Management of Vaginal Hypoplasia in Disorders of Sexual Development: Surgical and Non-Surgical Options

Sex Dev 2010;4:292–299 Published online: July 24, 2010 DOI: 10.1159/000316231 Management of Vaginal Hypoplasia in Disorders of Sexual Development: Surgical and Non-Surgical Options R. Deans M. Berra S.M. Creighton University College Hospital, Institute of Women’s Health, London , UK Key Words gardless of the vaginal reconstruction technique, patients Disorders of sexual development ؒ Surgery ؒ should be managed in a multidisciplinary team where there Vaginal hypoplasia is adequate emotional and psychological support available. Copyright © 2010 S. Karger AG, Basel Abstract Patients with disorders of sexual development (DSD) requir- Introduction ing vaginal reconstruction are complex and varied in their presentation. Enlargement procedures for vaginal hypo- Patients with disorders of sexual development (DSD) plasia include self-dilation therapy or surgical vaginoplasty. requiring vaginal reconstruction are complex and varied There are many vaginoplasty techniques described, and in their presentation. Enlargement procedures for vagi- each method has different risks and benefits. Reviewing the nal hypoplasia include self-dilation therapy or surgical literature on management options for vaginal hypoplasia, vaginoplasty. These interventions are offered to improve the results show a number of techniques available for the psychological and sexual outcomes. The concept of sur- creation of a neovagina. Studies are difficult to compare due gery for DSD conditions has become increasingly contro- to their heterogeneity, and the indications for surgery are versial in the last decade. Clinicians and patients have not always clear. Psychological support improves outcomes. become involved in the debate, with strong views on both There is a paucity of evidence to inform management re- sides of the fence, with minimal evidence to inform man- garding the optimum surgical technique to use, and long- agement. There is now a consensus that vaginal dilation term data on success is lacking, particularly with respect to therapy is the first-line treatment for vaginal hypoplasia sexual function. In conclusion, vaginal dilators remain the [ACOG, 2002] due to the absence of surgical risk, but suc- cornerstone of treatment of women with vaginal hypoplasia cess depends on the motivation of the patient, and the and should be used as the first-line technique. Surgical vag- appropriate time to start treatment must be decided on inoplasty has a role in complex patients with previous failed an individual basis. Concomitant psychological support dilation and surgical intervention, particularly those cases is necessary and improves outcomes. Surgical vagino- where there is significant scarring from previous surgery. Re- plasty methods depend on the genital configuration, pre- © 2010 S. Karger AG, Basel Dr. Rebecca Deans 1661–5425/10/0045–0292$26.00/0 University College Hospital Fax +41 61 306 12 34 Institute of Women’s Health E-Mail [email protected] Accessible online at: 250 Euston Rd, London NW1 2PG (UK) www.karger.com www.karger.com/sxd Tel. +44 785 237 4634, Fax +44 207 380 9565, E-Mail rebeccad @ med.usyd.edu.au SXD316231.indd 292 27.09.2010 11:12:06 vious attempts at genital surgery and the surgeon’s for cosmesis [Alizai et al., 1999; Creighton et al., 2001]. All personal expertise and preference. There are many vagi- of the indications for early genital surgery are now being noplasty techniques described: tension via an external re-evaluated, and new recommendations suggest delay- traction device, peritoneal grafting, amnion grafting, ing unnecessary genital surgery till an age of informed skin grafting, bowel grafting, and muscle and skin flaps. consent and to better individualise care [Hughes et al., Each method has different risks and benefits. The surgi- 2006; Lee et al., 2006]. cal risks include malignancy (in graft material), contrac- ture leading to introital stenosis or loss of vaginal length, Surgery in the Adolescent and Adult vaginal prolapse, dry vagina, or excessive vaginal dis- Gynaecologists are most often involved in the care of charge. There is a lack of evidence to inform management the adolescent patient developing ambiguous genitalia at regarding the optimum surgical technique to use, and re- puberty, vaginal hypoplasia or in follow-up of adults who liable long-term data on success is not available in the underwent feminising genital surgery as children. In literature, particularly with respect to sexual function. many subjects born with ambiguous genitalia, there will be an associated vaginal hypoplasia or agenesis, and the gynaecologist will need to discuss the treatment options Indications for Surgery at the appropriate time. Where childhood surgery has been performed, there is a strong possibility that repeat Currently, surgery is performed in 2 instances, first to surgery may be required for vaginal stenosis, hypoplasia correct the appearance of ambiguous genitalia at birth, or genital cosmesis. In cases of failed dilation, surgery is and second to reconstruct a vagina where the genital ap- also indicated. Treatment