It is always exciting to get new data on exstrophy from Johns Hopkins, but especially when sexual development is the subject . It is the only unit with enough patients on continuous follow-up to overcome the difficulties of researching such a rare condition.
In the last 40 years, patients born with exstrophy have achieved a near normal life-expectancy. Reconstructive techniques for the bladder are now such that incontinence is rare, although often bladder emptying depends on clean intermittent self-catheterisation . As with all fit young men, their minds turn frequently to sex and, occasionally, its natural consequence – pregnancy.
Current data have established that the men have a normal libido, orgasms, and erections. It is probable that the testes are normal at birth but often are damaged by recurrent infections. The penis is short, broad and has a characteristic chordee. Other erectile deformities are probably the result of corporeal damage during reconstruction in infancy. Most of these are surgically correctable. Ejaculation is poor or absent [3, 4].
Data on the men’s own satisfaction are contradictory and there are none on the partner’s opinions. Masturbation is almost universal. The incidence of erectile dysfunction is more than double that of controls (58% vs 23%) . Much the commonest cause is fear of rejection by a partner because of the obvious penile anomalies. Most series show that men like to establish a good partnership before starting intercourse, although at least one group report that random and short-term relationships are common . Unfortunately the published series are small and few of them address sexuality in a structured manner.
At Johns Hopkins the exstrophy database now has >1 200 patients and there is a programme for close and indefinite review. This is good for the patients and good for outcomes research. Sexual function has been investigated in 113 adult men (53% of those eligible) using a 42-question survey, which incorporated four validated instruments and additional questions related to sexuality .
In all, 85% had been sexually active at some time and 62% were currently in a relationship; three were homosexual and three bisexual. The divorce rate was lower than the norm in the USA! Amongst much other data, it was found that only 58% were moderately-to-very satisfied with their sex life. The mean penile perception score (PPS) was 6.2 (maximum possible 12) and most men were dissatisfied with their penile appearance to some degree. However, there was no relationship between the PPS and sexual activity or satisfaction. In all, 32 of 113 men had tried to achieve a pregnancy, of whom 72% were successful, with half of them requiring reproductive technology. Another 27% had a confirmed fertility problem.
With these new data, we can say that men born with exstrophy have a normal ambition for their sexual activity and form solid partnerships. Their overall level of satisfaction is lower than normal and the appearance of the penis is a major contributory cause. The fertility rate is significantly lower than normal. We still know nothing about the feelings of the partners.
Can anything be done to improve this situation? On the positive side, correction of the penile deformities, prompt management of urinary infections (to avoid epididymo-orchitis), and reproductive technology are helpful. It is most important not to damage the penis or its nerve supply during reconstructive surgery. At present, there are inadequate data to say whether the formation of a new phallus incorporating the native penis (similar to female–male gender reassignment) would generally be beneficial . Psycho-sexual support is often recommended but the techniques used and outcomes rarely reported. However, paediatric and adolescent urologists have a vital role in discussing sexual function with their patients, encouraging ‘normality’ and providing practical help when possible.