Advertisement

Cervical Agenesis

  • Jovana LekovichEmail author
  • Samantha M. Pfeifer
Chapter

Abstract

Cervical agenesis is a rare mullerian anomaly with less than 100 cases described in the literature. The etiology of cervical agenesis is not well understood. Presentation is typically lower abdominal pain that may be cyclic or sporadic around of the time of expected menarche, but no menstruation occurs due to the obstruction at the level of the cervix. Diagnosis is confirmed usually by MRI, which must differentiate cervical agenesis from a transverse vaginal septum as the treatments differ. Retrograde menstruation usually results in development of endometriosis, endometriomas, hematosalpinx, and adhesions sooner than with conditions such as imperforate hymen. Cervical agenesis is associated with vaginal agenesis in <50 % of cases. Treatment options traditionally been hysterectomy as earlier attempts to create a fistulous tract between the vagina and cervix resulted in high morbidity with subsequent reoperations and hysterectomy and even death. Recent treatment with uterovaginal anastomosis has revealed encouraging results, with subsequent pregnancies and deliveries reported. Optimal patients for this treatment method may include those with cervical dysgenesis or distal cervical agenesis, rather than complete cervical agenesis. Careful counseling regarding fertility sparing treatment should be undertaken before deciding on the optimal treatment modality for the individual.

Keywords

Cervical agenesis Cervical dysgenesis Cervical aplasia Retrograde menstruation Uterovaginal anastomosis Transverse vaginal septum Primary amenorrhea 

