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Surgical management and postoperative outcome of children with bladder exstrophy

Surgical management and postoperative outcome of children with bladder exstrophy
Literature review current through: Jan 2024.
This topic last updated: May 02, 2023.

INTRODUCTION — Bladder exstrophy is a complex congenital anomaly that is treated surgically (picture 1A and figure 1). Surgery is challenging, as bladder exstrophy involves the urinary, reproductive, and musculoskeletal systems, and, in some patients, the intestinal tract. Major goals of reconstruction are preservation of normal kidney function, development of adequate bladder storage and function (ie, urinary continence and emptying), and provision of acceptable genital cosmetic appearance and function.

The surgical management and postoperative outcomes of children with bladder exstrophy are discussed here. The clinical manifestations and initial management of infants with bladder exstrophy are discussed separately. (See "Clinical manifestations and initial management of infants with bladder exstrophy".)

SURGICAL APPROACHES — The following approaches, or variations of such, are used to manage bladder exstrophy. The first two interventions result in correction of the underlying defect.

Modern staged repair of bladder exstrophy (MSRBE) involves three staged operations and was initially developed in the 1970s [1,2]. Subsequent modifications have been made over the past several years.

Complete primary repair of bladder exstrophy (CPRBE) was introduced in 1989 with the hope that a single operation would achieve the goals of continence and preservation of renal function [3]. However, follow-up studies demonstrate that many children still require multiple procedures following CPRBE [4]. (See 'Choice of surgical approach' below.)

Urinary diversion is the least common of the surgical options and involves creating a functional connection between the urinary and intestinal tracts. Although there are several variations, the basic approach is to divert urine away from the urinary bladder and into the bowel (large intestine/colon) for temporary storage, and then intermittent passage with the stool through the rectum. When successful, passage of urine is under the control of the child. The bladder and urethra are closed but are "dry," and they are not exposed to or necessary for handling the urine.

The first two surgical approaches are based on successful initial bladder closure either at birth or within the first two months of life. Early bladder closure, especially when coupled with epispadias repair (CPRBE), is hypothesized to ensure initiation of bladder cycling in the newborn or young infant. It is thought that this will optimize bladder growth and development, resulting in an increased probability of continence and adequate bladder capacity, which may reduce the need for future bladder augmentation and urinary diversions. Another view is that the initial bladder size is the best indicator of ultimate bladder capacity and function.

Modern staged repair of bladder exstrophy — MSRBE consists of three specific components scheduled at the following approximate ages:

Newborn – Closure of the bladder, posterior urethra, and abdominal wall defect results in the creation of complete (penopubic) epispadias and is performed within the first two months of life [5]. In the newborn, closure may not be possible because of a stiff bladder "template" or because the bladder is too small if the infant is preterm. In the latter setting, time may allow for adequate growth so that closure is feasible [6,7].

Six months to one year of age – Epispadias repair is performed. The goal is to fashion a straight and functional penis with a glanular meatus in male patients and an acceptable cosmetic appearance.

Four to five years of age – Bladder neck reconstruction (BNR) and bilateral ureteral reimplantation are performed when there is documented adequate bladder capacity and motivation of the patient to participate in a postoperative voiding program. The goal of this last stage is to provide urinary continence.

Case series report that a continence rate following BNR was approximately 70 percent [8,9].

Complete primary repair of bladder exstrophy — CPRBE combines bladder closure and epispadias repair (and in some cases ureteral reimplantation) into a single operation (picture 2A-C) [3,10-14]. This surgical approach includes wide exposure of the proximal urethra, and the transfer of the posterior urethra, prostate, and bladder neck deep into the pelvis. The goal of CPRBE is to provide early and adequate bladder outlet resistance that promotes growth of the bladder. Similar to the first stage of the MSRBE approach, CPRBE may be performed within the first 72 hours of life, with or without pelvic bone osteotomy, or as a purposely-delayed procedure at approximately two months of age with pelvic osteotomies (picture 1B). A multiinstitutional bladder exstrophy consortium including the author's center is focused on refining the CPRBE technique, streamlining follow-up care, and optimizing outcome in patients with bladder exstrophy [15,16].

