Multi-Institutional Bladder Exstrophy Consortium After 8 Years: The Short- and Intermediate-Term Outcomes.

PURPOSE: Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real-time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long-term protection of the upper tracts. This study reports on our short-term complications and intermediate-term continence outcomes. MATERIALS AND METHODS: A single prospective database for all patients undergoing surgery with a BE epispadias complex diagnosis at 3 institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies, and immobilization techniques, and subsequent surgeries. Data on short-term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate-term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound, presence of vesicoureteral reflux, cortical defects on nuclear scintigraphy, and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization, and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded. RESULTS: CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (interquartile range) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 patients who did not have an osteotomy), 6 in modified Bryant's traction, and 2 in external fixation with Buck's traction. Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short-term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate-term outcomes were available for 40 of the 46 patients, who have between 4 and 8 years of follow-up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals. CONCLUSIONS: Cumulative efforts of prospective data collection have provided granular data for evaluation. Short-term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate-term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow-up are voiding with dry intervals of > 1 hour.

Male epispadias repair: Outcomes at three sites prior to the establishment of a multi-institutional collaboration.

INTRODUCTION: Epispadias, which occurs on the more mild end of the Bladder Exstrophy Epispadias Complex (BEEC) spectrum, presents still with a wide range of severity in boys, from mild glanular epispadias to penopubic epispadias with severe urethral and bladder neck defects. Surgical management ranges from isolated epispadias repair to epispadias repair with bladder neck reconstruction (BNR) with or without pelvic osteotomies. OBJECTIVES: We aimed to evaluate outcomes in epispadias treated at three institutions prior to formation of a formal collaboration. In addition, we sought to delineate outcomes based on anatomic severity at time of diagnosis, and initial procedure performed in cases of penopubic epispadias. METHODS: IRB approved databases were retrospectively queried at three institutions for patients who underwent repair of epispadias between 1/1993 and 1/2013. Degree of epispadias, age and technique at initial repair, and self-reported continence status at last follow-up were recorded. Continence was categorized as: wet, intermediate (dry 2-3 h), or dry, while also distinguishing those who void and those who require clean intermittent catheterization (CIC). Those not seen since 1/1/2015, younger than 10 years at last follow up, or in whom continence data were not recorded were excluded. RESULTS: A total of 48 boys were identified; 36 met inclusion criteria. The epispadias cohort consisted of 8 glanular epispadias (GE) (22%); 8 penile epispadias (PE) (22%), and 20 penopubic epispadias (PPE) (56%) with a median follow-up of 11.3 years (3.2-26.2 years). Overall, 33 of 36 (92%) boys void per urethra. Within the group that voids, 19/33 (58%) are completely dry, while 6/33 (18%) are wet. Among patients who underwent initial epispadias repair without concurrent or subsequent bladder neck reconstruction, continence rates were: GE 63% (5/8); PE 75% (6/8); PPE 71% (5/7). Among the 9 boys with PPE who underwent initial epispadias repair with concurrent BNR, 22% (2/9) were dry with no further surgeries. Overall, 8/20 (40%) of boys with PPE void with complete dryness. DISCUSSION: This multi-center retrospective review of continence in epispadias demonstrates that even some boys with glanular and penile epispadias can have challenges with continence, and boys with penopubic epispadias may remain wet despite careful preoperative assessment of bladder neck functionality and concurrent BNR. CONCLUSION: Continence outcomes in boys with all degrees of epispadias can be variable. Even boys with more distal defects may have significant bladder neck deficiency. And those with the most severe form of epispadias may require bladder neck reconstruction to achieve continence.

Prenatal Diagnosis of Bladder Exstrophy and OEIS over 20 Years.

