Skip to main content

Advertisement

SpringerLink
Log in

Gynecologic Disorders in Women With Ehlers-Danlos Syndrome

  • Published:
The Journal of the Society for Gynecologic Investigation: JSGI Aims and scope Submit manuscript

Abstract

Objective

We characterized the population with Ehlers-Danlos syndrome with regard to genital prolapse, urinary incontinence, and other gynecologic disorders.

Methods

Forty-one adult women who had registered for a first-ever Ehlers-Danlos multidis-ciplinary clinic participated in the study. Each had a comprehensive standardized evaluation, including gynecologic history, physical examination, urodynamic testing, and physical therapy evaluation. Qualitative and quantitative data were analyzed to determine means for various gynecologic disorders of Ehlers-Danlos syndrome.

Results

The frequencies of incontinence complaints (59%), endometriosis (27%), dyspa-reunia (57%), and previous hysterectomy (44%) were higher than expected for a population with a mean age of 41 years. Incontinence could not be demonstrated objectively. Prolapse was diagnosed in 12 (29.3%).

Conclusions

Careful attention should be paid to women with Ehlers-Danlos syndrome because of an association with many gynecologic complaints. Women with Ehlers-Danlos syndrome should be questioned regarding incontinence, genital prolapse, endometriosis, and dyspa-reunia. (J Soc Gynecol Invest 1995;2:559-64)

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Stoddard JF, Myers RE. Connective tissue disorders in obstetrics and gynecology. Am J Obstet Gynecol 1968;102:240–3.

    Article  CAS  PubMed  Google Scholar 

  2. Byers PH, Holbrook KA, Barsh GS. Ehlers-Danlos syndrome. In: Emery AEV, Rimion DL, eds. Principles and practices of medical genetics. Vol. 2. Edinburgh: Churchill Livingstone, 1990:1065–81.

    Google Scholar 

  3. Beighton P, de Paepe A, Danks D, et al. International nosology of heritable disorders of connective tissue, Berlin, 1986. Am J Med Genet 1988;29:581–94.

    Article  CAS  PubMed  Google Scholar 

  4. Rabin JM, Hirschfield L, Badlani GH. Type IX Ehlers-Danlos syndrome: Bladder diverticula with transitional cell carcinoma. Urology 1991;38:563–6.

    Article  CAS  PubMed  Google Scholar 

  5. Beighton P. The Ehlers-Danlos syndrome. In: Beighton P, ed. McKusick’s heritable disorders of connective tissue. 5th ed: St. Louis: CV Mosby, 1993:189–253.

    Google Scholar 

  6. Beighton P. Lethal complications of the Ehlers-Danlos syndrome. Br Med J 1968;3:656–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Hunter GC, Malone JM, Moore WS, et al. Vascular manifestations in patients with Ehlers-Danlos syndrome. Arch Surg 1982;117:495–8.

    Article  CAS  PubMed  Google Scholar 

  8. Klipple GL, Riordan KK. Rare inflammatory and hereditary connective tissue diseases. Rheum Dis Clin North Am 1989;15:383–98.

    CAS  PubMed  Google Scholar 

  9. Leduc L, Wasserstrum N. Successful treatment with the Smith-Hodge pessary of cervical incompetence due to defective connective tissue in Ehlers-Danlos syndrome. Am J Perinatol 1992;9:25–7.

    Article  CAS  PubMed  Google Scholar 

  10. Rudd NL, Holbrook KA, Nimrod C, Byers PH. Pregnancy complications in type IV EDS. Lancet 1983;i:50–3.

    Article  Google Scholar 

  11. Peaceman AM, Cruikshank DP. Ehlers-Danlos syndrome and pregnancy: Association of type IV with maternal death. Obstet Gynecol 1987;69:428–31

    CAS  PubMed  Google Scholar 

  12. Smith CV, Phelan JP. Pregnancy and the Ehlers-Danlos syndrome. A report of 2 cases. J Reprod Med 1982;27:757–60.

    CAS  PubMed  Google Scholar 

  13. Beighton P, Horan F. Orthopedic aspects of the Ehlers-Danlos syndrome. J Bone Joint Surg 1969;51:444–53.

    Article  CAS  Google Scholar 

  14. Rivera-Alsina ME, Kwan P, Zavisca FG, Hopkins S, Abouleish E. Complications of the Ehlers-Danlos syndrome in pregnancy: A case report. J Reprod Med 1984;29:757–9.

