Abstract
The major congenital anomalies of the genitourinary tract may result in disturbances of sexual and reproductive function. In general the children grow up with the same aspirations as their more normal peers, which are to marry, have intercourse and produce children. Some achieve this despite the deformities and in others specific reconstructive surgery may be needed. In exstrophy the vagina lies parallel to the floor when the girl is standing and the introitus is seen on the lower abdominal wall rather than in the perineum. Episiotomy is required in 34% and formal vaginoplasty in 23% of the cases. The exstrophy penis has a tight dorsal chordee that must be corrected to allow intercourse. The neurological and social consequences of myelomeningocele do not prevent patients from having an interest in sex. Those who are able to walk are likely to have normal sexual function compared to about 50% of those who are wheelchair bound. The recurrence risk for neural tube defects in their offspring is 1:50 for sons and 1:13 for daughters regardless of the sex of the affected parent. The physiological consequences of posterior urethral valves result in weak ejaculation in 50% and highly viscous and alkaline semen in 50% of the cases. Of male patients with ambiguous genitalia or micropenis 75% have normal intercourse.