Long-term bladder outcomes using a prescriber pattern scoring system for posterior urethral valves.

BACKGROUND: Urinary drainage for posterior urethral valves can be achieved with valve ablation (VA) or diversion by vesicostomy (VES) or cutaneous ureterostomy (CU). The effect of these interventions on long-term bladder function remains debated, and voiding symptomatology after VES or CU reversal has been poorly characterized. OBJECTIVE: The objective of this study was to examine the prevalence and scope of physician treatment patterns as a surrogate for retention or incontinence symptomatology among PUV patients undergoing primary VA or diversion by VES/CU and determine rates of progression to augmentation. STUDY DESIGN: This is a single-institution retrospective cohort study. Retention Scores (R) were calculated 1 point for: retention behavior (double/timed void), alpha-blocker, intermittent catheterization, or overnight indwelling catheter. Incontinence Scores (I) were calculated 1 point for: incontinence behavior (double/timed void), oral medication, or botulinum toxin. Patients with R score above 3 or I score above 2 were deemed to have severe retention or incontinence symptomatology respectively. End stage bladder (ESB) was defined as need for bladder augmentation. RESULTS: We identified 76 patients between 5 and 40 years old with median follow-up of 14.6 [5.0-40.4) years. There was no difference in the rates of severe retention or incontinence treatment pattern scoring between VA versus VES/CU (Figure). Rates of achieving R(1) status are similar between VA and VES/CU groups, though age of reaching R(1) was younger for those with VES/CU (4.8 years) compared to VA (6.6 years). There was no significant difference in rate of ESB by intervention category VA (9.4%) versus VES/CU (17.4%; p = 0.323). DISCUSSION: Treatment of retention symptomatology was more common than treatment of incontinence symptomatology regardless of primary management, VA or VES/CU. This study also indicates that VES/CU patients were just as responsive as VA patients to conservative treatments (behavioral changes, pharmacotherapy) for any type of bladder symptomatology as the progression to treatment of severe symptomatology and ESB were similar between cohorts. In this cohort, bladder outcomes were not associated with type of urinary diversion (VA or VES/CU). CONCLUSION: Long term bladder outcomes for valve patients demonstrated similar treatment patterns and progression to end-stage bladder regardless of diversion status. Patients went on to ESB approximately 4.4 years after diagnosis at similar rates between groups.

The role of anatomic pelvic dissection in the successful closure of bladder exstrophy: an aid to success.

INTRODUCTION: Classic bladder exstrophy is one of the rarest congenital anomalies compatible with life. Surgical treatment of bladder exstrophy has progressed, but the goal of surgery remains a successful primary bladder closure. Several factors have been identified to decrease the risk of failed closure, including appropriate use of osteotomy and adequate postoperative immobilization and analgesia. However, the role of the radical anatomic pelvic dissection, including dissection of the urogenital diaphragm fibers, in a successful closure has not yet been extensively explored. OBJECTIVE: The objective of this study was go examine the role of radical anatomic pelvic dissection, including dissection of the urogenital diaphragm fibers, in patients with classic bladder exstrophy. STUDY DESIGN: This was a retrospective study based on an institutional database. METHODS: A retrospective review from an institutional approved database of more than 1,300 patients with epispadias-exstrophy complex was performed. The inclusion criteria included patients with classic bladder exstrophy with at least one failed bladder closure and a reclosure at the authors' institution with a single senior surgeon. Data collection included demographics, clinical variables, and status of urogenital diaphragm fibers. Magnetic resonance imaging (MRI) scans, if available, were reviewed with a pediatric radiologist to identify urogenital diaphragm fibers. RESULTS: From the database, 93 patients met inclusion criteria. Of these patients, 74 had urogenital diaphragm fibers completely intact at the time of repeat closure, whereas 19 patients did not. There was no association with age or gender and status of urogenital diaphragm fibers. There was no association with osteotomy, the type of primary bladder closure, surgeon subspecialty, and the status of the urogenital fibers. Fourteen patients had at least two prior closures; surprisingly, 11 of these repeat closure patients still had intact urogenital fibers even after two prior closures. DISCUSSION: The recent development and application of 3D MRI-guided pelvic dissection in a large group of patients led the authors to investigate whether adequate pelvic floor dissection had been accomplished at primary or secondary closure. Several patients had MRI scans performed before repeat closure in which the urogenital diaphragm fibers were identified to be intact on imaging; this was corroborated with surgical findings. Approximately 80% of patients had their urogenital diaphragm fibers completely intact and, therefore, did not have an adequate pelvic dissection during their primary or secondary bladder closure, putting the success of their previous closures at risk. CONCLUSION: Inadequate pelvic diaphragm dissection, defined as intact urogenital diaphragm fibers, demonstrated in a large group of patients with failed exstrophy closure, may be a decisive factor in bladder closure failure. The use of 3D intra-operative image guidance may aid in a safer and more successful pelvic dissection.

