The Uterus pp 272-293 | Cite as

Congenital Absence of the Uterus and Vagina

  • Albert Altchek
Part of the Clinical Perspectives in Obstetrics and Gynecology book series (CPOG)

Abstract

Congenital absence of the vagina is referred to as Rokitansky syndrome, Mayer-Rokitansky-Kuster-Hauser syndrome, vaginal agenesis, and vaginal aplasia. There are several recent reviews of the condition.1–4 The classical reference is the Jones and Rock text.5 Absence of the uterus in genetic females occurs as part of the Rokitansky syndrome. About 9–10% of cases of this syndrome may have a functioning uterus but without a vagina. The syndrome is focused on a vagina because its absence is more readily noticed than the absence of the uterus.

Keywords

Depression Estrogen Foam Rubber Testosterone 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Griffin JE, Edwards C, Madden JD, et al: Congenital absence of the vagina. The Mayer-Rokitansky-Kuster-Hauser syndrome. Ann Intern Med 1976;85:224–236.PubMedGoogle Scholar
  2. 2.
    Rock JA: Anomalous development of the vagina. Semin Reprod Endocrinol 1986;4:13–32.CrossRefGoogle Scholar
  3. 3.
    Edmonds DK: Congenital malformations of the vagina and their management. Semin Reprod Endocrinol 1988;6:91–98.CrossRefGoogle Scholar
  4. 4.
    Berg PA, Breen JL, Gregori CA: Congenital absence of the vagina—the Mayer-Rokitansky-Kuster-Hauser syndrome. Adolesc Pediatr Gynecol 1989;2:73–85.Google Scholar
  5. 5.
    Jones HW Jr, Rock JA: Anomalies of the mullerian ducts, in: Reparative and Constructive Surgery of the Female Generative Tract. Baltimore, Williams & Wilkins, 1983, pp 146–186.Google Scholar
  6. 6.
    Strubbe EH, Thijn CJ, Willemsen WN, et al: Evaluation of radiographic abnormalities of the hand in patients with the Mayer-Rokitansky-Kuster-Hauser syndrome. Skeletal Radiol 1987;16:227–231.PubMedCrossRefGoogle Scholar
  7. 7.
    Biedel CW, Pagon RA, Zapata JO: Mullerian anomalies and renal agenesis: autosomal dominant urogenital adysplasia. J Pediatr 1984;104:861–864.PubMedCrossRefGoogle Scholar
  8. 8.
    Ghirardini G, Segre A: Vaginal agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome): recent etiopathogenetical and anatomical views. Clin Exp Obstet Gynecol 1982;2:98–102.Google Scholar
  9. 9.
    McPherson E, Carey J, Kramer A, et al: Dominantly inherited renal adysplasia. Am J Med Genet 1987;26:863–872.PubMedCrossRefGoogle Scholar
  10. 10.
    Opitz JM: Editorial comment: Vaginal atresia. (von Mayer-Rokitansky-Kuster or MRK anomaly) in hereditary renal adysplasia (HRA). Am J Med Genet 1987;26:873–876.PubMedCrossRefGoogle Scholar
  11. 11.
    Cramer, DW, Ravnikar VA, Craighill M, et al: Mullerian aplasia associated with maternal deficiency of galactose-1-phosphate uridyl transferase. Fertil Steril 1987;47:930–934.PubMedGoogle Scholar
  12. 12.
    Markham SM, Huggins GR, Parmely TH, et al: Cervical agenesis combined with vaginal agenesis diagnosed by magnetic resonance imaging. Fertil Steril 1987;48:143–145.PubMedGoogle Scholar
  13. 13.
    Rock JA, Jones HW: Construction of a neovagina for patients with a flat perineum. Am J Obstet Gynecol 1989;160:845–853.PubMedGoogle Scholar
  14. 14.
    Mclndoe AH, Bannister JB: An operation for the cure of congenital absence of the vagina. J Obstet Gynaecol Br Emp 1938;45:490–494.CrossRefGoogle Scholar
  15. 15.
    Abbe R: New method of creating a vagina in a case of congenital absence. Med Rec 1898;64:836–838.Google Scholar
  16. 16.
    Counseller VA: Congenital absence and traumatic obliteration of the vagina and its treatment with inlaying Thiersch grafts. Am J Obstet Gynecol 1938;36:632–638.Google Scholar
  17. 17.
    Buss JG, Lee RA: Mclndoe procedure for vaginal agenesis: results and complications. Mayo Clin Proc 1989;64:758–761.PubMedGoogle Scholar
  18. 18.
    Frank RT: The formation of an artificial vagina without operation. Am J Obstet Gynecol 1938;35:1053–1055.Google Scholar
  19. 19.
    Ingram JM: The bicycle seat stool in the treatment of vaginal agenesis and stenosis: a preliminary report. Am J Obstet Gynecol 1981;140:867–873.PubMedGoogle Scholar
