References

Adib N, Davies K, Grahame R, Woo P, Murray KJ. Joint hypermobility syndrome in childhood. A not so benign multisystem disorder?. Rheumatology. 2005; 44:(6)744-50 https://doi.org/https://doi.org/10.1093/rheumatology/keh557

Arendt-Nielsen L, Kaalund S, Bjerring P, Høgsaa B. Insufficient effect of local analgesics in Ehlers Danlos type III patients (connective tissue disorder). Acta Anaesthesiol Scand. 1990; 34:(5)358-61 https://doi.org/https://doi.org/10.1111/j.1399-6576.1990.tb03103.x

Arunkalaivanan AS, Morrison A, Jha S, Blann A. Prevalence of urinary and faecal incontinence among female members of the Hypermobility Syndrome Association (HMSA). J Obstet Gynaecol. 2009; 29:(2)126-8 https://doi.org/https://doi.org/10.1080/01443610802664747

Atalla A, Page I. Ehlers-Danlos syndrome type III in pregnancy. Obstet Gynecol. 1988; 71:(3 Pt 2)508-9

Baeza-Velasco C, Gély-Nargeot MC, Bulbena Vilarrasa A, Bravo JF. Joint hypermobility syndrome: problems that require psychological intervention. Rheumatol Int. 2011; 31:(9)1131-6 https://doi.org/https://doi.org/10.1007/s00296-011-1839-5

Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ. Ehlers-Danlos syndromes: revised nosology. am. J Med Genet. 1998; 77:(1)31-7

Bird HA. Joint hypermobility. Musculoskelet Care. 2007; 5:(1)4-19 https://doi.org/https://doi.org/10.1002/msc.91

Blitshteyn S, Poya H, Bett GCL. Pregnancy in postural tachycardia syndrome: clinical course and maternal and fetal outcomes. J Matern Fetal Neonatal Med. 2012; 25:(9)1631-4 https://doi.org/https://doi.org/10.3109/14767058.2011.648671

Bloom L, Byers P, Francomano C, Tinkle B, Malfait F The international consortium on the Ehlers–Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017; 175:(1)5-7 https://doi.org/https://doi.org/10.1002/ajmg.c.31547

Borrelli SE. What is a good midwife? Insights from the literature. Midwifery. 2014; 30:(1)3-10 https://doi.org/https://doi.org/10.1016/j.midw.2013.06.019

Castori M. Ehlers-danlos syndrome, hypermobility type: an underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012; 2012:1-22 https://doi.org/https://doi.org/10.5402/2012/751768

Castori M, Camerota F, Celletti C Natural history and manifestations of the hypermobility type Ehlers-Danlos syndrome: A pilot study on 21 patients. Am J Med Genet A. 2010a; 152A:(3)556-64 https://doi.org/https://doi.org/10.1002/ajmg.a.33231

Castori M, Camerota F, Celletti C, Grammatico P, Padua L. Quality of life in the classic and hypermobility types of Elhers-Danlos syndrome. Ann Neurol. 2010b; 67:(1)145-6 https://doi.org/https://doi.org/10.1002/ana.21934

Castori M, Morlino S, Dordoni C Gynecologic and obstetric implications of the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome hypermobility type) in 82 Italian patients. Am J Med Genet A. 2012; 158A:(9)2176-2182 https://doi.org/https://doi.org/10.1002/ajmg.a.35506

Castori M, Tinkle B, Levy H, Grahame R, Malfait F, Hakim A. A framework for the classification of joint hypermobility and related conditions. Am J Med Genet C Semin Med Genet. 2017; 175:(1)148-57 https://doi.org/https://doi.org/10.1002/ajmg.c.31539

Chetty S, Norton ME. Obstetric care in women with genetic disorders. Best Pract Res Clin Obstet Gynaecol. 2017; 42:86-99 https://doi.org/https://doi.org/10.1016/j.bpobgyn.2017.03.006

Coster PJ, Martens LC, Paepe A. Oral health in prevalent types of Ehlers-Danlos syndromes. J Oral Pathol Med. 2005; 34:(5)298-307 https://doi.org/https://doi.org/10.1111/j.1600-0714.2004.00300.x

