At least 80 % of female patients treated for bladder exstrophy require revision genital surgery [4]. Given that such a high proportion of female patients require revision surgery, routine vaginal assessment at puberty should be undertaken in order to evaluate the potential for further reconstruction. The earlier any potential problems are addressed, the better the chance of normal psychosexual and social development. A common functional complaint is of introital stenosis, inadequate vaginal length or introital stenosis can result in dyspareunia or an inability to have penetrative intercourse. In one series, 32 % of young women underwent revision genitoplasty for this problem alone [5].

The mean age to uterine prolapse in patients with exstrophy is 16 years [4] with a prevalence of 18 %; this can rise up to 50 % later in life. The prolapse is thought to occur due to the pelvic floor deficiency described above combined with a failure of the bony pelvis to form a complete ring anteriorly. Whilst the early osteotomy was not shown to decrease risk of prolapse, decreased pubic diastasis correlates to lower risk. It remains to be seen whether early mobilisation and reconstruction of the pelvic soft tissues (Kelly procedure) [6, 7] will reduce the incidence of prolapse. The traditionally described pessary treatment is often impossible due to a short vaginal length and the lack of pelvic floor musculature

Numerous techniques have been described for the treatment and prevention of prolapse, including fixing of the uterus to the abdominal wall in childhood [8]. This prophylactic measure proved to be a success and did not complicate pregnancy; however, this technique is not an effective treatment for existing prolapse. Robust long-term evidence regarding the correction of prolapse in the exstrophy population is sparse; a recent consensus suggests that suspension to the sacrum, when present, is likely to give the best results [9]. Various techniques have been described to achieve this, for example, the Gore-Tex® (W.L. Gore and Associates Ltd., Scotland, UK) wrap technique using a length of Gore-Tex® passed through the broad ligaments, around the cervix, and fixed to the sacral promontory. Data have shown a 75 % success rate over a mean follow-up of 8 years have been reported using this technique [10]. The technique of sacral suspension under its varied nomenclature (sacro-colpopexy, hysteropexy, sacrocervicopexy, etc.) is thought to have little or no deleterious effects on fertility; however, it should be deferred until a couple have completed their family. The risk is that during pregnancy or delivery the sacro-colpopexy would be disrupted leading to a recurrence of the prolapse and the need for a repeat procedure in a very complex surgical environment. When treating the prolapse, it is important not to remove the uterus as this not only renders the patient infertile, but is the only structure able to fill the pelvic floor defect. Without the uterus the potential for a large enterocoele exists, the treatment of which can be extremely challenging.

Historically, genital reconstruction in male patients was delayed until complete bladder closure or puberty but may now be undertaken with the initial surgery in the neonatal period [11]. Growing up with a normal appearing pendulous penis following reconstruction in the neonatal period has been said to have a positive effect on psychosexual and social development in these boys, the ‘true’ appearance and function of the penis is, however, not clear until puberty [12].

The classic appearance of the penis in a male with exstrophy is of a short (due to short corpora), broad, dorsally tethered penis, an open glans, absent dorsal prepuce, with a dorsal or ventral urethral meatus dependent upon the type and timing of reconstruction in infancy. Many erectile deformities have been described [13], but the majority are a consequence of surgery in infancy where the corpora may have been damaged, only dorsal chordee is an integral part of the exstrophy complex. Asymmetrical corpora are often encountered, where a scarred and fibrotic corpus can compound the deformity (Fig. 6.2).

The beginning of relationships and sexual function become important to all during adolescence. It is normal for any adolescent to question their function in relation to this and for many there will be moments or periods of insecurity. This is part of normal development. It is also normal to ask how a congenital anomaly and the treatment of it, including surgery, will affect sexual function. There is often a significant focus on cosmesis and in the case of a male patient penile size. In exstrophy, the bony diastasis may lead to a significant dip in the skin and a midline separation of the pubic hair. The long-term management issues with respect to sexual function have not been extensively studied but disorders or anxiety surrounding sexual function in adolescence can have a significant psychological impact on patients. Reports suggest that some 1/4–1/2 of patients are dissatisfied with penile length or chordee [16]. Libido in exstrophy patients is normal and they form normal relationships; in the author’s experience a key component in sexual function is the partner’s understanding and acceptance of the patient’s reconstructive history. In a society where casual sexual encounters are more common, this can create a pressure on these patients. A desire on their part to conform to the behaviour of their peer group is met with a fear that a new partner may not understand their condition and rejection may result. The treating urologist needs to be aware of this background and prepared to manage it. A multidisciplinary approach, including psychosexual counselling and support, should be adopted as discussing and addressing issues around sexual function can be challenging for both the patient and clinicians.