References

  1. 1.
    Rock JA, Roberts CP, Jones HW. Congenital anomalies of the uterine cervix: lessons from 30 cases managed clinically a common protocol. Fertil Steril. 2010;94:1858–63.CrossRefPubMedGoogle Scholar
  2. 2.
    Fujimoto VY, Miller JH, Klein NA, Soules MR. Congenital cervical atresia: report of seven cases and review of the literature. Am J Obstet Gynecol. 1997;177:1419–25.CrossRefPubMedGoogle Scholar
  3. 3.
    Deffarges JV, Haddad I, Musset R, Paniel BJ. Utero-vaginal anastomosis in women with uterine cervix atresia: long-term follow-up and reproductive performance. A study of 18 cases. Hum Reprod. 2001;16:1772–5.CrossRefGoogle Scholar
  4. 4.
    Bakri Y, Al-Sugair A, Hugosson C. Bicornuate non-fused rudimentary uterine horns with functioning endometria and complete cervical-vaginal agenesis: magnetic resonance diagnosis. Fertil Steril. 1992;58:620–1.CrossRefPubMedGoogle Scholar
  5. 5.
    Buttram Jr VC, Gibbons WE. Müllerian anomalies: a proposed classification (An analysis of 144 cases). Fertil Steril. 1979;32:40–6.CrossRefPubMedGoogle Scholar
  6. 6.
    The American Fertility Society. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, Mullerian anomalies and intrauterine adhesions. Fertil Steril. 1988;49:944–55.CrossRefGoogle Scholar
  7. 7.
    Oppelt P, Renner SP, Brucker S, Strissel PL, Strick R, Oppelt PG, Doerr HG, Schott GE, Hucke J, Wallwiener D, Beckmann MW. The VCUAM (Vagina Cervix Uterus Adnex-associated Malformation) classification: a new classification for genital malformations. Fertil Steril. 2005;84:1493–7.CrossRefPubMedGoogle Scholar
  8. 8.
    Engstad JE. Artificial vagina. Lancet. 1917;37:329–31.Google Scholar
  9. 9.
    Brayan AL, Nigro JA, Counseller VS. One hundred cases of congenital absence of the vagina. Surg Gynecol Obstet. 1942;129:361–7.Google Scholar
  10. 10.
    Griffin JE, Edwards C, Madden JD, Harrod M, Wilson JD. Congenital absence of the vagina: the Mayer-Rokitansky-Kuster-Hauser Syndrome. Ann Intern Med. 1979;85:224–36.CrossRefGoogle Scholar
  11. 11.
    Markham SM, Parmley TH, Murphy AA, Huggins GR, Rock JA. Cervical agenesis combined with vaginal agenesis diagnosed by magnetic resonance imaging. Fertil Steril. 1987;48:143–5.CrossRefPubMedGoogle Scholar
  12. 12.
    Sherwood M, Speed T. Congenital atresia of the cervix. Tex Med. 1941;37:215–9.Google Scholar
  13. 13.
    Rotter C. Surgical correction of the congenital atresia of the cervix. Am J Obstet Gynecol. 1958;76:643–6.CrossRefPubMedGoogle Scholar
  14. 14.
    Zarou G, Acken H, Brevetti R. Surgical management of congenital atresia of the cervix. Am J Obstet Gynecol. 1961;82:923–8.CrossRefPubMedGoogle Scholar
  15. 15.
    Geary W, Weed J. Congenital atresia of the uterine cervix. Obstet Gynecol. 1973;42:213–7.PubMedGoogle Scholar
  16. 16.
    Zarou G, Esposito J, Zarou D. Pregnancy following the surgical correction of the congenital atresia of the cervix. Int J Gynecol Obstet. 1973;11:143–6.CrossRefGoogle Scholar
  17. 17.
    Maciulla G, Heine M, Christian C. Functional endometrial tissue with vaginal agenesis. J Reprod Med. 1978;21:373–6.PubMedGoogle Scholar
  18. 18.
    Dillon W, Mudaliar N, Wingate M. Congenital atresia of the cervix. Obstet Gynecol. 1979;54:126–9.CrossRefPubMedGoogle Scholar
  19. 19.
    Monks P. Uterus didelphys associated with unilateral cervical atresia and renal agenesis. Aust N Z J Obstet Gynaecol. 1979;19:245–6.CrossRefPubMedGoogle Scholar
  20. 20.
    Niver D, Barrett G, Jewelewicz R. Congenital atresia of the uterine cervix and vagina: three cases. Fertil Steril. 1980;33:25–9.CrossRefPubMedGoogle Scholar
  21. 21.
    Valdes C, Malini S, Malinak L. Sonography in the surgical management of vaginal and cervical atresia. Fertil Steril. 1983;40:263–5.CrossRefPubMedGoogle Scholar
  22. 22.
    Jacob J, Griffin W. Surgical reconstruction of the congenitally atretic cervix: two cases. Obstet Gynecol Surv. 1989;44:556–68.CrossRefPubMedGoogle Scholar
  23. 23.
    Fraser I. Successful pregnancy in a patient with congenital partial cervical atresia. Obstet Gynecol. 1989;74:443–5.PubMedGoogle Scholar
  24. 24.
    Thijssen R, Hollander J, Willemsen W, van der Heyden P, van Dongen P, Rolland R. Successful pregnancy after ZIFT in a patient with congenital cervical atresia. Obstet Gynecol. 1990;76:902–4.PubMedGoogle Scholar
  25. 25.
    Suganuma N, Furuhashi M, Moriwaki T, Tsukahara S, Ando T, Ishihara Y. Management of missed abortion in a patient with congenital cervical atresia. Fertil Steril. 2002;77:1071–3.CrossRefPubMedGoogle Scholar
  26. 26.
    Creighton SM, Davies MC, Cutner A. Laparoscopic management of cervical agenesis. Fertil Steril. 2005;85:1510–3.Google Scholar
  27. 27.
    Nunley WC, Kitchin JD. Congenital atresia of the uterine cervix with pelvic endometriosis. Arch Surg. 1980;115:757–8.CrossRefPubMedGoogle Scholar
  28. 28.
    Mericskay M, Kitajewski J, Sassoon D. Wnt5a is required for proper epithelial-mesenchymal interactions in the uterus. Development. 2004;131:2016–72.CrossRefGoogle Scholar
  29. 29.
