Bladder exstrophy is a congenital anomaly that exists on a spectrum between epispadias to cloacal exstrophy.¹ Children affected by this condition are born with an open bladder plate, a hemiclitoris or epispadias, low umbilicus, pubic diastasis, and anteriorly displaced anus. The incidence of bladder exstrophy has been reported as 2.15 in 100,000 live births in the United States and affects 2.3 males for every female.² Common immediate postnatal care includes keeping the bladder plate moist with saline irrigation and covered with plastic wrap as well as avoiding mucosal irritation from foreign bodies like clamps controlling the umbilical stump.

Surgical management for these individuals can vary considerably. Typically, primary closure of the bladder with bilateral osteotomy is performed. This can be done shortly after birth if the bladder is an appropriate size or can be delayed 6-12 months to allow further growth. Historically, primary urinary diversion was more commonly performed, though it is still done for children not suitable for closure or who have failed bladder closure in the past. Beyond bladder closure these children also typically undergo epispadias repair, possible bladder neck repair or reconstruction, and bilateral ureteral reimplantation. These can be completed in multiple stages or in 1 procedure. These repairs are technically challenging and complications that arise can include bladder dehiscence, urethral stricture, glans necrosis, vesicocutaneous fistula, bladder prolapse, and more.

As these patients transition into adolescence and adulthood, sexual function and fertility become an important aspect for many. The anatomical impact of exstrophy can affect their reproductive organs, leading to a shortened vagina in women and male penis with an anterior corporal length about half of that compared to controls, even after bladder closure. Individuals with a history of bladder exstrophy can struggle with low satisfaction with their genital appearance. Suomen et al surveyed 21 men with a history of bladder exstrophy treated between 1956 and 1992 compared with age matched controls.³ They reported similar erectile function, desire, and sexual satisfaction between groups, however only 12/21 men were satisfied with the appearance of their genitals. Rubenwolf et al surveyed 39 males with a history of bladder exstrophy, all of whom underwent urinary diversion rather than bladder closure.⁴ They found relatively high rates of erectile and orgasmic dysfunction (50%) and reduced sexual desire (86%) with similar low rates of satisfaction with cosmesis (30%). However, greater than 90% of the men surveyed were sexually active and satisfied with intercourse. On average these men had 10.4 surgeries, 3.4 of which were for genital reconstruction specifically.

Exstrophy male genital reconstructive procedures can include skin grafting, tissue expansion, and radial forearm free flap phalloplasty. Harris et all reported outcomes for 28 males on the exstrophy epispadias spectrum who underwent one of these 3 reconstructive procedures.⁵ Of the 25 men who completed answers pertaining to penile length, 23 were preoperatively dissatisfied with their penile length, with 18 noting improvement postoperatively. Ultimately, the importance of preoperative counseling should be emphasized as only 61% of patients were satisfied with their reconstruction.

Unassisted male fertility rates have been reported at 10% or lower. Fertility rates are impacted by antegrade ejaculate volume, which is at least somewhat dependent on number and character of prior surgical procedures but may simply be a result of an incompetent bladder neck. These patients do have an increased risk of low sperm counts: Ebert et all reported only 3 of 16 men with a history of bladder exstrophy had antegrade ejaculation with normal sperm counts.⁶ Use of assisted reproductive technology (ART) has had good success. One series reported 11 out of 39 males with classic bladder exstrophy who fathered children. Ten of the 16 children were conceived using ART in the form of homologous insemination or testicular sperm extraction with intracytoplasmic sperm injection.⁴

Female exstrophy patients have been reported as having comparable sexual pleasure when compared with age matched controls in one series of 11 patients.³ Of these 11 women, 4 underwent procedures for vaginal stenosis. In another series of 29 women with continent urinary diversion, 90% regularly had intercourse.⁷ However, only 44% were satisfied with the cosmetic appearance of their genitals and repeat vaginoplasty was required in 33% due to dyspareunia. High rates of fertility are reported in this series with 12 women having 16 healthy children, all of whom were delivered via cesarean section. Yet, 12 of these pregnancies had some manner of complication. Pelvic organ prolapse is common, likely due to a widened diastasis and altered pelvic floor anatomy, with 11/29 developing this in this series. Surgical repair can be challenging for these individuals, however Everett et all described successful abdominal sacral colpopexy in 9 of 11 patients in their series.⁸

Adults with a history of bladder exstrophy can have satisfying sexual function and successfully conceive children. Considerations for males include low rates of genital appearance satisfaction. Many go through multiple genital reconstructive procedures but there are a variety of options to allow for not only cosmesis, but also function. Males can go on to father children, but with the majority requiring ART. Considerations for females include the importance for long-term urogynecologic follow-up given the high rates of vaginal reconstruction and prolapse. It is very feasible, however, for these patients to have a satisfying sexual life. Most females can become pregnant if they so desire, though they should be considered high risk and plan for cesarean section. Utilizing this information, we can not only provide surgical counseling for the initial procedures performed, but also help support these patients to achieve a high sexual quality of life and educate them on reproductive opportunities available.