is indicated to improve psycho- pearance is unambiguously female, but little or no natu- logical and sexual outcomes; however, there have been no ral vagina is present, the later being performed in the ma- studies to provide evidence that improvement in these jority of cases during adolescence or adulthood. outcomes are achieved, particularly in the long term, and in a number of these procedures ongoing vaginal dilata- Surgery for Ambiguous Genitalia tion is required. The concept underlying the surgical management of DSD conditions arises from the Optimal Gender Policy proposed by John Money in the 1950s, in which it was Vaginal Dilation stated that the phenotype must match the sex of rearing for optimal gender development [Money et al., 1955]. In Non-surgical vaginal dilation for vaginal agenesis was addition, they hypothesised that children were sexually ﬁrst reported by Frank [1938], who described the use of neutral until the age of 2 and therefore any corrective sur- vaginal moulds of increasing width and length to suc- gery should take place before this time. Further argu- cessfully create a neovagina for 5 out of 6 women. Since ments for the surgical correction in childhood of ambig- this time, it has been shown to be an effective technique uous genitalia included the belief that the child will be [Ismail-Pratt et al., 2007], although dilation treatment psychologically damaged by the virilised external ap- may take several months to achieve the final result. This pearance and correction before the age of permanent regime has been criticised as ‘distasteful’ in interview memory was desirable [Engert, 1989; Hrabovszky and studies with women [Boyle et al., 2005], and it has been Hutson, 2002]. Many neonates with ambiguous genitalia suggested that compliance and patient satisfaction is gen- are assigned as females. Traditional rationale for early erally low [Minto et al., 2003]. Motivation for reconstruc- feminising genital surgery for the more severely virilised tive surgery is often based on aspirations for patient nor- cases included relative technical ease of surgery, negating mality not just in terms of sex anatomy, but also sexual the need to disclose the disorder with patient, and an as- activities and experiences [Minto et al., 2003; Boyle et al., sumed ‘one stage’ procedure, with the aim of aiding pa- 2005; Liao et al., 2006]. Some women may perceive sur- rental acceptance of the child’s assigned gender, and im- gery to be a quicker and less emotionally involved ap- proving the psychological outcomes for the child [Crouch proach to create a neovagina. However, post-operative et al., 2004; Warne et al., 2005]. The majority of infants surgical dilation is almost always required and thus sur- undergoing genital surgery will require repeat genital gery is not necessarily the ‘quick fix’ operation that it ini- treatment (surgery or vaginal dilatation therapy) at or af- tially appears to be. Given the stigma attached to genital ter puberty, mainly for vaginal introital stenosis but also anomalies and the potential drawbacks in approaches to Management of Vaginal Hypoplasia Sex Dev 2010;4:292–299 293 SXD316231.indd 293 27.09.2010 11:12:11 vaginal reconstruction, specialist emotional and psycho- may have a scarred perineum and inelasticity of the tissue logical support must be planned alongside expert medi- or significant pelvic adhesions make surgery more tech- cal care. Within such a broad approach to care provision, nically complicated [Davies et al., 2005]. Comparing the barriers to dilation treatment and adherence are more outcomes of studies where prior complex procedures likely to be overcome, rendering it viable as an alternative have been performed such as the aforementioned group, to surgery as a ﬁrst-line approach. against studies where women with isolated vaginal agen- Vaginal dilator therapy has been shown as highly suc- esis are treated surgically without prior dilation therapy cessful in a recent prospective study by Ismail-Pratt et al. gives a biased picture of the results of the techniques re- [2007]. A standardised approach was used where patients ported. Secondly, individual surgeon ability and experi- were shown how to apply gentle pressure to vaginal dim- ence affects the choice of the procedure. Not least, there ple using graduated dilators for 30 min daily and attend is still no concordance in defining and recording the up to 5 follow-up visits at 6–8 weeks apart with a clinical main outcomes and long-term results of the different nurse specialist. Psychological support was available as vaginoplasties. All these factors play a role in the actual needed before, during or after treatment, as were oppor- impossibility of providing a comprehensive review of the tunities for education in sexual health and wellbeing. The proposed surgical approaches. With the limitations above success of dilator therapy was as high as 86% for achiev- mentioned, we will attempt to give an updated review of ing a normal vaginal length, and 81% of patients were able the current techniques. to have intercourse free of pain [Ismail-Pratt et al., 2007].

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