Closure Of Classic Bladder Exstrophy Research Articles

Classic Bladder Exstrophy Closure Without Osteotomy or Immobilization: An Exercise in Futility?

To review the outcomes of classic bladder exstrophy (CBE) closure without the use of osteotomy or lower extremity/pelvic immobilization. A prospectively maintained institutional approved exstrophy-epispadias complex database of 1487 patients was reviewed for patients with CBE who had undergone closure without osteotomy nor immobilization. All patients were referred to the authors' institution for reconstruction later in life or for failed closure. Of a total of 1016 CBE patients, 56 closure events were identified that met inclusion with a total of 47 unique patients. Thirty-eight closures were completed prior to 1990 (67.9%). Forty-five closure events developed eventual failure (45/56, 80.4%) (Table 1). Thirteen closure events were secondary closures (13/56, 23.2%). The primary closure failure rate was 83.7% (36/43) while the secondary closure failure rate was 69.2% (9/13). Failures were attributed to one or more of dehiscence, bladder prolapse, and vesicocutaneous fistula (25/45, 55.6%) (23/45, 51.1%) (6/45, 13.3%), respectively. Thirty-seven patients developed social continence (37/47, 78.7%), while only 8 patients developed spontaneous voided continence (7/47,17.0%) (Table 2). The most common methods of voiding were continent catheterizable channels (25/47, 53.2%) of which all were socially continent. These results illustrate the critical role osteotomy and postoperative immobilization can play in both primary and secondary exstrophy closure. While this is a historical case series, the authors believe that these results remain relevant to contemporary exstrophy surgeons.

A Single Center's Changing Trends in the Management and Outcomes of Primary Closure of Classic Bladder Exstrophy: An Evolving Landscape

To investigate management trends in a single institution with a large referral population in classic bladder exstrophy (CBE) over the past twenty years. An institutional database of 1415 exstrophy-epispadias complex patients was retrospectively reviewed for CBE patients with primary closure between 2000 and 2019. Osteotomy, location of closure, age of closure, and outcome of closures were reviewed. A total of 278 primary closures were identified, with 100 occurring at author's hospital (AH) and 178 at outside hospitals (OSH). Osteotomies were performed in 54% of cases at AH and 52.8% of cases at OSH. Osteotomy use increased over 20 years from 48.6% in 00's to 62.1% in 10's (p=0.046). The total success rate at AH was 96% and 62.9% at OSH. The median age at primary closure at AH increased from 5 days (00's) to 20 days (10's), compared to the OSH which increased from 2 days (00's) to 3 days (10's). Closure of CBE may be delayed for several reasons including insurance difficulties, transfer to another hospital, desire for second opinions, or surgeon preference. Delaying primary closure of bladder exstrophy gives families time to adjust lifestyle, arrange travel, and seek care at centers of excellence.

Complications of delayed and newborn primary closures of classic bladder exstrophy: Is there a difference?

The authors aim to compare single institutional 30-day complication rates between delayed and neonatal closure of classic bladder exstrophy (CBE). 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. 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). 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.

Delayed primary closure of bladder exstrophy without osteotomy: 12 year experience in a safe and gentle alternative to neonatal surgery

BackgroundSuccessful primary closure of bladder exstrophy is of utmost importance for bladder capacity and urinary continence. We evaluated our concept of delayed primary closure that challenges the role of neonatal surgery, pelvic osteotomy, and perioperative pain management. Material and MethodsWe reviewed the medical records of patients with classic bladder exstrophy (CBE) who had undergone delayed primary closure without osteotomy at our institution between January 2008 and May 2020. Data to be analyzed included patient demographics, intraoperative pelvic laxity, blood transfusion, postoperative ventilation time, requirement of pain medication, time to full feeds, length of ICU stay, postoperative complications, and total hospital stay. Results66 patients (44 boys) met the inclusion criteria. Mean age at surgery was 64.8 days (SD±24.7). Pelvic approximation < 5 mm was possible in 66 (100%) patients. Blood transfusion was required by 31 (47%) patients. 14 (21.2%) patients needed postoperative ventilation for a mean time of 2.7 h. 45 (68.2%) children required intravenous opioids in addition to an epidural catheter. Oral feeding started on average 17.6 h after surgery. Mean ICU stay was 1.3 day. The initial success rate of delayed closure was 93.9%. None of the patients had bladder dehiscence. Girls developed more often minor postoperative complications than boys (m/f: 12 [27.3%] vs. 8 [36.4%]. Mean overall time of hospitalization was 19 days (13–34 d). ConclusionDelayed primary closure of CBE without osteotomy but with continuous epidural blockage is a safe and promising procedure that has crucial advantages in the pre- and postoperative management of CBE. Level of EvidenceLevel III.