    CAS  PubMed  Google Scholar 

  15. Donlan P, Siski F, Riley E. Anesthetic considerations for Ehlers-Danlos syndrome. Anesthesiology 1980;52:266–9.

    Article  Google Scholar 

  16. National Center for Health Statistics. Health, United States, 1992. Hyattsville, Maryland: Public Health Service, 1993.

    Google Scholar 

  17. Walters MD, Shields LE. The diagnostic value of history, physical examination, and the Q-tip cotton swab test in women with urinary incontinence. Am J Obstet Gynecol 1988;159:145–9.

    Article  CAS  PubMed  Google Scholar 

  18. Nemir A, Middleton RP. Stress incontinence in young nulliparous women. A statistical study. Am J Obstet Gynecol 1954;68:1166–8.

    Article  CAS  PubMed  Google Scholar 

  19. Wolin LH. Stress incontinence in young, healthy nulliparous female subjects. J Urol 1969;101:545–9.

    Article  CAS  PubMed  Google Scholar 

  20. Iosif CS, Bekassy Z, Rydhstrom H. Prevalence of urinary incontinence in middle-aged women, Int J Gynecol Obstet 1988;26:255–9.

    Article  CAS  Google Scholar 

  21. Elving LB, Foldspang A, Lam GW, Mommsen S. Descriptive epidemiology of urinary incontinence in 3,100 women age 30–59. Scand J Urol Nephrol Suppl 1989;125:37–43.

    CAS  PubMed  Google Scholar 

  22. Thomas TM, Plymat KR, Blannin J, Meade TW. Prevalance of urinary incontinence. BMJ 1980;281:1243–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Bergman A, McCarthy TA, Ballard CA, Yanai J. Role of the Q-tip test in evaluating stress urinary incontinence. J Reprod Med 1987;32:273–5.

    CAS  PubMed  Google Scholar 

  24. Diokno AC, Normolle DP, Brown MB, Herzog AR. Urodynamic tests for female geriatric urinary incontinence. Urology 1990;36:431–9.

    Article  CAS  PubMed  Google Scholar 

  25. Ulmsten U, Ekman G, Malmstrom A. Different biochemical composition of connective tissue in incontinent and stress incontinent women. Acta Obstet Gynecol Scand 1987;66:455–7.

    Article  CAS  PubMed  Google Scholar 

  26. Nichols DH, Randall CL. Types of genital prolapse. In: Vaginal surgery. 3rd ed. Baltimore: Williams and Wilkins, 1989:69.

    Google Scholar 

  27. Makinen J, Soderstrom KO, Killholm P, Hirvonen T. Histological changes in the vaginal connective tissue of patients with and without uterine prolapse. Arch Gynecol 1986;239:17–20.

    Article  CAS  PubMed  Google Scholar 

  28. Wagh PV, Leverich AP, Sun CN, White HJ, Read RC. Direct inguinal herniation in men: A disease of collagen. J Surg Res 1974;17:425–33.

    Article  CAS  PubMed  Google Scholar 

  29. Wagh PV, Read RC. Defective collagen synthesis in inguinal herniation. Am J Surg 1972;124:819–22.

    Article  CAS  PubMed  Google Scholar 

  30. Al-Rawi ZS, Al-Rawi ZT. Joint hypermobility in women with genital prolapse. Lancet 1982;1439–41.

    Google Scholar 

  31. Norton P, Baker J, Sharp H, Warenski J. Genitourinary prolapse: Relationship with joint mobility. Obstet Gynecol 1995;85:225–8.

    Article  CAS  PubMed  Google Scholar 

  32. Wheeler JM. Epidemiology of endometriosis-associated infertility. J Reprod Med 1989;34:41–6.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Gynecology, Department of Obstetrics and Gynecology, and the Departments of Molecular Biology and Pathology, Hutzel Hospital/Wayne State University, 4707 St. Antoine Boulevard, Detroit, MI, 48201, USA

    Lisa J. Mcintosh MD, Veronica T. Mallett MD, Jane D. Frahm PT, David A. Richardson MD & Mark I. Evans MD

Authors
  1. Lisa J. Mcintosh MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Veronica T. Mallett MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jane D. Frahm PT
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. David A. Richardson MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Mark I. Evans MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mcintosh, L.J., Mallett, V.T., Frahm, J.D. et al. Gynecologic Disorders in Women With Ehlers-Danlos Syndrome. Reprod. Sci. 2, 559–564 (1995). https://doi.org/10.1177/107155769500200309

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1177/107155769500200309

Key words

Search

Navigation