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.

Does Preoperative Testosterone Administration Decrease Complications in Distal Hypospadias Repair With Urethroplasty?

PURPOSE: Testosterone administration prior to hypospadias repair is common practice among pediatric urologists; however, its impact on surgical outcomes remains controversial. We hypothesize that testosterone administration prior to distal hypospadias repair with urethroplasty significantly decreases postoperative complications. MATERIALS AND METHODS: We queried our hypospadias database for primary distal hypospadias repairs with urethroplasty from 2015 to 2021. Patients undergoing repair without urethroplasty were excluded. We collected information on patient age, procedure type, testosterone administration status, initial visit and intraoperative glans width, urethroplasty length, and postoperative complications. To determine the role of testosterone administration on incidence of complications, a logistic regression adjusting for initial visit glans width, urethroplasty length, and age was performed. RESULTS: A total of 368 patients underwent distal hypospadias repair with urethroplasty. One hundred thirty-three patients received testosterone and 235 did not. Initial visit glans width was significantly larger in the no-testosterone vs testosterone group (14.5 mm vs 13.1 mm, P = .001). Testosterone patients had significantly larger glans width at the time of surgery (17.1 mm vs 14.6 mm [no-testosterone group], P = .001). On multivariable logistic regression analysis after controlling for age at surgery, preoperative glans width, testosterone status, and urethroplasty length, testosterone administration did show significant association with reduced odds of postoperative complications (OR 0.4, P = .039). CONCLUSIONS: This retrospective review of patients shows that on multivariable analysis there is significant association between testosterone administration and decreased incidence of complications in patients undergoing distal hypospadias repair with urethroplasty. Future studies on testosterone administration should focus on specific cohorts of patients with hypospadias as benefits of testosterone may be more evident in some subgroups than others.

Robot-Assisted Laparoscopic Ureterocalicostomy in the Setting of Ureteropelvic Junction Obstruction: A Multi-Institutional Cohort.

PURPOSE: Recurrent ureteropelvic junction obstruction (UPJO) after failed pyeloplasty is a complex surgical dilemma. The robot-assisted laparoscopic ureterocalicostomy (RALUC) is a potential surgical approach, but widespread adoption is limited due to the perceived technical challenge of the procedure. We present a multi-institutional pediatric cohort undergoing RALUC for recurrent or complex UPJO, and hypothesize that the procedure is reproducible, safe and efficacious. MATERIALS AND METHODS: A 3-center multi-institutional collaboration was initiated and medical records of children undergoing RALUC between 2012 and 2020 were retrospectively reviewed. The details on baseline demographics, perioperative characteristics and postoperative outcomes were aggregated. RESULTS: During the study period 24 patients, 7 (29%) females and 17 (71%) males, were identified. Of the patients 21 (86%) had a history of previous pyeloplasty prior to RALUC, of whom 5 (24%) had 2 prior failed ipsilateral pyeloplasties. The reason for performing RALUC was short ureter in 3 (13%), intrarenal pelvis in 5 (21%) and extensive scarring at the ureteropelvic junction locus in 16 (67%) patients. The median age of patients at time of surgery was 5.1 years (IQR: 1.9, 14.7). Of the patients 9 (38%) had percutaneous nephrostomy prior to surgery; if percutaneous nephrostomy tubes were placed for relief of obstruction, an antegrade contrast study was done postoperatively to confirm resolution of obstruction. No 30-day Clavien-Dindo Grade III-V complications were noted. During the median followup of 16.1 months (IQR: 6, 47.5), 22 (92%) had improved symptoms and hydronephrosis with no further intervention; 2 (8%) patients underwent endoscopic interventions after RALUC and both ultimately underwent nephrectomy. CONCLUSIONS: This multi-institutional cohort demonstrates that RALUC is a safe and efficacious salvage option for failed pyeloplasty or complex anatomy with an acceptable success profile, especially in cases of extensive scarring at the UPJO or an intrarenal pelvis.

Quantifying Glans Width Changes in Response to Preoperative Androgen Stimulation in Patients Undergoing Hypospadias Repair.

