Introduction of a modified single stage reconstruction technique of male penopubic epispadias.

OBJECTIVE: To represent the long-term outcomes of our modified single-stage technique for the reconstruction of isolated penopubic epispadias in male patients. PATIENTS AND METHODS: Data from 113 patients were obtained from bladder-exstrophy-epispadias database of our tertiary center. A total of seven boys with isolated penopubic epispadias with no prior history of surgery and any other anomaly underwent our modified surgical approach from February 1997 to September 2019. The mean ± SD age at surgery was 6.5 ± 2.4 years. Volitional voiding status and cosmetic appearance were evaluated at each follow-up interval. Postoperative follow-up was performed at quarterly intervals in the first year and once a year in subsequent years. RESULTS: The mean ± SD of follow-up was 8.5 ± 6 years. All boys who were incontinent achieved urinary control and the ability of normal transurethral micturition following the surgery. Four boys became completely dry, and the other three attained social dryness. Postoperative mean (SD) bladder capacity was significantly increased from 54.5 (11) to 124 (40.0) within 6 months, and to 194 (47.5) at 18 months after surgery. Dorsal curvature has been resolved in all cases, and no postoperative complications were noted except for surgical site infection in one patient treated with antibiotics and bilateral vesicourethral reflux resolved after injection of bulking agents. Four patients had normal erectile function and ejaculation, while the others have not reached puberty yet. Moreover, none of the patients developed urethrocutaneous fistula, stricture, or penile ischemia. CONCLUSION: The present findings suggest the safety and effectiveness of the combination of single-stage urethro-genitoplasty, bladder neck plication, and fat pad pedicled flap in management of boys with isolated penopubic epispadias that can lead to the achievement of urinary control, acceptable sexual function, and cosmetically satisfactory genitalia. Minimal morbidity, low complication rate, and promising outcomes are essential factors, supporting the notion of introducing this technique as a valid option for management of this entity.

A less invasive technique for delayed bladder exstrophy closure without fascia closure and immobilisation: can the need for prolonged anaesthesia be avoided?

INTRODUCTION: It is believed that the main factors enhancing security of the bladder exstrophy closure are use of osteotomy, pubic bones approximation or transferred flaps for rectus fascia closure. However, these methods increase operating time, surgical trauma and carry risks for the patient. OBJECTIVES: To demonstrate that the goal of secure bladder exstrophy closure can be achieved easier technically and safer for the child than previously thought. The paper examines the hypothesis that less invasive bladder exstrophy closure achieved without fascia closure can reduce pain and avoid the need for immobilization and prolonged analgesia. STUDY DESIGN: Patients aged 34 days to 15 years (n = 36) from 37 who consecutively referred to the institution with classical bladder exstrophy between 2004 and 2016 underwent modified delayed primary (25) or redo (11) closure. One boy with low weight was excluded. Patient and treatment features were analysed to determine needs for immobilisation and anaesthesia in the postoperative period, and outcomes. PROCEDURE: Bladder exstrophy closure with proximal urethroplasty was performed with the detachment of crura from the ishiopubic rami and levators-from obturator internus muscle. Abdominal wall closure was accomplished with skin and subcutaneous fat mobilisation without rectus fascia closure. No method of immobilization was applied. RESULTS AND LIMITATIONS: Bladder closures have been successful in all 36 children in this report after 37 months (22-138) follow up. The surgeries took time between 126 and 215 min (mean - 148). After 1 day in the ICU the majority of the patients (34/36) were returned to the ward. No bladder spasms or signs of acute pain were noted in the ward; therefore, no local anesthesia or opioids were needed. Intravenous analgesia with non-narcotic analgesics was used for all patients in the ward for an average period 2.2 days (95% CI 2-4 days). COMPLICATIONS: Minor complications: two fistulas, which closed spontaneously; three bladder outlet obstructions, each required one endoscopic incision. No major complications of exstrophy closure such as dehiscence or bladder prolapse were occurred. CONCLUSIONS: The proposed less invasive technique with relieved postoperative program is the way to obtain successful bladder exstrophy closure as well as to reduce some risks for the patients. Absence of major complications, and avoiding the need for immobilisation and prolonged analgesia, contribute to the benefits of this approach.

