Heather N. Di Carlo

Modern Management of the Exstrophy-Epispadias Complex

The exstrophy-epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair epispadias, classic bladder exstrophy, and cloacal exstrophy have increased the success of achieving urinary continence, satisfactory cosmesis, and quality of life. Unfortunately, these procedures are not without their own complications. This review provides readers with an overview of the management of the exstrophy-epispadias complex and potential surgical complications.

Modern Management of Bladder Exstrophy Repair

The exstrophy–epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall musculature, and pelvic bones. Historically, surgical outcomes in patients with classic bladder exstrophy, the most common presentation of the exstrophy–epispadias complex, were poor. However, modern techniques have increased the success of achieving urinary continence, satisfactory cosmesis, and improved quality of life. Still, recent studies recognize complications that may occur during management of these patients. This review provides readers with an overview of the exstrophy–epispadias complex, the modern management of bladder exstrophy, and potential surgical complications.

Initial bladder closure of the cloacal exstrophy complex: Outcome related risk factors and keys to success

PURPOSE This study examines a large single-institution experience with cloacal exstrophy patients, analyzing patient demographics and surgical strategies predictive of bladder closure outcomes. METHODS One hundred patients with cloacal exstrophy were identified. Complete closure history including demographics, operative history, and outcomes was available on 60 patients. Twenty-six patients with a history of failed initial bladder closure were compared to 34 with a history of successful initial bladder closure. Univariate logistic regression analysis was used to compare the two groups. RESULTS Median follow up time after initial closure was 9years (range: 13months-29years). A 1cm increase in pre-closure diastasis resulted in a 2.64 increase in the odds of initial closure failure (p=0.004). Protective strategies against failure included delaying closure (per month) (OR=0.894, p=0.009), employing pelvic osteotomies (OR=0.095, p<0.001), and applying external fixation (OR=0.024; p=0.001). Among patients who underwent osteotomy (31% of patients in the failed group, 82% in the successful group), a longer delay between osteotomy and closure (OR=0.033; p=0.005) was also protective against failure. CONCLUSION Patients with a large diastasis are more likely to fail initial closure. Delaying initial closure for at least 3months, performing pelvic osteotomy, and using an external fixation device post-operatively are strategies that improve closure success.

Urologic Considerations and Complications in Kidney Transplant Recipients

Urologic considerations during the kidney transplantation process, starting with initial recipient evaluation and continuing through the post-transplant, long-term follow-up, are critical for minimizing urologic complications and improving graft survival. Appropriate, targeted, preoperative urologic evaluation of the recipient allows for an optimized urinary tract to accept the graft, whereas post-transplant urologic follow-up and monitoring decrease the risk of graft lost secondary to a urologic cause, particularly in patients with a urologic reason for their kidney failure and in those patients with concomitant urologic diagnoses. Urologic complications comprise the second most common adverse post-transplant event, occurring in 2.5% to 14% of patients and are associated with high morbidity, graft loss, and mortality. Early and late urologic complications, including hematuria, hematoma, lymphocele, urine leak, ureteral stricture, nephrolithiasis, and vesicoureteral reflux, and their causes and treatment options are explored. A multidisciplinary team approach to kidney transplantation, including transplant surgery, urology, and nephrology, optimizes outcomes and graft survival. Although the current role of the urologist in kidney transplantation varies greatly by institution, appropriate consultation, participation, and monitoring in select patients is essential.

Long-term sexual health outcomes in men with classic bladder exstrophy

To identify the long‐term sexual health outcomes and relationships in men born with classic bladder exstrophy (CBE).

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.

The Fate of the Complete Female Epispadias and Exstrophy Bladder—Is There a Difference?

PURPOSE Complete female epispadias, which occurs much more rarely than classic bladder exstrophy in females, is thought to have a more benign clinical course. We hypothesized that patients with complete female epispadias are more likely to have a larger bladder capacity and achieve voiding continence than females with classic bladder exstrophy. MATERIALS AND METHODS After obtaining institutional review board approval, females with complete female epispadias or classic bladder exstrophy were identified from an institutionally approved prospective database. We retrospectively reviewed the charts of 22 patients with complete female epispadias and 23 with female classic bladder exstrophy, including 3 with delayed primary closure. RESULTS Patients with complete female epispadias presented later and underwent the first reconstructive procedure at an older age than patients with classic bladder exstrophy. Patients with complete female epispadias had lower initial and final age adjusted bladder capacity than those with classic bladder exstrophy but the bladder growth rate did not differ between the groups. When patients with complete female epispadias were stratified by age at initial reconstruction, there was no difference in final age adjusted bladder capacity or the bladder growth rate. There was also no statistical difference between the groups in the number of surgeries, continence rate from initial reconstruction, bladder neck reconstruction success or need for a continent stoma. CONCLUSIONS This study suggests that females with classic bladder exstrophy who undergo successful primary closure have higher initial and final age adjusted bladder capacity than females with complete female epispadias but with a similar growth rate. This may reflect the creation of outlet resistance at a younger age than in those with complete female epispadias. However, no difference was identified between patients with complete female epispadias who initially underwent repair before vs after age 1 year. Patients with complete female epispadias undergo procedures to achieve continence that are similar to those in patients with classic bladder exstrophy.

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.

Use of muscle pedicle flaps for failed bladder neck closure in the exstrophy spectrum

PURPOSE The authors have reviewed the use of muscle pedicle flaps for the treatment of failed bladder neck closure in exstrophy spectrum patients. METHODS A retrospective review of all exstrophy spectrum patients who underwent continence procedures with the use of muscle pedicle flaps at our institution during the last 15 years was performed. Patient characteristics, surgical history, and outcomes, including complications, continence, morbidity, and infection, were assessed. The authors utilized muscle pedicle flaps in eight exstrophy patients, including four patients with classic bladder exstrophy and four patients with cloacal exstrophy. Seven of eight patients had failed at least one prior bladder neck closure, and they had undergone a median of three prior urologic procedures. To achieve continence, five rectus muscle flaps and three gracilis muscle flaps were utilized in combination with bladder neck closure. RESULTS There were no major intraoperative or postoperative complications. All patients were initially continent, and after a median follow-up of 18.7 months seven of eight patients were continent. One patient required continent urinary stoma revision and one patient developed perineal incontinence after perineal trauma. No patients required revision of, or additional, continence procedures at the bladder neck. DISCUSSION The use of pedicle muscle flaps appears to be a safe and feasible option for exstrophy spectrum patients with failed bladder neck closure. Although achieving continence can be difficult in this population, use of muscle flaps and bladder neck closure is a viable and effective option in this challenging subset of patients.

New advances in the pathophysiologic and radiologic basis of the exstrophy spectrum.

The exstrophy-epispadias complex is a rare spectrum of anomalies affecting the genitourinary system, anterior abdominal wall, and pelvis. Recent advances in the repair of classic bladder exstrophy (CBE) and cloacal exstrophy (CE) have resulted in significant changes in outcomes of surgical management (including higher continence rate, fewer surgical complications, and better cosmesis) and health-related quality of life in these patients. These noteworthy changes resulted from advances in the pathophysiological and genetic backgrounds of this disease and better radiologic assessment of the three-dimensional anatomy of the bony pelvis and its musculature. A PubMed search was performed with the keyword exstrophy. The resulting literature pertaining to genetics, stem cells, imaging, tissue engineering, epidemiology, and endocrinology was reviewed. The following represents an overview of the advances in basic science understanding and imaging of the exstrophy-epispadias spectrum and discusses their possible and future effects on the management of CBE and CE.