A Single Center's Changing Trends in the Management and Outcomes of Primary Closure of Classic Bladder Exstrophy: An Evolving Landscape.

OBJECTIVE: To investigate management trends in a single institution with a large referral population in classic bladder exstrophy (CBE) over the past twenty years. METHODS: An institutional database of 1415 exstrophy-epispadias complex patients was retrospectively reviewed for CBE patients with primary closure between 2000 and 2019. Osteotomy, location of closure, age of closure, and outcome of closures were reviewed. RESULTS: A total of 278 primary closures were identified, with 100 occurring at author's hospital (AH) and 178 at outside hospitals (OSH). Osteotomies were performed in 54% of cases at AH and 52.8% of cases at OSH. Osteotomy use increased over 20 years from 48.6% in 00's to 62.1% in 10's (P = .046). The total success rate at AH was 96% and 62.9% at OSH. The median age at primary closure at AH increased from 5 days (00's) to 20 days (10's), compared to the OSH which increased from 2 days (00's) to 3 days (10's). CONCLUSION: Closure of CBE may be delayed for several reasons including insurance difficulties, transfer to another hospital, desire for second opinions, or surgeon preference. Delaying primary closure of bladder exstrophy gives families time to adjust lifestyle, arrange travel, and seek care at centers of excellence.

Complications of delayed and newborn primary closures of classic bladder exstrophy: Is there a difference?

INTRODUCTION: The authors aim to compare single institutional 30-day complication rates between delayed and neonatal closure of classic bladder exstrophy (CBE). MATERIALS AND METHODS: An institutional database of 1415 exstrophy-epispadias patients was reviewed retrospectively for CBE patients who underwent primary closures at the authors' institution between 1990 and 2020. Patients were identified as having received either neonatal or delayed (at age >28 days) closures. All 30-day complications were recorded, including wound infection and dehiscence, genitourinary and non-genitourinary infections, bowel obstruction, blood transfusions, and others. Descriptive statistics were performed to summarize patient level data. Categorical variables were reported by count and percentages and were compared using Exact Cochran-Armitage trend analysis by decade, or with Fisher's Exact Test and Chi-square test when directly comparing categories and outcomes. Continuous variables were analyzed via Mann Whitney U and one-way ANOVA as appropriate. RESULTS: The cohort included 145 patients: 50 delayed and 95 neonatal closures. The total complication rate was 58% in delayed closures compared to 48.4% for neonatal closures (p = 0.298), with the majority being Clavien-Dindo grade I or II. Excluding blood transfusion, complication rates fell to 26% and 34.7% in delayed and neonatal closures, respectively (p = 0.349). The most common single complication was unplanned post-operative blood transfusion (38% delayed; 26.3% neonatal; p = 0.34), followed by pyelonephritis (2% delayed; 8.4% neonatal), and urinary fistula (6% delayed; 1.1% neonatal). Grade III Clavien-Dindo complications occurred in 2% delayed and 7.4% neonatal groups (n = 1; n = 7 respectively; p = 0.263). A single delayed patient had grade IV complications compared to three neonatal patients (p = 0.66). CONCLUSIONS: Delayed primary closure has become a frequently performed alternative in the modern treatment of bladder exstrophy for patients who do not undergo newborn closure because of prohibitive circumstances or surgeon's discretion. The majority of the complications associated with delayed closure are a low Clavien-Dindo grade and easily managed during the postoperative inpatient hospital stay. Families should be counseled about the possibility of minor, conservatively managed complications and likelihood of a blood transfusion with osteotomy.

Sexual health outcomes after penile reconstruction in the exstrophy-epispadias complex.

