The Psychosocial Impact of Hypospadias in Adulthood

Publications looking at the psychosocial, sexual, and reproductive impact of hypospadias in patients operated on, using twenty-first century techniques, really do not exist. There are studies from the 1980s that suggest that, in adult men operated on for hypospadias during childhood, there was a less than satisfactory psychosocial adjustment as compared with age-matched controls. The patients with hypospadias were more timid and embarrassed and appeared to be more socially isolated as adults. They had lower self-esteem and decreased capacity for social and emotional relationships, as well as being less qualified for stressful occupations, when compared with a control population.

In 1989, Sandberg and colleagues at Columbia University, in a study of 69 children with a history of hypospadias showed that these children had more behavioral problems and lower social skills than children in the general population. A number of older studies, including Sandberg and colleagues’ data, tend to support the fact that children of this era who had a history of multiple surgeries for hypospadias had less social competency than their peers. It was also found that children with hypospadias were less likely to verbalize their problems. In a more recent questionnaire-based review looking at the social and sexual life of adult patients operated on for hypospadias during childhood, the group with hypospadias and control group gave similar responses and did not differ in terms of success in sexual and social activities. Patients with hypospadias were more dissatisfied with the cosmetic results of their surgery than control patients, however, and they were more likely to have problems with urinary function.

In another study of more than 11,000 men age 18 or older, there were 42 individuals with a history of hypospadias with a similar number of controls without hypospadias. The hypospadias group was found to be significantly more inhibited in seeking sexual contacts and had a significantly lower number of patients who had already had full sexual intercourse by age 18. In addition, men with hypospadias were also significantly more likely than control patients to have a negative appraisal of their own genitalia. In terms of patient satisfaction with the cosmetic results of their surgery, the overwhelming majority of patients operated upon in childhood for hypospadias consider their penile appearance to be abnormal. Overall, patients with hypospadias seem to be somewhat less satisfied than their surgeons with the overall cosmetic appearance following hypospadias surgery.

All of these studies, however, included individuals who were operated on some years ago when hypospadias techniques were not as sophisticated as they are today and often involved operations over several years. The number of operations alone can have a significant impact on psychosocial development. The adult satisfaction rates will hopefully improve as the population that has undergone repairs with more modern techniques becomes adult and sexually active.

Micturition

Micturition after hypospadias repair has been reviewed in a number of studies, and in general most report significantly more urinary issues than in controls. Most studies looking at voiding function in individuals operated on in childhood for hypospadias suggest that concerns generally include spraying of urination more than 50% of the time, as well as postvoid dribbling, and the feeling of incomplete emptying. In a few studies where a uroflow has been used to measure micturition, the Qmax was found to be abnormal in 13.5% of patients with hypospadias compared with 2.9% of controls. In general, the more proximal the hypospadias, the more likely voiding issues were to occur. Recently, some concerns have been raised regarding long-term meatal issues in the TIP (Snodgrass) repair.

Ejaculation

In a number of studies from 1975 to 2000, there were difficulties with ejaculation reported in between 6% and 37% of individuals after hypospadias repair. The problems included weak or dribbling ejaculation, retained ejaculate having to be expressed manually, and delayed ejaculation or anejaculation. Once again, the more severe or proximal the original lesion, the more severe the ejaculatory issues.

Hypospadias in Adult Males

The surgical correction of hypospadias may at times be delayed for cultural, religious, or socioeconomic reasons. We have previously reported our experience with adult hypospadias repair, indicating the complexity of the surgical procedure and relatively poor outcome as compared with childhood hypospadias repair. There is little information concerning impact on pychosocial development of these individuals or information about sexual function before or after correction. It has been reported that about 40% of adult patients with hypospadias have not attempted sexual intercourse before the operation. Complications after childhood repairs, such as fistula and strictures, most often seen in the immediate postoperative period, can develop late and be seen in the adult. These complications are not simply missed problems in childhood but, de novo events in the adult. The correction of late complications is not straightforward, and clear disclosure of the complexity of repair must be given to the patient.