However, it has become apparent that a significant proportion of patients following CPRBE will require additional surgical intervention [13,17,18]. This was illustrated in a multicenter case series of 216 patients, including 212 patients who underwent CPRBE between 1980 and 2016 [17]. After primary closure, approximately one-half required bladder augmentation and 5 percent urinary diversion. By 18 years of age, almost 90 percent of patients with CPRBE underwent a bladder neck procedure (BNR, bladder neck closure, artificial urinary sphincter, or sling). The probability of subsequent surgical intervention varied amongst the five centers. Two-thirds of this cohort performed clean intermittent catheterization (CIC).

Timing — The initial reconstructive surgery of bladder closure may be performed within the first two to three days of life (immediate) or at approximately 6 to 12 weeks of age (delayed). In our center, the preferred timing for CPRBE is at 6 to 12 weeks of age (ie, delayed closure). One advantage of delayed closure is that the time between birth and initial repair will allow for growth, development, and additional maturation of some organs and systems prior to the complex initial reconstructive surgery and anesthesia [19]. For small preterm newborns in particular, delayed closure allows time for their anatomy to grow, which makes repair safer. In addition, delayed closure allows for the initiation of breastfeeding. Another advantage of delaying CPRBE is allowing infant bonding with parents/caregivers before initial reconstructive surgery and the lengthy recovery period that follows.

In a study based on the Pediatric Health Information System database that identified 381 patients undergoing primary closure within the first 120 days of life from 1999 to 2010, 279 neonates (73 percent) underwent repair within the first three days of life, 67 were repaired between 4 and 30 days of life, and 35 between 31 and 120 days of life [20]. Approximately one-third of the cohort (n = 119) underwent pelvic osteotomy, including 51 patients who underwent closure within the first three days of life.

Choice of surgical approach — It is unclear which surgical approach (MSRBE versus CPRBE) best meets the goals of preservation of normal kidney function, urinary continence, and provision of an acceptable cosmetic genital appearance and function. In general, the success of surgical management of bladder exstrophy is determined by the rates of urinary continence, the need for additional operation(s) (eg, bladder augmentation), and renal function.

Data are lacking that directly compare these two approaches based upon these outcome measures, partly because bladder exstrophy is an uncommon anomaly, and because most tertiary centers with the surgical expertise to treat this condition typically choose one approach over the other. The latter factor potentially leads to center and publication bias. However, it is clear that patients who undergo CPRBE are likely to have additional bladder neck surgical procedures [17]. It remains uncertain whether or not CPRBE reduces the number of overall surgical procedures and whether it results in a similar or higher rate of continence compared with MSRBE. (See 'Continence' below.)

Neonatal iliac osteotomy and pelvic immobilitzation — The need for iliac osteotomy as part of successful bladder closure in the first few days of life is independent of which surgical approach is selected. In general, bladder closure can be performed within the first 72 hours after birth without osteotomy if there is no extreme diastasis. This is because the pelvic ring is relatively malleable in the first two or three days of life. If the pelvis is not malleable or there is extreme diastasis, osteotomies should be performed to increase the likelihood of successful bladder closure.

Regardless of whether osteotomy is performed and which surgical approach is chosen, the pelvis is typically immobilized with traction following bladder closure. A common form of traction for newborns is modified Bryant's traction (picture 3). This is typically employed for two to four weeks following the initial reconstructive surgical procedure. Other reported alternate options include spica casting and a padded Velcro strap wrap technique, which has been reported to decrease length of stay and enable parents/caregivers to hold the infant shortly after repair [21-23].

