Optimizing prenatal diagnosis and referral of classic bladder exstrophy: Lessons from a single-institution experience.

INTRODUCTION: Classic bladder exstrophy (CBE) is a malformation of the genitourinary system that occurs due to failure of abdominal wall closure. Unlike other malformations of similar incidence, prenatal diagnosis of CBE relies on suggested, rather than formal, diagnostic criteria. OBJECTIVE: This report describes prenatal diagnosis of CBE in the largest single-institutional cohort to date and delineates key sonographic findings and protocols for specialist referral. MATERIALS AND METHODS: A single-institutional database was reviewed for CBE patients born since 2000. Data on screening ultrasound use, gestational age at ultrasound, and abnormal findings were extracted. Where possible, time of prenatal diagnosis (pre- or postnatal and gestational age), ultrasound findings and other imaging data, specialist referral, institution of birth and closure, and outcome of primary closure attempt were compared. RESULTS: Of 557 patients born with CBE between 2000 and 2022, 284 met inclusion criteria and complete data were available for 280 (229 born domestically and 51 born internationally) who were included for analysis. Abnormal sonography suggestive of CBE was present for 48% (n = 134) of patients, for whom absent bladder was the most common abnormal finding (76% [102/134]). Of domestic patients, 46% (n = 106) were diagnosed prenatally at a median gestational age of 22 weeks (inter-quartile range [IQR]: 20-24), and 14% (n = 32) underwent confirmatory fetal magnetic resonance imaging. Of domestic patients with abnormal prenatal findings, 75% (n = 80/106) consulted with maternal-fetal medicine and 58% (n = 62/106) consulted with pediatric urology. On univariate analysis, prenatal diagnosis was positively associated with primary repair at Association for the Bladder Exstrophy Community-recognized centers of excellence (54% vs. 38%, p = 0.02) and negatively associated with osteotomy at primary closure (41% vs 59%, p = 0.003) but not success of primary closure (74% vs. 82%, p = 0.07). DISCUSSION: Rates of prenatal diagnosis in this cohort were similar to previous reports of smaller cohorts. Diagnosis allows for comprehensive pre- and postnatal follow-up with a pediatric urologist, with implications on birth planning and decisions on termination of pregnancy. Because of the previously-reported association between exstrophy and in vitro fertilization, these pregnancies should undergo detailed sonography. Any nonvisualization of the fetal bladder should prompt a detailed exam, and any finding characteristic of bladder exstrophy warrants referral to pediatric urology. CONCLUSIONS: Although CBE is a rare disorder, it is underdiagnosed during pregnancy. Sonographers and obstetricians should be aware of characteristic findings and best practices following diagnosis. Early referral to pediatric urology and maternal-fetal medicine is important for counseling and postnatal planning.

Clear as Mud: Readability Scores in Cloacal Exstrophy Literature and Its Treatment.

Purpose: This study examines the readability of online medical information regarding cloacal exstrophy (CE). We hypothesize that inappropriate levels of comprehension are required in these resources, leading to poor understanding and confusion amongst caregivers. Methods: The Google and Bing search engines were used to search the terms "cloacal exstrophy" and "cloacal exstrophy treatment". The first 100 results for each were collected. Each webpage was analyzed for readability using four independent validated scoring systems: the Gunning-Fog index (GFI), SMOG grade (Simple Measure of Gobbledygook), Dale-Chall index (DCI), and the Flesch-Kincaid grade (FKG). Results: Forty-seven unique webpages fit the inclusion criteria. Mean readability scores across all websites were GFI, 14.6; SMOG score, 10.8; DCI, 9.3; and FKG, 11.8, correlating to adjusted grade levels of college sophomore, 11th grade, college, and 11th grade, respectively. There were significant differences across all readability formulas. Non-profit websites were significantly less readable than institutional and commercial webpages (GFI p = 0.012, SMOG p = 0.018, DCI p = 0.021, FKG p = 0.0093). Conclusion: Caregiver-directed health information regarding CE and its treatment available online is written at the 11th grade reading level or above. Online resources pertaining to CE must be simplified to be effective.