Predictors and outcomes of perioperative blood transfusions in classic bladder exstrophy repair: A single institution study.

BACKGROUND: Primary bladder closure of classic bladder exstrophy (CBE) is a major operation that occasionally requires intraoperative or postoperative (within 72 h) blood transfusions. OBJECTIVE: This study reported perioperative transfusion rates, risk factors for transfusion, and outcomes from a high-volume exstrophy center in primary bladder closure of CBE patients. STUDY DESIGN: A prospectively maintained, institutional exstrophy-epispadias complex database of 1305 patients was reviewed for primary CBE closures performed at the authors' institution (Johns Hopkins Hospital) between 1993 and 2017. Patient and surgical factors were analyzed to determine transfusion rates, risk factors for transfusions, and outcomes. Patients were subdivided into two groups based upon the time of closure: neonatal and delayed closure. RESULTS: A total of 116 patients had a primary bladder closure during 1993-2017. Seventy-three patients were closed in the neonatal period, and 43 were delayed closures. In total, 64 (55%) patients received perioperative transfusions. No transfusion reactions were observed. Twenty-five transfusions were in the neonatal closure group, yielding a transfusion rate of 34%. In comparison, 39 patients were transfused in the delayed closure group, giving a transfusion rate of 91%. Pelvic osteotomy, delayed bladder closure, higher estimated blood loss (EBL), larger pubic diastasis, and longer operative time were all associated with blood transfusion. In multivariable logistic regression, pelvic osteotomy (OR 5.4; 95% CI 1.3-22.8; P < 0.001), higher EBL-to-weight ratio (OR 1.3; 95% CI 1.1-1.6; P = 0.029), and more recent years of primary closure (OR 1.1; 95% CI 1.0-1.2; P = 0.018) remained independent predictors of receiving a transfusion (Summary Table). No adverse transfusion reactions or complications were observed. DISCUSSION: This was the first study from a single high-volume exstrophy center to explore factors that contribute to perioperative blood transfusions. Pelvic osteotomy as a risk factor was unsurprising, as the osteotomy may bleed both during and immediately after closure. However, it is important to use osteotomy for successful closure, despite the increased transfusion risk. The risks accompanying contemporary transfusions are minimal and osteotomies are imperative for successful bladder closure. CONCLUSIONS: More than half of CBE patients undergoing primary closure at a single institution received perioperative blood transfusions. While there was an association between transfusions and osteotomy, delayed primary closure, larger diastasis, increased operative time, and increased length of stay, only the use of pelvic osteotomy, higher EBL-to-weight ratio, and recent year of closure independently increased the odds of receiving a transfusion on multivariate analysis.

Back to the future: The Cecil-Culp technique for salvage penile reconstructive procedures.

INTRODUCTION: Re-operative penile reconstruction is challenging and requires tension-free skin closure. The repair popularized by Cecil and Culp in the 1940s, using the scrotum to provide a temporary vascularized bed for complex hypospadias repairs, fell out of favor due to temporal trends towards single-stage repairs and concern for utilizing hair-bearing skin on the penile shaft. OBJECTIVE: It was hypothesized that a modified Cecil-Culp (CC) concept of penile scrotalization, leaving the penis attached to the scrotum for 1 year rather than 6 weeks as originally described, improves outcomes with this reconstruction for ventral skin deficiency or poor vascular support. METHODS: Institutional Review Board-approved registries were reviewed to identify patients who underwent a CC repair during 1987-2016 at two institutions. Cecil-Culp technique was utilized in multi-stage hypospadias complication repairs or for insufficient ventral penile shaft skin coverage. Anatomic abnormality, number and type of prior surgeries, and complications before and after CC were recorded. RESULTS: Thirty-nine patients underwent CC: 23 failed hypospadias repairs, three hypospadias after bladder exstrophy, 10 penile curvature following circumcision, and three with skin loss from trauma. Mean age at CC was 61.8 months (hypospadias), and 59.8 months (non-hypospadias). Hypospadias patients underwent a mean of 3.6 surgeries (range 1-9) prior to CC. Four of the 39 patients (10.3%) had perioperative complications after CC, including scrotal abscess, skin infections, and difficulty removing the urethral stent. Eight of 37 (21.6%) patients had longer-term complications related to their hypospadias repair, including fistulae, diverticula, dehiscence, and stricture. Mean time from CC placement to release was 345 and 473 days for hypospadias and non-hypospadias cases, respectively. There was no apparent scrotal skin transferred to the penile shaft at the final take-down. Mean follow-up was 22.3 months. DISCUSSION: Embedding the penis into the scrotum for added vascularity and ventral skin coverage has been used effectively in cases of the most tenacious fistulas and for significant skin loss and trauma. Limitations of this study were its retrospective approach at two institutions over an extended period of time by multiple surgeons, so patient selection and procedure may have varied. CONCLUSIONS: Modification of CC repair by delaying 9-12 months before CC take-down enhanced the benefits of a robust vascular bed for wound healing, and helped to avoid transfer of hair-bearing scrotal skin to the penile shaft. The CC technique is an important tool for penile reconstructive surgery of complex hypospadias repairs with inadequate skin, and for traumatic injuries.