However, in a British case series, primary bladder closure without osteotomy and postoperative immobilization was successful in 70 of 74 patients (95 percent) [24]. These patients were managed postoperatively by either epidural anesthesia or with temporary paralysis and ventilatory support. These results need to be confirmed at other centers to see if the traditional approach of pelvic immobilization with or without osteotomy should be modified, and outcomes in respect to urinary continence need to be assessed. It should be noted that this approach to management is with initial surgery (first stage of MSRBE), later followed by a radical soft tissue ("Kelly") procedure that is typically planned for 9 to 12 months of age. The Kelly procedure is not a favored technique in the United States [25].

COMPLICATIONS — Complications following surgical correction of bladder exstrophy include:

Bladder dehiscence – Bladder dehiscence is a relatively rare complication [26]. Some experts in the field advocate the routine use of osteotomies to prevent this complication [27,28]. Others, including the author, believe that bladder closure can be performed successfully, often without osteotomies, if the procedure is performed within the first 72 hours of life when the pelvic ring is malleable and if there is not extreme diastasis. Bladder dehiscence is associated with an increased risk of incontinence [29].

Urinary tract infection – Urinary tract infection (UTI) is common in patients with bladder exstrophy. UTI may include pyelonephritis with the infection affecting either one or both kidneys. This is especially true in patients with bladder augmentation or ileal conduits who have reported incidences of UTI of 63 and 48 percent, respectively [30]. Vesicoureteral reflux (VUR) is another common finding in patients with bladder exstrophy, and it increases the risk of pyelonephritis.

In our series of 32 patients who underwent complete primary repair of bladder exstrophy (CPRBE), nine patients (28 percent) had one to four episodes of pyelonephritis [4].

Bladder stone – Formation of a bladder stone may occur due to urinary stasis and/or recurrent UTI. Because of increased mucus production and bacterial colonization, stone formation is more common in patients with bladder augmentation, with a reported incidence that ranges from 24 to 72 percent [31,32].

Bladder perforation – Bladder perforation rarely occurs. Patients are at increased risk for this complication following procedures that increase bladder outlet resistance such as bladder neck reconstruction (BNR). This complication may also occur in a patient that has had bladder augmentation [31].

Fistula – Urethrocutaneous or vesicocutaneous fistula (abnormal connection of the urethra or bladder to the skin) is the most commonly reported complications after initial bladder closure [33]. The reported fistula rate is approximately 9 percent in patients managed by CPRBE [28,34], and between 4 and 16 percent in those managed by modern staged repair of bladder exstrophy (MSRBE) [35,36]. It should be noted that in one of the MSRBE series, all of the patients had spontaneous resolution of their fistulae [35].

Hypospadias – Hypospadias appears to be a more common result at the initial closure in patients with CPRBE versus those with MSRBE [34,37]. In our initial series of patients managed with CPRBE, the rate of hypospadias was 43 percent. However, with modification of the procedure (interrupted suture closure of the urethra at epispadias repair), the rate of hypospadias has been reduced to 5 percent. With the delayed approach to CPRBE, hypospadias is rare [16,17]. Of note, repair of hypospadias after CPRBE with a Thiersch-Duplay urethroplasty (a relatively simple urethral tubularization technique) resulted in a high rate of urethrocutaneous fistula, and thus, we do not recommend this technique for hypospadias repair following CPRBE [34].

Penile injury – Ischemic penile injury, typically characterized by hemiglans and hemicorporeal body atrophy, may occur after exstrophy closure combined with epispadias repair (CPRBE), particularly when osteotomy is not performed. Compression of the pudendal vessels after pubic apposition and/or direct injury to the pudendal vessels may play an important role in the pathogenesis of this complication [38].

Long-term complications

Epididymitis – Epididymitis is an infrequent long-term complication, with a reported incidence of 19 to 33 percent in adult male patients [39-41]. In some cases, orchiectomy, vasectomy, or an epididymectomy is required for treatment and pain relief.

Vaginal and rectal prolapses – Vaginal and rectal prolapses are also long-term complications that occur in approximately 20 percent of female patients at a mean age of 16 years [42].