    Warot X, Fromental-Ramain C, Fraulob V, Chambon P, Dollé P. Gene dosage-dependent effects of the Hoxa-13 and Hoxd-13 mutations on morphogenesis of the terminal parts of the digestive and urogenital tracts. Development. 1997;124:4781–91.PubMedGoogle Scholar
  30. 30.
    Grecco TL, Furlow JD, Duello TM. Immunodetection of estrogen receptors in fetal and neonatal female mouse reproductive tracts. Endocrinology. 1991;129:1326–32.CrossRefGoogle Scholar
  31. 31.
    Fedele L, Bianchi S, Frontino G, Berlanda N, Montefusco S, Borruto F. Laparoscopically assisted uterovestibular anastomosis in patients with uterine cervix atresia and vaginal aplasia. Fertil Steril. 2008;89:212–6.CrossRefPubMedGoogle Scholar
  32. 32.
    Kriplani A, Kachhawa G, Awasthi D, Kulsherestha V. Laparoscopic-assisted uterovaginal anastomosis in congenital atresia of uterine cervix: a follow up study. J Minim Invasive Gynecol. 2012;19:477–84.CrossRefPubMedGoogle Scholar
  33. 33.
    Graham D, Nelson MW. Combined perineal-abdominal sonography in the evaluation of vaginal atresia. J Clin Ultrasound. 1986;14:735–8.CrossRefPubMedGoogle Scholar
  34. 34.
    Scanlan KS, Pozniak MA, Fagerhom M, Shapiro S. Value of transperineal sonography in the assessment of vaginal atresia. Am J Roentgenol. 1990;154:545–8.CrossRefGoogle Scholar
  35. 35.
    Fedele L, Portuese A, Bianchi S. Transrectal ultrasonography in the assessment of congenital vaginal canalization defects. Hum Reprod. 1999;14:359–62.CrossRefPubMedGoogle Scholar
  36. 36.
    Reinhold C, Hricak H, Forstner R, Ascher SM, Bret PM, Meter WR, Semelka R. Primary amenorrhea: evaluation with MRI imaging. Radiology. 1997;203:383–90.CrossRefPubMedGoogle Scholar
  37. 37.
    Letterie GS. Combined congenital absence of the vaginal and cervix. Diagnosis with magnetic resonance imaging and surgical management. Gynecol Obstet Invest. 1998;46:65–7.CrossRefPubMedGoogle Scholar
  38. 38.
    Lang IM, Babyn P, Oliver GD. MR imaging of pediatric utero-vaginal anomalies. Pediatr Radiol. 1999;29:163–70.CrossRefPubMedGoogle Scholar
  39. 39.
    Casey CA, Laufer MR. Cervical agenesis: septic death after surgery. Obstet Gynecol. 1997;90:706–7.CrossRefPubMedGoogle Scholar
  40. 40.
    Rock JA, Schlaff WD, Zacur HA, Jones HW. The clinical management of congenital absence of the uterine cervix. Int J Gynaecol Obstet. 1984;22:231–5.CrossRefPubMedGoogle Scholar
  41. 41.
    Hampton HL, Meeks GR, Bates GW, Wiser WL. Pregnancy after successful vaginoplasty and cervical stenting for partial atresia of the cervix. Obstet Gynecol. 1990;76:900–1.CrossRefPubMedGoogle Scholar
  42. 42.
    Chakravarty B, Konar H, Chowdhury NN. Pregnancies after reconstructive surgery for congenital cervicovaginal atresia. Am J Obstet Gynecol. 2000;183:421–3.CrossRefPubMedGoogle Scholar
  43. 43.
    Anttila L, Penttila TA, Suikkari AM. Successful pregnancy after in-vitro fertilization and transmyometrial embryo transfer in a patient with congenital atresia of cervix. Hum Reprod. 1999;14:1647–9.CrossRefPubMedGoogle Scholar
  44. 44.
    Lai T, Wu M, Hung K, Cheng Y, Chang F. Successful pregnancy by transmyometrial and transtubal embryo transfer after IVF in a patient with congenital cervical atresia who underwent uterovaginal canalization during Caesarean section. Hum Reprod. 2001;16:268–71.CrossRefPubMedGoogle Scholar
  45. 45.
    Callans N, De Cuypere G, De Sutter P, Monstrey S, Weyers S, Hoebeke P, Cools M. An update on surgical and non-surgical treatment for vaginal hypoplasia. Hum Reprod Update. 2014;20:775–801.CrossRefGoogle Scholar
  46. 46.
    Grimsby GM, Baker LA. The use of autologous buccal mucosa grafts in vaginal reconstruction. Curr Urol Rep. 2014;15:428.CrossRefPubMedGoogle Scholar
  47. 47.
    Lima M, Ruggeri G, Randi B, Domini M, Gargano T, La Pergola E, Gregori G. Vaginal replacement in the pediatric age group: a 34-year experience of intestinal vaginoplasty in children and young girls. J Pediatr Surg. 2010;45:2087–91.CrossRefPubMedGoogle Scholar
  48. 48.
    Rock JA, Zacur HA, Dlugi AM, Jones HW, TeLinde RW. Pregnancy success following surgical correction of imperforate hymen and complete transverse vaginal septum. Obstet Gynecol. 1982;59:448–51.PubMedGoogle Scholar
  49. 49.
    Huberlant S, Tailland ML, Poirey S, Mousty R, Ripart-Neveu S, Mares P, de Tayrac R. Congenital cervical agenesis: pregnancy after transmyometrial embryo transfer. J Gynecol Obstet Biol Reprod. 2014;43:521–5.CrossRefGoogle Scholar
  50. 50.
    Xu C, Xu J, Gao H, Huang H. Triplet pregnancy and successful twin delivery in a patient with congenital cervical atresia who underwent transmyometrial embryos transfer and multifetal pregnancy reduction. Fertil Steril. 2009;91:e1–3.Google Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Open Access This chapter is distributed under the terms of the Creative Commons Attribution Noncommercial License, which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.

Authors and Affiliations

  1. 1.Ronald O. Perelman and Claudia Cohen Center for reproductive Medicine Weill Cornell Medical CollegeNew YorkUSA
  2. 2.Weill Cornell Medical CollegeNew YorkUSA

Personalised recommendations