OBJECTIVE: To examine the prenatal diagnosis rates of bladder exstrophy (BE) and Omphalocele-Exstrophy-Imperforate anus-Spinal Defect Syndrome (OEIS) in a large cohort of patients over a 20-year period. We hypothesized that prenatal diagnosis rates improved over time due to evolving techniques in fetal imaging. METHODS: A multi-institutional database was queried to identify BE or OEIS patients who underwent primary closure between 2000 and 2020. We retrospectively determined prenatal or postnatal diagnosis. Those with unknown prenatal history were excluded. Multivariable logistic regression was used to investigate temporal pattern in rate of prenatal diagnosis while adjusting for sex and treating institution. RESULTS: Among 197 BE and 52 OEIS patients, 155 BE and 45 OEIS patients had known prenatal history. Overall prenatal diagnosis rates of BE and OEIS were 47.1% (73/155) and 82.2% (37/45), respectively. Prenatal diagnosis rate was significantly lower in BE compared to OEIS (P <.0001). The prenatal diagnosis rate for BE significantly increased over time (OR 1.10; [95%CI: 1.03-1.17]; P = .003). Between 2000 and 2005, the prenatal diagnosis rate of BE was 30.3% (10/33). Between 2015 and 2020, prenatal diagnosis rate of BE was 61.1% (33/54). Prenatal diagnosis rate for OEIS did not change over time. Rates of prenatal diagnosis did not differ by sex or treating institution. CONCLUSION: Rates of prenatal diagnosis of BE and OEIS are higher than previously reported. Prenatal diagnosis rate of BE doubled in the last 5 years compared to the first 5 years of the study period. Nonetheless, a significant proportion of both BE and OEIS patients remain undiagnosed prior to delivery.

Technical details and long-term outcomes of P.A.D.U.A. for congenital urethral narrowing; a case series and review of the literature.

INTRODUCTION: The P.A.D.U.A. technique is a method of addressing congenital urethral narrowing. It involves passive dilation with a series of progressively larger indwelling catheters. Utilization is limited by scant literature, particularly regarding technical details and long-term durability. Tools for achieving safe and reliable urinary drainage are critical in these patients, who require careful stewardship of their kidney and bladder function. OBJECTIVE: To describe long-term urethral patency and urinary function following P.A.D.U.A., and to provide sufficient technical detail to reproduce the technique. STUDY DESIGN: Patients with congenital urethral narrowing managed with P.A.D.U.A. were identified and chart review was performed. Details of catheter exchange sequences were compiled and described. The primary outcome was the attainment of adequate urethral caliber by successful completion of P.A.D.U.A., and the secondary outcome was voiding per urethra at most recent follow-up. RESULTS: P.A.D.U.A. achieved adequate urethral caliber in 9/11 (82%) of patients. This included seven patients with Prune Belly Syndrome, one with isolated urethral atresia, and one with a cloacal anomaly. P.A.D.U.A. failed to achieve urethral patency in one patient with urethral duplication, who was unable to progress through the catheter sequence, and one patient with Prune Belly Syndrome, who completed P.A.D.U.A. but developed recurrent narrowing one week later. There were no delayed failures of urethral patency. Patients who achieved patency underwent a median of seven catheter placements over 92 days. Median (range) initial and final catheter sizes were 3.5F (1.9-8F) and 14F (8-16F). While 82% achieved patency, only 3/11 (27%) were voiding spontaneously per native urethra at most recent follow-up. DISCUSSION: This series of patients undergoing P.A.D.U.A. for primary treatment of congenital urethral narrowing is the largest to date and provides granular technical details. It aligns with prior reports suggesting that P.A.D.U.A. achieves urethral patency in most patients with Prune Belly Syndrome or isolated urethral atresia, but has limited application in the hypoplastic duplicated urethra. Despite high rates of urethral patency in this select population, many patients will not achieve and maintain spontaneous voiding without catheterization. This is likely due to ongoing deterioration of bladder function caused by the prenatal developmental insult, paralleling the phenomenon seen in posterior urethral valves. CONCLUSION: P.A.D.U.A. is an effective and durable technique for achieving urethral patency. However, due to complicating factors such as the underlying bladder pathology present in many patients, urethral patency achieved with P.A.D.U.A. does not guarantee long-term safe and reliable spontaneous emptying per urethra.

Repurposing tools: A peña stimulator as a tool to map the limits of dissection and continence zone during bladder exstrophy repair.

