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Ahmad Haffar,1 Alexander Hirsch,1 Christian Morrill,1 Adelaide Garcia,1 Zachary Werner,2 John P Gearhart,1 Chad Crigger1 1Robert D. Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institute, Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Charlotte Bloomberg Children’s Hospital, Baltimore, MD, USA; 2West Virginia University School of Medicine, Department of Urology, Division of Pediatric Urology, Morgantown, WV, USACorrespondence: Ahmad Haffar, The Johns Hopkins University School of Medicine, The James Buchanan Brady Urological Institute, Robert D. Jeffs Division of Pediatric Urology, Charlotte Bloomberg Children’s Center, Baltimore, MD, 21287, USA, Tel +1 (304) 993-2237, Email ahaffar1@jh.eduPurpose: 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.Plain Language Summary: Cloacal exstrophy is a rare severe birth defect that does not always show up in prenatal screening. Parents are often unfamiliar with this disease and must learn about the disease, procedures, and outcomes their child may have in a short amount of time. Online medical information has been recommended to be written at a 6th grade reading level so that the general public may understand. The authors of this study wanted to investigate if average websites that parents might find using common search engines would be easily understood. The authors used different search phrases on Google and Bing search engines, and used them to look at the top 100 search results for each. The text from each page was graded based on 4 different readability scoring systems. The authors found 47 unique webpages that matched the criteria and found that most pages had reading levels of about 11th grade-college level. Due to its rarity, the information available about cloacal exstrophy are similar and would likely be poorly understood by most parents. Hospital institutions and non-profit exstrophy programs need to make a concerted effort to work together to develop simple yet helpful information regarding this disease.Keywords: cloacal exstrophy, readability, online health information, exstrophy treatment

Chad Crigger, Jake Kuzbel, Osama Al-Omar Department of Urology, Division of Pediatric Urology, West Virginia University, Morgantown, WV, 26506, USACorrespondence: Osama Al-OmarDepartment of Urology, Division of Pediatric Urology, West Virginia University, Suite 1400, Health Sciences Center South, Morgantown, WV, 26506, USAEmail osalomar@hsc.wvu.eduAbstract: Determining the need for bladder decompression and urinary diversion in the perioperative pediatric surgical patient can cause a clinical conundrum for the surgical team. Add in the several different types of urinary diversion devices possible, and the various materials associated therein and the process can suddenly seem unnecessarily daunting given the lack of concise recommendations and broad consensus. The decision to divert urine, though seemingly trivial, is associated with inherent risks. Managing and mitigating certain risks are best approached through proper education, selection, and technique. We provide a broad overview of pediatric catheter selection, indications, and pitfalls to streamline the process so that energy and attention can best be focused on the planned intervention at hand.Keywords: pediatric surgery, pediatric urology, urinary diversion, quality improvement, CAUTI

Objectives: To determine if the administration of standardized opioid disposal instructions with focused parental education improves proper disposal of leftover opioid medication among families of children undergoing ambulatory urologic surgery compared to routine postoperative instructions., Methods: A prospective, double-blinded, single-center randomized controlled trial was conducted in children 6-18 years undergoing ambulatory urology procedures between October 2021 and April 2023. Patients were randomized (1:1) to receive either the Food and Drug Administration (FDA) opioid disposal best practices worksheet plus nursing parental education or routine postoperative instructions alone. All patients were prescribed acetaminophen and ibuprofen and a per-protocol rescue opioid prescription. The primary outcome was rate of proper opioid disposal at 10-14 days post-procedure. Secondary outcomes included parents' postoperative pain measure (PPPM) scores, numerical pain scale (NPS) scores, and weight-based opioid utilization at 48 hours and 10-14 days., Results: We randomized 104 participants (53 intervention, 51 control) with 97% (101/104) complete follow-up data at 10-14 days. Patient demographics, procedural characteristics, and analgesia use were similar between groups. We observed no significant difference in proper opioid disposal rates between arms (31% intervention vs 18% control; estimated difference in proportion 13% [95% CI, -4%-29%]; P = .1). There were no increased odds of proper disposal of leftover opioid medication at 10-14 days with the intervention compared to the control (OR 2.0 [95% CI 0.8-5.1]; P = .1). We observed no differences in PPPM scores, NPS scores, or opioid utilization at 48 hours or 10-14 days., Conclusion: Providing formal opioid disposal instructions with parental education did not improve proper disposal of leftover opioid medication nor did it alter post-discharge opioid utilization after pediatric urologic surgery., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