Perioperative management of primary classic bladder exstrophy: A single institutional pathway to success.

PURPOSE: Appropriate perioperative management is crucial in patients undergoing classic bladder exstrophy closure (CBE). Therefore, the authors sought to review their intra and postoperative management of patients with CBE undergoing primary closure and examine the impact of this pathway on patient outcomes. METHOD: A prospectively maintained institutional approved exstrophy-epispadias complex database was reviewed for patients with CBE who had undergone primary closure between 2016 and 2022 and whose closure was performed within one year of age. Electronic medical records for eligible patients were retrospectively reviewed to examine patient demographics, use of pelvic osteotomy, immobilization status, pediatric intensive care unit (PICU) admission and management, perioperative analgesia and sedation, nutritional support, drainage tubes, blood transfusions, antibiotic coverage, hospital length of stay, postoperative complications, and closure failure. RESULTS: A total of 25 patients were identified, 22 with CBE and 3 with variant CBE. Closure was performed at a median age of 84 days with patients ranging in age from 9 to 351 days. All patients underwent osteotomy and immobilization with modified Buck's traction and external fixation for a median duration of 41 days. A suprapubic tube was placed in all patients for a median duration of 46.5 days. All patients underwent PICU admission following closure for a median duration of 8 days. Ventilator support was required in 68 % of patients for a median of 3 days. Epidural analgesia was used in all patients and catheters were maintained for a median duration of 19 days. All patients received a blood transfusion over the course of their admission. Patient-controlled analgesia was used in most patients as an adjunct for a median duration of 38.5 days. Other commonly used analgesic adjuncts included acetaminophen, diazepam, clonidine, and dexmedetomidine. TPN was used in 80 % of patients for a median of 7 days with a return of oral feeding thereafter. Overall, the closure success rate in this cohort of patients was 100 %. DISCUSSION: The outcome of primary bladder closure can have inauspicious consequences that can affect a child's continence for years. The incidence of failed bladder closure can be minimized with the implementation of a detailed plan for immobilization, analgesia, and nutrition guided by an experienced multi-disciplinary team. CONCLUSION: We have identified several guiding principles for perioperative success in exstrophy patients at our center including Buck's traction with external fixation, provision of adequate postoperative analgesia and sedation, aggressive nutritional support, renal and bladder drainage, and robust antibacterial support. Our high success rate in managing this complex pathology demonstrates its validity and use as a pathway to success.

Higher prevalence of benign tumors in men with testicular tumors and history of treated cryptorchidism.

OBJECTIVES: To identify if surgically treated cryptorchidism correlated with testicular tumor pathology at presentation. MATERIALS AND METHODS: An institutional database of patients treated for testicular cancer between 2003 and 2020 was reviewed. Inclusion criteria included orchiectomy patients. Exclusion criteria included unknown cryptorchidism history or pathology or laterality of orchiectomy. Data collection included demographics, surgical history, and tumor marker status. RESULTS: A total of 435 patients were included. Thirty-three of these patients had a history of UDT. There was no statistical difference in age at orchiectomy, laterality of orchiectomy, or lymphovascular invasion with regard to UDT history. There was a statistical difference in tumor pathology after orchiectomy, P = 0.03. On secondary analysis, benign pathology was significantly more common in patients with a history of UDT (15.2%) than without (4.7%), P = 0.01. Mixed GCT was also found at a significantly lower rate in patients with a history of UDT (18.2%) compared to those with no history of UDT (37.3%), P = 0.03. There were no statistically significant differences in other pathology. CONCLUSION: Previous studies have shown that there is a greater rate of seminoma in patients with testicular cancer in an undescended testis. This study shows that in patients with a history of UDT compared to those without a history of UDT, there is a greater percentage of patients with benign testicular masses after orchiectomy. Guideline based practices including monthly self-examination and testis-sparing surgery for appropriate patients may reduce rates of radical orchiectomy for benign tumors.

Long-Term Management of Problems in Cloacal Exstrophy: A Single-Institution Review.