Congenital renal anomalies in cloacal exstrophy: Is there a difference?

INTRODUCTION: Cloacal exstrophy (CE) is the most severe manifestation of the epispadias-exstrophy spectrum. Previous studies have indicated an increased rate of renal anomalies in children with classic bladder exstrophy (CBE). Given the increased severity of the CE defect, it was hypothesized that there would be an even greater incidence among these children. OBJECTIVE: The primary objective was to characterize renal anatomy in CE patients. Two secondary objectives were to compare these renal anatomic findings in male and female patients, and female patients with and without Müllerian anomalies. STUDY DESIGN: An Institutional Review Board-approved retrospective review of 75 patients from an institutional exstrophy database. Data points included: age at analysis, sex, and renal and Müllerian anatomy. Abnormal renal anatomy was defined as a solitary kidney, malrotation, renal ectopia, congenital cysts, duplication, and/or proven obstruction. Abnormal Müllerian anatomy was defined as uterine or vaginal duplication, obstruction, and/or absence. RESULTS: The Summary Table presents demographic data and renal anomalies. Males were more likely to have renal anomalies. Müllerian anomalies were present in 65.7% of female patients. Girls with abnormal Müllerian anatomy were 10 times more likely to have renal anomalies than those with normal Müllerian anatomy (95% CI 1.1-91.4, P = 0.027). DISCUSSION: Patients with CE had a much higher rate of renal anomalies than that reported for CBE. Males and females with Müllerian anomalies were at greater risk than females with normal uterine structures. Mesonephric and Müllerian duct interaction is required for uterine structures to develop normally. It has been proposed that women with both Müllerian and renal anomalies be classified separately from other uterine malformations on an embryonic basis. In these patients, an absent or dysfunctional mesonephric duct has been implicated as potentially causal. This provided an embryonic explanation for uterine anomalies in female CE patients. There were also clinical implications. Women with renal agenesis and uterine anomalies were more likely to have endometriosis than those with isolated uterine anomalies, but were also more likely to have successful pregnancies. Males may have had an analogous condition with renal agenesis and seminal vesicle cysts. Future research into long-term kidney function in this population, uterine function, and possible male sexual duct malformation is warranted. CONCLUSION: Congenital renal anomalies occurred frequently in children with CE. They were more common in boys than in girls. Girls with abnormal Müllerian anatomy were more likely to have anomalous renal development. Mesonephric duct dysfunction may be embyologically responsible for both renal and Müllerian maldevelopment.

Inflatable penile prosthesis technique and outcomes after radial forearm free flap neophalloplasty.

The aim of this study was to describe the technical aspects and short-term outcomes of inflatable penile prosthesis (IPP) implantation after neophallus reconstruction at a single institution. Nine men with previously constructed radial forearm neophalli underwent IPP implantation. The etiologies of their penile anomaly were bladder exstrophy complex in five, disorder of sexual differentiation in two and genital obliteration secondary to ballistic trauma in two. Median follow-up was 9.6 months (range 1.5-139.7). The records for these patients were retrospectively reviewed and outcomes recorded. Mean age was 23.6 (range 18-31) years, and mean time interval from neophalloplasty to IPP implantation was 22.1 months (range 3-48). In all cases, 3-piece IPPs were employed, with eight of patients having one cylinder implanted in the native corporal body and extending into the neophallus. Mean surgical time was 222 min (range 142-409). Median length of implanted device was 22 cm. No intraoperative complications were observed. At the most recent follow-up, six patients (66.7%) had functional devices, with acceptable surgical outcomes. Three patients (33.3%) sustained device infections, and three (33.3%) sustained cylinder erosion. In three patients in whom neo-tunica albuginea were fashioned by ensheathing the cylinder with allograft human dermal tissue matrix, no erosions occurred. One patient underwent two revisions, the first for the associated erosion and infection and the second for genital pain, and was left with a semi-rigid prosthesis. IPP implantation affords the best opportunity for functionality for patients with a radial forearm free flap neophallus. Caution must be taken to ensure viability of the neophallus intraoperatively, and protocols to minimize the risk of infection should be followed. Fashioning neo-tunica albuginea using graft material may reduce risk of erosion.