Failed Primary Bladder Exstrophy Closure with Osteotomy: Multivariable Analysis of a 25-Year Experience.

PURPOSE: Successful primary bladder exstrophy closure provides the best opportunity for patients to achieve a functional closure and urinary continence regardless of the method of repair. Use of osteotomy during initial closure has significantly improved success rates. However, failures can still occur. We identify factors that contribute to a failed primary exstrophy closure with osteotomy. MATERIALS AND METHODS: We reviewed a prospectively maintained institutional database for classic bladder exstrophy cases primarily closed with osteotomy at our institution or referred after primary closure between 1990 and 2015. Data were collected regarding patient gender, closure, osteotomy, immobilization, orthopedics and perioperative pain control. Univariate and multivariable analyses were performed to determine predictors of failure. RESULTS: A total of 156 patients met inclusion criteria. Overall failure rate was 30% (13% from our institution and 87% from referrals). On multivariable analysis use of Buck traction (OR 0.11, 95% CI 0.02-0.60, p = 0.011) and immobilization time greater than 4 weeks (OR 0.19, 95% CI 0.04-0.86, p = 0.031) had significantly lower odds of failure. Osteotomy performed by general orthopedic surgeons had significantly higher odds of failure (OR 23.47, 95% CI 1.45-379.19, p = 0.027). Type of osteotomy and use of epidural anesthesia did not significantly impact failure rates. CONCLUSIONS: Proper immobilization with modified Buck traction and external fixation, immobilization time greater than 4 weeks and undergoing osteotomy performed by a pediatric orthopedic surgeon are crucial factors for successful primary closure with osteotomy.

Sexual Function in Patients Operated on for Bladder Exstrophy and Epispadias.

PURPOSE: Bladder exstrophy and epispadias complex is a rare congenital malformation that may have detrimental effects on sexual function. We evaluated sexual function of patients with bladder exstrophy and epispadias complex using validated questionnaires and compared the results with age matched controls. MATERIALS AND METHODS: Patients with bladder exstrophy and epispadias complex treated between 1956 and 1992 were identified from our hospital operative database. A total of 63 patients were mailed questionnaires up to 3 times, resulting in 32 replies (51%). Men were mailed the International Index of Erectile Function-15 questionnaire and women were mailed the Female Sexual Function Index questionnaire, and all patients were asked auxiliary questions regarding children, satisfaction with external genitalia and urinary continence. RESULTS: There were no differences in sexual function between sexually active men with bladder exstrophy and epispadias complex and age matched controls on different erectile function domain scores according to the International Index of Erectile Function-15 questionnaire. The Female Sexual Function Index questionnaire among women yielded comparable results on different domain scores, except for median total score, which was higher in patients (33.6, IQR 29.4 to 34.9) than in controls (30.1, IQR 26.4 to 32.4, p = 0.049), suggesting better sexual function in patients with bladder exstrophy and epispadias complex. A greater proportion of patients with bladder exstrophy and epispadias complex had not become sexually active, compared to controls (35% vs 11%, p = 0.008). Patients with bladder exstrophy and epispadias complex were less likely to have children than controls (22% vs 45%, p = 0.021). CONCLUSIONS: Men and women with bladder exstrophy and epispadias complex had good long-term outcomes on erectile and general sexual function tests. However, there is a likelihood that these individuals start their sexual life later than the general population, and fewer have children compared to controls.

Classic bladder exstrophy: Frequent 22q11.21 duplications and definition of a 414 kb phenocritical region.