INTRODUCTION: Cloacal exstrophy (CE) is the most severe malformation of the exstrophy-epispadias complex. This study aims to discuss long-term sequela in a single major institution with a high volume of CE patients. MATERIALS AND METHODS: A prospectively maintained database of 1490 patients on the exstrophy epispadias spectrum (145 cloacal exstrophy) from 1974 to 2023. The patient database was reviewed for CE patients >10 years of age for genitourinary, gastrointestinal, orthopedic, and psychosocial outcomes. RESULTS: A total of 63 patients (43.4%) with ≥10 years of follow up were included for analysis. Thirty-nine (61.9%) patients were 18 years or older. Twenty-two (34.9%) patients were female and 39 (61.9%) male, 14 of whom were gender converted at birth. Two female patients conceived naturally and delivered via cesarean section. No male born CE patients had biological children. Catheterizable channels were common (45/63, 71.4%) and most (88.9%) were continent. Gastrointestinal diversion was managed mostly by colostomy (37/63, 58.7%). Three out of five (60.0%) patients who underwent PSARP were continent of stool. Twenty-two (34.9%) patients were wheelchair-bound. Psychosocial diagnoses included 52.4% (33/63) patients with anxiety/depression and 27.0% with chronic pain. Out of 56 patients evaluated by physical therapy, 75% were independent in ADL performance. Of patients older than 18, 79.5% (31/39) had attended college and 82.1% (32/39) were gainfully employed. CONCLUSION: Advances in critical care, nutrition, gastrointestinal, orthopedic, and urologic management have resulted in survival rates approaching 100% among patients with CE. While these children face long-term sequela spanning various organ systems, many lead independent and fully-functional lives. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: Level IV.

Classic Bladder Exstrophy Closure Without Osteotomy or Immobilization: An Exercise in Futility?

OBJECTIVE: To review the outcomes of classic bladder exstrophy (CBE) closure without the use of osteotomy or lower extremity/pelvic immobilization. METHODS: A prospectively maintained institutional approved exstrophy-epispadias complex database of 1487 patients was reviewed for patients with CBE who had undergone closure without osteotomy nor immobilization. All patients were referred to the authors' institution for reconstruction later in life or for failed closure. RESULTS: Of a total of 1016 CBE patients, 56 closure events were identified that met inclusion with a total of 47 unique patients. Thirty-eight closures were completed prior to 1990 (67.9%). Forty-five closure events developed eventual failure (45/56, 80.4%) (Table 1). Thirteen closure events were secondary closures (13/56, 23.2%). The primary closure failure rate was 83.7% (36/43) while the secondary closure failure rate was 69.2% (9/13). Failures were attributed to one or more of dehiscence, bladder prolapse, and vesicocutaneous fistula (25/45, 55.6%) (23/45, 51.1%) (6/45, 13.3%), respectively. Thirty-seven patients developed social continence (37/47, 78.7%), while only 8 patients developed spontaneous voided continence (7/47,17.0%) (Table 2). The most common methods of voiding were continent catheterizable channels (25/47, 53.2%) of which all were socially continent. CONCLUSION: These results illustrate the critical role osteotomy and postoperative immobilization can play in both primary and secondary exstrophy closure. While this is a historical case series, the authors believe that these results remain relevant to contemporary exstrophy surgeons.

Mucosal Violations and Their Effect on Successful Bladder Neck Closure in Cloacal Exstrophy.