PURPOSE: Testosterone (T) administration prior to hypospadias surgery to increase glans size remains controversial. Understanding T's effect on glans width (GW) is essential to understanding its potential impact on surgical outcomes. We hypothesized that preoperative T in prepubertal boys significantly increases GW at the time of hypospadias surgery. MATERIALS AND METHODS: Our single institutional database was queried to identify patients who underwent hypospadias surgery from 2016 to 2020, in which data for T administration and GW were available. Descriptive, nonparametric and categorical statistics were performed as indicated. RESULTS: A total of 579 patients were eligible for analysis. Median age at surgery was 0.9 years (IQR 0.6-1.6). A total of 247/579 patients (42.7%) received T. The median GW at surgery was 15 mm (IQR 13-17). When comparing patients who had T administered to those who did not, we found a significant difference in GW at surgery (16 mm vs 14 mm, p <0.001). The median change in GW from the office to surgery was 4 mm for those receiving T vs 0 mm for those not receiving T (p <0.001). We identified a greater change in GW from preoperative to intraoperative measurements in patients who received 2 doses of T vs 1 dose (4 mm vs 2 mm, p <0.001). A histogram plot revealed the distribution of GW change at surgery. CONCLUSIONS: In our prospectively collected cohort of patients undergoing hypospadias surgery, we were able to quantitate the change in GW from preoperative T. Two doses of T resulted in a significant increase in GW vs 1 dose.

Total Motile Sperm Count in Adolescent Boys with Varicocele is Associated with Hormone Levels and Total Testicular Volume.

PURPOSE: The risk factors for future infertility in adolescents with varicocele are controversial, and little is known about the association between hormone levels and semen parameters. Semen analysis is likely the closest marker of fertility but may be difficult to obtain in some boys secondary to personal, familial or religious reasons. Identifying other clinical surrogates for abnormal semen parameters may offer an alternative for assessing varicocele severity in these boys. We hypothesized that hormone levels and total testicular volume are predictive of abnormal total motile sperm count. MATERIALS AND METHODS: We retrospectively reviewed Tanner 5 boys with palpable left varicoceles who underwent a semen analysis and had serum hormone levels tested (luteinizing hormone, follicle-stimulating hormone, inhibin B, anti-müllerian hormone and/or total testosterone) within a 6-month period. Total testicular volume was also calculated. Abnormal total motile sperm count was defined as <9 million sperm per ejaculate. RESULTS: A total of 78 boys (median age 17.2 years, IQR 16.5-18.0) were included. Luteinizing hormone, anti-müllerian hormone and total testosterone were not correlated with any semen analysis parameter. There was a negative correlation between follicle-stimulating hormone and total motile sperm count (ρ -0.35, p=0.004) and positive correlation between inhibin B and total motile sperm count (ρ 0.50, p <0.001). Total testicular volume was significantly positively correlated with total motile sperm count (ρ 0.35, p=0.01). ROC analyses revealed an optimal follicle-stimulating hormone cutoff of 2.9, an optimal inhibin B cutoff of 204 and an optimal total testicular volume cutoff of 34.4 cc to predict abnormal total motile sperm count. CONCLUSIONS: Total motile sperm count is inversely associated with follicle-stimulating hormone levels, and directly associated with inhibin B levels and total testicular volume. Optimized cutoffs for serum follicle-stimulating hormone, inhibin B and total testicular volume may prove to be reasonable surrogates for total motile sperm count in boys who defer semen analysis for personal or religious/cultural reasons.

Time to Complication Detection after Primary Pediatric Hypospadias Repair: A Large, Single Center, Retrospective Cohort Analysis.

PURPOSE: Controversy remains within the pediatric urology community regarding adequate duration of followup after hypospadias repair. Some have suggested that minimal long-term followup is necessary due to a low incidence of late complications. The objective of this study was to delineate time to complication detection for primary hypospadias repairs. MATERIALS AND METHODS: We queried our prospectively maintained hypospadias database and identified all patients undergoing primary hypospadias repair from June 2007 to June 2018. Patients were excluded if they had undergone primary repair elsewhere or did not have a followup visit. Complications were defined by the need for an additional unplanned surgical procedure. Kaplan-Meier analysis was performed to assess time to complication by degree of hypospadias. RESULTS: A total of 1,280 patients met inclusion criteria, of whom 976 (68.9%) underwent distal, 64 (4.9%) mid shaft and 240 (18.8%) proximal hypospadias repair. Complication rates were 10.7% (104 patients), 18.8% (12) and 53.8% (129, p<0.0001) for distal, mid shaft and proximal hypospadias repair, respectively. Only 47% of complications were detected within the first year postoperatively. Median time to complication for all repair types was 69.2 months (IQR 23 to 131.9), ie 83.1 months (IQR 42.0 to 131) for patients undergoing distal repair and 29.4 months (IQR 11.9 to 82.1) for patients undergoing proximal repair (p <0.001). CONCLUSIONS: In our large single institution series of pediatric patients undergoing hypospadias repair fewer than half of the complications presented within the first year postoperatively. Long-term followup is recommended for patients undergoing hypospadias repair to adequately detect and address complications.