INTRODUCTION: The penis in exstrophy-epispadias complex (EEC) males is diminutive and patients are often dissatisfied with appearance and length. Due to an increased risk of psychosexual dysfunction, patients desire penile reconstruction. Surgical options include penile lengthening with a full thickness skin graft (SG) or tissue expansion (TE) used for cutaneous coverage or neophalloplasty using a radial forearm free flap or pedicled antero-lateral thigh flap. OBJECTIVE: The aim of this study was to assess sexual health outcomes before and after SG or TE assisted lengthening and neophalloplasty. STUDY DESIGN: Patients aged 18 years or older, who underwent penile reconstruction were identified using an institutionally approved database of EEC patients. Patient perception of penile appearance, sexual function, their frequency of sexual intercourse and overall satisfaction with reconstruction were assessed using a survey consisting of validated and non-validated questions. RESULTS: Eighty-three patients underwent penile reconstruction, 57 met the inclusion criteria and 28 responded to the survey (49.1%). Fourteen were reconstructed using a SG, 6 with TE and 8 underwent neophalloplasty. Median time from reconstruction to survey completion was 4.4 years (range 1.2-13.2) Four patients had a diagnosis of epispadias, 1 cloacal exstrophy, 23 classic bladder exstrophy (CBE). Before reconstruction patients were dissatisfied with their penile appearance with a median penile perception score of 4.5 compared to 7.5 after surgery (p = 0.0034, Fig. 1). Twenty-three patients were dissatisfied with penile length, with 18 reporting an improvement following reconstruction (p = 0.0002). There was no correlation in time after reconstruction with PPS or satisfaction with penile length (p = 0.86 and p = 0.55, respectively). Overall, the median Sexual Health Inventory for Men (SHIM) score was 19, with no difference between the surgical groups (p = 0.33). Nine patients engaged in sexual intercourse before reconstruction which increased to 17 afterwards. CONCLUSION: All three surgical methods improved patient perception of penile appearance and length. Patients who previously never engaged in intercourse were able to and those who had were doing so more frequently, of which a greater proportion were in a relationship. The results are encouraging though must be interpreted with caution due to the small number of responders and possible risk of bias. These findings provide vital information for future patients and routine psychological assessment will help manage patient expectations to improve satisfaction.

Osteotomy in the newborn classic bladder exstrophy patient: A comparative study.

INTRODUCTION: Pelvic osteotomy is indicated in classic bladder exstrophy (CBE) patients with a wide pubic diastasis or non-malleable pelvis. While the safety of pelvic osteotomy in delayed and failed closures is established, there remains less clarity on their safety in newborns. The authors herein sought to present their experience with CBE patients who underwent pelvic osteotomy for assistance with bladder closure during both the newborn and delayed time periods. OBJECTIVE: The authors hypothesize that pelvic osteotomy during exstrophy closure may be performed safely in newborns with few perioperative or post-operative negative sequelae. STUDY DESIGN: A prospectively maintained IRB-approved database was reviewed for CBE patients who underwent osteotomy during primary closure. Patient demographics, performing institution (authors' or outside), closure outcome, diastasis width, and post-operative complications were noted. Patient subgroups included newborn and delayed (>28 days of life) closures. Failure was defined as bladder dehiscence, prolapse, outlet obstruction, or vesicocutaneous fistula requiring reoperation. Orthopedic complications included nerve palsies, superficial pin-site infection, and bladder neck erosion by orthopedic hardware. Analyses were performed using a Chi-square test. RESULTS: 286 patients were included: 186 newborn and 100 delayed closures. The authors' institution performed 109 cases (44 newborn and 65 delayed). Within the overall newborn closure cohort, no significant differences were found in outcomes among the osteotomy types with success rates of 80%, 60.8%, and 71.4% in the combined, posterior iliac, and anterior innominate groups, respectively (p = 0.24). In the delayed group, success rates were significantly different with rates of 100%, 72.4%, and 93.8% in the combined, posterior iliac, and anterior innominate groups, respectively (p < 0.001). Febrile urinary tract infection (UTI) was the most common complication at 8% (23/286). Only 1.7% (5/286) of patients had orthopedic complications with 3 patients in the newborn cohort, 2 patients in the delayed cohort, and only one patient requiring reoperation. DISCUSSION: Orthopedic complications are rare in CBE patients who undergo osteotomies regardless of the closure period. No clinically significant difference in orthopedic complication rate was found between newborn and delayed closure periods. CONCLUSIONS: While current trends have moved toward delayed primary closures, there remains a role for osteotomy during exstrophy closure in select newborn patients and can be performed safely with few complications.

