Bladder capacity and growth in classic bladder exstrophy: A novel predictive tool.

INTRODUCTION: Bladder capacity (BC) is an important metric in the management of patients with classic bladder exstrophy (CBE). BC is frequently used to determine eligibility for surgical continence procedures, such as bladder neck reconstruction (BNR), and is associated with the likelihood of achieving urinary continence. OBJECTIVE: To use readily available parameters to develop a nomogram that could be used by patients and pediatric urologists to predict BC in patients with CBE. STUDY DESIGN: An institutional database of CBE patients was reviewed for those who have undergone annual gravity cystogram 6 months after bladder closure. Candidate clinical predictors were used to model BC. Linear mixed effects models with random intercept and slope were used to construct models predicting log transformed BC and were compared with adjusted R2, Akaike Information Criterion (AIC), and cross-validated mean square error (MSE). Final model evaluated via K-fold cross-validation. Analyses were performed using R version 3.5.3 and the prediction tool was developed with ShinyR. RESULTS: In total, 369 patients (107 female, 262 male) with CBE had at least one BC measurement after bladder closure. Patients had a median of 3 annual measurements (range 1-10). The final nomogram includes outcome of primary closure, sex, log-transformed age at successful closure, time from successful closure, and interaction between outcome of primary closure and log-transformed age at successful closure as the fixed effects with random effect for patient and random slope for time since successful closure (Extended Summary). DISCUSSION: Using readily accessible patient and disease related information, the bladder capacity nomogram in this study provides a more accurate prediction of bladder capacity ahead of continence procedures when compared to the age-based Koff equation estimates. A multi-center study using this web-based CBE bladder growth nomogram (https://exstrophybladdergrowth.shinyapps.io/be_app/) will be needed for widespread application. CONCLUSION: Bladder capacity in those with CBE, while known to be influenced by a broad swath of intrinsic and extrinsic factors, may be modeled by the sex, outcome of primary bladder closure, age at successful bladder closure and age at evaluation.

Volumetric and acetabular changes in the bony pelvis associated with primary closure of classic bladder exstrophy.

INTRODUCTION: Repair of classic bladder exstrophy (CBE) is known to alter dimensions of the bony pelvic ring. Pelvic volume and acetabular configuration are additional metrics which merit analysis in the reconstruction process. Advances in magnetic resonance imaging (MRI) allow for precise elucidation of such anatomy in pediatric patients, providing enhanced knowledge of how primary reconstruction may impact factors in pelvic health. METHODS: An IRB-approved exstrophy-epispadias database of 1337 patients was reviewed for patients with CBE who had pelvic MRI performed before and after repair. Pelvic MRIs were analyzed by a pediatric radiologist, and three-dimensional volumetric renderings of the true pelvis were calculated. Pre- and post-closure imaging were compared, in addition to imaging from age-matched controls without pelvic pathology. Cartilaginous acetabular index and version angles were also calculated and compared between groups. RESULTS: Eighteen patients with post-closure imaging, 14 of whom also had pre-closure imaging, and 23 control patients (ages 0-365 days) were included. The median ages at pre- and post-closure scan were 2 and 178 days, respectively. Osteotomy was performed in 6 (33%) of the closures. The median segmented pelvic volumes were 89 cm3 in the pre-closure group, 105 cm3 in the post-closure group, and 72 cm3 in the control group. At a given age, patients with CBE pre-closure had the largest pelvic volume, and those without bladder exstrophy demonstrated the smallest pelvic volumes (Summary Figure). CBE patients' pelvic volumes were overall lower following repair, relative to age (p = 0.007). Cartilaginous acetabular version angle increased following closure, with acetabular orientation converting from retroversion to anteversion in all cases. However, only acetabular version angles were significantly different between groups. DISCUSSION: This study found that pelvic volume significantly decreased relative to age following primary repair of CBE, but that it did not correct to control levels. Similarly, acetabular retroversion that is naturally seen in exstrophy patients was converted to anteversion post-closure but did not become completely normal. Knowledge of these persistent anatomical anomalies may be useful in treatment of future concerns related to exstrophy, such as continence achievement, pelvic organ prolapse, and potential gait disturbances. Lack of significant differences between study groups regarding acetabular index angles can give assurance to providers that hip dysplasia is neither a natural concern nor iatrogenically inflicted through reconstruction. CONCLUSIONS: The primary closure of exstrophy results in pelvic volumes and anteverted acetabula that more closely resemble those without bladder exstrophy, compared to pre-closure findings.

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.

Urinary Continence Outcomes in Classic Bladder Exstrophy: A Long-Term Perspective.

PURPOSE: We investigated surgical approaches to urinary incontinence and long-term continence outcomes after successful bladder reconstruction in a heterogeneous patient population with classic bladder exstrophy. We hypothesized that while most patients will achieve urinary continence after surgery, only a select group will void volitionally per urethra. MATERIALS AND METHODS: An institutional database of 1,323 patients with exstrophy-epispadias complex was reviewed for patients with classic bladder exstrophy who underwent successful bladder closure and a subsequent continence procedure between 1975 and 2017. Procedures included bladder neck reconstruction, bladder neck reconstruction with augmentation cystoplasty or continent catheterizable stoma, and bladder neck closure with continent catheterizable stoma. Cloacal exstrophy, epispadias and variant exstrophy cases were excluded from analysis. Continence at last followup was defined as a dry interval of 3 or more hours without nighttime leakage. Those patients with more than 3 months of followup were assessed. RESULTS: Overall 432 patients underwent successful bladder closure (primary 71.5%, repeat 28.5%) and a urinary continence procedure. At last followup 162 (37%) underwent bladder neck reconstruction, 76 (18%) underwent bladder neck reconstruction with augmentation cystoplasty or continent catheterizable stoma, 173 (40%) underwent bladder neck closure with continent catheterizable stoma and 18 underwent other procedures. Median followup from the first continence procedure was 7.2 years (IQR 2.3-13.7). Continence was assessed in 350 patients. After isolated bladder neck reconstruction 91 of 142 patients were continent (64%, 95% CI 56-72). After bladder neck closure with continent catheterizable stoma 124 of 133 patients evaluated were continent (93%, 95% CI 87-97). CONCLUSIONS: Most patients with classic bladder exstrophy require multiple reconstructive procedures to achieve continence. Only about 25% of patients are expected to void normally per urethra without reliance on catheterization or urinary diversion.