BACKGROUND: Cloacal exstrophy (CE) is rare and challenging to reconstruct. In the majority of CE patients voided continence cannot be achieved and so patients often undergo bladder neck closure (BNC). Prior mucosal violations (MVs), a surgical event when the bladder mucosa was opened or closed, significantly predicted failed BNC in classic bladder exstrophy with an increased likelihood of failure after 3 or more MVs. The aim of this study was to assess predictors for failed BNC in CE. METHODS: CE patients who underwent BNC were reviewed for risk factors for failure including osteotomy use, successful primary closure, and number of MVs. Chi-squared and Fisher's exact tests were used for comparing baseline characteristics and surgical details. RESULTS: Thirty-five patients underwent BNC. Eleven patients (31.4%) failed BNC including a vesicoperineal fistula in nine, vesicourethral and vesicocutaneous fistula in one each. The fistula rate in patients with 2 or more MVs was 47.4% (p = 0.0252). Two patients subsequently developed a vesicocutaneous fistula after undergoing repeated cystolithotomies. A rectus abdominis or gracilis muscle flap were used to close the fistula in 11 and 2 patients, respectively. CONCLUSIONS: MVs have a greater impact in CE with an increased risk of failed BNC after 2 MVs. CE patients are most likely to develop a vesicoperineal fistula while a vesicocutaneous fistula is more likely after repeat cystolithotomy. A prophylactic muscle flap should be considered at time of BNC in patients with 2 or more MVs. LEVELS OF EVIDENCE: Prognosis Study, Level III.

Bladder capacity and growth in classic bladder exstrophy: A novel predictive tool.

INTRODUCTION: Bladder capacity (BC) is an important metric in the management of patients with classic bladder exstrophy (CBE). BC is frequently used to determine eligibility for surgical continence procedures, such as bladder neck reconstruction (BNR), and is associated with the likelihood of achieving urinary continence. OBJECTIVE: To use readily available parameters to develop a nomogram that could be used by patients and pediatric urologists to predict BC in patients with CBE. STUDY DESIGN: An institutional database of CBE patients was reviewed for those who have undergone annual gravity cystogram 6 months after bladder closure. Candidate clinical predictors were used to model BC. Linear mixed effects models with random intercept and slope were used to construct models predicting log transformed BC and were compared with adjusted R2, Akaike Information Criterion (AIC), and cross-validated mean square error (MSE). Final model evaluated via K-fold cross-validation. Analyses were performed using R version 3.5.3 and the prediction tool was developed with ShinyR. RESULTS: In total, 369 patients (107 female, 262 male) with CBE had at least one BC measurement after bladder closure. Patients had a median of 3 annual measurements (range 1-10). The final nomogram includes outcome of primary closure, sex, log-transformed age at successful closure, time from successful closure, and interaction between outcome of primary closure and log-transformed age at successful closure as the fixed effects with random effect for patient and random slope for time since successful closure (Extended Summary). DISCUSSION: Using readily accessible patient and disease related information, the bladder capacity nomogram in this study provides a more accurate prediction of bladder capacity ahead of continence procedures when compared to the age-based Koff equation estimates. A multi-center study using this web-based CBE bladder growth nomogram (https://exstrophybladdergrowth.shinyapps.io/be_app/) will be needed for widespread application. CONCLUSION: Bladder capacity in those with CBE, while known to be influenced by a broad swath of intrinsic and extrinsic factors, may be modeled by the sex, outcome of primary bladder closure, age at successful bladder closure and age at evaluation.

Achieving goal capacity for continence surgery: A cumulative event analysis of bladder exstrophy patients.