Medical student misconceptions in cardiovascular physiology.

Students find cardiovascular physiology challenging. Misunderstandings can be due to the nature of the subject, the way it is taught, and prior knowledge, which impede learning of new concepts. Some misunderstood concepts can be corrected with teaching (i.e., preconceptions), whereas others are resistant to instruction (i.e., misconceptions). A set of questions, specifically created by a panel of physiology experts to probe difficult cardiovascular concepts, was used to identify preconceptions, misconceptions, and the effect of education level on question performance. The introductory cardiovascular lecture used in this study was created based on these questions. In-class polling of medical students' (n = 736) performance was performed using the Turning-Point clicker response system during lecture instruction. Results were compared with published data from undergraduates (n = 1,076) who completed the same questions but without prior instruction. To our knowledge, there have been no studies directly comparing performance using the same instrument and large numbers of undergraduate and medical students. A higher education level was associated with increased performance (preconceptions), whereas several concepts resistant to instruction (misconceptions) were identified. Findings suggest that prior knowledge interfered with the acquisition of medical knowledge. Based on these results, potential causes for these misconceptions and remedial teaching suggestions are discussed.

A New Paradigm for Outpatient Diagnosis and Treatment of Lower Urinary Tract Symptoms Utilizing a Mobile App/Software Platform and Remote and In-Office Visits: A Feasibility Study.

INTRODUCTION: This pilot/feasibility study evaluated a new diagnostic and treatment paradigm for patients with lower urinary tract symptoms using a software platform comprised of a mobile app, validated patient reported outcome questionnaires, bladder diaries and remote patient monitoring. METHODS: New urology referrals for lower urinary tract symptoms completed validated lower urinary tract symptoms questionnaires and 24-hour bladder diaries on a mobile app. Patients were triaged based on symptom severity into an urgent or routine office visit or a remote visit. Outcome metrics assessed include acceptability, practicality, implementation and integration. RESULTS: Of 500 urology consults there were 226 (45%) patients with new lower urinary tract symptoms referrals of whom 201 (89%) had email addresses. In all there were 182 men and 19 women, aged 18 to 87 years (mean 59, SD 17), of whom 71/201 (35%) registered, 57/71 (80%) completed the app and 41/57 (72%) elected remote visits. Mean time from urology referral to initiation of a remote or in-office diagnostic and treatment plan was 7.4 days (SD 5.9) and 7.7 days (SD 7.6), respectively. CONCLUSIONS: Of patients who completed the app 72% were triaged to a remote visit. Time from urology referral to initiation of remote or in-office diagnostic and treatment plan averaged 1 week. This paradigm for management of lower urinary tract symptoms has potential to optimize the clinical workflow, improve health care outcomes and reduce costs.

New Insights on the Basic Science of Bladder Exstrophy-epispadias Complex.

The exstrophy-epispadias complex is a rare congenital anomaly presenting as a wide spectrum of disorders. The complex nature of this malformation leads to continuous investigations of the basic science concepts behind it. Elucidating these concepts allows one to fully understand the mechanisms behind the disease in order to improve diagnosis, management, and treatment ultimately leading to improvement in patient quality of life. Multiple technological advancements within the last 10 years have been made allowing for new studies to be conducted. Herein, the authors conduct a literature review of studies from 2009 to 2019, considering novel theories regarding the genetics, embryology, bladder, bony pelvis, prostate, and genitalia of patients with bladder exstrophy-epispadias complex.

Diagnostic utility of axial imaging in the evaluation of hematuria: A systematic review and critical appraisal of the literature.