2D and 3D MRI features of classic bladder exstrophy.

The bladder exstrophy-epispadias complex (EEC) represents a spectrum of rare and surgically correctable congenital anomalies. Classic bladder exstrophy (CBE) stands between epispadias and cloacal exstrophy (CE) in the severity spectrum, and is the most commonly encountered type. CBE involves congenital defects of the bladder, abdominal wall, pelvic floor, and bony pelvis. With the growing understanding of the detrimental effects of radiation in children, magnetic resonance imaging (MRI) is progressively been utilized in the preoperative work-up and post-surgical follow-up of these patients. MRI provides valuable information for planning and evaluating the optimal surgical techniques for closure of CBE. The aim of this paper is to provide a review of the two- (2D) and three-dimensional (3D) MRI features of CBE including a detailed analytical description of the anatomy of the pelvic floor in affected patients.

Utility of tissue expansion in pediatric phallic reconstruction: a 10-year experience.

OBJECTIVE: Boys with complex penile anomalies often undergo multiple operations, leaving a paucity of unscarred skin for further reconstructive procedures. Our objective was to evaluate the ability of tissue expansion to provide local skin for successful phallic reconstruction. MATERIALS AND METHODS: Eighty boys (mean age of 11.9 years) with hypospadias (n = 42) or epispadias (n = 38) formed the study cohort. All patients had undergone at least one failed reconstructive operation. Indications for tissue expansion included scarcity of penile skin with urethral stenosis, urethrocutaneous fistula, chordee, and/or residual defect. One or two expanders were placed under the skin of the penile shaft and removed at the time of reconstruction. RESULTS: Average time between expander placement and reconstruction was 10.9 weeks. Mean follow-up time was 25.3 months. Complications during expansion occurred in 33 patients (41.3%). Twenty-two patients (27.5%) had at least one expander removed prematurely and 46.9% were replaced. Expansion yielded adequate tissue for reconstruction in 76 patients (95.0%). Successful outcomes were achieved in 39 patients after initial reconstruction and 25 patients after further intervention, yielding an overall success rate of 80.0%. CONCLUSION: Tissue expansion is a useful tool with an acceptable rate of complications for phallic reconstruction in patients who have failed prior surgical reconstruction.

The use of alloderm as an adjunct in the closure of the bladder - Cloacal exstrophy complex.

OBJECTIVE: To decrease the incidence of vesicocutaneous fistulae (VCF), intra-pubic stitch erosion (IPE) and intrasymphyseal plate erosion (ISE) of bladder or cloacal exstrophy in patients undergoing reclosure, we sought additional bulking material to place between the posterior urethral/bladder neck and pubic closures. MATERIAL AND METHODS: In 43 patients (2005-2009) undergoing exstrophy/cloacal exstrophy closure or reclosure, we placed human acellular dermis (HAD) between the posterior urethral/bladder neck and pubic closures. The thickest piece of HAD available was placed above the urethra and bladder neck, and attached to the pelvic floor with sutures of 4-0 Vicryl prior to pubic bone apposition. RESULTS: Twenty-three were primary and 20 were reclosures. Of the 23 primary closures, 17 were classic exstrophy and six were cloacal exstrophy. Of the 20 reclosures, 17 were classic exstrophy and three were cloacal exstrophy. Thirty-four had an osteotomy and nine did not, at the time of closure. No patient experienced failure of closure, a VCF, an IPE or an ISE into the urethra after pubic apposition, or other complication related to the use of HAD. One patient had a superficial wound infection, and one had premature suprapubic tube dislodgement requiring replacement in the operating room. CONCLUSION: From the success of this novel technique in failed closures, we have begun using HAD as an adjunct in all exstrophy closures.

Sexual function and fertility issues in cases of exstrophy epispadias complex.