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., Competing Interests: Conflicts of interest None of the authors have a direct or indirect commercial financial incentive associated with the publication of this article, and the manuscript is not under consideration elsewhere., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

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., Competing Interests: Conflict of interest The authors have no financial or personal relationships with other people or organizations that could inappropriately influence their work., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

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., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: We sought to determine if the addition of liposomal bupivacaine to bupivacaine hydrochloride improves opioid-free rate and postoperative pain scores among children undergoing ambulatory urologic surgery., Materials and Methods: A prospective, phase 3, single-blinded, single-center randomized trial with superiority design was conducted in children 6 to 18 years undergoing ambulatory urologic procedures between October 2021 and April 2023. Patients were randomized 1:1 to receive dorsal penile nerve block (penile procedures) or incisional infiltration with spermatic cord block (inguinal/scrotal procedures) with weight-based liposomal bupivacaine plus bupivacaine hydrochloride or bupivacaine hydrochloride alone. The primary outcome was opioid-free rate at 48 hours. Secondary outcomes included parents' postoperative pain measure scores, numerical pain scale scores, and weight-based opioid utilization at 48 hours and 10 to 14 days., Results: We randomized 104 participants, with > 98% (102/104) with complete follow-up data at 48 hours and 10 to 14 days. At interim analysis, there was no significant difference in opioid-free rate at 48 hours between arms (60% in the intervention vs 62% in the control group; estimated difference in proportion -1.9% [95% CI, -20%-16%]; P = .8). We observed no increased odds of patients being opioid-free at 48 hours with the intervention compared to the control group (OR 0.96 [95% CI 0.41-2.3]; P = .9). The trial met the predetermined futility threshold for early stopping. There was no difference in parents' postoperative pain measure scores, numerical pain scale scores, or opioid utilization at 48 hours or 10 to 14 days. No difference in adverse events was observed., Conclusions: The addition of liposomal bupivacaine to bupivacaine hydrochloride did not significantly improve opioid-sparing effect or postoperative pain compared with bupivacaine hydrochloride alone among children ≥ 6 years undergoing ambulatory urologic surgery.

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.

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., Competing Interests: Conflict of interest The authors have no financial or personal relationships with other people or organizations that could inappropriately influence their work., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Bladder exstrophy (BE), cloacal exstrophy (CE), and epispadias (E) are variants of the exstrophy-epispadias complex (EEC). These children require opioids and benzodiazepines to achieve pain management and immobilization for a lifetime of surgeries. It is hypothesized that these children would be sensitized to opiates and benzodiazepines as adults. The objective was to identify incidence of opiate and benzodiazepine use in adult EEC patients., Methods: A US Health network, TriNetX Diamond was queried from 2009 to 2022. Incidence of prescriptions for benzodiazepines and opioids were calculated for adults aged 18-60 years with a diagnosis of BE, CE, or E., Results: A total of 2627 patients were identified: 337 with CE, 1854 patients with BE, and 436 with E. Of these, 55.5% of CE, 56.4% of BE, and 41.1% of E had received any opioid prescription. Non-EEC controls had lower rates of opioids at 0.3%. E had a lower likelihood than BE or CE of receiving opioids (p < 0.0001, p < 0.0001). Benzodiazepines were prescribed in 30.3% of CE, 24.4% of BE, 18.3% of E, and 0.1% of controls. CE had a higher likelihood of benzodiazepines than both BE and E (p = 0.022, p < 0.001, respectively). E group had the lowest likelihood of benzodiazepine prescription (p = 0.007 when compared to BE) and all groups were significantly higher than controls (p < 0.0001 for all comparisons). For BE, females were more likely to be prescribed opioids (p = 0.039) and benzodiazepines (p = 0.027) than males. Sub-analyses revealed BE females had higher rates of surgical procedures (general, cardiac, gastrointestinal, and maternity) and chronic diagnoses (generalized anxiety disorder, major depressive disorder, chronic pain) compared to males with BE. Older age was associated with higher likelihood of opioid or benzodiazepine prescriptions in BE (p < 0.001), CE (p = 0.004), and E (p = 0.002)., Discussion: Across the EEC, adult patients with the most severe anomalies of CE were more likely to have received opioids and benzodiazepines. Females with BE were prescribed more opioid and benzodiazepines than males with BE. Mirroring the US population, female sex and increasing age were associated with higher rates of prescriptions, chronic diagnoses, and surgical procedures. Limitations include the lack of granular data and ability to correlate results with childhood surgeries., Conclusion: Adult EEC patients have higher rates of opioid and benzodiazepine prescriptions, with a high percentage of co-prescribing when compared to healthy controls. Across the spectrum, those with more severe anomalies, female sex, and increasing age were more likely to have received prescriptions., Competing Interests: Conflicts of interest None., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

