Introduction

Complex anomalies of the reproductive and genital tract occur in 1 in 2000–3000 women resulting in approximately 25 000 affected women in the UK (Table 1).1–4 These conditions include disorders of sex development such as androgen insensitivity syndrome (AIS) and congenital adrenal hyperplasia, anomalies of the Müllerian system such as the Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) where the vagina and uterus are absent, and various other abnormalities of development of the genital tract where menstruation is blocked.

Management of these women is complex and should be led by a multidisciplinary team with input from gynaecologists, urologists, colorectal surgeons, endocrinologists, geneticists, nurse specialists, radiologists, biochemists and psychologists. Patients should feel comfortable and empowered enough in the multidisciplinary team setting to contribute to making decisions regarding their own health care. The aims of management should be to achieve optimal physical and psychological health with good quality of life, sexual function and fertility options. To achieve this, it is often necessary to create a vagina for these women – a so-called ‘neovagina’.5

The National Health Service (NHS) Cervical Screening Programme screens over 3 million women each year to detect and treat precancerous cervical abnormalities. However, it is accepted that cervical smears are not useful in screening women with a neovagina unless a cervix is present, and most women with these conditions are advised to opt out of the national screening programme. In practical terms it is not possible to do a smear if no cervix is present. In addition, smears taken from the neovagina vary depending on the tissue used to line it and are difficult for cytologists to interpret. However the recent introduction of vaccination and testing for human papillomavirus (HPV) may be appropriate for women with a neovagina and this needs further evaluation to …