Mini-Review
Management of Vaginal Agenesis

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Abstract

Rokitansky syndrome and complete androgen insensitivity syndrome are the most common causes of vaginal agenesis. Treatment should be deferred until adolescence to allow informed consent and compliance. The best treatment for vaginal agenesis remains controversial although vaginal dilation therapy is still widely considered the first line treatment because success rates are high and associated risks are low. A variety of surgical options are also available, each with enthusiastic proponents. Long-term outcome studies on most surgical techniques, however, are still lacking and until recently most studies have reported on success rate in terms of anatomical success only, without including sexual function. Moreover, the medical literature lacks prospective comparative outcome studies, meaning that current choice of surgical procedure relies greatly on the surgeon’s preference and experience.

Introduction

Congenital vaginal agenesis is most commonly seen in women with Rokitansky syndrome (also referred to as Mayer-Rokitansky-Kuster-Hauser syndrome or Müllerian aplasia), and complete androgen insensitivity syndrome (CAIS). In addition it can be associated with other rarer complex conditions affecting the urinary and gastrointestinal tracts, such as cloacal and anorectal anomalies. Women with Rokitansky syndrome have a XX karyotype and normal functioning ovaries but an absent or rudimentary uterus and a short vagina resulting from failed embryonic development of the Müllerian duct. It occurs in one in 5,000–10,000 female births; however, the exact etiology is still unknown. Women with CAIS, which affects one in 13,000–40,000 live births, have 46,XY karyotype, testicular gonads, absent Müllerian structures, and a short vagina. This is attributed to a mutation in the androgen receptor gene which renders the body insensitive to testosterone and hence results in a female phenotype with female external genitalia. Since patients with Rokitansky syndrome and CAIS have normal breast development either from normal ovarian hormones in the former condition or from peripheral conversion of testosterone to estradiol in the latter one, these patients typically present in adolescence with primary amenorrhea. Detailed investigation is required to diagnose and differentiate these conditions; however, in a clinical setting the vaginal findings are identical with an absent or short vaginal dimple. Surgical and non-surgical treatments are available to lengthen the vagina and facilitate penetrative sexual intercourse.

Section snippets

Timing of Treatment

In the majority of cases these conditions present in adolescence which means the patient can be fully involved in decisions about the type and timing of treatment. Where presentation is earlier in childhood, it is accepted that both non-surgical and surgical methods of vaginal creation are best deferred until adolescence or even adulthood when the patient has reached physical and psychological maturity.1, 2 This allows for proper decision making and also increases the compliance with vaginal

Treatment Choice

The best treatment for vaginal agenesis is still controversial. In the USA and UK vaginal dilation therapy is considered the best first-line treatment2 and surgery is reserved for cases when vaginal dilation therapy fails or when a patient is ineligible for vaginal dilation due to previous perineal surgery. However, in many European countries, surgical vaginoplasty such as a laparoscopic Vecchietti or Davydov procedure is the first line treatment and vaginal dilation is only used

Vaginal Dilation

Vaginal dilation therapy for vaginal agenesis was first described by Frank in 1938.4 It involves the use of vaginal molds, initially made from pyrex and currently made from plastic, of increasing width and length. The patient is asked to apply gentle pressure on the vaginal dimple for at least 30 minutes daily for several months with the aim to achieve a vaginal length of 7–8 cm. This therapy works by the pressure effect which acts to progressively stretch the vagina with time. Thus the

Surgical Methods

Surgical vaginoplasties can be subdivided into the following categories:

  • 1.

    Creation of a perineal pouch. This includes the Williams vaginoplasty and subsequent modifications.

  • 2.

    Lining a neovaginal space. This includes procedures based on the McIndoe technique where a neovaginal space is dissected between the bladder and rectum, then lined with different types of tissue.

  • 3.

    Laparoscopic procedures. These methods include the Vecchietti procedure and Davydov procedures.

  • 4.

    Intestinal vaginoplasty.