Dutta I, Wilson H, Oteri O. Pregnancy and delivery in ehlersdanlos syndrome (hypermobility type): review of the literature. Obstet Gynecol Int. 2011; 2011:1-3 https://doi.org/https://doi.org/10.1155/2011/306413

Gazit Y, Nahir AM, Grahame R, Jacob G. Dysautonomia in the joint hypermobility syndrome. Am J Med. 2003; 115:(1)33-40 https://doi.org/https://doi.org/10.1016/S0002-9343(03)00235-3

Gazit Y, Jacob G, Grahame R. Ehlers-Danlos Syndrome-Hypermobility Type: A Much Neglected Multisystemic Disorder. Rambam Maimonides Med J. 2016; 7:(4) https://doi.org/https://doi.org/10.5041/RMMJ.10261

Glatter KA, Tuteja D, Chiamvimonvat N, Hamdan M, Park JK. Pregnancy in postural orthostatic tachycardia syndrome. Pacing Clin Electrophysiol. 2005; 28:(6)591-3 https://doi.org/https://doi.org/10.1111/j.1540-8159.2005.50026.x

Golfier F, Peyrol S, Attia-Sobol J, Marret H, Raudrant D, Plauchu H. Hypermobility type of Ehlers-Danlos syndrome: influence of pregnancies. Clin Genet. 2001; 60:(3)240-1 https://doi.org/https://doi.org/10.1034/j.1399-0004.2001.600312.x

Grigoriou E, Boris JR, Dormans JP. Postural orthostatic tachycardia syndrome (POTS): association with Ehlers-Danlos syndrome and orthopaedic considerations. Clin Orthop Relat Res. 2015; 473:(2)722-8 https://doi.org/https://doi.org/10.1007/s11999-014-3898-x

Hakim A, Grahame R. Joint hypermobility. Best Pract Res Clin Rheumatol. 2003; 17:(6)989-1004 https://doi.org/https://doi.org/10.1016/j.berh.2003.08.001

Hakim AJ, Sahota A. Joint hypermobility and skin elasticity: the hereditary disorders of connective tissue. Clin Dermatol. 2006; 24:(6)521-33 https://doi.org/https://doi.org/10.1016/j.clindermatol.2006.07.013

Hakim AJ, Grahame R, Norris P, Hopper C. Local anaesthetic failure in joint hypermobility syndrome. J R Soc Med. 2005; 98:(2)84-5 https://doi.org/https://doi.org/10.1177/014107680509800222

Hermanns-Lê T, Piérard GE. Skin Ultrastructural Clues on the Impact of Ehlers-Danlos Syndrome in Women. Journal of Dermatological Research. 2016; 1:(3)34-40 https://doi.org/https://doi.org/10.17554/j.issn.2413-8223.2016.01.9

Hermanns-Lê T, Piérard GE, Piérard-Franchimont C, Delvenne P. Gynecologic and obstetric impact of the Ehlers-Danlos syndrome: clues from scrutinizing dermal ultrastructural alterations. Gynecology. 2014; 2:(1) https://doi.org/https://doi.org/10.7243/2052-6210-2-1

Hurst BS, Lange SS, Kullstam SM Obstetric and gynecologic challenges in women with Ehlers-Danlos syndrome. Obstet Gynecol. 2014; 123:(3)506-13 https://doi.org/https://doi.org/10.1097/AOG.0000000000000123

Jones TL, Ng C. Anaesthesia for caesarean section in a patient with Ehlers-Danlos syndrome associated with postural orthostatic tachycardia syndrome. Int J Obstet Anesth. 2008; 17:(4)365-9 https://doi.org/https://doi.org/10.1016/j.ijoa.2008.04.003

Kålund S, Høgså B, Grevy C, Oxlund H. Reduced strength of skin in Ehlers Danlos syndrome, type III. Scand J Rheumatol. 1990; 19:(1)67-70 https://doi.org/https://doi.org/10.3109/03009749009092623

Kanjwal Y, Kosinski D, Grubb BP. The postural orthostatic tachycardia syndrome: definitions, diagnosis, and management. Pacing Clin Electrophysiol. 2003; 26:(8)1747-57 https://doi.org/https://doi.org/10.1046/j.1460-9592.2003.t01-1-00262.x

Kanjwal K, Karabin B, Kanjwal Y, Grubb BP. Outcomes of pregnancy in patients with preexisting postural tachycardia syndrome. Pacing Clin Electrophysiol. 2009; 32:(8)1000-3 https://doi.org/https://doi.org/10.1111/j.1540-8159.2009.02430.x