Malignancy – The risk of malignancy appears to be higher in patients with exstrophy than in the general population, especially in a high risk group of patients with bladder augmentation using intestinal components to create a colorectal reservoir (eg, ureterosigmoidostomies) [43,44]. This was illustrated in a large case series of 103 patients with bladder exstrophy [43]. In the high-risk group of 28 patients, there were seven cases of malignancies (colonic carcinoma [n = 3], bladder cancer [n = 3], and carcinoma in situ of the colon [n = 1]). In the low-risk group of 33 patients, one patient had bladder cancer, and one had renal cell carcinoma.

Inguinal hernia – Inguinal hernia is a common long-term complication following bladder closure. Understanding the timing of greatest risk of developing inguinal hernia is important, since incarceration resulting in strangulation of intra-abdominal contents can lead to significant morbidity. Although the incidence and risk factors of inguinal hernia have been reported, the timing of occurrence is not well understood. However, observational data suggest that the risk is greatest within the six months of bladder closure, and the rate of recurrence is higher than the general pediatric population [45].

In a follow-up study (median time of 6.5 years) from our center of 91 patients who underwent CPRE, inguinal hernia repair was performed in 34 of 53 males (64 percent) and 2 of 38 females (5 percent) [45]. The median time to inguinal hernia was 4.7 months following CPRE. In multivariate analysis, male sex was strongly associated with inguinal hernia (hazard ratio [HR] = 19.00, p = 0.0038) but not osteotomy and delay in closure. Of the 36 patients with an initial inguinal hernia, seven presented with recurrence on the ipsilateral side.

The decreased risk of inguinal hernia after one year of follow-up may reflect anatomic stability that is reached following major reconstruction of the pelvis.

OUTCOME

Continence — As discussed previously, urinary continence is one of the goals of successful surgical management of bladder exstrophy. However, comparing results of continence amongst studies is challenging because:

There is no standard definition used for continence in the bladder exstrophy literature. The most commonly used definition is maintenance of dry periods of three or more hours during the day without stress incontinence, although others have used two or more hours [13,46].

Confounding factors, including the age at bladder closure, type of bladder closure performed, the number and type of procedures required to establish continence, and ongoing surgical management changes.

Prior to the mid-1990s and the advent of routine neonatal bladder closure, a higher proportion of patients required bladder augmentation or urinary diversion (eg, ureterosigmoidostomies), urinary continent diversions, and the need for clean intermittent catheterization (CIC) in order to empty the bladder [47,48]. In some studies, patients who required these interventions were defined as achieving continence.

Successful initial bladder closure is an important factor for achieving continence [29,49,50]. Published reports of continence rates vary due to the surgical approach and subsequent need for additional surgical intervention (eg, bladder neck reconstruction).

Modern staged repair – Case series that use a modern staged repair of bladder exstrophy (MSRBE) with bladder neck reconstruction (BNR) report continence rates of approximately 70 percent without bladder augmentation and CIC [8,9,51]. Another center reported a higher rate of continence of 90 percent, but bladder augmentation and CIC were required in 70 and 67 percent of patients, respectively [31]. (See 'Modern staged repair of bladder exstrophy' above.)

Complete primary repair – Data on continence achievement in patients treated with complete primary repair of bladder exstrophy (CPRBE) are limited and suggest that a number of these patients will require BNR to achieve continence [4,28,52-55]. Variations may be due to center experience. With technical revisions of the CPRBE [16,17], toward creating a more anatomically correct bladder neck, the need for post CPRBE bladder neck reconstruction has decreased significantly. (See 'Complete primary repair of bladder exstrophy' above.)

Successful initial bladder closure is an important factor for achieving continence [9,29,49,50]. There appears to be a higher continence rate in female versus male patients [32,47]. However, in a small subset of patients, continence may not be achieved despite multiple surgical attempts. In these cases, further surgical interventions are necessary, including bladder augmentation or bladder removal and a continent diversion procedure.

Renal function — Early series reported poor renal outcome, particularly in patients who required bladder augmentation or ileal loop conduits, with 10 percent of deaths attributed to renal failure [30]. The loss of renal function was felt to be due to recurrent pyelonephritis resulting in significant renal scarring and loss of renal parenchyma.