EDITORIAL COMMENT

This is an interesting offering that adds to our knowledge of the problems that can be incurred in managing an exstrophy female during pregnancy. The most interesting vignette is in the lack of uterine prolapse in those patients who had an old fashioned posterior iliac osteotomy. The newer combined transverse innominate and anterior iliac osteotomies with pelvic fixation do a much better job of transferring the major pelvic musculature from behind the anus to a more anterior position to better support the anterior pelvic compartment seen on three-dimensional magnetic resonance imaging (3D MRI). 1 Stec AA Tekes A Ertan G et al. Evaluation of pelvic floor muscular redistribution after primary closure of classic bladder exstrophy by 3-dimensional magnetic resonance imaging. J Urol. 2012; 188: 1535-1542 Crossref PubMed Scopus (23) Google Scholar Also, we have seen very few post Cesarean section prolapses in this group regardless of whether they void from below or have a continent reservoir. We tell these ladies they usually can have one delivery without prolapse but afterwards the risk does increase regardless of having a prior osteotomy. That being said we have performed sacroculpopexies for these women to have subsequent pregnancies if they desire. Although pelvic osteotomies is a morbid procedure in adults, the authors have performed pelvic osteotomies to facilitate uterine suspension in patients with failed prior attempts at repairing uterine prolapse. 2 Ansari MS Gearhart JP Cervellione RM Sponseller PD. The application of pelvic osteotomy in adult female patients with exstrophy: applications and outcomes. BJU Int. 2011; 108: 908-912 PubMed Google Scholar Recently reported 3D MRI-guided surgery combined with pelvic osteotomy may allow for better recapitulation of the pelvic floor thus decreasing the rate or prolapse in adult life. 3 Di Carlo HN Maruf M Massanyi EZ Shah B Tekes A Gearhart JP. 3-Dimensional magnetic resonance imaging guided pelvic floor dissection for bladder exstrophy: a single arm trial. J Urol. 2019; 202: 406-412 Crossref PubMed Scopus (5) Google Scholar More data in the future like this paper is needed as modern exstrophy surgery along with better pelvic reconstructive osteotomies will hopefully decrease the complication rate in this interesting group of patients.

Expression of Low Affinity Nerve Growth Factor Receptor p75 in Classic Bladder Exstrophy.

Successful primary closure of classic bladder exstrophy (BE) is crucial for development of bladder capacity and voided continence. It is universally agreed that an intensive pain management including the use of caudal epidural anesthesia is an essential cornerstone for the outcome of this complex surgery. Whether and to what extent pain is caused by structural or functional changes is not yet known. The nerve growth factor (NGF) is regarded as a marker for pain in different bladder disorders. This prospective study investigated the role of histological alterations and NGF in patients with BE including 34 patients with BE and 6 patients with congenital vesicoureterorenal reflux (VUR) who served as controls. Between January 2015 and April 2020 transmural bladder biopsies were taken from the posterior bladder wall during delayed primary bladder closure. The samples were stained for histological evaluation and subjected to immunohistochemistry to analyze NGFR p75. Differences in histological alterations were examined with Fisher's exact test, and Mann-Whitney-U-test was used to compare the NGFR p75 staining intensity between patients with BE and controls. Patients with BE showed significantly more often acute inflammation (p < 0.001), squamous metaplasia (p = 0.002), and cystitis glandularis (p = 0.005) as well as NGFR p75 in the urothelium (p = 0.003) than patients with VUR. A limitation of this study is the small number of participants due to the rare disease entity. Similar to other painful bladder disorders, pain transmission in BE after intitial closure may in part be facilitated by elevated NGF signaling through its receptor.

Editorial Comment on: Volumetric and acetabular changes in the bony pelvis associated with primary closure of classic bladder exstrophy

This manuscript examines a small subset of bladder exstrophy patients and describes pelvic bony anatomy. 18 patients underwent an MRI after closure, between 2 and 188 days after surgery, and 1/3 of these patients had osteotomies. All patients who were included had a successful closure, but there are no selection criteria provided. Fourteen patients had a pre and post closure MRI, and found that pelvic volumes increased, however, the authors admitted that this is likely due to normal patient somatic growth. Both pre and post closure pelvic volumes were different from controls. However, the authors demonstrate that acetabular angles more closely resembled controls after closure than pre-operatively. Volumetric and acetabular changes in the bony pelvis associated with primary closure of classic bladder exstrophyJournal of Pediatric UrologyVol. 16Issue 6PreviewRepair 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. Full-Text PDF

Delaying primary closure of classic bladder exstrophy: When is it too late?

