Effectiveness of Liposomal Bupivacaine With Bupivacaine Hydrochloride vs Bupivacaine Hydrochloride Alone as a Local Anesthetic for Children Undergoing Ambulatory Urologic Surgery: The Baby ORIOLES Randomized Clinical Trial.

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.

The utilization of perfused cadaver simulation in urologic training: a pilot study.

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.

Spermatic cord dedifferentiated liposarcoma presenting as a recurrent inguinal hernia.

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 Aging Mountaineer: PSA screening in older men--of value or should we skip this test?

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.

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.

National Postoperative Opioid Prescribing Rates Following Pediatric Urology Procedures Before and After the 2018 American Academy of Pediatrics Challenge to Reduce Opioid Prescribing: A Claims Database Analysis.

OBJECTIVE: To evaluate trends in opioid prescribing rates following pediatric urologic surgery. METHODS: We queried the TriNetX Research database for patients under age 18 who underwent one of seven common pediatric urology procedures. We identified the proportion of patients that received an oral opioid prescription within 5days of surgery. The primary analysis evaluated the trend in postoperative opioid prescriptions using 3-month intervals from January 2010 to December 2022. We performed an interrupted time series analysis assessing trends in opioid prescribing patterns both before and after the American Academy of Pediatrics challenge. RESULTS: Of the 81,644 pediatric urology procedures, 29,595 (36.2%) received a postoperative opioid prescription, including 29.8% of circumcisions, 25.8% of hydrocelectomies, 39.6% of hypospadias repairs, 42.7% of pyeloplasties, 42.8% of ureteral reimplants. For all procedures we observed rising rates of opioid prescribing, increasing by 0.9% per 3-month interval prior to the challenge statement release from 2010 to 2018. We observed an overall significant decrease in opioid prescribing by 2.2% per 3-month interval following the challenge statement release. Additionally, since 2018, there was a significant decrease in opioid prescribing in all of the race, ethnicity, and age cohorts. CONCLUSION: Opioid prescribing following pediatric urology procedures has sharply decreased following the 2018 American Academy of Pediatrics challenge statement which underscores the value of cross-specialty quality improvement initiatives. Nonetheless, opioid prescribing remains high with potential racial or age disparities that warrant further investigation.

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.

A Rocky Road: Bladder Stones in the Augmented Exstrophy-Epispadias Complex Patient.

OBJECTIVES: To determine the rate of stone formation amongst patients of the exstrophy-epispadias complex with augmentation cystoplasty. We hypothesize that bowel segment choice influences the rate of stone formation after bladder augmentation and the rate of complications from bladder stone surgery. METHODS: An IRB approved institutional database of 1512 exstrophy-epispadias patients was reviewed retrospectively. Patients that had a history of bladder augmentation and were seen at our institution between 2003 and 2023 were included. RESULTS: Out of 259 patients, bladder stones developed in 21.6% (56), of which the bowel segment used was colon in 147 patients and ileum in 100. Stones formed in 19% of colon augments compared to 29% ileal augments, however this was not statistically significant (p=0.07). The most common primary stone component was dahllite, followed by struvite for all augments. The median time to stone treatment after augmentation was 4.14 years (0.75-31). 74% of patients had a recurrence that required a second surgery. The median time from first to second surgery and second to third surgery was 1.4 years and 2.22 years, respectively. Bladder stone surgery complications occurred in 14% of patients, vesicocutaneous fistula being the most common, and complications did not differ by augment type. Median follow up after first stone intervention was 6.07 years (0-19.5). CONCLUSION: The treatment of bladder stones in the exstrophy-epispadias complex remains challenging. Interventions to prevent recurrence are crucial as the majority of patients will require two or more stone surgeries in their lifetime.

Interposing Rectus and Gracilis Muscle Flaps For Pelvic Reconstruction in Bladder Exstrophy After Bladder Neck Closure.