BACKGROUND: Following successful closure of patients with classic bladder exstrophy (CBE), the next major milestone is the establishment of urinary continence. Prior to determining the most appropriate continence surgery, it is imperative to reach an adequate bladder capacity minimum of 100 cc in order to make the decision between bladder neck reconstruction (BNR) or continent stoma, with or without augmentation cystoplasty (AC). OBJECTIVE: To examine the timing of when patients achieve threshold bladder capacity for BNR eligibility. We hypothesize most patients will achieve an adequate bladder capacity (100 cc) by 7 years old when continence surgeries will begin to be considered. STUDY DESIGN: An institutional database of 1388 exstrophy patients was retrospectively reviewed for CBE patients after successful primary bladder closure. Bladder capacities were measured via gravity cystography and data presented as descriptive statistics. The cohort was stratified by location, neonatal (≤28 days) or delayed closure period and osteotomy status. The bladder capacities were categorized to either reaching goal or not and a cumulative event analysis was performed. The event being reaching 100 cc capacity or greater and time being the number of years between bladder closure and attainment of goal capacity. RESULTS: 253 patients met inclusion criteria between 1982 and 2019. The majority were of male gender (72.9%), had their closure performed at the authors' institution (52.5%), within the neonatal period (80.7%), and without an osteotomy (51.7%). 64.9% of patients reached goal bladder capacity. There were no significant differences in those who did or did not achieve goal except for clinical follow up. Cumulative event analysis demonstrated a median time of 5.73 years (95% CI 5.2-6.20) corresponded with a 50% event probability of reaching goal capacity. Cox-proportional hazards showed location of closure was significantly associated with hazards of reaching goal bladder capacity (HR = 0.58, CI 0.40-0.85, p = 0.005). Based on this model, the median time to event would be 5.20 years (95% CI 4.76-5.80) for cases done at the authors' hospital and 6.26 years for those performed at an outside hospital (95% CI 5.77-7.24). CONCLUSIONS: These findings help surgeons counsel families appropriately on the odds of attaining goal capacity at various ages. For those who do not reach 100 cc by five years of age, it helps further characterize the odds of requiring a continent stoma with bladder augmentation and the best timing for reconstructive surgery in order to safely gain urinary continence. Families may also be assured that most patients would have the breadth of surgical options when it comes to continence as more than half of patients reached the bladder capacity threshold.

A Single-Institutional Experience With Prenatal Diagnosis of Cloacal Exstrophy: Room for Improvement.

INTRODUCTION: A single institutional study characterizes the rate of prenatal diagnosis of cloacal exstrophy (CE) and examines its role on successful primary closures. MATERIALS AND METHODS: An institutional database of 1485 exstrophy-epispadias patients was reviewed retrospectively for CE patients with confirmed presence/absence of prenatal diagnostics, primary exstrophy closure since 2000, institution of closure, and at least 1 year of follow up following closure. RESULTS: The cohort included 56 domestic patients and 9 international patients. Overall, 78.6% (n = 44) of domestic patients were prenatally diagnosed while 21.4% (n = 12) were diagnosed postnatally. A positive trend was observed in the rate of prenatal diagnosis across the study period, 56.3%, 84.2%, 88.9% respectively (p = 0.025). Confirmatory fMRI was obtained in 40.9% (n = 18) of prenatally diagnosed cases. Patients diagnosed prenatally were found to be more likely to undergo treatment at exstrophy centers of excellence (72.1% v 33.3%, p = 0.020). Prenatal diagnosis was not predictive of increased rate of successful primary closure (75.6% vs 75.0%; p = 1.00; OR: 1.03, 95% CI: 0.23-4.58). Primary closures undertaken at exstrophy centers of excellence were significantly more likely to be successful compared to outside hospitals (90.9% v 50.0%, p = 0.002). CONCLUSIONS: The rate of prenatal diagnosis of CE in patients referred for management to a high-volume exstrophy center is improving. Despite this improvement, patients continue to be missed in the prenatal period. While prenatal diagnosis offers the ideal opportunity to educate, counsel, and prepare expectant families, patients diagnosed at birth are not disadvantaged in their ability to receive a successful primary closure. Further research should investigate the benefit of patient referral to high-volume exstrophy centers of care to ensure optimal care and outcomes.

Bladder exstrophy-epispadias complex related litigation: A legal database review.