INTRODUCTION: The complete primary repair of bladder exstrophy (CPRE) aims to restore normal anatomy through complete mobilization and reapproximation of the bladder neck and proximal urethra. METHODS: The Peña stimulator has previously been used to identify musculature in the pelvis. The device is now used to distinguish intersymphyseal bands from pelvic floor and urethral sphincteric musculature during CPRE. The ability to distinguish the levator ani muscles from fibrotic bands assists the surgeon in identifying the appropriate extent of deep pelvic dissection during CPRE. RESULTS: During the anatomic bladder neck recreation, the muscle stimulator demonstrated the striated muscle at the level of the bladder neck and urethral sphincter. CONCLUSION: Localization of the muscles at the bladder neck and proximal urethra corroborates the concept that pelvic floor physical therapy could enhance the strength of the pelvic floor in children with bladder exstrophy.

Bilateral ureteral reimplantation at complete primary repair of exstrophy: Post-operative outcomes.

BACKGROUND AND STUDY OBJECTIVE: The value of bilateral ureteral reimplant (BUR) at the time of complete primary repair of bladder exstrophy (CPRE) has been suggested, however, outcomes are poorly characterized in current medical literature. We hypothesize that BUR at time of CPRE will decrease the rate of recurrent pyelonephritis, post-operative vesicoureteral reflux (VUR), and the need for subsequent ureteral surgery. STUDY DESIGN: We analyzed 64 consecutive patients with a diagnosis of classic bladder exstrophy (BE) who underwent CPRE at three institutions from 2013 to 2019.15 patients underwent cephalotrigonal BUR-CPRE and 49 patients underwent CPRE alone. Our primary outcome was >1 episode of pyelonephritis as documented in the medical record. Secondary outcomes were persistent vesicoureteral reflux (VUR), with a sub-analysis of number of refluxing renal units and presence of dilating VUR, and the need for subsequent ureteral surgery. Descriptive statistics in addition to standard, two tailed univariate statistics, were used to compare the groups where appropriate. RESULTS: BUR-CPRE was associated with a significant decrease in the rates of post-operative VUR, number of refluxing renal units, and need for subsequent ureteral surgery (p = 0.002, p = 0.001, and p = 0.048 respectively). There was a reduction in the rates of recurrent pyelonephritis and dilating reflux in patients undergoing BUR-CPRE, though it did not reach significance. Female gender was significantly associated with recurrent pyelonephritis regardless of BUR-CPRE status (p = 0.005). There were no reports of distal ureteral obstruction or other complications following BUR-CPRE. The mean post-operative follow up for the BUR-CPRE group was 46.33 (10.26) months vs. 53.76 (26.05) months for CPRE (p = 0.11). DISCUSSION: Recurrent pyelonephritis following bladder closure in patients with BE is a well-documented surgical complication, with centers performing CPRE reporting rates of post-operative pyelonephritis from 22 to 50%. Our series demonstrates similar efficacy of BUR-CPRE compared to other contemporary series and provides additional detail about need for subsequent ureteral surgeries and increased long term follow-up of these complex patients. Limitations of the study include male predominance of the cohort and lack of randomization of BUR-CPRE. CONCLUSIONS: BUR-CPRE decreases postoperative VUR and the need for additional ureteral surgery in select BE patients; it should be considered when technically feasible. While results continue to suggest a trend toward decreased recurrent pyelonephritis and dilating reflux, further longitudinal follow-up in our cohort will be needed.

The Richard Grady Monsplasty: A vertical Z-plasty technique.

Classic bladder exstrophy in the female results in an exstrophic bladder and urethra, an anterior introitus with a bifid clitoris and short labia minora. During closure, the lower abdominal wall is closed and the bifid clitori are brought into close apposition, but are often not completely closed to prevent injury to the clitoral bodies, thus leaving a persistent gap between the clitoral bodies that grows over time. We demonstrate a vertical z plasty closure to provide a 2 layer closure of the mons that decreases tension and improves cosmetic appearance by recreating a clitoral hood that provides a more normal appearance of the external genitalia for girls with bladder exstrophy.

Timing of inguinal hernia following complete primary repair of bladder exstrophy.

