Mini-ReviewManagement of Vaginal Agenesis
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
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Long-term Results of Sexual Function and Body Image After Vaginoplasty With Acellular Dermal Matrix in Women With Mayer-Rokitansky-Küster-Hauser Syndrome
2024, Journal of Minimally Invasive GynecologyThe Sexuality of Adolescents and Young Women With MRKH Syndrome: A Qualitative Study
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2021, International Journal of Surgery Case ReportsCitation Excerpt :In many cases, only a short vagina or small duct-like structure mimicking the vagina can be seen, and the uterus may be rudimentary. The disconnection of the malformed vagina with the uterus is a common finding [18,19,20]. Patients with a congenital cloacal malformation may need to undergo complex reconstruction of the rectourogenital tracts.
Robotic assisted ileo-vaginoplasty for vaginal atresia
2021, Journal of Pediatric UrologyNeovagina Creation: A Novel Improved Laparoscopic Vecchietti Procedure in Patients with Mayer-Rokitansky-Küster-Hauster Syndrome
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The authors report no conflicts of interest.