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., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

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., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: The two main objectives of this study were to explore the rate of spinal dysraphism within bladder and cloacal exstrophy and to analyze the relationship between spinal dysraphism surgery, including timing of spinal dysraphism surgery, with urological and neurological outcomes., Methods: A prospectively maintained IRB-approved database of pediatric exstrophy patients treated from 1982 to 2021 was retrospectively reviewed for patients with spinal dysraphism. Spinal dysraphism was categorized into the following 7 subtypes: lipoma-based closed defect, myelomeningocele, meningocele, diastematomyelia, myelocystocele, low-lying conus with tethered cord/fatty filum, and sacral bony defect. Other factors assessed included patient demographic characteristics, type of spinal dysraphism procedure, reoperation, complication, presence of other neurological problems (e.g., hydrocephalus, Chiari malformation), neurological status, and urological function., Results: Analysis revealed that 114/1401 patients had coexisting spinal dysraphism. Of these 114, sufficient records including type of dysraphism were available for 54. Spinal dysraphism was most common within cloacal exstrophy (83.3% [45/54 patients]), followed by cloacal exstrophy variants (9.3% [5/54]), classic bladder exstrophy (3.7% [2/54]), and classic bladder exstrophy variants (3.7% [2/54]). Within spinal dysraphism, lipoma-based closed defects (63.0% [34/54]) and low-lying conus with tethered cord/fatty filum (11.1% [6/54]) were most common. Hydrocephalus and Chiari malformation occurred in 24.1% (13/54) and 11.1% (6/54) of patients. All 13 patients with hydrocephalus underwent shunt placement. Among those who underwent neurosurgical intervention, the complication rate for spinal dysraphism was 14.6% (7/48). Motor function data were available for 41 patients and revealed that motor function declined for 2/41 (4.8%) patients and improved for 6/41 (14.6%) after neurosurgery. There was no statistical difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure., Conclusions: The authors have reported the surgical management and outcomes of patients with exstrophy and coexisting spinal dysraphism (n = 54). In 54 patients, spinal dysraphism was most common in the subset of patients with cloacal exstrophy (83.3%). Lipoma-based closed defects (63.0%) and low-lying conus with tethered cord/fatty filum (11.1%) were the most common, and the rates of hydrocephalus and Chiari malformation were 24.1% and 11.1%, respectively. There was no difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.

