Mayer-Rokitansky Syndrome Follow-up

  • Author: Andrew J Kirsch, MD, FAAP, FACS; Chief Editor: Andrea L Zuckerman, MD   more...
 
Updated: Mar 5, 2012
 

Further Inpatient Care

  • Removal of the rudimentary uterus is suggested if hematometra develops.
Next

Further Outpatient Care

  • Monitor how well patients comply with postsurgical dilation regimens.
  • Recommend condoms to help prevent human papilloma virus infection.[8] Regularly examine patients to assess for stenosis.
Previous
Next

Complications

  • Excessive skin scarring at donor graft site
  • Postoperative rectovaginal and urethrovaginal fistulas
  • Vaginal discharge and dyspareunia
  • Vaginal stenosis
  • Inadequate vaginal lubrication
  • Poor patient compliance with postsurgical dilation schedule
Previous
Next

Prognosis

  • The patient may have normal sexual functioning after surgical reconstruction.
  • Surgical reconstruction does not establish the ability to conceive through natural means. Conception cannot occur without the aid of assisted reproductive techniques.
Previous
Next

Patient Education

  • To achieve optimal results, stress compliance with a home dilation schedule.
  • Thoroughly discuss the embryonic development of the reproductive system with the patient if the patient is interested and is able to use the information.
  • Genetic offspring are possible through use of a gestational carrier as the ovaries are normal.
  • If indicated, refer the patient for psychological counseling to explore gender identity issues.
  • For excellent patient education resources, visit eMedicine's Women's Health Center. Also, see eMedicine's patient education articles Amenorrhea and Female Sexual Problems.
Previous
 
Contributor Information and Disclosures
Author

Andrew J Kirsch, MD, FAAP, FACS  Clinical Professor of Urology, Chief of Pediatric Urology, Emory University School of Medicine, Children's Healthcare of Atlanta; Partner, Georgia Urology, PA

Andrew J Kirsch, MD, FAAP, FACS is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, and Society for Fetal Urology

Disclosure: Nothing to disclose.

Coauthor(s)

Jonathan D Kaye, MD  Fellow in Pediatric Urologic Surgery, Department of Urology, Emory University School of Medicine

Jonathan D Kaye, MD is a member of the following medical societies: American Medical Association, American Urological Association, and Endourological Society

Disclosure: Nothing to disclose.

Suzanne M Carter, MS  Senior Genetic Counselor, Associate, Department of Obstetrics and Gynecology, Division of Reproductive Genetics, Montefiore Medical Center, Albert Einstein College of Medicine

Suzanne M Carter, MS is a member of the following medical societies: American Bar Association

Disclosure: Nothing to disclose.

Susan J Gross, MD, FRCS(C), FACOG, FACMG  Codirector, Division of Reproduction Genetics, Associate Professor, Department of Obstetrics and Gynecology, Albert Einstein College of Medicine

Susan J Gross, MD, FRCS(C), FACOG, FACMG is a member of the following medical societies: American College of Medical Genetics, American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, American Medical Association, American Society of Human Genetics, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Elizabeth Alderman, MD  Director of Fellowship Training Program, Director of Adolescent Ambulatory Service, Professor of Clinical Pediatrics, Department of Pediatrics, Division of Adolescent Medicine, Albert Einstein College of Medicine and Children's Hospital at Montefiore

Elizabeth Alderman, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, North American Society for Pediatric and Adolescent Gynecology, and Society for Adolescent Medicine

Disclosure: Merck Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Wayne Wolfram, MD, MPH  Associate Professor, Department of Emergency Medicine, Mercy St Vincent Medical Center

Wayne Wolfram, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Pediatrics, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Andrea L Zuckerman, MD  Assistant Professor of Obstetrics/Gynecology and Pediatrics, Tufts University School of Medicine; Division Director, Pediatric and Adolescent Gynecology, Tufts Medical Center

Andrea L Zuckerman, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, Association of Professors of Gynecology and Obstetrics, Massachusetts Medical Society, North American Society for Pediatric and Adolescent Gynecology, and Society for Adolescent Medicine

Disclosure: Nothing to disclose.

References

  1. Sultan C, Biason-Lauber A, Philibert P. Mayer-Rokitansky-Kuster-Hauser syndrome: recent clinical and genetic findings. Gynecol Endocrinol. Jan 2009;25(1):8-11. [Medline].

  2. Morcel K, Guerrier D, Watrin T, Pellerin I, Leveque J. [The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome: clinical description and genetics]. J Gynecol Obstet Biol Reprod (Paris). Oct 2008;37(6):539-46. [Medline].

  3. Edmonds DK, Rose GL, Lipton MG, Quek J. Mayer-Rokitansky-Küster-Hauser syndrome: a review of 245 consecutive cases managed by a multidisciplinary approach with vaginal dilators. Fertil Steril. Mar 2012;97(3):686-90. [Medline].