Opioid tolerance impacts compliance with enhanced recovery pathway after major abdominal surgery.

BACKGROUND: Opioid tolerant patients have been shown to have poor postoperative outcomes. Enhanced recovery pathways are evidence-based interventions that focus on optimizing recovery, and their effectiveness depends on the degree of compliance with the pathway. We wish to determine pathway compliance and its impact on postoperative outcomes in opioid tolerant patients undergoing abdominal surgery on an enhanced recovery pathway. METHODS: From December 2014 to June 2017, 646 patients undergoing major abdominal surgery on an enhanced recovery pathway were included. Patients <18 years and having emergency surgery were excluded. Compliance was measured to 14 perioperative pathway standards and high-compliance was defined as adhering to ≥75% standards. Opioid tolerance was defined as any patient taking a prescribed opioid medication equivalent to 60 mg of oral morphine per day for 1 week prior to surgery. The Colorectal Physiological and Operative Severity Score for the Enumeration of Mortality and Morbidity was used for risk-adjusted analyses. Outcomes of interest include length of stay, major complications (Clavien-Dindo ≥2), 30-day readmission rates, and mortality. RESULTS: Overall, 114 (18%) patients were opioid tolerant and 532 (82%) were not opioid tolerant. Opioid tolerant patients were less likely to be highly compliant with enhanced recovery pathway standards than non-tolerant patients (35% vs 54%; P < .001); particularly postoperative care standards. On adjusted analysis, opioid tolerance was associated with a 2-fold increase in readmissions following major abdominal surgery. Examining only those patients with opioid tolerance, adjusted analysis demonstrated that high compliance with the enhanced recovery pathway standards was independently associated with a 26% reduction in length of stay, over a 90% reduction in major complications, and mitigated the effect on readmissions. CONCLUSION: The authors provide evidence that opioid tolerance is associated with less favorable outcomes in patients undergoing major abdominal surgery on an enhanced recovery pathway, and this is likely due to a lack of pathway compliance. Establishing strategies to improve compliance in this challenging patient cohort may serve to mitigate the negative impact of opioid tolerance.

Anatomy of Classic Bladder Exstrophy: MRI Findings and Surgical Correlation.

PURPOSE OF REVIEW: The exstrophy-epispadias complex (EEC) represents a group of congenitally acquired malformations involving the musculoskeletal, gastrointestinal, and genitourinary systems. Classic bladder exstrophy (CBE) is the most common and best studied entity within the EEC. In this review, imaging features of CBE anatomy will be presented with surgical correlation. RECENT FINDINGS: Magnetic resonance imaging (MRI) has emerged as a useful modality for pre- and postnatal assessment of the abdominal wall, pelvic floor, and gastrointestinal and genitourinary systems of children with CBE. The authors' experience supports use of preoperative MRI, in conjunction with navigational software, as a method for identifying complex CBE anatomy. Imaging facilitates surgical approach and improves visualization of complex anatomy, potentially helping to avoid complications. Continued investigation of imaging guidance in CBE repair is needed as surgical techniques improve.

Exploration of Practice Patterns in Exstrophy Closures: A Comparison Between Surgical Specialties Using a National and Institutional Database.

OBJECTIVE: To compare the surgical subspecialties performing bladder exstrophy closures and characterize their practice patterns using both a national and institutional database. METHODS: The National Surgical Quality Improvement Program Pediatric (NSQIPP) database was reviewed for all bladder exstrophy closures performed from 2012 to 2017. A single institutional exstrophy-epispadias complex database of 1337 patients was reviewed for patients with a bladder closure at a referring institution from 1975 to 2018. Patients with cloacal exstrophy were excluded. The subspecialties of the surgeons performing the closures were identified. Practice patterns such as the use of a pelvic osteotomy and postoperative immobilization, and perioperative outcomes were compared for each subspecialty group. RESULTS: A total of 84 bladder exstrophy patients from NSQIPP and 263 from the author's institutional database met the inclusion criteria. From NSQIPP, 88% of closures were performed by pediatric urologists while 12% were done by other subspecialists. From the institutional database, 75% of referred bladder exstrophy closures were done by a pediatric urologist, and 25% by other services. Gender, race, operation time, length of stay, and postoperative complications were not significantly different between the groups. In one database, pediatric surgeons performed closures earlier, and in another database, pediatric urologists had greater utilization of osteotomy with different immobilization techniques. Pediatric urologists had a higher success rate. CONCLUSION: Pediatric urologists performed the most bladder exstrophy closures in both databases; they operated on more delayed closures with a greater use of adjunctive procedures and a higher success rate. Differences in surgical training may contribute to the differences in practice patterns.