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., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: In patients with classic bladder exstrophy (CBE), posterior urethral strictures after primary bladder closure can be difficult to manage and therefore necessitate alternative techniques in reconstruction of the proximal urethra. There is a paucity of literature describing treatment and management of posterior urethral stricture arising after repair of classic bladder exstrophy., Objective: To describe the technique of a bladder neck reconstruction (BNR) with a modified Tanagho anterior detrusor flap in the treatment of posterior urethral stricture arising after repair of classic bladder exstrophy., Patients and Methods: A prospectively maintained, IRB-approved database of 1401 exstrophy-epispadias patients was reviewed for patients who underwent bladder neck or proximal urethral reconstruction with modified Tanagho flap for proximal urethral stricture disease from the time period of 1975-2021. A total of 5 patients (1 female and 4 males) with classic bladder exstrophy who underwent proximal urethral reconstruction with modified Tanagho flap were identified. Of these 5 patients, the 1 female patient was treated for ongoing incontinence, not posterior urethral stricture, and was thus excluded from this report. Outcomes measured included post operative bladder capacity, post-operative continence status, upper urinary tract status, and stricture recurrence., Results: Four male patients with CBE were treated for posterior urethral strictures and are reviewed here. Three patients underwent successful primary closure (1 via modern staged repair of exstrophy (MSRE) and 2 via complete primary repair of exstrophy (CPRE)), and 1 patient (MSRE) required repeat closure at the time of stricture repair. Mean age at BNR Tanagho flap was 3.8 (range 2.3-5.0) years. All patients remained stricture free with post-operative urinary incontinence; 2 patients underwent subsequent elective bladder neck transection to achieve continence. Mean follow up 9.9 (range 2.1-15.6) years., Discussion: All 4 patients had excellent long term success rates, comparable to results reported in several adult studies utilizing a modified Tanagho flap in women with post-traumatic bladder neck contractures. In addition, our technique described here shares similarities with both the originally described Tanagho flap as well as with a dorsal onlay graft urethroplasty., Conclusion: In this limited series, modified Tanagho flap reconstruction is effective in treating posterior urethral stricture disease in CBE. This technique adds a valuable adjunct to the armamentarium of surgical options for this difficult to manage condition., Competing Interests: Conflicts of interest The authors have no financial or personal relationships with other people or organizations that could inappropriately influence their work., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

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., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: The addition of pelvic osteotomy to the armamentarium of tools for correction of classic bladder exstrophy (CBE) and cloacal exstrophy (CE) has undeniably served as one of the most effective advancements in improving the likelihood of successful primary bladder closure. Osteotomy-related complications have been studied and documented extensively in patients with CBE, yet evaluation remains limited in CE concordant with its relative rarity., Objective: To compare orthopedic complications in patients with CBE and CE who underwent primary bladder closure with osteotomy., Methods: A prospectively maintained, IRB-approved database of 1401 exstrophy-epispadias patients was reviewed for patients with CBE or CE after primary closure and pelvic osteotomy performed at a single institution from 1975 to 2021. Failed closure was defined as dehiscence, bladder prolapse, or vesicocutaneous fistula at any point. Surgery or anesthesia-related complications were captured within 6 weeks of osteotomy or closure., Results: A total of 146 patients were included in the analysis with 109 and 37 patients with CBE and CE, respectively. Between the CBE and CE cohorts, there were significant differences in median age at primary closure (68 days [IQR 10-260] vs 597 [448-734]; p < 0.001), diastasis width (4 cm IQR [3.8-4.6] vs 6.1 [5.0-7.2]; p < 0.001), osteotomy at time of closure (99.1% vs 75.7%; p < 0.001), and utilization of external hip fixation (67.9% vs 89.2%; p = 0.011). There was no significant difference by gender, osteotomy technique, or hip immobilization technique. Regarding exstrophy closure outcomes, there were 5 failures in the CBE group and 1 in the CE group (p = 1.000). Complications were experienced in 38.5% and 56.8% of CBE and CE patients (p = 0.054) with a significant difference in orthopedic complications (primarily consisting of superficial pin-site infections) between the cohorts (4.6% vs 16.2%, p = 0.031). There was no significant difference in grade 3 or higher complications between cohorts (5.5% vs 13.5%, p = 0.147)., Discussion: This was the first study comparing orthopedic complications after osteotomy between CBE and CE, providing valuable insight into which factors vary among cohorts and which are associated with increased complication rates. Despite availability of high case numbers for these rare disorders, the analysis continued to be limited sample size and missing data for retrospective analysis., Conclusions: While exstrophy closure success and overall complications rates are similar in patients with CBE and CE, patients with CE experience more superficial pin-site infections after pelvic osteotomy. External hip fixation may be associated with the increase in orthopedic complications, though further research is required to elucidate the underlying cause of these complications., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Pediatric urology is a much-needed subspecialty with a breadth of complex disorders that can often prove challenging to diagnose and manage. Exacerbating this need is the minimal exposure medical trainees receive to pediatric urology. Pediatric urology arrived in West Virginia in 1983 but the subspecialty has been inconsistently represented since then. Currently there are 2 fellowship-trained pediatric urologists in the state of West Virginia, which has an area of approximately 24,038 square miles. We review our experience with the use of telemedicine in providing outreach to the wider parts of our medically underserved state and ultimately evaluate its efficacy from a patient-centric cost analysis and diagnosis concordance perspective. We hypothesized that the use of telemedicine would be cost and time-effective for patients in our rural state., Methods: We retrospectively reviewed our series of patients presenting from outside telemedicine "referral centers" in Martinsburg, Parkersburg and Wheeling for pediatric urological consultation. We evaluated reason for consultation, geographic driving distance, drive time and travel cost saved from telemedicine consultation., Results: A total of 92 patients presented to outside designated telemedicine centers from August 2018 to April 2020. The mean driving time saved utilizing telemedicine consultation was 4 hours and 46 minutes, and mean driving distance saved was 299.8 miles. Travel costs saved in terms of fuel averaged $173.88 per patient. The most common reason for consultation was undescended testis, followed by recurrent urinary tract infection and nocturnal enuresis. Of the 23 patients who required surgery, only 2 (8.7%) had an initial diagnosis that was not concordant with their operating room examination., Conclusions: While modest, our data indicate a modern solution to a historical need in our state. Our high diagnosis concordance rate (91.3%) shows that a well-trained advanced practice provider can adequately perform an operative evaluation via telemedicine.