BACKGROUND: Classic bladder exstrophy (CBE) is the most common form of the bladder exstrophy and epispadias complex. Previously, we and others have identified four patients with a duplication of 22q11.21 among a total of 96 unrelated CBE patients. METHODS: Here, we investigated whether this chromosomal aberration was commonly associated with CBE/bladder exstrophy and epispadias complex in an extended case-control sample. Multiplex ligation-dependent probe amplification and microarray-based analysis were used to identify 22q11.21 duplications in 244 unrelated bladder exstrophy and epispadias complex patients (including 217 CBE patients) and 665 healthy controls. RESULTS: New duplications of variable size were identified in four CBE patients and one control. Pooling of our previous and present data (eight duplications in 313 CBE patients) yielded a combined odds ratio of 31.86 (95% confidence interval, 4.24-1407.97). Array-based sequence capture and high-throughput targeted re-sequencing established that all breakpoints resided within the low-copy repeats 22A to 22D. Comparison of the eight duplications revealed a 414 kb phenocritical region harboring 12 validated RefSeq genes. Characterization of these 12 candidate genes through whole-mount in situ hybridization of mouse embryos at embryonic day 9.5 suggested that CRKL, THAP7, and LZTR1 are CBE candidate genes. CONCLUSION: Our data suggest that duplication of 22q11.21 increases CBE risk and implicate a phenocritical region in disease formation.

Short-term outcomes of the multi-institutional bladder exstrophy consortium: Successes and complications in the first two years of collaboration.

INTRODUCTION/BACKGROUND: Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care. OBJECTIVE: Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term. STUDY DESIGN: Boston Children's Hospital, Children's Hospital of Philadelphia and Children's Hospital of Wisconsin alternately served as the host, with observation, commentary and critique by visiting collaborating surgeons. CPRE with bilateral iliac osteotomy was performed at 1-3 months of age. High-definition video capture of the surgery allowed local and distant broadcast to facilitate real-time observation and teaching, and recording of all procedures. RESULTS: From February 2013 to February 2015, MIBEC participating surgeons performed CPRE on 27 consecutive patients (22 classic BE, five epispadias). There were no dehiscences in 27 patients (0%, 95% CI 0-12.5%). Thirteen girls and 14 boys underwent CPRE at a median age of 2.3 months (range 0.1-51.6). One boy had a hypospadiac urethral meatus at CPRE completion. Hydronephrosis of mild or moderate grade was present postoperatively in eight girls and two boys. Additional results, per gender, are presented in the Summary table below. DISCUSSION: Absence of dehiscence in this cohort was comparable or compared favorably with the literature. However, several girls had significant obstructive complications following CPRE. The rate of bladder outlet obstruction (BOO) in girls was increased compared with published reports. A low complication rate was noted in the boys following CPRE, which was comparable to reports in the literature, and early signs of continence and spontaneous voiding were noted in some boys and girls. Limitations included variation in patient age at presentation, thereby introducing a wide age range at CPRE. Outcome data were limited by short follow-up regarding voiding with continence. CONCLUSION: This collaborative effort proved beneficial regarding significantly increased surgeon exposure to CPRE, refinement of CPRE technique, surgeon learning and expertise. Technical refinement of CPRE is ongoing.

Complications of bladder closure in cloacal exstrophy: Do osteotomy and reoperative closure factor in?

BACKGROUND/PURPOSE: The aims of surgical management in cloacal exstrophy (CE) have shifted to optimizing outcomes and quality of life while minimizing morbidity. This report reviews the single-institution experience of complications of bladder closure in CE. METHODS: Patients with CE were identified from a prospectively-maintained bladder exstrophy-epispadias complex database. Operative and follow-up data were analyzed to compare complications and failure rates of bladder closure between closures performed with and without osteotomy and primary versus reoperative closures. RESULTS: Of 134 patients followed with CE, 112 met inclusion criteria. Median follow-up time was 3.05years. The failure rate among 112 primary closures (mean age 8.4months) was 31.3% versus 51.9% in reoperative closures (mean age 19.7months) (p=0.044). Complication rate among primary and reoperative closures was 17.9% and 33.3%, respectively (p=0.076). For closures with pelvic osteotomy, failure rate was 24.0% versus 45.9% without osteotomy (p=0.018). Among primary closures with osteotomy, the complication rate was 21.3% versus 10.8% without osteotomy (p=0.171). CONCLUSIONS: Complications of bladder closure are common in CE. Pelvic osteotomy reduces failure rates without a significant rise in complications, which are often minor. There was no statistically significant difference in complication rates between reoperative and primary closures. However, reoperative closures were more likely to fail, emphasizing the importance of a successful primary closure. LEVEL OF EVIDENCE: II: retrospective study.