  20. 20.
    Hughes EG, Spence JEH: Human amniotic membrane: its use as an allograft in vaginal construction. Adolesc Pediatr Gynecol 1988;1:39–41.Google Scholar
  21. 21.
    Wharton LR: A simple method of constructing a vagina: report of four cases. Ann Surg 1938;107:842–854.PubMedCrossRefGoogle Scholar
  22. 22.
    Sheares BH: Congenital atresia of the vagina: a new technique for tunnelling the space between bladder and rectum and construction of the new vagina by a modified Wharton technique. J Obstet Gynaecol Br Emp 1960;67:24–31.PubMedCrossRefGoogle Scholar
  23. 23.
    Williams EA: Congenital absence of the vagina. A simple operation for its relief. J Obstet Gynaecol Br Commow 1964;71:511–512.CrossRefGoogle Scholar
  24. 24.
    Baldwin JF: The formation of an artificial vagina by intestinal transplantation. Ann Surg 1904;40:398–403.PubMedGoogle Scholar
  25. 25.
    Novak F, Kos L, Plesko F: The advantages of the artificial vagina derived from sigmoid colon. Acta Obstet Gynecol Scand 1978; 57:95–96.PubMedCrossRefGoogle Scholar
  26. 26.
    Altchek A: Psychologic aspects of adolescent obstetric and gynecologic conditions, in Heacock DR (ed): A Psychodynamic Approach to Adolescent Psychiatry. The Mount Sinai Experience. New York, Marcel Dekker, 1980; pp 239–278.Google Scholar
  27. 27.
    Altchek A: What to do when there is no vagina. Contemp OB/GYN 1988;32:50–51,Google Scholar
  28. 27a.
    Altchek A: What to do when there is no vagina. Contemp OB/GYN 1988;32:54,Google Scholar
  29. 27b.
    Altchek A: What to do when there is no vagina. Contemp OB/GYN 1988;32:57–59,Google Scholar
  30. 27c.
    Altchek A: What to do when there is no vagina. Contemp OB/GYN 1988;32:63–64,Google Scholar
  31. 27d.
    Altchek A: What to do when there is no vagina. Contemp OB/GYN 1988;32:66,Google Scholar
  32. 27e.
    Altchek A: What to do when there is no vagina. Contemp OB/GYN 1988;32:68.Google Scholar
  33. 28.
    Altchek A: Pediatric and adolescent gynecology, in Nichols DH, Evrard JR (eds): Ambulatory Gynecology. Philadelphia, Harper & Row 1985, pp 20–62.Google Scholar
  34. 29.
    Salvatore CA, Lodovicci O: Vaginal agenesis: an analysis of ninety cases. Acta Obstet Gynecol Scand 1978;57:89–94.PubMedCrossRefGoogle Scholar
  35. 30.
    Rock JA, Azziz R: Genital anomalies in childhood. Clin Obstet Gynecol 1987;30:682–696.PubMedCrossRefGoogle Scholar
  36. 31.
    Niver DH, Barrett G, Jewelewicz R: Congenital atresia of the uterine cervix and vagina: three cases. Fertil Steril 1980;33:25–29.PubMedGoogle Scholar
  37. 32.
    Maciulla GJ, Heine MW, Christian CD: Functional endometrial tissue with vaginal agenesis. J Reprod Med 1978;21:373–376.PubMedGoogle Scholar
  38. 33.
    Jacob JH, Griffin WT: Surgical reconstruction of the congenitally atretic cervix: two cases. Obstet Gynecol Surv 1989;44:556–569.PubMedCrossRefGoogle Scholar
  39. 34.
    Fraser IS: Successful pregnancy in a patient with congenital partial cervical atresia. Obstet Gynecol 1989;74:443–445.PubMedGoogle Scholar
  40. 35.
    Farber M: Congenital atresia of the uterine cervix. Semin Reprod Endocrinol 1986;4:33–38.CrossRefGoogle Scholar
  41. 36.
    Cukier J, Batzofin JH, Conner JS, et al: Genital tract reconstruction in a patient with congenital absence of the vagina and hypoplasia of the cervix. Obstet Gynecol 1986;68:32s–36s.PubMedGoogle Scholar
  42. 37.
    Murphy AA, Krall A, Rock JA: Bilateral functioning uterine analgen with the Rokitansky-Mayer-Kuster-Hauser syndrome. Int J Fertil 1987;32:316–319.PubMedGoogle Scholar
  43. 38.
    Sing KJ, Devi L: Hysteroplasty and vaginoplasty for reconstruction of the uterus. Int J Gynecol Obstet 1980;17:457–459.Google Scholar
  44. 39.
    Singh J, Devi YL: Pregnancy following surgical correction of nonfused mullerian bulbs and absent vagina. Obstet Gynecol 1983; 61:267–269.PubMedGoogle Scholar

Copyright information

© Springer-Verlag New York Inc. 1991

Authors and Affiliations

  • Albert Altchek

There are no affiliations available

Personalised recommendations