Diphallia is an exceedingly rare anomaly characterized by partial or complete duplication of the phallus. Approximately 100 cases have been reported worldwide since its initial documentation, and incidence is estimated at 1 in 5 to 6 million live births. Therapeutic management is dependent on the extent of the anomaly, ranging from phallic excision to complex reconstructive procedures in cases of broader systemic involvement. We present the case of congenital true diphallia with associated penoscrotal transposition, bifid scrotum, partial urethral duplication, ventral chordee, large scrotal lipoma and sacral dimple. We further present a review of available literature pertaining to diphallia., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: We sought to determine if participating in a surgical training session using perfused fresh human cadavers (PFHC) had a positive effect on urology residents' confidence in performing open and endoscopic procedures., Methods: Urology residents at our institution participated in a surgical training session in the West Virginia University Fresh Tissue Training Program, which utilized fresh cadavers with vascular perfusion. The session consisted of performing different urologic procedures (open and endoscopic) on the perfused fresh human cadavers (PFHC). Residents were given a survey to rate their confidence in different urologic procedures before, after, and 6 months after the session. Each procedure on the survey had 3-6 questions associated with it, with scores ranging from 0 (no confidence) to 4 (great confidence). Scores for each procedure before and after the session were compared., Results: Six residents participated in the session. There was an increase in the score for every procedure performed after the session. Scores at 6 month follow up remained higher than the pre-session scores., Conclusion: PFHCs offer an excellent opportunity to teach a wide variety of urologic procedures to residents. Incorporation of PFHCs may be very useful in urologic training, and further studies on its use are warranted., (© 2021. The Author(s).)