Reproduction

Hypospadias is frequently associated with other genitourinary abnormalities, the most common being undescended testis, which ranges between 17% and 30% of patients born with hypospadias. The frequency of undescended testis varies with the severity of hypospadias and may be as high as 31% in severe forms of hypospadias. The frequency of undescended testis in the general population ranges between 3% and 5% and is reported to be between 5% and 10% of individuals presenting with infertility. Testicular atrophy is a known feature (complication) of surgery for undescended testis and testicular atrophy is obviously a risk factor for impaired infertility. Older literature suggests there may be abnormal levels of follicle-stimulating hormone in individuals with undescended testis and hypospadias; however, recent findings in 32 children with isolated hypospadias indicated that none of the patients presented with enzymatic abnormalities of testosterone biosynthesis or partial androgen insensitivity.

What is clear from recent data is that most men with hypospadias alone have normal semen quality; however, semen quality is reduced for men with hypospadias plus other genital abnormalities, including undescended testis. Reproductive hormone levels indicate a subtle impairment of testicular function in men with hypospadias alone. It has been observed that there are fewer fathers among men with hypospadias versus controls. This may be because of several factors, including psychosocial factors, sexual dysfunction, reduced semen quality, or most likely a combination of all of these factors. In summary, there is no clear-cut association of hypospadias, undescended testis, and reduced fertility potential.

Summary

Studies outlining the long-term effects of hypospadias repair in childhood using modern twenty-first century techniques are scarce, but it seems clear that surgery in early childhood with better techniques will improve overall functional and cosmetic results long term. As far as micturition is concerned, adult patients with hypospadias have significantly more urinary symptoms when compared with adult controls and, in general, the more severe the primary lesion, the more severe are the micturition issues. In terms of cosmesis, the results of hypospadias repair done in childhood are generally good; however, there are a significant number of individuals who following their surgery will consider their penile appearance to be abnormal. In terms of sexuality, adult patients with hypospadias are generally reported to be less satisfied with sexual function when compared with controls. This lower satisfaction may be related to cosmesis, issues with body image, or low self-esteem. Erectile dysfunction is more prevalent in patients with hypospadias as compared with controls, and patients with severe hypospadias in particular report more ejaculatory problems than controls. In terms of relationships, adult patients born with severe hypospadias have fewer intimate relationships, lower self-esteem, negative genital appraisal, and inhibition in sexual contact as compared with controls.

Posterior Urethral Valves

One of the most devastating congenital anomalies in the male infant is posterior urethral valves (PUV), which cannot only affect long-term bladder function, but also renal function in a significant number of the individuals so effected. The incidence of PUV ranges from 1 in 4000 to 1 in 7500 live male births. The postnatal sequelae range from minor to life threatening. The impact of this anomaly can involve relatively simple problems with lower urinary tract function with incontinence to devastating issues with chronic renal insufficiency and end-stage renal disease (ESRD). Total bladder outlet bladder obstruction by PUV before 20 weeks of gestation has been shown to result in bilateral renal dysplasia and severe pulmonary hypoplasia, leading to death (Potter syndrome). Less than complete obstruction often leads to varying degrees of bladder dysfunction and/or renal damage. The incidence of newborns with PUV has decreased dramatically over 20 years, most probably because of antenatal screening and early termination. There has been a significant amount of interest generated over the past 3 decades in antenatal screening and intervention for PUV. The most common intervention to date has been the vesicoamniotic shunt. The long-term results are not convincing in terms of any real benefit being added by the use of antenatal shunting. Although there is some evidence that bladder function and perhaps renal function can be salvaged by early intervention and decompression, the pulmonary injury has not shown to be effected in a positive way. There are scattered reports of individual successes; however, long-term data to date are not terribly hopeful.

There are few if any data to suggest that the initial approach to PUV makes any difference whatsoever in terms of long-term renal function and progression to ESRD. Most reports suggest that primary valve ablation and allowing the bladder to cycle is superior to primary supravesical diversion in terms of normalizing long-term bladder function. In addition, there are no data to suggest that the upper tract outcome is any different in children who have had prenatal intervention as compared with those who have had early postnatal intervention.