OBJECTIVE: To review the nature and extent of bladder exstrophy-epispadias related malpractice litigation in the United States. METHODS: Two legal databases (Nexis Uni, WestLaw) were reviewed for state and federal cases using the terms "bladder exstrophy", "cloacal exstrophy", "epispadias", in combination with "medical malpractice", or "negligence", or "medical error", or "complication", or "malpractice", or "tort". Databases were queried from 1948 to 2022 and reviewed for medical and legal details. RESULTS: Our search yielded 16 unique legal cases with 6 fitting established criteria for analysis. Urology and paediatric urologists were named in 50% of cases as were community medical systems. Cause for lawsuit included negligence in surgical performance (50%), primary closure of exstrophy (33%), and post-operative care (50%). Settlement agreement was reached in one case (17%). Outcomes favoured the physician in 60% of trials. Lawsuits alleging negligent surgical performance and/or post-operative care exclusively named urologists with outcomes favouring the surgeon in 66% of cases. The settlement payment (n = 1) was $500,000 and monetary damages (n = 1) equated to $1.3 million. CONCLUSIONS: Malpractice litigation related to BEEC treatment is rare. Trial outcomes favour the medical provider. Cases that resulted in financial liability successfully alleged avoidable negligence resulting in irreversible physical damage. The authors recommend families with BEEC seek board-certified paediatric urologists experienced in treating this complex and/or Bladder Exstrophy Centers of Excellence. Further, we recommend surgeons treating BEEC properly educate patients and families on the severity of this major birth defect including its lifelong implications and need for surgical revisions.

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.

Effect of Distance From Fertility Center on Utilization of Fertility Preservation Referral in Men.

PURPOSE: Fertility preservation (FP) is underutilized in males with cancer or other diseases requiring gonadotoxic therapies. We sought to evaluate whether patient distance from FP center affected rates of providing a semen analysis after referral. MATERIALS AND METHODS: We performed a retrospective analysis of all males who were referred for FP at a single institution between 2013 and 2021. A multiple logistic regression model was conducted with semen sample submission as the variable of interest. Predictor variables were disease type, distance, and payment method. Secondary outcomes were number of semen samples submitted and number of vials collected. RESULTS: Records of 461 males referred to our center were analyzed. Of these patients, 326 (71%) provided a semen sample after referral and 135 (30%) did not. Further distance from our center was associated with lower odds of submitting a semen sample (OR, 0.85; 95% CI, 0.75 to 0.97; P < .05). For patients who submitted at least one sample, distance did not affect the total number of samples submitted but was associated with a small increase in total vials cryopreserved. CONCLUSION: Men referred for FP exhibit a high rate of sperm cryopreservation. Further distance from FP center was associated with decreased odds to provide semen sample after referral. Our model estimated a 15% decrease in odds of collection with every doubling of distance from our center. Efforts must be made to improve FP utilization for patients traveling far distances, but distance alone should not preclude referral.

The Role of Tissue Expanders in the Reconstruction of Proximal Hypospadias.

OBJECTIVE: To report the technique and outcomes of tissue expansion (TE) for generating additional penile skin for urethroplasty and/or skin coverage during primary or redo hypospadias repair in penoscrotal transposition (PST) patients with a proximal hypospadias. METHODS: Proximal hypospadias and PST patients with a lack of penile skin, congenitally or after failed repair, who underwent TE assisted reconstruction were reviewed. TE were placed under the penile shaft and expanded skin was used for tubularized incised plate repair. Success was defined as urethral advancement to the corona or more distal with tension free skin coverage. RESULTS: A total of 24 patients underwent reconstruction including 16 as part of primary repair and for redo repairs in 8. Nine patients experienced expander extrusion and posturethroplasty complications occurred in 43.8% of primary repairs and 75.0% of redo repairs; a urethrocutaneous fistula was most common (n = 8). Overall, success was achieved in 87.5% of patients with postoperative meatal locations almost all coronal (45.8%) or glanular (50.0%). CONCLUSION: Proximal hypospadias reconstruction is challenging, and complications are not infrequent. TE is a useful alternative for complex patients with a skin paucity such that cutaneous coverage would be difficult following urethroplasty. Although the complication rate was 43.8% for primary repair, TE generated sufficient residual skin for success after additional reconstruction. For redo repair early use is most beneficial as there is more expandable skin. The pseudocapsules provide vascularized coverage to reinforce the urethra while there is sufficient skin to minimize the need for a skin graft for penile coverage.

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.

Fixation with lower limb immobilization in primary and secondary exstrophy closure: A saving grace.