Bladder rupture occurs in only 1.6% of blunt abdominopelvic trauma cases. Although rare, bladder rupture can result in significant morbidity if undiagnosed or inappropriately managed. AUA Urotrauma Guidelines suggest that urethral catheter drainage is a standard of care for both extraperitoneal and intraperitoneal bladder rupture regardless of the need for surgical repair. However, no specific guidance is given regarding the length of catheterization. The present study seeks to summarize contemporary management of bladder trauma at our tertiary care center, assess the impact of length of catheterization on bladder injuries and complications, and develop a protocol for management of bladder injuries from time of injury to catheter removal. A retrospective review was performed on 34,413 blunt trauma cases to identify traumatic bladder ruptures over the past 10 years (January 2008-January 2018) at our tertiary care facility. Patient data were collected including age, gender, BMI, mechanism of injury, and type of injury. The primary treatment modality (surgical repair vs. catheter drainage only), length of catheterization, and post-injury complications were also assessed. Review of our institutional trauma database identified 44 patients with bladder trauma. Mean age was 41 years, mean BMI was 24.8 kg/m 2 , 95% were Caucasian, and 55% were female. Motor vehicle collision (MVC) was the most common mechanism, representing 45% of total injuries. Other mechanisms included falls (20%) and all-terrain vehicle (ATV) accidents (13.6%). 31 patients had extraperitoneal injury, and 13 were intraperitoneal. Pelvic fractures were present in 93%, and 39% had additional solid organ injuries. Formal cystogram was performed in 59% on presentation, and mean time to cystogram was 4 hours. Gross hematuria was noted in 95% of cases. Operative management was performed for all intraperitoneal injuries and 35.5% of extraperitoneal cases. Bladder closure in operative cases was typically performed in 2 layers with absorbable suture in a running fashion. The intraperitoneal and extraperitoneal injuries managed operatively were compared, and length of catheterization (28 d vs. 22 d, p =0.46), time from injury to normal fluorocystogram (19.8 d vs. 20.7 d, p =0.80), and time from injury to repair (4.3 vs. 60.5 h, p =0.23) were not statistically different between cohorts. Patients whose catheter remained in place for greater than 14 days had prolonged time to initial cystogram (26.6 d vs. 11.5 d) compared with those whose foley catheter was removed within 14 days. The complication rate was 21% for catheters left more than 14 days while patients whose catheter remained less than 14 days experienced no complications. The present study provides a 10-year retrospective review characterizing the presentation, management, and follow-up of bladder trauma patients at our level 1 trauma center. Based on our findings, we have developed an institutional protocol which now includes recommendations regarding length of catheterization after traumatic bladder rupture. By providing specific guidelines for initial follow-up cystogram and foley removal, we hope to decrease patient morbidity from prolonged catheterization. Further study will seek to allow multidisciplinary trauma teams to standardize management, streamline care, and minimize complications for patients presenting with traumatic bladder injuries., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2019 John Barnard et al.)

Background/aim: Platinum-based chemotherapy often fails due to its severe adverse effects. The aim of this study was to examine the adverse effects profile and efficacy of dicycloplatin and compare them to those of cisplatin and carboplatin., Materials and Methods: Cystoscopy surveillance of the first American cancer patient treated with dicycloplatin was performed quarterly. In vitro and in vivo studies were conducted using immunoblotting and flow cytometry to assess immune status of spleen and bone marrow of mice treated with dicycloplatin, cisplatin and carboplatin., Results: The American patient did not suffer clinically significant myelosuppression; dicycloplatin has sustained remission in this patient to date. Experimental studies showed that dicycloplatin is less toxic to bone marrow and spleen of mice than cisplatin and carboplatin., Conclusion: Dicycloplatin is a promising drug in cancer chemotherapy with less aggressive side-effects than those typically associated with cisplatin and carboplatin. This is an important therapeutic advantage in cancer chemotherapy. Clinical investigation of dicycloplatin as an alternative to cisplatin or carboplatin is warranted., (Copyright© 2019, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Paratesticular sarcomas are a rare entity and provide a unique clinical challenge due to their slow growing, often painless natural course. Adding to this challenge is the complex anatomy of the scrotum that allows these masses to mimic other conditions, including inguinal hernia, cysts, or fluid collections. We report such a case and our approach to an 83-year-old male with dedifferentiated liposarcoma of the spermatic cord with a history of inguinal hernia. In doing so, we highlight the need for thorough evaluation of scrotal masses and the management of these rare, though well-described, tumors.