Robotic surgery in infants and children: an argument for smaller and fewer incisions.

PURPOSE: Robotic-assisted laparoscopic (RAL) surgery has gained momentum in pediatric urology. Technological adaptations such as the development of 5 mm instruments have led to robotic procedures being performed on younger children and those having smaller body habitus, with improved cosmesis. However, concerns have been raised regarding decreased intra-abdominal working space and the absence of monopolar curved scissors (hot endoshears®) when using 5 mm instruments. The aim of this study is to examine the overall experience at a single pediatric urology center using 5 mm instruments with no planned additional assistant ports during common robotic procedures. We hypothesized this approach is safe and feasible for a variety of pediatric urologic reconstructive procedures. METHODS: We retrospectively reviewed all major robotic procedures entered into an IRB approved data registry. The analysis was performed only for procedures in which 5 mm instruments were used exclusively with hook diathermy. Procedures that utilized 8 mm instrumentation were excluded from the study. Data were abstracted according to patient age, weight and robotic surgery performed. Outcomes included post-operative complications (Clavien-Dindo classification), operative time, operative blood loss, need for assistant port placement and conversion rates to open or pure laparoscopic surgery. RESULTS: From 2012 to 2016, 220 consecutive pediatric RAL urological surgical cases were performed on 201 patients. These comprised pyeloplasty (n = 102) 46.4%, ureteral reimplants (n = 84) 38.2% and ipsilateral ureteroureterostomy (n = 34) 15.5%. Median age at surgery was 4 years (3 months to 18 years). There were no conversions to open or laparoscopic surgery. Placement of an additional Assist port was documented in seven cases. Severe (Clavien grade 4) complications occurred in two patients requiring ICU admission: one for sepsis and one ventilator-dependent patient having increased work of breathing post-op. Intra-operative blood loss was minimal ( < 50 ml) in 97% of cases. Patients ≤ 1 year of age comprised 28.6% of the study population. Univariate analysis revealed no association between age and occurrence of complications (p = 0.957) CONCLUSIONS: This study represents one of the largest series of consecutive RAL surgery using 5 mm instruments in pediatric urology. Acceptable complication rates, OR times and blood loss were achieved using this technique. We conclude that the use of 5 mm instruments gives excellent operative outcomes in pediatric reconstructive procedures.

Multi-Institutional Review of Outcomes and Complications of Robot-Assisted Laparoscopic Extravesical Ureteral Reimplantation for Treatment of Primary Vesicoureteral Reflux in Children.

PURPOSE: Robot-assisted laparoscopic extravesical ureteral reimplantation has been proposed as a minimally invasive alternative to open ureteral reimplantation for correcting primary vesicoureteral reflux in children. However, in the current literature there are conflicting data regarding the safety and efficacy of this approach. Amid ongoing debate we analyzed outcomes and complications from this procedure in a large multi-institutional cohort. MATERIALS AND METHODS: We reviewed the records of children who underwent robot-assisted laparoscopic extravesical ureteral reimplantation at 9 academic centers from 2005 to 2014. Radiographic failure was defined as persistent vesicoureteral reflux on postoperative voiding cystourethrogram or radionuclide cystogram. Complications were graded using the Clavien-Dindo scale. RESULTS: A total of 260 patients (363 ureters) underwent robot-assisted laparoscopic extravesical ureteral reimplantation for primary vesicoureteral reflux during the study period. The cohort included 90 patients with either duplex ureter (42), failed endoscopic treatment (40) or concomitant diverticulectomy (8). Of the 280 ureters with postoperative voiding cystourethrogram or radionuclide cystogram available radiographic resolution was seen in 246 (87.9%). There were 25 complications overall (9.6%), with 7 grade 3 complications (2.7%) and no grade 4 or 5 complications. Four patients (3.9%) had transient urinary retention following bilateral reimplantation. CONCLUSIONS: Robot-assisted laparoscopic extravesical ureteral reimplantation has a low complication rate consistent with published series of open ureteral reimplantation. Radiographic success rates are approaching those of the open procedure but continue to fall short of the gold standard approach. These findings necessitate critical thinking regarding potential technique improvements and further prospective investigation into the efficacy of this procedure.