In patients with EEC, the issues such as sexuality, sexual function and fertility gain more importance once theses patients advance from puberty to adulthood. The aim of this review is to critically examine the available evidence on these issues. A systemic literature search was performed in Medline over the last 25 years using the key words: Exstrophy, sexual function and pregnancy. Search results were limited to studies of patients with exstrophy published in English literature. A total of 1500 publications were found and subsequently screened by title and when appropriate by abstracts. Of these, 40 publications pertinent to the subject were included for the analysis. The publications were supplemented by an additional 15 publications obtained from their bibliographies. The studies were rated according to the guidelines published by the US department of health and human services. Heterosexuality is usually expressed in both the sexes and most of them have adequate sexual function. Urinary diversion in some series seems to result in better ejaculatory hence fertility outcome in male patients. Recent series have shown equally good results with primary reconstruction. Most of the female patients have normal fertility while male patients have significantly low fertility. Most of the male and female patients with EEC have adequate sexual function. Most of the female patients have normal fertility while most of the male patients have significantly low fertility.

Mitrofanoff continent catheterizable conduits: top down or bottom up?

OBJECTIVE: During augmentation and Mitrofanoff procedures, conduits are usually implanted into the posterior bladder wall. Anatomical considerations may necessitate an anterior conduit. To compare the relative drainage efficiency in patients with posterior and anterior conduits, we studied their rates of bladder stone formation and urinary tract infection (UTI). MATERIALS AND METHODS: A retrospective chart review identified exstrophy patients who underwent augmentation and Mitrofanoff between 1991 and 2003. Patients with 3 years or greater follow-up were included. Fifty-four patients fit this criterion, with a conduit implanted anteriorly (33) or posteriorly (21). We compared rates of bladder stone formation and UTI. Stomal revisions and the status of the bladder neck were also noted. RESULTS: Stone formation and UTI rates were higher in the anterior conduits, although only UTI showed a statistically significant difference. Patient demographics were similar between the two groups, including age and sex. The rates of stomal complications and the bladder neck status were also similar. CONCLUSIONS: Patients with anterior conduits had an increased risk of UTI and bladder stone formation compared to those with posterior conduits, although this was not significant in the case of bladder stone rate. This may indicate sub-optimal bladder drainage and should be addressed with careful preoperative counseling and close follow-up.

Epidemiological survey of 214 families with bladder exstrophy-epispadias complex.

PURPOSE: We sought to identify causative nongenetic and genetic risk factors for the bladder exstrophy-epispadias complex. MATERIALS AND METHODS: A total of 237 families with the bladder exstrophy-epispadias complex were invited to participate in the study, and information was obtained from 214 families, mainly from European countries. RESULTS: Two families showed familial occurrence. Male predominance was found among all subgroups comprising epispadias, classic bladder exstrophy and cloacal exstrophy, with male-to-female ratios of 1.4:1, 2.8:1 and 2.0:1, respectively (p = 0.001). No association with parental age, maternal reproductive history or periconceptional maternal exposure to alcohol, drugs, chemical noxae, radiation or infections was found. However, periconceptional maternal exposure to smoking was significantly more common in patients with cloacal exstrophy than in the combined group of patients with epispadias/classic bladder exstrophy (p = 0.009). Only 16.8% of mothers followed the current recommendations of periconceptional folic acid supplementation, and 17.6% had started supplementation before 10 weeks of gestation. Interestingly, in the latter group mothers of patients with cloacal exstrophy were more compliant with folic acid supplementation than were mothers of the combined group of patients with epispadias/classic bladder exstrophy (p = 0.037). Furthermore, mothers of children with cloacal exstrophy knew significantly more often prenatally that their child would have a congenital malformation than did mothers of children with epispadias/classic bladder exstrophy (p <0.0001). CONCLUSIONS: Our study corroborates the hypothesis that epispadias, classic bladder exstrophy and cloacal exstrophy are causally related, representing a spectrum of the same developmental defect, with a small risk of recurrence within families. Embryonic exposure to maternal smoking appears to enforce the severity, whereas periconceptional folic acid supplementation does not seem to alleviate it. There is a disproportional prenatal ultrasound detection rate between severe and mild phenotypes, possibly due to the neglect of imaging of full bladders with a focus on neural tube defects.

Modern staged repair of bladder exstrophy: a contemporary series.

