New Insights on the Basic Science of Bladder Exstrophy-epispadias Complex.

The exstrophy-epispadias complex is a rare congenital anomaly presenting as a wide spectrum of disorders. The complex nature of this malformation leads to continuous investigations of the basic science concepts behind it. Elucidating these concepts allows one to fully understand the mechanisms behind the disease in order to improve diagnosis, management, and treatment ultimately leading to improvement in patient quality of life. Multiple technological advancements within the last 10 years have been made allowing for new studies to be conducted. Herein, the authors conduct a literature review of studies from 2009 to 2019, considering novel theories regarding the genetics, embryology, bladder, bony pelvis, prostate, and genitalia of patients with bladder exstrophy-epispadias complex.

Osteotomy in the newborn classic bladder exstrophy patient: A comparative study.

INTRODUCTION: Pelvic osteotomy is indicated in classic bladder exstrophy (CBE) patients with a wide pubic diastasis or non-malleable pelvis. While the safety of pelvic osteotomy in delayed and failed closures is established, there remains less clarity on their safety in newborns. The authors herein sought to present their experience with CBE patients who underwent pelvic osteotomy for assistance with bladder closure during both the newborn and delayed time periods. OBJECTIVE: The authors hypothesize that pelvic osteotomy during exstrophy closure may be performed safely in newborns with few perioperative or post-operative negative sequelae. STUDY DESIGN: A prospectively maintained IRB-approved database was reviewed for CBE patients who underwent osteotomy during primary closure. Patient demographics, performing institution (authors' or outside), closure outcome, diastasis width, and post-operative complications were noted. Patient subgroups included newborn and delayed (>28 days of life) closures. Failure was defined as bladder dehiscence, prolapse, outlet obstruction, or vesicocutaneous fistula requiring reoperation. Orthopedic complications included nerve palsies, superficial pin-site infection, and bladder neck erosion by orthopedic hardware. Analyses were performed using a Chi-square test. RESULTS: 286 patients were included: 186 newborn and 100 delayed closures. The authors' institution performed 109 cases (44 newborn and 65 delayed). Within the overall newborn closure cohort, no significant differences were found in outcomes among the osteotomy types with success rates of 80%, 60.8%, and 71.4% in the combined, posterior iliac, and anterior innominate groups, respectively (p = 0.24). In the delayed group, success rates were significantly different with rates of 100%, 72.4%, and 93.8% in the combined, posterior iliac, and anterior innominate groups, respectively (p < 0.001). Febrile urinary tract infection (UTI) was the most common complication at 8% (23/286). Only 1.7% (5/286) of patients had orthopedic complications with 3 patients in the newborn cohort, 2 patients in the delayed cohort, and only one patient requiring reoperation. DISCUSSION: Orthopedic complications are rare in CBE patients who undergo osteotomies regardless of the closure period. No clinically significant difference in orthopedic complication rate was found between newborn and delayed closure periods. CONCLUSIONS: While current trends have moved toward delayed primary closures, there remains a role for osteotomy during exstrophy closure in select newborn patients and can be performed safely with few complications.

Practice patterns in classic bladder exstrophy: A global perspective.

INTRODUCTION: While evaluation and management options for classic bladder exstrophy (CBE) patients are numerous and varied, little is known regarding the relative utilization of these different methods throughout the world. A large group of exstrophy surgeons practicing globally was surveyed, seeking to document their methods of care. METHODS: A list of international exstrophy surgeons' email addresses was compiled using professional contacts and referral networking. An online survey was sent to each email address. Surgeons who had not performed a CBE closure within the previous 5 years were excluded. Survey questions queried the respondents' surgical practice type, years since training, and their preferred methods of preoperative evaluation, operative management, and postoperative management. Survey invitations were sent out starting in December 2014 and responses were collected for approximately 6 months. RESULTS: A total of 1152 valid email addresses were invited, resulting in 293 respondents (25%) from 39 countries and every American Urological Association (AUA) section. Seventy-six were excluded, leaving 217 respondents (Table). Respondents reported a median of 17 years since finishing their surgical training (IQR 8-25 years). Practice types included pediatric urology (n = 209), general urology (n = 9), pediatric surgery (n = 59), and other practice makeup (n = 3). On subgroup analyses, there were no significant regional practice differences, with the exception of complete primary repair of exstrophy (CPRE) and oral opioid prescribing being significantly higher in North America compared to other regions. DISCUSSION: Findings indicate that there may be diversity in CBE practice patterns globally. While most responding surgeons from regions outside of North America indicated modern staged repair of exstrophy (MSRE) as their preferred closure technique, a relatively equal distribution of respondents from North America selected CPRE and MSRE. A majority of North American surgeons chose performing osteotomies for both newborn and delayed closures, while an appreciable number of respondents from other regions selected never using osteotomies in their closures. Limitations to this study include a low survey response rate, particularly from surgeons outside of the United States, which may have significantly impacted the ability to draw meaningful global comparisons. CONCLUSIONS: Global variation among practices of surgeons performing CBE closures may exist. The wide range of methods demonstrated by this survey suggests the need for more conclusive comparative studies to elucidate whether an optimal standard exists. Local social factors, access to surgical expertise and transportation to referral centers, and finances play a role in what constitutes the best operative approach.