PURPOSE: A pivotal factor in the success of bladder closure in patients with classic bladder exstrophy (CBE) is the postoperative immobilization of the pelvis and lower extremities after pelvic osteotomy. This study investigates the outcomes of closure among patients with lower limb immobilization using many techniques. The authors hypothesize that the addition of external fixation (pelvic immobilization) in patients with any form of limb immobilization will be associated with improved outcomes. METHODS: A prospectively maintained institutional exstrophy-epispadias complex database of 1415 patients was reviewed for patients with CBE who had undergone closure with available immobilization and osteotomy data. Association between closure outcomes and immobilization techniques were determined. Univariate analysis was performed using Chi-Square or Fischer-Exact test as appropriate for categorical variables. Multivariate analysis via binomial logistic regression was used to identify factors leading to successful closure. RESULTS: A total of 747 closure events matching the inclusion criteria were identified. Patients included 508 males and 239 females. There were 597 primary closures (79.9%) with 150 reclosure events (20.1%). Limb immobilization was used in 627 (83.9%) of closure events. Successful closures were associated with osteotomy use (p < 0.0001) and limb immobilization (p < 0.0001); specifically, the combined anterior innominate with posterior vertical iliac osteotomy and modified Buck's traction with external fixation (p < 0.0001, p < 0.0001). Among the group of 33 patients who received external fixation alone and no other type of immobilization, the failure rate was 33.3%, comparatively, patients with any form of combined immobilization (external fixation with lower limb immobilization) had a failure rate of 7.1% ( Table 1). Among patients immobilized with mummy wrap, spica casting, or knee immobilizers, external fixation was associated with 3.76 increased odds of successful closure (p = 0.0005, 95% CI 1.79-7.90). In a unique group of 67 patients without pelvic osteotomy or any form of pelvic or limb immobilization, the failure rate was 74.6%. DISCUSSION: This study confirms, in a larger series, previous findings of improved outcomes when patients are immobilized with modified Buck's traction and external fixation. The authors apply this technique in most all closures and recommend this technique be utilized whenever feasible. However, regardless of the manner of lower limb immobilization, external fixation is a critical factor to optimize closures and ensure success. CONCLUSION: The results of this study clearly suggest the use of external fixation can be protective against bladder closure failure. The use of pelvic immobilization, in addition to post-operative lower limb immobilization should be strongly considered.

Application of Tunica Vaginalis Flap for Epispadias Repair in the Epispadias-Exstrophy Complex.

OBJECTIVE: To describe long-term outcomes and experience with the tunica vaginalis flap (TVF) as a tissue augment for complex epispadias repair. METHODS: A prospectively maintained institutional database was reviewed for patients who underwent epispadias repair with TVF from 2010 to 2021. Evaluation of patient characteristics between those who developed dehiscence or UCF and those who did not was performed via Mann-Whitney U, Kruskal-Wallis, or Fisher's exact test, as appropriate. RESULTS: A total of 47 male patients were identified, of whom, 1 (2.1%) had cloacal exstrophy, 38 (80.9%) had classic bladder exstrophy or a variant, and 8 (17.0%) had epispadias as their primary exstrophy-epispadias complex diagnosis. The median age at epispadias repair was 13 months (IQR 11 - 19). The overall rate of fistula or dehiscence development was 19.1% (9 patients), of whom, 7 developed urethrocutaneous fistulae, 1 dehiscence, and 1 both. Notably, 0 of the 5 patients who had undergone prior epispadias repair and 0 of the 8 patients with a diagnosis of isolated epispadias developed a UCF or dehiscence after repair with TVF. Differences in age at repair, primary EEC diagnosis, prior epispadias repair, pre-repair testosterone, fibrin sealant utilization, closure layers, stent removal time, and bladder capacity at repair were not statistically significant (P>.05) CONCLUSION: Our expanded evaluation indicates that utilization of the tunica vaginalis as an adjunct to epispadias repair may provide durable protection against fistula development in EEC patients who have undergone prior epispadias repair and in primary repair of patients with isolated epispadias.

Perioperative Reflection: An Understudied Yet Common Practice Among Surgical Residents.