INTRODUCTION: Increasing severity of hematuria is instinctively associated with higher likelihood of urological malignancy. However, the robustness of the evidentiary base for this assertion is unclear, particularly as it relates to the likelihood of upper urinary tract pathology. Thus, the value of axial imaging in the diagnostic workup of hematuria is unclear due to differences in the underlying patient populations, raising concern for sampling bias. We performed a systematic review to characterize the literature and association between severity of hematuria and likelihood of upper urinary tract cancer based on axial imaging. METHODS: MEDLINE, EMBASE, and Cochrane were systematically searched for all studies reporting on adult patients presenting with hematuria. We used Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) for reporting of this systematic review and meta-analysis and the Newcastle-Ottawa Scale for risk of bias assessment. Degree of hematuria was classified as "microscopic," "gross," or "unspecified." Three urological malignancies (bladder, upper tract urothelial, and renal cancer) were considered both individually and in aggregate. Random-effects model with pairwise comparisons was employed to arrive at the axial imaging diagnostic yields. RESULTS: Twenty-nine studies were included, of which six (20.7%) reported on patients with gross hematuria only, four (13.8%) reported on patients with microscopic hematuria only, seven (24.1%) included both, and 12 (41.4%) did not define or specify the severity of hematuria. Of 29 studies, two (6.9%) were at high-risk of bias, 21 (72.4%) at intermediate-risk, and six (20.7%) at low-risk of bias using the Newcastle-Ottawa criteria. Based on axial imaging, rates of diagnoses of renal, upper tract urothelial, and bladder cancers differed with differing severity of hematuria. Notably, rates of renal and upper tract urothelial carcinoma were higher in studies of patients with unspecified hematuria severity (3.6% and 10.4%, respectively) than among patients with gross hematuria (1.5% and 1.3%, respectively). When all urological malignancies were pooled, patients with unspecified hematuria were diagnosed more frequently (19.5%) compared to those with gross (15.3%) and microscopic hematuria (4.5%, difference=1.51%, 99% confidence interval 3.6-26.5%). CONCLUSIONS: Lack of granularity in the available literature, particularly with regards to patients with unspecified hematuria severity, limits the generalizability of these results and highlights the need for future studies that provide sufficient baseline information, allowing for firmer conclusions to be drawn.

Pedicled Anterolateral Thigh and Radial Forearm Free Flap Phalloplasty for Penile Reconstruction in Patients With Bladder Exstrophy.

PURPOSE: Male patients with exstrophy-epispadias complex have a congenitally short penis. Adolescent and adult patients often desire penile reconstruction to enable penetrative intercourse. When penile lengthening using local tissue and/or skin graft is unsuitable, phalloplasty is performed. We provide updated experiences using radial forearm free flap and pedicled anterolateral thigh flap phalloplasty techniques over a 6-year period in exstrophy-epispadias complex patients. MATERIALS AND METHODS: Consecutive patients who underwent radial forearm free flap or pedicled anterolateral thigh flap phalloplasty between 2013 and 2019 were reviewed. A total of 11 patients were identified, including 8 with classic bladder exstrophy and 3 with cloacal exstrophy. In each case, flap dimensions, donor and recipient vessels and nerves, complications, outcomes and subsequent surgical procedures are reported. RESULTS: A radial forearm free flap was performed in 6 patients and a pedicled anterolateral thigh flap in 5. Flap survival was observed in all cases and 5 patients subsequently received an inflatable penile prosthesis. Among patients undergoing a pedicled anterolateral thigh flap, there was 1 mortality from acute pulmonary embolism, and the other 4 required at least 1 debulking procedure. CONCLUSIONS: Phalloplasty is an important procedure in patients with exstrophy-epispadias complex with severe penile inadequacy. Both techniques have favorable long-term outcomes, although the pedicled anterolateral thigh flap frequently requires a later debulking procedure. The radial forearm free flap is preferred but if the radial forearm is overly thin and the risk of inflatable penile prosthesis extrusion is high, the pedicled anterolateral thigh flap is an acceptable alternative.

Delaying primary closure of classic bladder exstrophy: When is it too late?