Khalil H, Rafi J, Hla T. A case report of obstetrical management of a pregnancy with hypermobile Ehlers–Danlos syndrome and literature review. Obstet Med. 2013; 6:(2)80-2

Kimpinski K, Iodice V, Sandroni P, Low PA. Effect of pregnancy on postural tachycardia syndrome. Mayo Clin Proc. 2010; 85:(7)639-44

Kishta W, Abduljabbar FH, Gdalevitch M, Rauch F, Hamdy R, Fassier F. Hip dysplasia in children with osteogenesis imperfecta. J Pediatr Orthop. 2017; 37:(7)479-83 https://doi.org/https://doi.org/10.1097/BPO.0000000000000644

Knoepp LR, McDermott KC, Muñoz A, Blomquist JL, Handa VL. Joint hypermobility, obstetrical outcomes, and pelvic floor disorders. Int Urogynecol J Pelvic Floor Dysfunct. 2013; 24:(5)735-40 https://doi.org/https://doi.org/10.1007/s00192-012-1913-x

Lawrence EJ. The clinical presentation of Ehlers-Danlos syndrome. Adv Neonatal Care. 2005; 5:(6)301-14

Levy HP. Ehlers-Danlos Syndrome, Hypermobility Type. In: Adam MP, Ardinger HH, Pagon RA (eds). GeneReviews. Seattle (WA): University of Washington; 1993

Levy H. Ehlers-Danlos syndrome, Hypermobility type. In: Adam MP, Ardinger HH, Pagon RA (eds). GeneReviews. Seattle (WA): University of Washington; 2004

Lind J, Wallenburg HCS. Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population. Acta Obstet Gynecol Scand. 2002; 81:(4)293-300 https://doi.org/https://doi.org/10.1034/j.1600-0412.2002.810403.x

Malfait F, Francomano C, Byers P The 2017 international classification of the Ehlers–Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017; 175:(1)8-26 https://doi.org/https://doi.org/10.1002/ajmg.c.31552

Marsden G, Perry M, Kelley K, Davies AH. Diagnosis and management of varicose veins in the legs: summary of NICE guidance. BMJ. 2013; https://doi.org/https://doi.org/10.1136/bmj.f4279

McEvoy MD, Low PA, Hebbar L. Postural orthostatic tachycardia syndrome: anesthetic implications in the obstetric patient. Anesth Analg. 2007; 104:(1)166-7 https://doi.org/https://doi.org/10.1213/01.ane.0000246815.39197.2b

Molloholli M. Implications for obstetric care. BMJ. 2011; https://doi.org/https://doi.org/10.1136/bmj.d1003

Motiaa Y, Doumiri M, El Ouadghiri N, AnasTazi S. Postural orthostatic tachycardia syndrome: anesthetic management in the obstetric patient. Journal of Obstetric Anaesthesia and Critical Care. 2016; 6:(2) https://doi.org/https://doi.org/10.4103/2249-4472.191599

Murray ML, Pepin M, Peterson S, Byers PH. Pregnancy-related deaths and complications in women with vascular Ehlers–Danlos syndrome. Genet Med. 2014; 16:(12)874-80 https://doi.org/https://doi.org/10.1038/gim.2014.53

Antenatal care for uncomplicated pregnancies.London: NICE; 2017

The Code: Professional standards of practice and behaviour for nurses and midwives.London: NMC; 2015

Paepe AD, Malfait F. Bleeding and bruising in patients with Ehlers-Danlos syndrome and other collagen vascular disorders. Br J Haematol. 2004; 127:(5)491-500 https://doi.org/https://doi.org/10.1111/j.1365-2141.2004.05220.x

Rombaut L, Malfait F, De Paepe A Impairment and impact of pain in female patients with Ehlers-Danlos syndrome: A comparative study with fibromyalgia and rheumatoid arthritis. Arthritis Rheum. 2011; 63:(7)1979-87 https://doi.org/https://doi.org/10.1002/art.30337

Roop KA, Brost BC. Abnormal presentation in labor and fetal growth of affected infants with type III Ehlers-Danlos syndrome. Am J Obstet Gynecol. 1999; 181:(3)752-3 https://doi.org/https://doi.org/10.1016/S0002-9378(99)70524-7