INTRODUCTION/BACKGROUND: Bladder exstrophy patients have a high prevalence of inguinal hernia that often become clinically evident following bladder closure. Understanding when the bladder exstrophy patient is under greatest risk of developing an inguinal hernia following bladder closure is important, since incarceration resulting in strangulation of intra-abdominal contents can lead to significant morbidity if not addressed in a timely fashion. Although the incidence and risk factors of inguinal hernia have been reported, the timing of occurrence is not well understood. OBJECTIVE: The primary objective of this study was to assess the timing of inguinal hernia following complete primary repair of bladder exstrophy (CPRE). In addition, we aimed to evaluate possible risk factors associated with inguinal hernia, including sex, age at bladder closure and iliac osteotomy status. STUDY DESIGN: A multi-institutional retrospective review identified patients with bladder exstrophy repaired by CPRE under 6 months of age while excluding those who underwent inguinal hernia repair before or during bladder closure. Timing of inguinal hernia following bladder closure was evaluated using Kaplan-Meier methods. Cox proportional hazards model was used to investigate association of sex, age at bladder closure, and osteotomy on the risk of developing of inguinal hernia while clustering for institution. RESULTS: 91 subjects were included in our analysis with median follow-up time of 6.5 years. 34 of 53 males (64.2%) and 2 of 38 females (5.3%) underwent inguinal hernia repair. The median time to inguinal hernia was 4.7 months following closure. The greatest hazard of inguinal hernia was within the first six months following closure. In multivariate analysis, male sex was strongly associated with inguinal hernia (HR = 19.00, p = 0.0038). Osteotomy and delay in closure were not significantly associated with inguinal hernia. 7 of 36 patients (19.4%) who underwent inguinal hernia repair presented with recurrence on the ipsilateral side. DISCUSSION: Our results suggest that the greatest risk of inguinal hernia is within the first six months following bladder closure. 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. While male bladder exstrophy patients are significantly more susceptible to inguinal hernias following CPRE, osteotomy and delayed bladder closure do not appear to be protective factors for inguinal hernia development following initial bladder closure. CONCLUSIONS: There is a heightened risk of inguinal hernia in the first six months following closure. The rate of recurrence following inguinal hernia repair is significantly elevated compared to the general pediatric population.

Key anatomic findings on fetal ultrasound and MRI in the prenatal diagnosis of bladder and cloacal exstrophy.

INTRODUCTION: Prenatal determination of bladder exstrophy (BE) or cloacal exstrophy (CE), known also as the omphalocele-exstrophy-imperforate anus-spinal anomaly complex (OEIS), is challenging. Distinguishing between BE and CE is important because children with CE have many more challenges initially and during their lifetime. An accurate diagnosis is critical when counselling expectant parents. We hypothesized that there are key imaging features that can distinguish BE from CE, and that there are areas of diagnostic concordance and discordance between fetal ultrasound (fUS) and fetal MRI (fMRI) among these entities. MATERIALS AND METHODS: We queried a single institutional IRB-approved registry of children with BE and CE to identify those with accessible fetal imaging from 2000 to 2018, and formal interpretations were collected. Two pediatric radiologists performed independent retrospective blinded review of the images. Criteria evaluated included: genitalia, kidneys, bowel appearance, presence of anal dimple, location of insertion of umbilical cord into the abdomen relative to the abdominal wall defect, umbilical vessels, bladder protuberance, presence of omphalocele, and spine/neural cord abnormalities. We evaluated concordance between radiologic interpretations and postnatal diagnosis, as well as between specific findings in the two diagnostic modalities. RESULTS: Twenty-one infants born between 2000 and 2018 with BE or CE had fetal imaging for review: 15 had both fUS and fMRI, 2 had fUS alone, and 4 fMRI alone. There was 100% concordance between fUS and fMRI in evaluating kidneys, presence of anal dimple, location of abdominal insertion of umbilical cord relative to the defect, number of umbilical vessels, and spine abnormalities/level of neural cord termination. The following discrepancies were observed: 1) genitalia and bowel appearance, and bladder protuberance in 1/15 (6.7%); 2) presence of an omphalocele in 2/15 (13.3%). Of the initial radiologic interpretations, 4/17 (23.5%) of fUS and 2/19 (10.5%) of fMRI erroneously were interpreted as on the OEIS spectrum when the post-natal diagnosis was BE. Errors in diagnosis were due to a protuberant bladder plate extending beyond the plane of the abdominal wall with bowel loops posteriorly mimicking an omphalocele. In all of these BE cases, the abdominal wall defect was located inferior to the umbilical cord insertion on the abdominal wall. CONCLUSION: An everting bladder plate with bowel loops posterior to the plate in classic BE may be misdiagnosed as CE. Identification of the location of umbilical cord insertion relative to the abdominal wall defect, with fetal US or MRI, results in the correct differentiation between BE and CE.