It is unknown what impact the newer surgical approaches that include early bladder closure will have on renal function [4,8]. In our series of 32 patients with complete primary repair, renal scan demonstrated cortical defects in six patients, which were bilateral in three patients [4]. Seven patients had evidence of mild to moderate hydronephrosis on follow-up renal ultrasonography.

Prospective evaluation that includes serial renal nuclear scans and ultrasounds, and renal functional studies (eg, creatinine clearance) are needed to determine the long-term renal outcome of patients with bladder exstrophy who are managed with either modern staged or complete primary repair. This information will be helpful to determine the optimal surgical approach in the care of patients with bladder exstrophy.

Psychological outcome — Although the major early focus has been on surgical techniques, surgical outcomes, and bladder function, follow-up studies have shown that these patients must deal with psychological ramifications of their disorder, including issues of body image and the impact of multiple and complex surgical procedures. As a result, it is important that these patients undergo psychological evaluation and, if needed, psychological support and therapy.

Although several studies over the last 10 to 20 years have examined the physiological outcomes of bladder exstrophy, they are flawed by faulty or inadequate methodology because of self-selection bias and the lack of normal controls. Nevertheless, studies of children and young adults with bladder exstrophy demonstrate that these patients are at risk for developing a poor body image, anxiety, depression, and adjustment disorder.

Genitalia and body image — Several studies based upon patient surveys and interviews report that adolescents and young adults with bladder exstrophy are dissatisfied with their genital and body appearance [26,42,56-59]. Their poor body image results in avoidance of undressing or showering in front of others, low rates of masturbation and genital touching, anxiety in establishing a close sexual relationship, and restricting sexual activity.

Anxiety and depression — Studies that used validated survey tools for psychological evaluation report that patients with bladder exstrophy are at risk for anxiety, depression, and adjustment disorder [60]. Results also suggest that incontinence was associated with increased psychological dysfunction [26]. Other reports based on patient interviews or study-designed surveys also demonstrated increased anxiety and need for psychological support [57,61]. One retrospective study reported that 15 percent of their 121 patients expressed suicidal ideation; however, a control group was not available for comparison [62].

Sexual function and fertility

Sexuality — Although patients with bladder exstrophy are generally dissatisfied with their genitalia and body image, they appear to have adequate sexual function.

Male patients – Several studies in postadolescent males report erection rates between 94 and 100 percent, and ejaculation rates between 82 and 88 percent [26,39,40,57]. However, ejaculatory abnormalities (including retrograde ejaculation) occur frequently [63], which may be a potential cause of infertility. Despite these concerns, a majority of postpubertal males experienced satisfactory intercourse [63,64].

Female patients – The data are more limited regarding sexual function in postpubertal females.

In one case series of 42 females with bladder exstrophy, 34 engaged in sexual activity. However 28 required a modified episiotomy, and two required vaginoplasty to allow for intercourse [65].

In one study that surveyed 16 female patients greater than 18 years of age, all patients reported appropriate sexual desire, and 10 were sexually active at a mean age of 19.9 years [42]. Three patients reported dyspareunia (genital pain with intercourse) with all sexual activity, and two with occasional dyspareunia. Seven patients reported having orgasms.

Survey results from 28 of 44 patients (64 percent) showed that sexual function and quality-of-life scores were lower for the respondents than normative data [66].

Fertility

Male patients — Case reports and small case series suggest that male patients with bladder exstrophy have decreased fertility due to oligospermia, azoospermia, asthenospermia, decreased sperm motility, and low ejaculate volumes [39,41,64,67,68].

In one case series, semen analyses from 8 of 25 men with bladder exstrophy showed low ejaculate volume in six specimens, normal sperm count in five, and oligospermia in three [40]. Normal sperm motility and viability were maintained in half of the specimens. In a long-term follow up study of 53 male patients, five have fathered children.