BACKGROUND: The exstrophy-epispadias complex is a spectrum of ventral wall malformations including classic bladder exstrophy (CBE) and cloacal exstrophy (CE). Patients undergo multiple soft-tissues procedures to achieve urinary continence. If unsuccessful bladder neck closure (BNC) is performed, muscle flaps may be used to reinforce BNC or afterwards for fistula reconstruction. In this study, patients reconstructed using a rectus abdominis or gracilis muscle flap were reviewed. METHODS: A retrospective cohort study of exstrophy-epispadias complex patients who underwent BNC and had a muscle fap was performed. Indication for flap use, surgical technique, risks for BNC failure including mucosal violations (MVs) were reviewed. MVs were prior bladder mucosa manipulation for exstrophy closure, repeat closure(s) and bladder neck reconstruction. Success was defined as BNC without fistula development. RESULTS: Thirty-four patients underwent reconstruction. Indications included during BNC (n=13), fistula closure after BNC (n=17), following BNC during open cystolithotomy (n=1) or fistula closure after open cystolithotomy (n=3). A vesicourethral fistula developed most frequently in CBE (88.9%) and vesicoperineal fistula in CE (87.5%). Thirty-three rectus flaps and 3 gracilis flap were used with success achieved in 97.1% and 66.7%, respectively. All 34 patients achieved success and 2 CE patients required a second flap. CONCLUSION: The rectus flap is preferred as it covers the antero-inferior bladder and pelvic floor to prevent urethral, cutaneous, and perineal fistula formation. The gracilis flap only reaches the pelvic floor to prevent urethral and perineal fistula development. Increased MVs, increase the risk of fistula formation and may influence the need for prophylactic flaps.

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.

Stomal Stenosis After Continent Urinary Diversion in Bladder Exstrophy: Risk Factors and Management.

OBJECTIVE: To identify risk factors for stenosis and compare management strategies for stenosis etiology and to examine the efficacy of each approach. Patients with classic bladder exstrophy (CBE), a rare genitourinary malformation, may require construction of a continent urinary stoma (CUS) if incontinence persists. Stomal stenosis is a challenging complication as it is common, progressive and recurrent. METHODS: CBE patients who underwent CUS were retrospectively reviewed for risk factors for stenosis including stoma type, prior midline laparotomy number, and umbilicoplasty suture material. Stenosis etiology and management strategies were further reviewed. RESULTS: A total of 260 CBE patients underwent CUS creation. Stenosis developed in 65 patients (25.0%) at a median interval of 1.9 years. Etiology included scar contracture (n=41), keloid (n=17), and hypertrophic scar (n=7). Multifilament suture was the only variable associated with an increased risk of stenosis compared to monofilament suture (p=0.009). Almost all patients required surgical intervention. Most scar contractures underwent stomal incision with success in 100%. Hypertrophic scars and keloids responded best to excision with local tissue rearrangement (66.7%). At last follow-up, all patients achieved success. CONCLUSION: Stomal stenosis is common and challenging for the reconstructive surgeon. Strategies to prevent and effectively manage this are greatly desired. Use of multifilament suture for the umbilicoplasty increased stenosis perhaps from a greater inflammatory response and scarring, while monofilament suture may reduce its incidence. Stomal incision for treating scar contractures, and excision with local tissue rearrangement for hypertrophic scars and keloids may improve successful primary surgical intervention.

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.

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.

The use of a modified tanagho flap in the repair of posterior urethral stricture after primary exstrophy closure.

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.

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.

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.

Pelvic osteotomy in cloacal exstrophy: A changing perspective.

BACKGROUND: The type of osteotomy and pelvic fixation in the management of primary cloacal exstrophy (CE) closure is variable. The purpose of this study was to evaluate primary CE closure outcomes with osteotomy, immobilization, and multi-staging procedure trends over time. METHODS: An institutional database was retrospectively reviewed for patients who underwent primary CE closure from 1960 to 2020. Demographics, osteotomy, fixation, and outcomes were noted. Subanalyses by location of primary closure (AH=author's hospital; OH=outside hospital). RESULTS: Out of 122 patients, multi-stage became more common than single-stage procedures (p = 0.019), with multi-stage associated with higher success rates (77.4% v 45.7%; p = 0.001). The use of any osteotomy increased over time (p = 0.007), with a posterior approach falling out of favor and increasing prevalence of a combined osteotomy (p<0.001). The use of any osteotomy compared to no osteotomy was associated with successful closure (77.6% v 41.7%; p = 0.007). The combined, posterior, and anterior approaches were associated with 90%, 76.2%, and 60.9% successful primary closure rates, respectively (p<0.001). Fixation modalities changed over time as Buck's traction (p<0.001) and external fixation (p<0.001) became more prevalent. Spica casting has become less common (p = 0.0002). Immobilization type was associated with success rates with Buck's (92.1%; p<0.001) and external fixation (86.0%; p<0.001) performing best. CONCLUSIONS: The use of osteotomy and fixation in the CE spectrum has changed markedly. In this cohort, a staged approach with combination osteotomy was associated with better outcomes when using a multidisciplinary team approach. LEVEL OF EVIDENCE: This is a retrospective comparative study (Type of Study: Treatment; Evidence Level: III).