Evaluation of outcomes following complete primary repair of bladder exstrophy at three individual sites prior to the establishment of a multi-institutional collaborative model.

PURPOSE: The Multi-Institutional Bladder Exstrophy Consortium (MIBEC) was established in 2013 to refine technical aspects of the complete primary repair of bladder exstrophy (CPRE), to decrease complications, and to improve outcomes. In order to place outcomes from the consortium into context of historic outcomes, we evaluated continence and dry intervals in children who were repaired prior to the beginning of the consortium at these institutions. We hypothesized that continence (voiding with dryness) is rarely achieved after primary CPRE and surgery following CPRE improves dryness but may hinder voiding per urethra. MATERIALS AND METHODS: We reviewed prospectively maintained IRB approved databases of children who underwent CPRE for classic bladder exstrophy (BE) between 5/1993 and 1/2013 at 3 institutions. Exclusion criteria included: lack of continence documentation, and lack of follow up subsequent to January 2014. We recorded age at closure, method of bladder emptying, bladder capacity and surgical history. We used a 3 part dryness scale for both patients who void volitionally and those on CIC. Children were considered dry if they could hold urine for over 3 h. An intermediate group was defined as having a dry interval of 2-3 h, with minimal dampness in between voids. If dry intervals were <2 h with frequent leakage, children were considered wet. RESULTS: A total of 54 of 73 (38 M) children met inclusion criteria. 35 of 54 (64.8%) children void per urethra, while 18 (33.3%) perform clean intermittent catheterization (CIC) and 1 underwent a vesicostomy due to incomplete emptying and UTI's. 25/35 (71.4%) of those voiding per urethra underwent CPRE only, while the remaining 10 underwent secondary continence procedures. In total, 26 of 54 (48.1%) are dry, that is either continent or not wet for > 3 h, while only 11/54 (20.4%) are truly continent, i.e., voiding per urethra. Only 9/54 (16.7%) are continent after a single surgery (CPRE). 14/54 children who are dry (25.9%) underwent a bladder neck procedure with or without augmentation and empty with CIC. DISCUSSION AND CONCLUSION: Granular detail about the specifics of emptying, surgical history, and dry intervals is crucial to understand the true outcomes from the repair of BE. Children with BE undergoing CPRE prior to the institution of MIBEC experienced variable results, with only 17% achieving continence while spontaneously voiding per urethra without additional reconstruction. We are now engaged in MIBEC to identify factors that contribute to continence and to attempt to render such findings reproducible.

The implications of fellowship expansion on future pediatric urologist surgical volumes.

INTRODUCTION: Over the past 25 years, Pediatric Urology fellowship programs accredited by the Accreditation Council for Graduate Medical Education (ACGME) have more than doubled. This increase may lead to a significant decrease in the number of operative cases per surgeon and therefore impact the current practice of pediatric urology. OBJECTIVE: The objective in conducting this study is to try and predict the effect of the current number of pediatric urology fellowship training positions on future case volume per surgeon using a mathematical model and to discuss future management of the pediatric urology workforce. DESIGN: The current study employed a mathematical model to predict the effect of the number of fellowship graduates on future "case volume per surgeon". We incorporated population growth rates, to calculate incidence rates of key procedures/conditions and the anticipated retirement rate of the current pool of pediatric urologists to help calculate this. RESULTS: There is a possibility to increase the number of practicing board-certified pediatric urologists in the next 30 years from approximately 325 to 900 (figure). There will be a twofold reduction in case volume per surgeon compared to the present in model 1. In model 2 the decrease in case volumes is less significant. The annual number of fellows needed to obtain a future-to-current ratio equal to 1 is 16 for model 1, and 26 for model 2. DISCUSSION: Our study demonstrates, by using two different models that the current number of pediatric urology fellowship training positions in the United States will ultimately lead to a significant decrease in the case volume per surgeons. Our model has limitations as it relies on multiple assumptions. We are assuming that all fellowship positions would be filled every year and that all fellows would graduate, establish their practices in the United States, and devote 100% of their assumed 30-year professional career to pediatric urology. We also made assumptions of disease occurrence and need for surgical correction. The final assumption we made was that the birth rate would stay static over the next 30 years even though it has been declining for many decades. CONCLUSION: This exercise, even with its inherent limitations, is still sufficient to demonstrate that fellowship expansion warrants thoughtful discussion.

