59: Long Term Complications of Urogential Reconstruction

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Introduction

Urogenital reconstruction is a broad description of surgeries that many of the most complex pediatric and adult urology patients have undergone. This chapter addresses the long-term considerations for adolescents and young adults (AYA) who have undergone reconstruction for urogenital (UG) sinuses, cloacal anomalies, exstrophy (including cloacal exstrophy and epispadias), and proximal hypospadias with a view to their reconstructive surgical outcomes later in life. Controversies regarding timing of reconstruction are discussed.

Of note, long-term outcomes are inherently challenging to study in these complex populations because there have been such radical shifts in how we counsel and manage patients over time. For example, in bladder exstrophy, the modern staged repair became popularized in the early 1970s.1,2,3 In cloacal anomalies and UG sinuses, the posterior sagittal approach and total urethral mobilization (TUM) were published in the 1980s and 1990s.4,5 Thus, studying an adolescent and adult population today might make conclusions about outdated techniques and attitudes.

Another major limitation to the study and collation of data regarding urogenital reconstruction is that all the disease states summarized in this chapter exist on wide spectrums and are rare. For example, the complications and outcomes for a patient with a short confluence UG sinus or short common channel cloaca will likely be vastly different from a patient where the vagina and/or rectum insert into the bladder neck. Alternatively, a patient with bladder exstrophy and a large bladder plate without polyps and a narrow diastasis is vastly different from a patient with a demure plate covered in polyps and a wide diastasis. However, studies often include patients throughout a spectrum together because:

1. it can be challenging to quantify the severity of disease on an individualized basis and,
2. the number of patients in each disease state are often so small that making any meaningful conclusions requires some grouping of patients.

Regardless, we must assess the data as they currently stand and use them to advance and improve the way we study long-term outcomes for these unique and important populations.

Table 1 Summary of long-term considerations in urogenital reconstruction

UG Sinus	Cloacal Anomalies	BEEC	Proximal Hypospadias
Vaginal stenosis Genital cosmesis Continence Menstruation Obstetrics	Vaginal stenosis Genital cosmesis Continence Menstruation Obstetrics Bowel function	Continence Penile reconstruction Erectile/ejaculatory dysfunction Vaginal stenosis Genital cosmesis Prolapse Menstruation Obstetrics	Recurrent chordee Erectile/ejaculatory dysfunction Complications (fistulae, stricture, diverticulae) Genital cosmesis

Urogenital

For females with a UG sinus secondary to congenital adrenal hyperplasia (CAH) and those with a pure UG sinus, repair of the UG sinus may be performed in infancy. Controversies surrounding the timing of repair and concomitant feminizing genitoplasty are discussed in a later section. This section assumes a UG sinus repair was performed and will discuss the associated long-term outcomes and complications.

Patients with short confluences measured on endoscopy may be managed simply with a skin-flap (Fortunoff) introitoplasty. For those with longer confluences in which more mobilization is required, options include total urogenital sinus mobilization (TUM), partial urogenital sinus mobilization (PUM), or vaginal pull through with the common channel used as urethra. TUM involves radical dissection of the entire UG sinus circumferentially with division of the pubourethral ligaments to allow for greater mobilization. PUM aims to preserve the pubourethral ligaments. Regardless, the surgical approach, is to mobilize as little as necessary, balanced against the need to achieve a tension free closure to the perineum. In both cases, mobilized sinus tissue can be used to recreate a mucosa-lined vestibule.

In patients best served with a vaginal pull through and use of the common channel as the urethra, continence may be compromised as the true urethra and continence mechanism may be short and underdeveloped. TUM similarly can cause incontinence and a neurogenic component due to the radical mobilization required. Often a bladder neck surgery (sling, reconstruction, or transection) will be required to achieve continence. Urethrovaginal fistulas are a dreaded complication of vaginal pull through repair, the true incidence of these in the long-term is unknown. When minimal mobilization occurs as with patients undergoing a posterior channel mobilization or PUM, AYA tend to have excellent continence outcomes.6,7

Neonatally performed vaginoplasty, for UG sinus repair, has a high rate of stenosis (25-100%) that will often require a secondary introitoplasty at puberty.8,9,10 These tend to be relatively simple procedures that heal well, with patient participation in postoperative dilation. After feminizing genitoplasty, sexual and psychological outcomes are grossly understudied despite the significant controversy that exists today.