Posterior Urethral Valves and End-Stage Renal Disease

Obstructive uropathy, which is caused by posturethral valves, continues to be a common cause of chronic renal insufficiency in children and adolescents. The rate of progression of renal insufficiency to ESRD in these children with PUV is probably somewhere between 13% and 20%. The most important prognostic factor in these children going forward is their nadir serum creatinine. A serum creatinine of less than 0.8 mg/dL at 1 year of age is associated with essentially normal long-term real function, whereas a nadir creatinine of greater than 1.2 mg/dL after 12 months of age has a much more rapid acceleration to ESRD. Findings would suggest that, in most boys, the die for ultimate renal function is cast at presentation. Those with good renal function will do well and those with poor renal function will not. The time frame for ESRD is largely dependent on the level of renal function to begin with. In most situations, the time to progression ranges between 11 and 15 years, which corresponds with pubertal development. The other important cofactors affecting the rate of progression to ESRD would include high-grade vesicoureteral reflux and persistent bladder dysfunction.

Posterior Urethral Valves and Bladder Dysfunction

Bladder dysfunction has an enormous impact not only on toilet training in children with PUV but also on progression to ESRD. Most clinicians agree that the long-term outcome in patients with PUV revolves around the management of their bladder dysfunction. It has been estimated that up to 75% of children born with PUV have underlying bladder dysfunction in spite of adequate valve fulguration early on. The long-term use of clean intermittent catheterization, as well as the use of anticholinergics, can be extremely helpful in these patients, especially those with more chronic and slow renal deterioration caused by increased bladder pressure. In addition, Koff and colleagues have shown that overnight bladder drainage will further decrease bladder dysfunction, lower intravesical pressure, and delay renal insufficiency.

The bladder dysfunction seen in patients with PUV in association with ongoing renal deterioration has been labeled the “valve bladder syndrome.” This is characterized by myogenic failure of the bladder with poor compliance and detrusor hyperactivity. The renal failure seen in conjunction with the valve/bladder syndrome is characterized by a lack of concentrating ability, leading to increased urine production. This association was noted first by Mitchell in 1982. It is exceedingly important that clinicians following patients with PUV understand that the valve bladder is not stable. It has been noted for a long time that valve bladders that are hypocompliant in infancy and early childhood can decompensate rather rapidly in adolescent years. This is extremely important in following these patients through adolescence. The typical urodynamic patterns seen in adolescent patients with valve/bladders include hyperreflexia, hypertonia, and myogenic failure.

The other significant change that has taken place in the management of these individuals born with PUV is the sharp reduction in the use of augmentation cystoplasty. In our database, which encompasses 33 years of data collection, there are 157 augmentation cystoplasties and 91 continent diversions. Of that number, 24 of the augmentation cystoplasties were in patients with PUV, including 19 gastric cystoplasties and 6 ureterocystoplasties; however, since 2000, there have been only 3 augmentation cystoplasties done in patients with PUV at our institution, and each of the 3 were patients referred long into the course of their treatment (Hensle, unpublished data, 2001). This decrease in the use of augmentation cystoplasty reflects the better management of the high-pressure noncompliant bladder with anticholinergics, clean intermittent catheterization, and overnight drainage.

In patients with ESRD secondary to posterior urethral valves, renal transplantation into valve/bladders has shown to be safe in a number of recent studies. These studies have concluded that a history of PUV does not portend a worse prognosis for renal graft survival compared with individuals transplanted for nonvalve issues. In patients with a valve bladder undergoing transplantation, the treatment plan has to be individualized to prevent lower tract deterioration. This often includes intermittent catheterization, anticholinergics, and overnight drainage. This allowed safe and effective renal transplantation in those individuals requiring it.

The single most important feature in dealing with these children as they move to adolescence and adulthood is to customize the urologic management of their bladder to decrease the risk of ongoing renal insufficiency and make success of renal transplantation optimal. This often requires close monitoring of these individuals with frequent video urodynamic evaluations and attention to detail in terms of the individual’s commitment to clean-intermittent catheterization and, if appropriate, nocturnal urinary drainage. What is of critical importance to the clinical urologist following these youngsters as they move from childhood to adolescence to adulthood with a hostile bladder is to be sure that the bladder management in terms of filling, emptying, and pressure management is under good control to protect what is either left of native renal function or to protect the native or transplanted kidney.