Antibiotic Prophylaxis with Trimethoprim-Sulfamethoxazole versus No Treatment after Mid-to-Distal Hypospadias Repair: A Prospective, Randomized Study.

PURPOSE: To evaluate the impact of prophylactic antibiotics after distal hypospadias repair on postoperative bacteriuria, symptomatic urinary tract infection, and postoperative complications in a prospective, randomized trial. MATERIALS AND METHODS: Consecutive patients aged 6 months to 2 years were enrolled at our institution between June 2013 and May 2017. Consenting patients were randomized to antibiotic prophylaxis with trimethoprim-sulfamethoxazole versus no antibiotic. Patients had catheterized urine samples obtained at surgery and 6-10 days postoperatively. The primary outcome was bacteriuria and pyuria at postoperative urine collection. Secondary outcomes included symptomatic urinary tract infection and postoperative complications. RESULTS: 70 patients consented to the study, of which 35 were randomized to receive antibiotics compared to 32 who did not. Demographics, severity of hypospadias, and type of repair were similar between the groups. Patients in the treatment group had significantly less pyuria (18%) and bacteriuria (11%) present at stent removal compared to the nontreatment group (55% and 63%; p=0.01 and p < 0.001, resp.). No patient had a symptomatic urinary tract infection. There were 11 postoperative complications. CONCLUSIONS: Routine antibiotic prophylaxis appears to significantly decrease bacteriuria and pyuria in the immediate postoperative period; however, no difference was observed in symptomatic urinary tract infection or postoperative complications. Clinical Trial Registration Number NCT02593903.

Clinical pathway for early discharge after complete primary repair of exstrophy and epispadias by using a spica cast.

INTRODUCTION/OBJECTIVE: Secure closure of the pubic diastasis during bladder exstrophy and epispadias repair decreases the abdominal wall tension at the time of reconstruction. Pelvic osteotomies are routinely performed at the time of abdominal wall and bladder reconstruction in order to more easily facilitate pubic symphyseal diastasis approximation. Postoperative pelvic immobilization is performed by methods that include modified Buck's traction, modified Bryant's traction, and spica casting. People undergoing closure often require inpatient hospitalization for 2-8 weeks because of the pelvic immobilization. The present study examined the findings from a clinical pathway for early discharge after complete primary repair of exstrophy (CPRE) and proximal epispadias repair with spica casting. METHODS: The present study is a retrospective review of patients that underwent pelvic osteotomies with spica casting at the time of CPRE or proximal epispadias repair from November 2006 to March 2013. All patients had anterior innominate osteotomies and spica cast pelvic immobilization. RESULTS: Pelvic osteotomies and spica cast pelvic immobilization were performed on 17 children. The median postoperative stay was 6.0 days and the subdivided results are in Table. No children experienced an abdominal or orthopedic complication. A few children required minor cast adjustments to relieve pressure. After cast removal, no skin breakdown, pressure necrosis, or nerve palsy were found. The median length of casting without pinning was 31 (26-48) days. DISCUSSION: The use of spica cast pelvic immobilization after exstrophy and epispadias repair is safe and allows for earlier discharge when compared to other methods of pelvic immobilization. However, although the family appreciates early discharge and additional bonding, the priority is the success of the closure. The present findings demonstrate, and are corroborated by other spica cast publications, that spica casting is as effective as modified Bryant's traction or modified Buck's traction. The success rates for CPRE with spica casting are similar to published staged repairs and have the benefit of allowing for bladder cycling, which potentially enables better bladder growth and development. If success and complication rates are comparable amongst the different pelvic immobilization groups, then variables including hospital length of stay and cost become appropriate comparisons. CONCLUSION: The shortened discharge time, along with a significant decrease in acuity of care leads to significant decreases in hospital costs. Additional hospital stay when using modified Bryant's traction or modified Buck's traction with external fixation will accrue significant hospital costs.