INTRODUCTION: With current trends towards delaying the closure of classic bladder exstrophy (CBE), bladder growth rate or ultimate capacity may be impacted. OBJECTIVE: To examine consecutive bladder capacities in CBE patients who had primary closures at differing ages and determine whether there is an optimal age for closure, with reference to bladder capacity. STUDY DESIGN: A retrospective review was performed using an institutional database. INCLUSION CRITERIA: CBE, successful neonatal (i.e. ≤28 days old) or delayed (i.e. >28 days old) primary closure, at least three consecutive bladder capacities or two measures taken 18 months apart, and first bladder capacity measured ≥3 months after closure. Only capacities prior to continence surgery and before 14 years of age were considered. Two cohorts were created: neonatal and delayed closure. To account for repeated measurements per patient, a linear mixed model evaluated effects of age and length of delay on bladder capacity based on closure cohort. Individuals in the delayed closure group were further stratified into quartiles to assess for detriment to the bladder based on length of delay. RESULTS: The cohort included 128 neonatal and 38 delayed patients. Median age at closure for the delayed group was 193 days (IQR 128-299). Based on univariate analysis, for the first three capacity measurements, the delayed group had significantly lower capacities despite having a similar median age when the measurements were taken. Linear mixed effects model showed significantly decreased total bladder capacity in delayed closure compared to neonates. The 2nd and 4th quartile groups had the most significant decreases in capacity. DISCUSSION: Time points for the most significant decline appear after the 2nd and 4th quartiles, representing 4-6 months and beyond 9 months, respectively. From this, the authors theorize that the appropriate time to close an exstrophy patient is as early as possible (1st quartile), or, if a delay is needed for growth of a bladder template, then between 6 and 9 months (3rd quartile). There may be a detriment to growth rate, however, statistical power may be lacking to discern this. Study limitations include the single-centered, retrospective design. However, results described here fill an important deficit in the knowledge of managing CBE. CONCLUSIONS: All patients in the delayed bladder closure group demonstrated a decline in bladder capacity compared to the control neonatal closure group, with significant differences in the 2nd and 4th quartiles. Thus, closing the bladder prior to nine months of age is recommended.

Volumetric and acetabular changes in the bony pelvis associated with primary closure of classic bladder exstrophy.

INTRODUCTION: Repair of classic bladder exstrophy (CBE) is known to alter dimensions of the bony pelvic ring. Pelvic volume and acetabular configuration are additional metrics which merit analysis in the reconstruction process. Advances in magnetic resonance imaging (MRI) allow for precise elucidation of such anatomy in pediatric patients, providing enhanced knowledge of how primary reconstruction may impact factors in pelvic health. METHODS: An IRB-approved exstrophy-epispadias database of 1337 patients was reviewed for patients with CBE who had pelvic MRI performed before and after repair. Pelvic MRIs were analyzed by a pediatric radiologist, and three-dimensional volumetric renderings of the true pelvis were calculated. Pre- and post-closure imaging were compared, in addition to imaging from age-matched controls without pelvic pathology. Cartilaginous acetabular index and version angles were also calculated and compared between groups. RESULTS: Eighteen patients with post-closure imaging, 14 of whom also had pre-closure imaging, and 23 control patients (ages 0-365 days) were included. The median ages at pre- and post-closure scan were 2 and 178 days, respectively. Osteotomy was performed in 6 (33%) of the closures. The median segmented pelvic volumes were 89 cm3 in the pre-closure group, 105 cm3 in the post-closure group, and 72 cm3 in the control group. At a given age, patients with CBE pre-closure had the largest pelvic volume, and those without bladder exstrophy demonstrated the smallest pelvic volumes (Summary Figure). CBE patients' pelvic volumes were overall lower following repair, relative to age (p = 0.007). Cartilaginous acetabular version angle increased following closure, with acetabular orientation converting from retroversion to anteversion in all cases. However, only acetabular version angles were significantly different between groups. DISCUSSION: This study found that pelvic volume significantly decreased relative to age following primary repair of CBE, but that it did not correct to control levels. Similarly, acetabular retroversion that is naturally seen in exstrophy patients was converted to anteversion post-closure but did not become completely normal. Knowledge of these persistent anatomical anomalies may be useful in treatment of future concerns related to exstrophy, such as continence achievement, pelvic organ prolapse, and potential gait disturbances. Lack of significant differences between study groups regarding acetabular index angles can give assurance to providers that hip dysplasia is neither a natural concern nor iatrogenically inflicted through reconstruction. CONCLUSIONS: The primary closure of exstrophy results in pelvic volumes and anteverted acetabula that more closely resemble those without bladder exstrophy, compared to pre-closure findings.