OBJECTIVE: Many changes have occurred in the treatment of bladder exstrophy over the last few years and many repairs are now offered. The purpose of this study was to evaluate long-term outcomes in a select group of patients in whom modern staged repair (MSRE) was undertaken. PATIENTS AND METHODS: From an institutionally approved database were extracted 189 patients who had undergone primary closure between 1988 and 2004. The records of 131 patients (95 males) who underwent MSRE with a modified Cantwell-Ransley repair by a single surgeon in 1988-2004 were reviewed with a minimum 5-year follow up. RESULTS: Sixty-seven patients with a mean age of 2 months (range 6 h to 4 months) underwent primary closure, and 18 underwent osteotomy at the same time. Mean age at epispadias repair was 18 months (8-24). Mean age at bladder neck reconstruction (BNR) was 4.8 years (40-60 months) with a mean capacity of 98 cc (75-185). Analysis of bladder capacity prior to BNR revealed that patients with a mean capacity greater than 85 cc median had better outcomes. Seventy percent (n=47) are continent day and night and voiding per urethra without augmentation or intermittent catheterization. Social continence defined as dry for more than 3h during the day was found in 10% (n=7). Six patients required continent diversion after failed BNR. Seven patients are completely incontinent. The mean time to daytime continence was 14 months (4-23) and the mean time to night-time continence was 23 months (11-34). No correlation was found between age at BNR and continence. CONCLUSIONS: Patients with a good bladder template who develop sufficient bladder capacity after successful primary closure and epispadias repair can achieve acceptable continence without bladder augmentation and intermittent catheterization.

Reconstructive lower urinary tract surgery in incontinent adolescents with exstrophy/epispadias complex.

OBJECTIVES: To outline the management strategies applied to the adolescent patient population with exstrophy/epispadias and incontinence at our institution. These patients present a difficult management problem. At the same time they are dealing with difficult issues, including body image and sexual awareness, the added burden of ongoing incontinence causes major anxieties and lifestyle restrictions. In many, incontinence has persisted despite numerous operations. METHODS: A total of 25 (19 male and 6 female) patients who remained incontinent into adolescence or early adulthood were reviewed. Of the 25 patients, 19 had exstrophy, 4 had cloacal exstrophy, and 1 male and 1 female had epispadias. Six patients had undergone eight prior attempts at continent reconstruction, one had undergone cutaneous diversion, and one had problems after ureterosigmoidostomy. RESULTS: The mean age at continence surgery was 12.9 years. Of the 25 patients, 18 underwent bladder augmentation, with a continent stoma in 17 and an artificial sphincter in 1. An additional 5 patients underwent bladder neck transection, with a new continent stoma in 3. The ureterosigmoidostomy was converted to a Mainz II pouch. One patient underwent continent neobladder formation. Nine patients (36%) developed complications during follow-up. Three required stoma revision for stenosis and one for prolapse. Pouch stones occurred in 4 patients, and vesicocutaneous fistula developed in 1. All achieved full urinary continence. The mean follow-up was 72.4 months. CONCLUSIONS: Some children with exstrophy/epispadias reach adolescence and remain incontinent. For these patients, modern reconstructive techniques provide hope of continence. With careful preoperative assessment, exact surgical precision, and regular follow-up, a successful outcome can be expected in virtually all cases without the need for external urine collection devices.

Polyps in the exstrophic bladder. A cause for concern?

PURPOSE: The role of environmental injury in carcinogenesis is widely recognized. Malignancy in exstrophic bladders has been reported most frequently in untreated adults and those undergoing surgical treatments which involve the mixing of fecal and urinary streams. The question of whether the closed exstrophic bladder has a similar potential for malignancy has not been resolved. The polypoid appearance of the exstrophic bladder template raises the concern of premalignant lesions. We characterized the histology of these lesions and analyzed their microscopic features with particular reference to predisposition for dysplasia. In doing so, we attempt to address the aforementioned question and set the stage for definitive quantification of the risk of malignancy in these patients with careful, long-term followup. MATERIALS AND METHODS: Under institutional board review, the slides of 38 patients with classic bladder exstrophy who had polyps excised at the time of closure were reviewed by a single genitourinary pathologist (JIE). The most common findings were reported for polyps resected at primary and secondary closure, respectively, and a comparative analysis was performed. RESULTS: Of the 38 cases 24 were primary closures and 14 were secondary closures. Six of the primary closures were delayed by 6 weeks or greater. The 2 basic types of polyps observed were fibrotic and edematous. Both types were associated with overlying reactive squamous metaplasia in approximately 50% of cases. Varying degrees of fixed on file Brunn's nests, cystitis cystica and cystitis glandularis were noted. Cystitis glandularis was observed in a significantly greater percentage of secondary closures (p = 0.0014). CONCLUSIONS: Although no dysplasia was noted, cystitis glandularis is associated with the development of adenocarcinoma of the bladder. The finding of cystitis glandularis suggests a more severe epithelial injury and it follows that the significant majority of these cases (10 of 14, 71.4%) were observed with polyps resected during secondary closure. These patients warrant future surveillance with urine cytology and cystoscopy as they enter adult life.