  4. Carrard C, Chevret-Measson M, Lunel A, Raudrant D. Sexuality after sigmoid vaginoplasty in patients with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril. Mar 2012;97(3):691-6. [Medline].

  5. Hensle TW, Chang DT. Vaginal reconstruction. Urol Clin North Am. Feb 1999;26(1):39-47, vii. [Medline].

  6. Hensle TW, Shabsigh A, Shabsigh R, Reiley EA, Meyer-Bahlburg HF. Sexual function following bowel vaginoplasty. J Urol. Jun 2006;175(6):2283-6. [Medline].

  7. Fedele L, Bianchi S, Frontino G, Fontana E, Restelli E, Bruni V. The laparoscopic Vecchietti's modified technique in Rokitansky syndrome: anatomic, functional, and sexual long-term results. Am J Obstet Gynecol. Apr 2008;198(4):377.e1-6. [Medline].

  8. Frega A, Scirpa P, Sopracordevole F, Biamonti A, Bianchi P, De Sanctis L, et al. Impact of human papillomavirus infection on the neovaginal and vulval tissues of women who underwent surgical treatment for Mayer-Rokitansky-Kuster-Hauser syndrome. Fertil Steril. Oct 2011;96(4):969-73. [Medline].

  9. Acien P, Lloret M, Chehab H. Endometriosis in a patient with Rokitansky-Kuster-Hauser syndrome. Gynecol Obstet Invest. 1988;25(1):70-2. [Medline].

  10. Blask AR, Sanders RC, Rock JA. Obstructed uterovaginal anomalies: demonstration with sonography. Part II. Teenagers. Radiology. Apr 1991;179(1):84-8. [Medline].

  11. Chervenak FA, Stangel JJ, Nemec M, Amin HK. Mayer-Rokitansky-Küster-Hauser syndrome. Congenital absence of vagina. N Y State J Med. Jan 1982;82(1):23-6. [Medline].

  12. Edmonds DK. Congenital malformations of the genital tract and their management. Best Pract Res Clin Obstet Gynaecol. Feb 2003;17(1):19-40. [Medline].

  13. Farber M, Mitchell GW. Surgery for congenital absence of the vagina. Obstet Gynecol. Mar 1978;51(3):364-7. [Medline].

  14. Fedele L, Dorta M, Brioschi D, et al. Magnetic resonance imaging in Mayer-Rokitansky-Küster-Hauser syndrome. Obstet Gynecol. Oct 1990;76(4):593-6. [Medline].

  15. Folgueira G, Perez-Medina T, Martinez-Cortes L, et al. Laparoscopic creation of a neovagina in Mayer-Rokitansky-Küster-Hauser syndrome by modified Vecchietti's procedure. Eur J Obstet Gynecol Reprod Biol. Aug 2006;127(2):240-3. [Medline].

  16. Garcia J, Jones HW. The split thickness graft technic for vaginal agenesis. Obstet Gynecol. Mar 1977;49(3):328-32. [Medline].

  17. Graziano K, Teitelbaum DH, Hirschl RB, Coran AG. Vaginal reconstruction for ambiguous genitalia and congenital absence of the vagina: a 27-year experience. J Pediatr Surg. Jul 2002;37(7):955-60. [Medline].

  18. Griffin JE, Edwards C, Madden JD, et al. Congenital absence of the vagina. The Mayer-Rokitansky-Kuster-Hauser syndrome. Ann Intern Med. Aug 1976;85(2):224-36. [Medline].

  19. Ikuta K, Iida T, Okada H, et al. Laparoscopic-assisted creation of a vagina. J Am Assoc Gynecol Laparosc. Nov 1996;4(1):53-6. [Medline].

  20. Joseph VT. Pudendal-thigh flap vaginoplasty in the reconstruction of genital anomalies. J Pediatr Surg. Jan 1997;32(1):62-5. [Medline].

  21. Okada E, Iwahira Y, Maruyama Y. Treatment of vaginal agenesis with an expanded vulval flap. Plast Reconstr Surg. Sep 1996;98(3):530-3. [Medline].

  22. Oppelt P, Renner SP, Kellermann A, et al. Clinical aspects of Mayer-Rokitansky-Kuester-Hauser syndrome: recommendations for clinical diagnosis and staging. Hum Reprod. Mar 2006;21(3):792-7. [Medline].

  23. Rosenberg HK, Sherman NH, Tarry WF, et al. Mayer-Rokitansky-Kuster-Hauser syndrome: US aid to diagnosis. Radiology. Dec 1986;161(3):815-9. [Medline].

  24. Tolhurst DE, van der Helm TW. The treatment of vaginal atresia. Surg Gynecol Obstet. May 1991;172(5):407-14. [Medline].

  25. Troiano RN, McCarthy SM. Mullerian duct anomalies: imaging and clinical issues. Radiology. Oct 2004;233(1):19-34. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.