Further prospective studies are needed to determine whether or not there is a significant impact on fertility in male patients with bladder exstrophy. If there is a decrease in male fertility, possible causes include semen abnormalities, failure of proper ejaculatory function, or a combination of both. With advances in assisted reproductive technology, conception remains a viable possibility for male patients who have fertility problems. (See "Treatments for male infertility".)

Female patients — Bladder exstrophy appears to also have an impact on female infertility and fetal/neonatal outcome. Case reports and series have described successful pregnancies and delivery in women with bladder exstrophy [42,65]. However, impaired fertility, poor fetal/neonatal outcome, and a risk of significant maternal complications at delivery were reported in a case series of 52 women (mean age 33 years) with classic bladder exstrophy as follows [69]:

Of the women who had tried to become pregnant, approximately two-thirds (19 of 28 patients) were able to conceive.

For the 19 women who had conceived, there were a total of 57 pregnancies including three sets of twins. Outcomes of these pregnancies were 34 live births (56 percent), 21 miscarriages (35 percent), 4 still births or neonatal deaths, and 1 termination.

There were four major maternal delivery room complications including two women with postpartum hemorrhage, one patient with transection of the ureter, and one with fistula formation.

Urinary diversion — Limited data in male adults who underwent bladder removal and urinary diversion suggest that sexuality and fertility were comparable to patients in whom the bladder has been preserved [70].

Orthopedic outcome — There is one retrospective survey study that suggests that adult patients with bladder exstrophy may be at risk for back and hip pain, which may limit the degree of physical activity. In a questionnaire-based survey without physical exam with only a 52 percent response rate (n = 33 respondents), patients with bladder exstrophy were more likely to complain about back pain and an inability to run continuously than those with epispadias [71]. Among patients with bladder exstrophy, those who underwent pelvic osteotomy appeared to experience more hip pain than patients without pelvic osteotomy.

Professional, educational, and social outcome

Patients — Limited data demonstrate that patients with bladder exstrophy are capable of achieving a high educational level and satisfactory employment.

In one long-term follow up study of males with bladder exstrophy, all had attended high school, approximately half had a college education, and those who were not attending school had a full-time job [40].

In a series of 100 patients with a mean age of 14.5 years, only two patients attended schools for special needs children, while the remaining 98 patients attended normal primary and secondary schools and, in some cases, college [57].

In a long-term follow-up study, 25 of an original cohort of 42 patients with bladder exstrophy born between 1937 and 1968 were evaluated. These patients were treated with ureterosigmoidostomy [72]. Of these 25 evaluable patients (mean age 50 years), 21 were married or lived in a stable relationship. Eight were parents (six by normal conception and two by artificial reproductive technology). Nine patients reported no sexual activity or were unable to engage in intercourse. All but two patients were professionally and socially successful.

Parents/caregivers — Data are limited on the effect of bladder exstrophy on parents/caregivers. A survey of 20 parents showed that parents of children diagnosed experienced a significant amount of stress [73]. Answers to standardized questionnaires revealed parents used adaptive means of coping with routine care (ie, plan-based problem solving, seeking social support, positive reappraisal), and nonadaptive means (ie, escape/avoidance and distancing) during times of increased stress. These findings have highlighted the need for specific parental/caregiver evaluation and interventions that have now become a component of the routine comprehensive care at our institution.

Health-related quality of life — Despite the number of long-term sequelae associated with bladder exstrophy, limited data suggest that adolescent patients adapt to the challenges of their disorder. This was illustrated in a study that surveyed adolescents with bladder exstrophy and epispadias using a validated quality of health questionnaire [74]. Although the majority of patients reported incontinence and a median nine surgical procedures, patients with exstrophy/epispadias had similar scores in nearly all domains compared with those of a normal reference control population.