Outcomes of inflatable penile prosthesis insertion using a neotunica allograft in neophalluses of patients on the bladder exstrophy-epispadias complex spectrum.

BACKGROUND: Men born on the bladder exstrophy-epispadias complex (BEEC) spectrum may undergo neophallus reconstruction with a variety of free flaps, most commonly radial forearm. In order to achieve erectile function, a penile prosthesis may be inserted following neophallus construction. OBJECTIVE: In this study, we sought to describe the perioperative and clinical outcomes of inflatable penile prosthesis (IPP) insertion into neophalluses of patients along the BEEC spectrum. STUDY DESIGN: Using a prospectively maintained institutional BEEC database, patients who had undergone both neophallus creation and IPP placement were identified for this study. Surgery was performed by two surgeons with expertise in IPP implantation. Operative details and postoperative outcomes and complications were collected with review of the medical record. RESULTS: Overall, there were a total of 13 men who underwent neophallus reconstruction with subsequent IPP placement. Of these men, 76.9% (10/13) had experienced successful primary closure in childhood. One patient had corporal loss secondary to complete primary repair of exstrophy (CPRE) following a failed primary attempt. The average age at IPP placement was 23.2 years (SD ± 5.06). Mean OR time (n = 11) was 244.8 min (SD ± 46.2). 76.9% (10/13) of the cases used AlloDerm™ as a neotunica albuginea for support of the IPP. Three patients (23.1%) experienced complications all of which required revision surgery. Two of these complications were erosions in patients for whom AlloDerm™ was not used in the initial IPP placement. DISCUSSION: The results of this study suggest that IPP placement in neophalluses of males on the BEEC spectrum have low perioperative and postoperative morbidity with successful outcomes. While the rate of complications is notably higher in this group compared to the native penis IPP literature, the use of AlloDerm™ or other allograft materials may help to narrow this gap. CONCLUSIONS: IPP placement in neophalluses of men on the BEEC spectrum is successful in the majority of cases. Complications may be reduced with the use of AlloDerm™ in initial placement.

Continence after BNR in the complete repair of bladder exstrophy (CPRE): A single institution expanded experience.