An initial report of a novel multi-institutional bladder exstrophy consortium: a collaboration focused on primary surgery and subsequent care.

PURPOSE: For bladder exstrophy repair it is universally accepted that successful initial surgery is paramount to achieve the optimal outcome. Gaining the necessary surgical experience is challenging due to the rarity of bladder exstrophy. We report preliminary findings of a multi-institutional collaboration created to increase experience and proficiency with the care of bladder exstrophy. MATERIALS AND METHODS: Our 3 institutions alternatively served as the host site for scheduled surgeries with observation, commentary and critique by visitors from the other sites. The technique was complete primary repair with bilateral iliac osteotomy. The timing of complete primary repair at age 1 to 3 months facilitated collaboration. We recorded patient demographics and outcomes, and the impact of this collaboration on our technique and experience. Video recording was used for real-time observation and teaching, and future analysis, editing and review. RESULTS: A total of 16 site visits occurred from February 2013 through May 2014. Complete primary repair was performed in 9 males and 7 females with bladder exstrophy. Median age at complete primary repair was 2 months (range 0.1 to 28.8). Median followup was 8.9 months (range 2.8 to 18.2). All closures were successful with no dehiscence. Complications included urethrocutaneous fistula in 2 patients, 1 episode of pyelonephritis in 3 each and urethral obstruction in 2 females, of whom 1 required clean intermittent catheterization. CONCLUSIONS: We report a multi-institutional collaboration to standardize the surgical management of bladder exstrophy. This effort increased the annual experience of each institution involved from threefold to ninefold and it has accelerated the physician knowledge base to ultimately benefit patient care.

Urinary proteome analysis in patients with stable SFU grade 4 ureteropelvic junction obstruction differs from normal.

OBJECTIVE: To evaluate and analyze the urinary proteome in infants with stable grade 4 ureteropelvic junction obstruction (UPJO) and compare to age-matched normal controls. METHODS: Bladder urine specimens were obtained from 21 healthy infants with normal maternal/fetal ultrasound and 25 infants with grade 4 unilateral UPJO. All patients had >40% ipsilateral individual kidney function by renal scanning and the anteroposterior (AP) diameter of the hydronephrotic kidney ranged from 1.6-3.9 cms at presentation. Over a 5-year follow-up period, the disease progressed in 7 infants (28%), resolved in 4 (16%), and remains stable in the majority (56%). The urinary specimens were prepared using standard methods and subjected to LC/MS/MS analysis. The normalized data were annotated utilizing the Ingenuity Pathways Analysis (IPA; www.Ingenuity.com) knowledge platform. RESULTS: In the stable UPJO group, the urinary proteomes obtained in infancy differed significantly from the age-matched controls. Analysis revealed important differences in a number of biologic functions including inflammation, apoptosis, tubular injury and fibrosis, and reactive oxygen species response. CONCLUSION: The urinary proteomes from the bladder in patients with stable grade 4 UPJO (by imaging criteria) are significantly different at birth and during the first year of life and seem to indicate the presence of an ongoing active renal response to UPJO. The imminent discovery of surrogate urinary biomarkers may result in reconsideration of the watchful waiting strategy during this critical period of renal maturation and development in infancy.

Colonic adenocarcinoma in a 13-year-old with cystic fibrosis.

The increase in longevity among patients with cystic fibrosis has brought to light comorbidities associated with the disease that are less acutely fatal than its pulmonary manifestations. Modern retrospective analysis has demonstrated an increased risk of gastrointestinal malignancy in patients with cystic fibrosis, with a marked elevation in malignancy rates among patients in their 20s and 30s. Here we report a case of colonic adenocarcinoma presenting as pneumaturia in a 13-year-old patient with cystic fibrosis.

Results of a 2-year multicenter trial of endoscopic treatment of vesicoureteral reflux with synthetic calcium hydroxyapatite.