The use of combined bladder and epispadias repair in boys with classic bladder exstrophy: outcomes, complications and consequences.

PURPOSE: Despite widespread use of modern staged reconstruction for classic bladder exstrophy, there remains a role for combined bladder closure and epispadias repair when primary closure is delayed or initial reconstruction has failed. The principle of combining bladder and urethral closure in 1 operation was first proposed more than 40 years ago, and represents a demanding technical procedure. We recount our experience to date with this approach. MATERIALS AND METHODS: A total of 38 boys underwent combined bladder and epispadias repair using pelvic osteotomies. Five cases were delayed primary closures owing to a bladder template unsuitable for newborn closure. A total of 30 cases were previous failed bladder closures, with concurrent epispadias repair in 6. The 3 remaining cases were staged closures where the epispadias repair failed, leading to bladder prolapse via the posterior urethra. Overall, there was a major bladder prolapse in 25 cases, and separation of the pubic symphysis with dehiscence of anterior abdominal wall structures and bladder in 8. RESULTS: Mean age at surgery was 26.5 months for the 33 reclosures. A total of 10 boys had development of a urethrocutaneous fistula and 4 had development of strictures. A total of 19 patients required additional procedures of the bladder neck (endoscopic), urethra or penis. A later bladder neck reconstruction was undertaken in 19 boys, of whom 12 are continent. Seven boys underwent continent diversion and 5 are considering the procedure. A total of 14 boys are awaiting adequate capacity for bladder neck reconstruction. Ureteral reimplantation was performed in 22 patients, and no patient was rendered hypospadiac. CONCLUSIONS: When combined closure is applied to failed previous exstrophy repair one can expect at best a 50% continence rate without continent diversion. Furthermore, based on the experience at this institution the majority of patients require ureteral reimplantation, and many require additional surgery to the penis or urethra. However, with the application of modern reconstructive techniques continence and a cosmetically pleasing phallus can be expected in most cases, although at the expense of multiple surgical procedures.

The place of pelvic osteotomy in the modern era of bladder exstrophy reconstruction.

BACKGROUND: The place of pelvic osteotomy in reconstructing bladder/cloacal exstrophy has been debated for some time; the experience with 'combined' osteotomy in primary and re-operative exstrophy closure at this institution is presented, with a discussion of the historical and scientific place of osteotomy in managing this condition. PATIENTS AND METHODS: Sixty-eight patients had bilateral vertical and transverse iliac osteotomy between 1992 and 2003, and with outcome data available. Of 58 patients with classic exstrophy, eight were newborns, eight were deliberately delayed primary closures, 36 were re-operative after previous failed closure and six were bladder neck reconstructions where the bladder outlet was very wide, such that bony closure was felt necessary for successful bladder neck coaptation. Of 10 patients with cloacal exstrophy, nine were primary closures and one was a re-operative closure. Data were collected relating to age at closure, complications and continence outcome. RESULTS: The mean (range) age (months) was 41 (5-179) for re-operative closures, 12.5 (3-32) for delayed primary closures, 64.1 (38-79) for bladder neck reconstruction, 51.4 (6-165) for cloacal exstrophy closure, and 15 (2-45) days for newborn exstrophy closure. There was a superficial wound infection in two patients, pin-site infection in one, loose pins in two, and two had transient femoral nerve palsy. In two patients the procedure failed and they required further re-operative closure with osteotomy. Sixteen patients are dry urethrally day and night, 12 have had and four are awaiting bladder augmentation, one has a colon conduit, and 35 are awaiting a definitive continence procedure. CONCLUSIONS: Osteotomy has a proven track record in the field of exstrophy reconstruction, and the benefit especially in re-operative closure is emphasized by the present results. The surgical morbidity with the 'combined osteotomy' is low, cosmetic results are excellent and the effect on success of closure is clearly advantageous.

Applications of the modified Cantwell-Ransley epispadias repair in the exstrophy-epispadias complex.