INTRODUCTION: Several surgical methods have been used for primary repair of bladder exstrophy in the newborn. Complete primary repair of exstrophy (CPRE) aims to prevent the need for surgeries beyond the newborn period. Due to the rarity of bladder exstrophy, it has proven difficult in the past to analyze whether use of this method of closure truly does confer acceptable continence outcomes and hence minimizes the requirement for additional surgeries later in life. OBJECTIVE: To describe the continence outcomes of CPRE patients who went on to receive bladder neck reconstruction (BNR), and secondarily, to compare clinical features between those patients who were able to receive undergo a BNR compared to those who were not. STUDY DESIGN: An IRB approved database of 1330 exstrophy-epispadias patients was used to identify referred patients after successful CPRE for management of continued urinary incontinence. Urinary continence outcomes were assessed in those who underwent modified Young Dees Leadbetter BNR following CPRE. RESULTS: Sixty-one patients were referred for treatment after successful CPRE between 1996 and 2016. None developed continence or a dry interval after primary closure. Of these, forty-two (68.9%) underwent BNR by a single surgeon at a mean age of 5.8 years (range 5-8.4). The mean bladder capacity at BNR was 147 mL (range 102-210 mL). Twenty-five (59.5%) achieved day and night continence, 7 (16.7%) gained daytime continence with nocturnal leakage, and 10 (23.8%) remain totally incontinent. Mean follow-up after BNR was 5.9 years. Combined CPRE and pelvic osteotomy were performed in 100% of patients who were continent and 75% of those who were daytime dry. No continent patient had a ureteral reimplantation before BNR, whereas 4 patients with daytime continence and nocturnal leakage and 7 patients who remained continuously incontinent did. DISCUSSION: This is the largest known series of BNRs in exstrophy patients closed by CPRE. Previous smaller studies have demonstrated mild to moderate success rates of BNR after CPRE, with many patients still requiring additional continence surgeries. The present study found similar results, with additional indication that successful primary closure and use of pelvic osteotomies may correlate with enhanced continence. This study includes outcomes from a single surgeon, with a maximum length of follow up of 13 years. CONCLUSIONS: CPRE alone often does not render patients continent of urine, based on the authors' referral population. However, following BNR continence rates in this subgroup were found to reach 76%. Surgeons who treat this population should keep these factors in mind when planning continence surgeries.

Delaying reclosure of bladder exstrophy leads to gradual decline in bladder capacity.

INTRODUCTION: After unsuccessful repair of bladder exstrophy, when to repeat surgical intervention is unclear. One must balance time required for tissue healing with the damaging effects of an exposed urothelium to the environment. OBJECTIVE: The authors aim to study whether a relationship exists between bladder growth/capacity and time till eventual successful closure. STUDY DESIGN: An institutional database of exstrophy-epispadias complex patients was queried for failed exstrophy closure with successful repeat reconstruction, at least three consecutive bladder capacity measurements, and measurements obtained at least three months following successful closure. Patients closed successfully in the neonatal period were used as a comparative group. Linear mixed effects models were used to study the effect of time and age on bladder capacity. RESULTS: Forty-seven patients requiring reclosure and 117 who had successful neonatal closures were included. Two models were created. The first linear mixed effects model found that for a given age, the bladder capacity declined approximately 9.6 mL per year (p = 0.016). The second model found that when time to successful closure was grouped by quartiles, compared to neonates, those in the fourth quartile had significantly decreased bladder capacity of 28.8 cc (p = 0.042). An interaction model comparing neonates and those requiring reclosure did not demonstrate a significant change in bladder growth rate (p = 0.098). A model stratified by quartiles similarly did not find any significant impact to bladder growth rate. DISCUSSION: From the general linear mixed effects models, the authors conclude when compared to neonates, (1) there was an approximate 9.6 cc loss of total bladder capacity per year taken until successful closure, and that (2) those who were delayed the longest had the most significant difference in bladder capacity. This study required stricter inclusion criteria compared to previous publications, and therefore the conclusions that can be drawn regarding bladder growth rates may be more reliable. Future studies will examine the effects of delayed closure on the bladder at the cellular level. CONCLUSIONS: There is a demonstrable significant impact on overall bladder capacity with increasing delay to successful reclosure. One should be cautious when prolonging reconstruction of the bladder as these data demonstrate a time dependent decline in overall capacity.

Practice patterns in classic bladder exstrophy: A global perspective.