PURPOSE: With no FDA approved material available for endoscopic treatment of vesicoureteral reflux, in 2001 we began a prospective multicenter trial of synthetic calcium hydroxyapatite as a subureteral bulking agent in children with traditional indications for surgical repair. MATERIALS AND METHODS: A total of 98 patients (155 ureters) with grades II to IV reflux were enrolled at 10 sites in the United States to obtain 86 patients with completed protocol end points at 3 months. Of the 86 patients 74 underwent renal and bladder ultrasonography, blood count and serum chemistry analysis, and VCUG at 1 year. A total of 46 patients (47%) completed 2-year study end points, including VCUG. RESULTS: At 1 and 2 years 24 of the 74 patients (32%) were cured. Ureteral cure rates were 46% and 40% at 1 and 2 years, respectively. With 35 patients treated and 85% compliance with the required 2-year VCUG the primary center achieved 2-year cure rates of 66% of patients and 72% of ureters. CONCLUSIONS: Synthetic calcium hydroxyapatite is a safe, durable and effective material for endoscopic treatment of VUR. Increased experience with the injection of synthetic calcium hydroxyapatite yields improved results.

Urinary continence is well preserved after total urogenital mobilization.

PURPOSE: Total urogenital sinus mobilization (TUM) has been advocated as a successful technique in the repair of common urogenital sinus anomalies. To our knowledge the long-term effects on continence, voiding pattern and bowel control have not been published. We present our experience and assessment regarding these issues. MATERIALS AND METHODS: We retrospectively reviewed our experience with TUM. Medical records were reviewed for each patient along with long-term followup by telephone questionnaire and clinical visits. Uroflowmetry was performed in recent patients who were old enough to cooperate in the study. RESULTS: A total of 13 females underwent TUM. Medical records were reviewed for all patients. Patients were divided into 2 groups. Group 1 consisted of 7 patients who underwent surgery before the age of achieving continence, and group 2 consisted of 6 patients who were incontinent before surgery. One patient in group 2 was lost to long-term followup. Two children in group 1 have enuresis, a 27-month-old with day and nighttime enuresis and a 3-year-old with only nocturnal enuresis. All patients in group 2 were continent immediately postoperatively. CONCLUSIONS: TUM preserves urinary continence immediately postoperatively among patients who are continent before surgery. Furthermore, it does not appear to delay the natural development of continence among patients undergoing surgery before the age of toilet training.

University of Wisconsin experience using the Doli S lithotriptor.

OBJECTIVES: To present our initial results using the Dornier Doli S lithotriptor with the 220 electromagnetic shock wave emitter to treat urinary calculi. At present, there is no published report of the efficacy of this instrument in service in the United States. METHODS: We retrospectively reviewed the outcome of shock wave lithotripsy in 270 consecutive patients with solitary renal and/or ureteral stones treated from September 1998 to October 2001 with the Dornier Doli S lithotriptor. Data were collected with respect to stone size, location, and fragmentation. RESULTS: Of the 270 patients treated, 204 had renal stones and 66 had ureteral stones. All patients had solitary stones. The renal stones averaged 9.7 mm in size (range 4 to 26). The ureteral stones averaged 7.8 mm (range 4 to 17). Of the renal stones, 51% were located in the renal pelvis, with 18%, 5%, and 25% located in the upper, middle, and lower poles, respectively. Of the ureteral stones, 68% were located in the proximal ureter, with 14% and 18% in the mid and distal ureter, respectively. In the renal group, 176 (86%) of 204 patients achieved clinical success. Of these patients, 148 were stone free (73%) and 28 had residual fragments less than 4 mm in size (14%). In the ureteral group, 52 (79%) of 66 patients achieved clinical success. Of these patients, 50 were stone free (76%) and 2 (3%) patients had fragments less than 4 mm in size. Thirteen (6%) of 204 patients in the renal group required retreatment. Four (6%) of 62 patients in the ureteral group required retreatment. Of the patients in whom treatment failed and who had stone analysis, 16 (76%) of 21 had stones composed of predominantly calcium oxalate monohydrate. Four patients developed steinstrasse. Two were treated with retrograde stent placement, and the other two with placement of percutaneous nephrostomy. One patient developed a known perinephric hematoma but did not require a blood transfusion. CONCLUSIONS: Shock wave lithotripsy using the Doli S in appropriately selected patients is an effective instrument for treating urinary calculi throughout the urinary tract.