OBJECTIVE: The Cantwell-Ransley technique is the most popular and widely used approach to epispadias repair. This is an evaluation and update of the long-term results of using the modified Cantwell-Ransley technique for epispadias repair. PATIENTS AND METHODS: The modified Cantwell-Ransley epispadias repair technique was performed on 129 boys of which 97 had classic bladder exstrophy and 32 complete epispadias. For 106 boys this was primary urethral repair (82 with classic exstrophy, 24 with epispadias) and for the other 23 boys it was a repeat repair (15 with exstrophy, eight with epispadias). RESULTS: At a mean follow-up of 88 months, 120 had a penis that was inclined downward or horizontally while standing. In patients with exstrophy, fistulae were noted in 16% and 33% after primary and repeat urethral repair, respectively. In patients with epispadias, fistulae were noted in 13% and 25% after primary and secondary repair, respectively. In total, five boys with a fistula appearing in the immediate postoperative period following primary urethral repair demonstrated spontaneous healing by 3 months' follow-up. Urethral stricture requiring treatment developed in nine patients. Minor wound infection and skin separation occurred in nine with exstrophy and three with epispadias. Endoscopic examination or catheterization in 120 cases revealed an easily manipulated neourethra. Of 15 sexually active patients, all reported orgasms and ejaculation with a straight penis on erection, although one has complained that his penis is shorter since surgery. CONCLUSIONS: The modified Cantwell-Ransley technique for epispadias repair produces durable functional and cosmetic results, and fewer major complications than seen with other repairs. Fistulae occurring after primary urethral repair may close spontaneously, but all those occurring after repeat closure will require further surgery.

Loss of the penile glans and/or corpora following primary repair of bladder exstrophy using the complete penile disassembly technique.

PURPOSE: The technique of penile disassembly is increasingly being used for the surgical repair of classical bladder exstrophy. We describe the complications and discuss the possible etiologies of genital injuries we have seen following this operation. MATERIALS AND METHODS: A review of the records of patients evaluated for management of genital complications following complete repair of bladder exstrophy from 1996 to 2003 was performed. RESULTS: Nine patients were evaluated for genital injuries following complete repair of bladder exstrophy using the penile disassembly technique. Injuries included the loss of 1 hemiglans and penile urethra in 2 cases; loss of 1 hemiglans and distal corpora in 2; loss of bilateral glans, distal corpora and penile urethra in 2; loss of 1 hemiglans, 1 corporal body, urethral plate and penile shaft skin in 1; loss of 1 hemiglans, distal corporal body and a portion of the urethra in 1; and loss of 1 hemiglans in 1. The exact etiology of these complications is unknown, and possibilities include, a technical mishap, induction of venous congestion/arterial spasm or disruption of a congenitally abnormal blood supply. CONCLUSIONS: The finding that primary repair of bladder exstrophy using the penile disassembly technique is associated with the risk of partial or complete penile loss dampens our enthusiasm for this procedure. The association of genital injuries with penile disassembly has resulted in a modification of this surgical technique that would hopefully lessen its risk.

Coping with bladder exstrophy: diverse results from early attempts at functional urinary tract surgery.

OBJECTIVES: To report the long-term surgical outcome in a group of patients with bladder exstrophy treated from 1960 to 1982, and to assess physical health, social integration and sexual function, as attempts at functional closure during the development of this surgery resulted in patients with differing surgical status of the genitourinary organs. PATIENTS AND METHODS: A review of medical record archives revealed 36 patients; of these, seven had died, six were untraceable and six declined to participate, leaving 15 evaluable subjects (seven men and eight women, mean age at follow-up, 35 years). Data were collected from medical records and direct interviews were conducted using a semi-structured questionnaire, after obtaining informed consent. The Short Form-36 (SF-36) v2 Health Survey instrument was used to assess health and well-being. RESULTS: Three patients have retained the use of their bladder, and 11 had initial bladder closure but required (at a mean age of 3.1 years) various urinary diversions. Eight patients had a pelvic osteotomy. Of 27 functioning renal units, 25 are in reasonable to good condition. Six men and six women had genital reconstruction. Four men are capable of penetrative intercourse and ejaculate; six women manage penetrative intercourse and five have orgasms. All patients attended mainstream school and 13 achieved examination success. Nine patients took vocational training and 11 work full-time. Five patients are married and five are in long-term relationships. Two men have achieved three pregnancies and one women has had a child. The mean total SF-36 score (maximum 3600) was 2763 in men and 2235 in women. CONCLUSIONS: Surgery for bladder exstrophy has been developing for more than 40 years and the legacy of early attempts at functional closure is a population of adults who have a diversity of lower urinary tracts, good preservation of renal function overall and acceptable sexual function. They are usually robust, healthy and well-adjusted individuals functioning well in society, often in full-time employment and long-term relationships. Adolescent follow-up must be clearly focused, incorporating a multidisciplinary team approach to facilitate a seamless transition into adulthood.