INTRODUCTION: While evaluation and management options for classic bladder exstrophy (CBE) patients are numerous and varied, little is known regarding the relative utilization of these different methods throughout the world. A large group of exstrophy surgeons practicing globally was surveyed, seeking to document their methods of care. METHODS: A list of international exstrophy surgeons' email addresses was compiled using professional contacts and referral networking. An online survey was sent to each email address. Surgeons who had not performed a CBE closure within the previous 5 years were excluded. Survey questions queried the respondents' surgical practice type, years since training, and their preferred methods of preoperative evaluation, operative management, and postoperative management. Survey invitations were sent out starting in December 2014 and responses were collected for approximately 6 months. RESULTS: A total of 1152 valid email addresses were invited, resulting in 293 respondents (25%) from 39 countries and every American Urological Association (AUA) section. Seventy-six were excluded, leaving 217 respondents (Table). Respondents reported a median of 17 years since finishing their surgical training (IQR 8-25 years). Practice types included pediatric urology (n = 209), general urology (n = 9), pediatric surgery (n = 59), and other practice makeup (n = 3). On subgroup analyses, there were no significant regional practice differences, with the exception of complete primary repair of exstrophy (CPRE) and oral opioid prescribing being significantly higher in North America compared to other regions. DISCUSSION: Findings indicate that there may be diversity in CBE practice patterns globally. While most responding surgeons from regions outside of North America indicated modern staged repair of exstrophy (MSRE) as their preferred closure technique, a relatively equal distribution of respondents from North America selected CPRE and MSRE. A majority of North American surgeons chose performing osteotomies for both newborn and delayed closures, while an appreciable number of respondents from other regions selected never using osteotomies in their closures. Limitations to this study include a low survey response rate, particularly from surgeons outside of the United States, which may have significantly impacted the ability to draw meaningful global comparisons. CONCLUSIONS: Global variation among practices of surgeons performing CBE closures may exist. The wide range of methods demonstrated by this survey suggests the need for more conclusive comparative studies to elucidate whether an optimal standard exists. Local social factors, access to surgical expertise and transportation to referral centers, and finances play a role in what constitutes the best operative approach.

Urinary Continence Outcomes in Classic Bladder Exstrophy: A Long-Term Perspective.

PURPOSE: We investigated surgical approaches to urinary incontinence and long-term continence outcomes after successful bladder reconstruction in a heterogeneous patient population with classic bladder exstrophy. We hypothesized that while most patients will achieve urinary continence after surgery, only a select group will void volitionally per urethra. MATERIALS AND METHODS: An institutional database of 1,323 patients with exstrophy-epispadias complex was reviewed for patients with classic bladder exstrophy who underwent successful bladder closure and a subsequent continence procedure between 1975 and 2017. Procedures included bladder neck reconstruction, bladder neck reconstruction with augmentation cystoplasty or continent catheterizable stoma, and bladder neck closure with continent catheterizable stoma. Cloacal exstrophy, epispadias and variant exstrophy cases were excluded from analysis. Continence at last followup was defined as a dry interval of 3 or more hours without nighttime leakage. Those patients with more than 3 months of followup were assessed. RESULTS: Overall 432 patients underwent successful bladder closure (primary 71.5%, repeat 28.5%) and a urinary continence procedure. At last followup 162 (37%) underwent bladder neck reconstruction, 76 (18%) underwent bladder neck reconstruction with augmentation cystoplasty or continent catheterizable stoma, 173 (40%) underwent bladder neck closure with continent catheterizable stoma and 18 underwent other procedures. Median followup from the first continence procedure was 7.2 years (IQR 2.3-13.7). Continence was assessed in 350 patients. After isolated bladder neck reconstruction 91 of 142 patients were continent (64%, 95% CI 56-72). After bladder neck closure with continent catheterizable stoma 124 of 133 patients evaluated were continent (93%, 95% CI 87-97). CONCLUSIONS: Most patients with classic bladder exstrophy require multiple reconstructive procedures to achieve continence. Only about 25% of patients are expected to void normally per urethra without reliance on catheterization or urinary diversion.

Adolescent Presentation of Posterior Urethral Valves.

Posterior urethral valves (PUVs) present clinically across a varied spectrum, ranging from severe obstruction with massive distension of the bladder and upper urinary tracts in the fetus, to a much more indolent course with minor secondary changes in the young or older child. Type III (diaphragm) PUVs are relatively uncommon, and are associated with difficulty in passing a transurethral catheter into the bladder as well as smooth dilation of the bladder. The following case examines the unusual experiences of both diagnosing PUVs in a teenager, and capturing visual evidence of type III valves during cystourethroscopy.