Ross J, Grahame R. Joint hypermobility syndrome. BMJ. 2011; https://doi.org/https://doi.org/10.1136/bmj.c7167

Sangsawang B. Risk factors for the development of stress urinary incontinence during pregnancy in primigravidae: a review of the literature. Eur J Obstet Gynecol Reprod Biol. 2014; 178:27-34 https://doi.org/https://doi.org/10.1016/j.ejogrb.2014.04.010

Smith C. Understanding Hypermobile Ehlers-Danlos Syndrome & Hypermobility Spectrum Disorder.Somerset: Redcliff-House Publications; 2017

Stewart JM, Medow MS, Montgomery LD, Glover JL, Millonas MM. Splanchnic hyperemia and hypervolemia during Valsalva maneuver in postural tachycardia syndrome. Am J Physiol Heart Circ Physiol. 2005; 289:(5)H1951-9 https://doi.org/https://doi.org/10.1152/ajpheart.00194.2005

Sundelin HEK, Stephansson O, Johansson K, Ludvigsson JF. Pregnancy outcome in joint hypermobility syndrome and Ehlers-Danlos syndrome. Acta Obstet Gynecol Scand. 2017; 96:(1)114-19 https://doi.org/https://doi.org/10.1111/aogs.13043

Terry RH, Palmer ST, Rimes KA, Clark CJ, Simmonds JV, Horwood JP. Living with joint hypermobility syndrome: patient experiences of diagnosis, referral and self-care. Fam Pract. 2015; 32:(3)354-8 https://doi.org/https://doi.org/10.1093/fampra/cmv026

Tinkle BT. Joint Hypermobility Handbook: a Guide for the Issues & Management of Ehlers-Danlos Syndrome Hypermobility Type and the Hypermobility Syndrome.Niles (IL): Left Paw Press; 2010

Tinkle B, Castori M, Berglund B Hypermobile Ehlers–Danlos syndrome (aka Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history. Am J Med Genet C Semin Med Genet. 2017; 175:(1)48-69 https://doi.org/https://doi.org/10.1002/ajmg.c.31538

Voermans NC, Knoop H, Bleijenberg G, van Engelen BG. Pain in Ehlers-Danlos syndrome is common, severe, and associated with functional impairment. J Pain Symptom Manage. 2010; 40:(3)370-8 https://doi.org/https://doi.org/10.1016/j.jpainsymman.2009.12.026

Volkov N, Nisenblat V, Ohel G, Gonen R. Ehlers-Danlos syndrome: insights on obstetric aspects. Obstet Gynecol Surv. 2007; 62:(1)51-7 https://doi.org/https://doi.org/10.1097/01.ogx.0000251027.32142.63

Wiesmann T, Castori M, Malfait F, Wulf H. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet J Rare Dis. 2014; 9:(1) https://doi.org/https://doi.org/10.1186/s13023-014-0109-5

Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth and beyond

02 April 2018
Clinical Practice
13 min read
02 April 2018
Volume 26 · Issue 4

Abstract

The Ehlers-Danlos Syndromes (EDS) are an underdiagnosed group of conditions with implications and risks associated with childbearing. Those with EDS suggest that health professionals lack of awareness in this area, and consequently describe delays in access to appropriate healthcare services. This article draws on the existing international evidence available to present evidence-based care considerations for childbearing women with hypermobile Ehlers-Danlos Syndrome (hEDS) throughout the antenatal, intrapartum and postnatal periods. Care considerations are also offered in relation to the care of the newborn infant. The management of hEDS in childbearing women and babies can be complex. Findings point to the need for a multidisciplinary approach to formulating individualised care plans in partnership with women. In understanding the evidence in relation to this issue, midwives will be better able to practice evidence-based and woman-centred care.

The 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).

Register now to continue reading

Thank you for visiting British Journal of Midwifery and reading some of our peer-reviewed resources for midwives. To read more, please register today. You’ll enjoy the following great benefits:

What's included

  • Limited access to our clinical or professional articles

  • New content and clinical newsletter updates each month

Register
Or continue by

Signing in with your registered email address

Midwifery
Pregnancy
Ehlers-Danlos Syndrome
Birth
Joint instability
Obstetric
Parturition