Creation of a Perineal Pouch

Initially described by Williams in 1964, this technique fell into general disuse until modified by Creatsas et al in 2001.15 It involves a U-shaped incision extending from the medial side of the labia at the level of the external urethral meatus down across the perineal body to form a skin flap. The tissues are then mobilized and sutured in layers to form a pocket in the perineum to allow coitus.3 In a study on 178 Rokitansky patients, anatomical success was achieved in 96% while 94% reported

Conclusion

At present there is still no agreement on the gold standard treatment for vaginal agenesis. Most gynecologists in the USA and the UK agree that vaginal dilation therapy should be the first line treatment as it is successful without the risks of surgery. However, vaginal dilation is time consuming and some patients find it distressing. Short term outcome data on the laparoscopic Vecchietti procedure has also confirmed good success rates and low morbidity and this may also be an appropriate first

References (63)

  • A. Keser et al.

    Treatment of vaginal agenesis with modified Abbé-McIndoe technique: long-term follow-up in 22 patients

    Eur J Obstet Gynecol Reprod Biol

    (2005)
  • M. Lima et al.

    Vaginal replacement in the pediatric age group: a 34-year experience of intestinal vaginoplasty in children and young girls

    J Pediatr Surg

    (2010)
  • C. Fotopoulou et al.

    Functional and anatomic results of amnion vaginoplasty in young women with Mayer-Rokitansky- Kauster-Hauser syndrome

    Fertil Steril

    (2010)
  • L. Fedele et al.

    Laparoscopic creation of a neovagina in Mayer-Rokitansky-Kuster-Hauser syndrome by modification of Vecchietti’s operation

    Am J Obstet Gynecol

    (1994)
  • L. Fedele et al.

    Neovaginal mucosa after Vecchietti’s laparoscopic operation for Rokitansky syndrome: Structural and ultrastructural study

    Am J Obstet Gynecol

    (2006)
  • J.L. Brun et al.

    Long-term results after neovagina creation in Mayer-Rokitanski-Kuster-Hauser syndrome by Vecchietti’s operation

    Eur J Obstet Gynecol Reprod Biol

    (2002)
  • F. Borruto et al.

    The laparoscopic Vecchietti technique for vaginal agenesis

    Int J Gynaecol Obstet

    (2007)
  • S. Brucker et al.

    Neovagina creation in vaginal agenesis: development of new laparoscopic Vecchietti-based procedure and optimized instruments in a prospective comparative interventional study in 101 patients

    Fertil Steril

    (2008)
  • L. Fedele et al.

    Creation of a neovagina in Rokitansky patients with a pelvic kidney: comparison of long-term results of the modified Vecchietti and McIndoe techniques

    Fertil Steril

    (2010)
  • A.M. El Saman et al.

    Laparoscopically assisted balloon vaginoplasty for management of vaginal aplasia

    Int J Gynaecol Obstet

    (2007)
  • A.M. El Saman et al.

    Enhancement balloon vaginoplasty for treatment of blind vagina due to androgen insensitivity syndrome

    Fertil Steril

    (2011)
  • D. Dargent et al.

    Gynecol Obstet Fertil

    (2004)
  • L.V. Adamyan et al.

    Application of endoscopy in surgery for malformations of genitalia

    J Am Assoc Gynecol Laparosc

    (1994)
  • L.M. Allen et al.

    Psychosexual and functional outcomes after creation of a neovagina with laparoscopic Davydov in patients with vaginal agenesis

    Fertil Steril

    (2010)
  • C. Gatti et al.

    Sexuality and psychosocial functioning in young women after colovaginoplasty

    J Urol

    (2010)
  • A. Karateke et al.

    Intestinal vaginoplasty: seven years’ experience of a tertiary center

    Fertil Steril

    (2010)
  • M.C. Davies et al.

    The pitfalls of vaginal construction

    BJU Int

    (2005)
  • P.H. Communal et al.

    Sexuality after sigmoid colpopoiesis in patients with Mayer-Rokitansky-Kuster-Hauser Syndrome

    Fertil Steril

    (2003)
  • T.W. Hensle et al.

    Sexual function following bowel vaginoplasty

    J Urol

    (2006)
  • N. Khen-Dunlop et al.

    Rokitansky syndrome: clinical experience and results of sigmoid vaginoplasty in 23 young girls

    J Urol

    (2007)
  • P. Christopoulos et al.

    Laparoscopic sacrocolpopexy to treat prolapse of the neovagina created by vaginal dilation in rokitansky syndrome

    J Pediatr Adolesc Gynecol

    (2011)
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