Abstract
In women with vaginal hypoplasia, such as in Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) and in Complete Androgen Insensitivity Syndrome (CAIS), surgical vaginoplasty and non-surgical self-dilation treatments are available to lengthen the vagina and facilitate sexual intercourse, but the best treatment remains controversial. Vaginal dilation has been recommended as a first-line treatment, because of its less invasive character and high success rate. However, the exploration of factors associated with compliance and long-term outcome is incomplete, including whether psychological counselling needs to be embedded in treatment to maximize efficacy. It is not known if failed vaginal dilation therapy jeopardizes further surgical success outcomes, especially because in a number of these procedures ongoing vaginal dilation is required. In addition, if surgery is needed, there is a lack of evidence to inform physicians regarding the optimum surgical technique to use. Also, it is unclear whether maintenance dilation therapy in case of sexual inactivity is crucial to ensure functional success. In view of this ongoing debate, we performed a search of all published literature (English language only) restricted to the management of vaginal hypoplasia in patients with MRKH or CAIS from 1898 to March 2013 using Pubmed, Cochrane Library and Web of Science. Of the 6700 articles initially identified, a total of 190 studies are analysed. More specifically, by establishing the risk/efficacy profile (vaginal capacity, complications and long-term durability in terms of sexual function) of the different surgical and non-surgical reconstruction techniques, we evaluate if vaginal dilation proposed as the first-line technique is justified based on the evidence. When anatomical success was defined as a length of ≥7 cm and functional success as coitus, all vaginoplasty techniques yielded significantly higher success rates (>90 versus 75% after vaginal dilation), irrespective of underlying diagnosis or start vaginal length. When functional success was defined as 'satisfaction with sex', including non-genital sex, differences disappeared. Failed dilation therapy does not preclude anatomical (nor functional) success if vaginoplasty afterwards is necessary. Traction vaginoplasty seems to have the highest anatomical (99%) and functional success rates (96%), whereas both split- and full-thickness skin graft procedures and intestinal procedures have the lowest successful outcomes (83-95%). Overall, complication rates were significantly lower within the vaginal dilation groups when compared with the different vaginoplasty techniques. Although no randomized control data exist regarding maintenance dilation, the available evidence suggests that continued dilation is needed to maintain patency in periods of coital inactivity. Despite the expectancy that the probability of further positive outcomes is maximized with psychological counselling, this could not be confirmed. As the medical literature lacks high-quality comparative outcome studies and prospective, longitudinal studies are scarce, no evidence-based treatment guidelines can be provided. However, because of the physically low complication rate and an overall success chance of 75%, vaginal dilation as first choice treatment seems to be justified. Overall, the laparoscopic Vecchietti procedure, becoming more and more available in specialized centres, is considered an appropriate surgical option in patients who are poorly compliant and failed dilation therapy, or for those who do not want to start with vaginal dilation therapy. Future approaches need to raise a wider range of psychosexually oriented questions, elucidate the relationship between vaginal depth and satisfactory outcomes and gain additional experience concerning the format of acceptable and efficient psychological care.
Highlights
(vaginal capacity, complications and long-term durability in terms of sexual function) of the different surgical and non-surgical reconstruction techniques, we evaluate if vaginal dilation proposed as the first-line technique is justified based on the evidence
The laparoscopic Vecchietti procedure, becoming more and more available in specialized centres, is considered an appropriate surgical option in patients who are poorly compliant and failed dilation therapy, or for those who do not want to start with vaginal dilation therapy
It is apparent that the clinical management of congenital disorders of sex development (DSD)—in which anatomical, gonadal or chromosomal sex is atypical—has been in a state of great flux, debate and controversy during the last decade (Zucker, 2002; Hughes et al, 2006)
Summary
It is apparent that the clinical management of congenital disorders of sex development (DSD)—in which anatomical, gonadal or chromosomal sex is atypical—has been in a state of great flux, debate and controversy during the last decade (Zucker, 2002; Hughes et al, 2006). The differential diagnosis of MRKH mainly includes 46,XY DSD, androgen insensitivity syndrome in which the lack of a functional androgen receptor results in phenotypically normal female external genitalia (Quigley et al, 1995) These women have testes, absent Mullerian structures and a blind short vaginal pouch due to the action of antiMullerian hormone (Sarpel et al, 2005; Hurme et al, 2009). Besides correct diagnosis of the underlying condition and evaluation for associated congenital anomalies, an important step in the effective medical management is psychosocial counselling before any treatment or intervention (ACOG, 2013) These conditions have devastating implications for fertility and sexual intercourse and affected women may experience variable levels of distress, connected to the perception of having a compromised womanhood and characterized by the fear of being devalued by others (Hecker and McGuire, 1977; Alderson et al, 2004). Regular coitus (Serment et al, 1969)
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