Cloacal Anomalies

Urogenital reconstruction in infancy for females with cloacal anomalies vary greatly depending on the length of common channel and where the vagina and rectum insert into the urethra. Most commonly, patients undergo a posterior sagittal anorecto-vagino-urethroplasty (PSARVUP) with or without UG sinus mobilization. As previously discussed, a TUM or PUM may be employed depending on where the vagina inserts into the urethra and thus, how much mobilization is needed to bring two separate orifices to the perineum. If the vagina enters near the bladder neck, a vaginal pull through might be considered and the common channel used as the urethra. Vaginal replacement with bowel can be considered where there is insufficient vaginal length or vaginal atresia.

In the long-term, a high suspicion for neurogenic bladder should be maintained throughout the AYA period. Radical mobilization during surgery is thought to lead to neuropraxia that often occurs early, but late presentations are always possible. Overall, 80-91% of adult females a history of cloacal anomaly, achieve social continence and 49-67% will require clean intermittent catheterization (CIC), either via urethra or through a continent catheterizable channel.11,12,13

About 22-36% of females will present at puberty with obstructed menstruation due to introital stenosis after infant vaginoplasty.14,15 If a vagina of adequate length is present, a skin-flap introitoplasty or buccal mucosa grafting are possible treatment options. If a suitable vaginoplasty was never accomplished in infancy, other options such as a bowel vaginoplasty, skin (McIndoe) vaginoplasty, or buccal mucosa graft vaginoplasty are all options.

Bladder Exstrophy Epispadias Complex

Long-term complications of urogenital reconstruction after closure of epispadias, bladder or cloacal exstrophy center around urinary continence and sexual function. Unsurprisingly, the definition of “continence” is variable depending on the study and often the patient population is so heterogeneous (severity of disease, closure technique, etc.) that conclusions are difficult to make. In general, achieving continence is possible for AYA with BEEC, although additional bladder neck procedures, augmentation cystoplasty, and catheterization are routinely required to accomplish continence goals. Augmentation cystoplasty is expected in 58-70% of adults, many of whom (88-95%) also will have a bladder neck reconstruction or transection and closure to aid in continence or dryness.16,17,18 Only 14-25% of adult patients report volitional voiding per urethra.18,19

Penile complications after epispadias repair include fistulae, diverticulae, need for skin revision and recurrent dorsal chordee. In a series of 30 patients with isolated epispadias, 17% required a skin revision and 20% had fistulae.20 89% expressed concern about their penile appearance, one required corporeal plication for dorsal chordee and one patient underwent a forearm flap phalloplasty. In males with bladder exstrophy, Rubenwolf et al showed a 49% incidence of dorsal chordee and 36% with reduced penile sensation.21 Neophallus creation is an option for males who have a phallus that is not suitable due to length or degree of chordee for intercourse.

For females with BEEC, sequelae following primary closure in infancy may include introital stenosis and a significant scar over the mons pubis. Roughly 25–57% of females will require some form of introitoplasty during adolescence or adulthood.22,23,24,25 Obstructed menstruation is rare in BEEC, however introitoplasty allows for use of tampons and ability to have penetrative sex. These are usually straightforward procedures: options include dilation, perineal skin-flap introitoplasty, or use of buccal mucosal grafts. The abnormal pelvic floor musculature and anterior displacement of the vagina, females with BEEC result in a high rate of pelvic organ prolapse (POP). About 30–50% of patients will have evidence of POP by age 16, regardless of parity.26,27

Proximal Hypospadias

Notably, long-term outcomes in this population are very difficult to parse out, as many of the studies group proximal and distal hypospadias repair together. In a similar theme to the other disease states noted in this chapter, the expected difference in outcomes and complications between a subcoronal or glanular hypospadias and a perineal hypospadias with penoscrotal transposition are great. In addition, it is estimated that there are over 300 types of repair noted in the literature and the exact type utilized is rarely noted.28

In the short term, we know that single-staged repairs for proximal hypospadias estimate a 56% complication rate, with a higher risk of unplanned procedures and multiple complications when compared to a two-stage repair.29 Notably, each subsequent redo repair comes with a higher risk of complications.30 Thus for patients presenting as AYA with a continued complication from a proximal hypospadias repair, the odds of success decrease with each attempt at repair.