OBJECTIVE: Directed reflection, a descriptive, analytical, and critical reasoning process, has been implemented in several professional fields as a method of developing expertise. In surgery, investigation of reflection has been limited. To date, we are unaware of any study that has sought to define or characterize the independent reflective practices of surgical trainees. In this small pilot study, we characterize the independent operative reflection activities among surgical residents at a single academic institution. DESIGN: An unvalidated survey was developed through cognitive interviews and pilot testing to assess 5 aspects of operative reflection. An IRB approved electronic survey was developed and distributed to all general surgery residents at an academic institution. Responses were divided by post-graduate year into junior residents (JR; PGY 1-3) and senior residents (SR; PGY 4-5). RESULTS: A response rate of 46% (23/50) was attained. All respondents self-identified as operative reflectors. Three primary methods of reflection were identified: mental, written, and oral. Mental reflection was the primary reflection method of 75% of our respondents as identified by step-by-step mental rehearsal, internal narration, and question series. Reflection was practiced everyday by 42% of respondents. Topics of reflection focused on weaknesses (50%), self-improvement (35%) and judgment (15%). Several respondents (57%) indicated that reflection-based learning tools would be useful. No statistically significant changes in the method or frequency were established between junior and senior residents (p = 0.378, p = 0.464, respectively). Variation in mental reflection activities among JR and SR groups were found (p = 0.029, p = 0.00004, respectively). CONCLUSIONS: The enduring role of reflection in experiential learning has been established in many professional fields but remains under-utilized in surgical education. Our study suggests surgical residents reflect independently of curriculum in individual and categorizable ways. Further investigation is required to adequately characterize reflective habits of the surgical resident and evaluate its impact on competency.

Buried Penis Reconstruction in the Management of Localized Penile Cancer.

INTRODUCTION: The objective of this article is to describe surgical techniques for the management of localized penile cancer concurrent with adult acquired buried penis. Penile cancer, while rare, invariably impacts quality of life as the primary surgical management ranges from local excision to total penectomy. Penile cancer has recently been linked to adult acquired buried penis (AABP) with the hypothesis that chronic inflammation of the penis contributes to risk of penile malignancy. Buried penis reconstruction is now a well-described procedure involving escutcheonectomy and split thickness skin grafting which has been shown to improve quality of life in individuals with adult acquired buried penis. TECHNICAL CONSIDERATIONS: This report describes a patient with AABP requiring partial penectomy and inguinal node dissection who also underwent adult acquired buried penis repair during his procedure. A review of cases of buried penis cases was carried out and four other cases of penile cancer were identified who were managed with penile preserving procedures. CONCLUSIONS: This combination of surgical techniques strives to improve quality of life and facilitate cancer surveillance without compromising oncologic outcomes.

The Gullwing Technique: A Novel Method of Transcorporal Artificial Urinary Sphincter Placement for the Fragile Urethra.

OBJECTIVE: To describe the novel gullwing technique for artificial urinary sphincter (AUS) placement. The transcorporal technique for AUS placement is beneficial in patients with 'fragile urethras' (previous failed AUS, urethroplasty or history of radiation) however limitations include insufficient lateral and ventral urethral support in addition to potential cinching during corporotomy closure which, in the absence of additional grafting may restrict our ability to conserve internal corporal capacity and limit options for future preservation of erectile function via penile prosthesis placement. The gullwing variation of the technique offers the potential to circumvent these disadvantages. MATERIALS AND METHODS: This case describes the gullwing variation of transcorporal AUS placement in a complex patient with a history of abdominopelvic trauma and prior failed AUS placements secondary to urethral erosion. RESULTS AND CONCLUSION: Transcorporal AUS placement in patients with prior urethral compromise has been shown to result in lower revision and erosion rates. The gullwing modification of the technique is a novel variation providing improved circumferential urethral protection and, with the addition of corporal grafting, aims to enable the preservation of the three-dimensional corporal volume necessary for ease of subsequent penile prosthesis implantation. However, studies assessing the long-term functional outcomes and durability of this technique are needed.