In a study from a single center that evaluated quality of life outcome in children between 8 and 17 years of age, patients with bladder exstrophy had higher health-related quality of life scores for physical functioning and overall quality of life compared with children with kidney stones and the general pediatric population [75]. These surprising findings were attributed to the aggressive and comprehensive medical, surgical, and psychosocial care provided to this cohort of patients with bladder exstrophy. However, patients with bladder exstrophy with lower scores for body image were more likely to have lower social and emotional function scores and less likely to have romantic relationships.

LONG-TERM MANAGEMENT — Life-long care is required for patients with bladder exstrophy because of the risk of long-term complications and the complexity of psychological issues that face these patients. Care is a shared responsibility between the patient and an experienced medical/surgical team of healthcare professionals.

Care includes periodic assessment of the following:

Bladder function and continence

Renal function with serial renal scans, ultrasonography, and plasma/serum creatinine

Psychological well-being with issues that include poor body image, anxiety and screening. If needed, appropriate referral for counseling

Postpubertal sexual function and fertility, and if appropriate, referral for artificial reproductive technology

SUMMARY AND RECOMMENDATIONS

Introduction ‒ Bladder exstrophy is a complex congenital anomaly that is treated surgically. Care for the individual with bladder exstrophy requires life-long care by an experienced and dedicated team of healthcare professionals.

Goals of corrective surgery ‒ Major goals of surgical correction are preservation of normal kidney function, development of adequate bladder storage and function, and provision of acceptable appearance and function of genitalia.

Surgical approaches ‒ Two surgical approaches are currently used to correct bladder exstrophy: modern staged repair of bladder exstrophy (MSRBE), and complete primary repair of bladder exstrophy (CPRBE). Although it is unknown which approach best achieves the goals of surgical outcome, bladder closure in the neonatal period is a major feature of both. In our center, we manage infants with bladder exstrophy with CPRBE. (See 'Surgical approaches' above.)

Bladder closure (Grade 2C) In our center, the preferred timing for CPRBE is at 6 to 8 weeks of age (see 'Surgical approaches' above). This allows for parental bonding and additional growth of the infant and maturation of the bladder.

Avoidance of osteotomy ‒ We do not suggest routine iliac osteotomy if the pelvis is malleable (before 72 hours of life) and there is no extreme diastasis (Grade 2C). Osteotomy is performed in patients with extreme diastasis or poorly malleable pelvis. Infants typically require immobilization with traction following bladder closure (figure 1 and picture 1A-B) regardless of whether an osteotomy is performed. (See 'Neonatal iliac osteotomy and pelvic immobilitzation' above.)

Complications ‒ Complications following surgical correction include bladder dehiscence, urinary tract infection (UTI), bladder stone and perforation, fistula, and hypospadias. Long-term complications include epididymitis in adult males, vaginal and rectal prolapse in female patients, inguinal hernia, and an increased risk of malignancy, especially in patients with bladder augmentation using intestinal components. (See 'Complications' above.)

Outcome

Continence ‒ Bladder continence continues to be one of the major outcome goals following surgery. Reasonable continence levels appear to be achievable following bladder neck reconstruction (BNR) in patients managed by MSRBE. It appears that a significant number of patients with CPRBE will also require BNR to achieve continence. (See 'Continence' above.)

Psychological outcome ‒ Because postpubertal patients with bladder exstrophy often have psychological problems that include poor body image, dissatisfaction with their genital appearance, anxiety, depression, and adjustment disorder, management should include psychological evaluation and, if needed, psychological support and referral. (See 'Psychological outcome' above.)

Sexuality and fertility ‒ Sexual function appears to be adequate in both postpubertal male and female patients with bladder exstrophy. Although data are limited, it does not appear that fertility is an issue for affected females. However, decreased fertility may be a concern in male patients. (See 'Sexual function and fertility' above.)

Adult outcome ‒ Patients with bladder exstrophy are capable of achieving satisfactory professional, educational, and social outcomes. (See 'Professional, educational, and social outcome' above.)

Long-term management ‒ Long-term management includes periodic assessment of bladder and renal function, psychological state, and postpubertal sexual function and fertility. (See 'Long-term management' above.)

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Topic 6582 Version 35.0

References

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