In general, males with a history of proximal hypospadias seem to have worse long-term outcome satisfaction when compared to males with a history of distal hypospadias. Repairs that involve more genital reconstruction appear to have a greater impact on sexual function due to recurrent chordee (5–23%), erectile dysfunction (0–73%) and ejaculatory difficulties (5–36%).31 Recurrent curvature is common in this cohort as well as dissatisfaction with penile length.32

Hennayake et al recently published that in a cohort of adolescents with a history of childhood one-stage proximal hypospadias repair, 39% had complications requiring operative repair.33 In this cohort, the mean HOSE score was 14.8/16 and the mean PPPS score was 17.8/18. Residual curvature was noted in a small percentage, almost 90% reported a firm glans with erection, and all patients reported normal ejaculation.

In patients requiring corporal incisions and/or grafting, resultant erectile dysfunction rates are poorly documented. A recent study suggests that a small number of patients who underwent small intestinal submucosal (SIS) corporal grafting, 5% reported moderate ED, with no difference compared to a control group.34

Controversy

One of the most debated and controversial topics in urology today centers around timing and consent for genital reconstruction. This has been most notable in the discussion of patients with differences in sex development (DSD), including patients with UG sinuses and proximal hypospadias. Questions such as when surgery should occur, what urogenital reconstruction is most appropriate and who decides (caregiver vs. patient) have been the major topics of debate.

The Chicago DSD consensus meeting and publication from 2006, agreed that infant clitoroplasty should be avoided.35 There are no data to support the claim that feminizing genitoplasty (namely clitoroplasty and labioplasty) confer any psychological or sexual benefit. A recent study by Bennecke et al demonstrated that views on infant genital surgery are still mixed among affected patients.36 They found that 66% of patients with CAH and 60% of those with an XY DSD thought that infancy is the appropriate age for surgery. 47% of males thought early hypospadias surgery is better. Those who had infant genital surgery were more favorable of it; conversely, patients who had recent contact with support groups were more likely to favor later surgery. The authors conclude a case-by-case shared decision-making model is better suited for the sensitive nature of infant genital surgery, rather than a blanket ban.

As stated in the introduction, these populations are an extremely heterogeneous group in infancy and as AYA. There are some decisions that are not as controversial such as mobilization of the rectum from the vagina in a patient with a cloacal anomaly or closure of the bladder in bladder exstrophy. But UG sinus mobilization in an otherwise healthy child, whether to make a neovagina in an infant knowing a revision surgery will be necessary, however, decisions around feminizing genitoplasty must be dealt with in a patient- and family-centered fashion. Caring for these patients, not only in infancy, but also into adolescence and young adulthood with a multi-disciplinary team, including medical, surgical and psychological input, is critical.

Conclusions

Data concerning long-term complications and outcomes after urogenital reconstruction have significant limitations including small sample sizes, gross heterogeneity of cohorts, and variable definitions of success. However, care for the AYA who has undergone a UG sinus, cloacal, exstrophy, or proximal hypospadias repair requires individualized management with an underlying knowledge of general principles from the literature presented in this chapter. Ultimately, multi-institutional studies of longitudinal cohorts of patients will help overcome some of the current limitations.

Key Points

• Understanding the balance between cosmetic improvement and functional reconstruction is very important in counseling patients or their families.
• There are very few absolute indications for infant surgery but parental consent for genital surgery remains controversial and an individualized approach is necessary.
• Patients should be cared for by providers working within a multidisciplinary team.
• An understanding of the potential complications of early surgery is important for those who have undertaken the surgery and those who will care for the patient as an adolescent/adult.

References

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