The most recent American Urologic Association (AUA) guidelines concerning prostate-specific antigen (PSA) screening have caused much controversy. One particular guideline, in regard to testing elderly men, is the center of intense debate. Currently, the AUA recommends against PSA screening in men aged 70 and older. West Virginia faces the challenge of a population aging at a rate that far exceeds the projected national rate. With such a substantial future proportion of older men, and increased life expectancy within this group, careful consideration of PSA screening in elderly men is warranted. Given the uncertainty surrounding the basis for the AUA guidelines, and the increased quality of life and remaining life expectancy for older men, we propose a view that supports PSA screening in carefully selected elderly men, and offer background to assist clinicians in arriving at a sound clinical decision in concert with the patient.

A class of neutral 18-electron molecules with planar tetracoordinate carbon (ptC) centers is introduced. We show computationally that when n = 3 the neutral singlet molecule C(BeH)(n)(BH(2))(4-n) and other isoelectronic (18-valence electron) molecules of main group elements collapse from locally tetrahedral arrangements at the C-center to (near) planar tetracoordinate structures. For C(BeH)(3)BH(2) and C(CH(3))(BH(2))Li(2), for example, the tetrahedral type conformation is not even a minimum on the potential energy surface at the B3PW91, MP2(full), or CCSD levels of theory. The Mg analogue C(MgH)(3)BH(2) of the Be system also features a completely flat global minimum (with even higher energy planar minima in both cases as well). Other neutral compounds that may prefer planar geometries are apparent, and new openings for experimental investigations and theoretical analyses of planar tetracoordinate main group systems are identified. The planar conformation persists at one center in the C(BeH)(3)BH(2) dimer, and may be identifiable in higher order clusters of ptC molecules as well.

A computational analysis of the charge distribution in halomethanes and their heavy analogues (MH(4-n)X(n): M = C, Si, Ge, Sn, Pb; X = F, Cl, Br, I) as a function of n uncovers a previously unidentified saturation limit for fluorides when M ≠ C. We examine the electron densities obtained at the CCSD, MP2(full), B3PW91, and HF levels of theory for 80 molecules for four different basis sets. A previously observed substituent independent charge at F in fluoromethanes is shown to be a move toward saturation that is restricted by the low polarizability of C. This limitation fades into irrelevance for the more polarizable M central atoms such that a genuine F saturation is realized in those cases. A conceptual model leads to a function of the form [q(M(n')) - q(M(n))] = a[χ(A') - χ(A)] + b that links the electronegativities (χ) of incoming and leaving atoms (e.g., A' = X and A = H for the halogenation of MH(4-n)X(n)) and the associated charge shift at M. We show that the phenomenon in which the charge at the central atom, q(M), is itself independent of n (e.g., at carbon in CH(4-n)Br(n)) is best described as an "M-neutral substitution"--not saturation. Implications of the observed X saturation and M-neutral substitutions for larger organic and inorganic halogenated molecules and polymeric materials are identified.

Halogen bonding between halide sites (in substituted organic molecules or inorganic halides) and Lewis bases is a rapidly progressing area of exploration. Investigations of this phenomenon have improved our understanding of weak intermolecular interactions and suggested new possibilities in supramolecular chemistry and crystal engineering. The capacity for halogen bonding is investigated at the MP2(full) level of theory for 100 compounds, including all 80 MH(4-n)X(n) systems (M = C, Si, Ge, Sn, and Pb; X = F, Cl, Br, and I). The charge redistribution in these molecules and the (in)stability of the sigma-hole at X as a function of M and n are catalogued and examined. For the mixed MH(3-m)F(m)I compounds, we identify a complicated dependence of the relative halogen bond strengths on M and m. For m = 0, for example, the H(3)C-I----NH(3) halogen bond is 6.6 times stronger than the H(3)Pb-I----NH(3) bond. When m = 3, however, the F(3)Pb-I----NH(3) bond is shorter and approximately 1.6 times stronger than the F(3)C-I----NH(3) bond. This substituent-induced reversal in the relative strengths of halogen bond energies is explained.