Intermediate-Term Followup of Proximal Hypospadias Repair Reveals High Complication Rate.

PURPOSE: Results following distal hypospadias repair are favorable. Grouping proximal and distal hypospadias repair artificially increases the perceived success rate of proximal hypospadias. We identified our complication rate of proximal hypospadias repair and hypothesized a higher complication rate for 1-stage repair. MATERIALS AND METHODS: We retrospectively reviewed the records of consecutive boys who underwent proximal hypospadias from 2007 to 2014. Proximal hypospadias was defined as a urethral meatus location at or more proximal than the penoscrotal junction after penile degloving. We further stratified boys into those with planned 1-stage vs 2-stage repair. Univariate and Cox regression analyses were performed to assess associations with covariates and compare time to the first complication, respectively. RESULTS: A total of 167 boys met study inclusion criteria. Median followup was 31.7 months for 1-stage repair in 86 patients and staged repair in 81. The overall complication rate was 56%. Complications developed in 53 of 86 1-stage (62%) vs 40 of 81 staged (49%) repairs (p = 0.11). The number of unplanned procedures per patient was higher in the 1-stage than in the staged group (0.99 vs 0.69, p = 0.06), as was the number of patients who had at least 2 complications (29 of 86 or 33% vs 13 of 81 or 16%, p = 0.03). Cox regression showed no difference in time to the first complication for staged compared to 1-stage repair (HR 0.77, 95% CI 0.43-1.39). CONCLUSIONS: Our 56% complication rate of proximal hypospadias warrants further long-term patient followup. More patients in the 1-stage group experienced at least 2 complications. However, when complications developed, they developed no differently in the 2 groups.

The missed crossing vessel during open pyeloplasty: a potential advantage of the robot-assisted approach in children.

OBJECTIVE: To investigate whether the panoramic view offered by robot-assisted laparoscopic pyeloplasty (RALP) reduces the likelihood of missing a crossing vessel compared to open pyeloplasty in cases where initial pyeloplasty fails. METHODS: A single institution redo-pyeloplasty database was reviewed for children treated between January 2012 to July 2023. Clinical history, imaging and operative details were reviewed to identify the etiology for the redo procedure. RESULTS: Cohort consisted of 45 patients undergoing a redo RALP during the study period. 29 of 45 patients had an initial open surgical approach, whereas 16 had an initial RALP. 10 patients were noted to have a missed crossing vessel on redo pyeloplasty - 9 had an initial open approach whereas 1 had an initial RALP (p<0.0001). CONCLUSIONS: RALP may reduce the risk of missing a crossing vessel due to the panoramic view of the surgical field intrinsic to an intraperitoneal RALP approach.

Prenatal Presentation of a Covered Cloacal Exstrophy Variant; Early Diagnostic Challenges Within the Broad Spectrum of the Exstrophy-Epispadias Complex.

There is a broad range of variant phenotypes that can occur within the bladder exstrophy and epispadias complex spectrum. Accurate prenatal detection helps prepare families and to coordinate subspecialty resources. Here, we present the case of a patient with prenatally diagnosed patient with covered cloacal exstrophy variant along with four additional cases illustrating the nonlinear spectrum from isolated epispadias to cloacal exstrophy. Given the rarity of these variants overall and of each subtype within the spectrum, there is a need for long-term multi-institutional outcomes data to improve detection, characterization, and prognostication for these patients.

Unmasking Ureteral Polyps: Insights From a Case Series.

Fibroepithelial polyps in the urinary tract are a rare cause of obstructive uropathy with fewer than 130 cases reported in the literature. In our series, we describe polyps that were missed on preoperative imaging and later found in the operating room during pyeloplasty. It is critical for urologists to be aware of polyps as a potential source of obstruction as they can increase the complexity of a reconstruction and, if missed, may result in a failed repair and persistent obstruction. We hypothesize that performing a retrograde pyelogram prior to ureteric reconstruction will facilitate diagnosis prior to surgical repair.

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.