochar, M.D. A 52-year-old woman was referred for aortic disease. Her mother died suddenly at age 41, and the autopsy showed an aortic dissection. In the past year, the patient's 17year- -old so Finally, aged 39, and following a collapse in hospital during a night shift working as a lab assistant, a visiting UK doctor ordered McClarin to be tested for vEDS - vascular Ehlers-Danlos.
Vascular EDS. This type can be life-threatening because it weakens the walls of your blood vessels, such as the arteries of your heart, kidneys, and spleen.This makes them more likely to burst Ehlers-Danlos syndromes are inherited connective tissue disorders, characterised by joint hypermobility, skin hyperextensibility, and tissue fragility. Hypermobile Ehlers-Danlos syndrome (EDS) is the most common of 13 subtypes. Many affected people are asymptomatic or develop only minor symptoms
Ehlers Danlos Syndrome has a range of types, all are collagen disorders. It is presently thought that most forms of EDS affect around 1 in 5000 people. The vascular form is EDS4, also called EDSIV or VEDS. This form is presently thought to affect 1 in around 25,000 people. Unlike most forms of EDS, VEDS is known to shorten life span. FAMILY HISTOR The vascular type of Ehlers-Danlos syndrome can cause severe symptoms, including the rupture of the walls of the blood vessels, uterus, or intestines. This damage can lead to serious complications. The vascular category of Ehlers Danlos syndrome is too connected with an improved hazard of organ rupture, Involving crack of the intestine plus the womb through pregnancy. Individuals with the kyphoscoliosis type of Ehlers Danlos syndrome or EDS go through harsh, progressive curving of the backbone that can obstruct with breathing Pepin MG et al. Survival is affected by mutation type and molecular mechanism in vascular Ehlers-Danlos syndrome (EDS type IV). Genet Med. 2014 Dec;16(12):881-8. Murray M et al. Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome. Genet Med. 2014 Dec;16(12):874-80. Pepin MG and Byers PH. Gene Reviews: Ehlers. Vascular EDS. The Vascular type of EDS is characterized by thin, translucent skin that bruises easily but is not necessarily particularly stretchy, and spontaneous dilation / rupture of arteries and organs (Byers et al, 2017). The problem lies in the production of type 3 collagen
Ehlers-Danlos syndrome: Definition The Ehlers-Danlos syndromes (EDS) refer to a group of inherited disorders that affect collagen structure and function. Genetic abnormalities in the manufacturing of collagen within the body affect connective tissues, causing them to be abnormally weak. Description Collagen is a strong, fibrous protein that. Women with vascular EDS who become pregnant may have an increased risk of vascular complications. There is an increased risk of womb rupture in later pregnancy