With current trends towards delaying the closure of classic bladder exstrophy (CBE), bladder growth rate or ultimate capacity may be impacted. To examine consecutive bladder capacities in CBE patients who had primary closures at differing ages and determine whether there is an optimal age for closure, with reference to bladder capacity. A retrospective review was performed using an institutional database. CBE, successful neonatal (i.e. ≤28 days old) or delayed (i.e. >28 days old) primary closure, at least three consecutive bladder capacities or two measures taken 18 months apart, and first bladder capacity measured ≥3 months after closure. Only capacities prior to continence surgery and before 14 years of age were considered. Two cohorts were created: neonatal and delayed closure. To account for repeated measurements per patient, a linear mixed model evaluated effects of age and length of delay on bladder capacity based on closure cohort. Individuals in the delayed closure group were further stratified into quartiles to assess for detriment to the bladder based on length of delay. The cohort included 128 neonatal and 38 delayed patients. Median age at closure for the delayed group was 193 days (IQR 128-299). Based on univariate analysis, for the first three capacity measurements, the delayed group had significantly lower capacities despite having a similar median age when the measurements were taken. Linear mixed effects model showed significantly decreased total bladder capacity in delayed closure compared to neonates. The 2nd and 4th quartile groups had the most significant decreases in capacity. Time points for the most significant decline appear after the 2nd and 4th quartiles, representing 4-6 months and beyond 9 months, respectively. From this, the authors theorize that the appropriate time to close an exstrophy patient is as early as possible (1st quartile), or, if a delay is needed for growth of a bladder template, then between 6 and 9 months (3rd quartile). There may be a detriment to growth rate, however, statistical power may be lacking to discern this. Study limitations include the single-centered, retrospective design. However, results described here fill an important deficit in the knowledge of managing CBE. All patients in the delayed bladder closure group demonstrated a decline in bladder capacity compared to the control neonatal closure group, with significant differences in the 2nd and 4th quartiles. Thus, closing the bladder prior to nine months of age is recommended.

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

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. 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. 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 89cm3 in the pre-closure group, 105cm3 in the post-closure group, and 72cm3 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. 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. 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

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. 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. 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. 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. 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.

Modern Management of the Failed Bladder Exstrophy Closure: A 50-yr Experience

BackgroundA failed closure of classic bladder exstrophy (CBE) has a negative long-term impact on the patient and the health care system. ObjectiveTo investigate the outcomes of CBE patients with failed primary bladder closure. Design, setting, and participantsA database of 1317 exstrophy-epispadias complex patients was retrospectively reviewed for CBE patients with failed primary bladder closure from 1965 to 2017 with subsequent repeat closure. InterventionRepeat bladder exstrophy closure and subsequent continence procedure. Outcome measurements and statistical analysisFailed exstrophy closures are defined as occurrence of bladder prolapse, dehiscence, vesicocutaneous fistula, outlet obstruction, or combination of these factors. Successful repeat closures are defined as closures that require no further operative intervention as a consequence of these factors. Kaplan-Meier to determine time to successful repeat closure and receiver operator characteristic curve to determine the optimal time for secondary closure were determined. Results and limitationsIn total, 170 CBE patients had at least one repeat closure following a failed primary closure (115 male/55 female). With continued closure attempts, 166/170 (97.6%) patients were successfully closed. The median time to successful closure from birth was 12.9 mo (95% confidence interval: 11.7–15.7). Furthermore, 52/153 (34%) patients had more than one osteotomy. Of 215 total osteotomies, 50 (29.4%) were performed during the 170 failed primary closures, 128 (75.3%) during the 170second closures, and 27 (64.3%) during the 42 third closures. Of 96 patients with available continence data, 74 (77.1%) achieved urinary continence. ConclusionsA successful repeat closure is possible, especially when used in conjunction with a pelvic osteotomy. Continent urinary diversion yielded the highest continence rate in this cohort. Patient summaryWe looked at outcomes of classic bladder exstrophy closure in a large population. Successful repeat closure is possible in the majority of cases when used with pelvic osteotomy. A majority of patients achieved urinary continence using a continent diversion.

Reply by Authors

No AccessJournal of UrologyPediatric Urology1 Dec 2018Reply by Authors View All Author Informationhttps://doi.org/10.1016/j.juro.2018.06.093AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "Reply by Authors." The Journal of Urology, 200(6), p. 1361 References 1 Di Carlo H, Tekes A and Gearhart JP: Intraoperative 3D MRI guided pelvic dissection in the closure of classic bladder exstrophy: a novel approach. Presented at annual meeting of European Society for Paediatric Urology, Prague, Czechoslovakia, October 14-17, 2015. Google Scholar 2 Kasprenski M, Maruf M, Jayman J et al: Penile disassembly in exstrophy: time to reevaluate? J Urol, suppl., 2018; 199: e326, abstract MP25-02. Google Scholar © 2018 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 200Issue 6December 2018Page: 1361 Advertisement Copyright & Permissions© 2018 by American Urological Association Education and Research, Inc.MetricsAuthor Information Expand All Advertisement PDF downloadLoading ...

Predictors and outcomes of perioperative blood transfusions in classic bladder exstrophy repair: A single institution study

Primary bladder closure of classic bladder exstrophy (CBE) is a major operation that occasionally requires intraoperative or postoperative (within 72h) blood transfusions. This study reported perioperative transfusion rates, risk factors for transfusion, and outcomes from a high-volume exstrophy center in primary bladder closure of CBE patients. A prospectively maintained, institutional exstrophy-epispadias complex database of 1305 patients was reviewed for primary CBE closures performed at the authors' institution (Johns Hopkins Hospital) between 1993 and 2017. Patient and surgical factors were analyzed to determine transfusion rates, risk factors for transfusions, and outcomes. Patients were subdivided into two groups based upon the time of closure: neonatal and delayed closure. A total of 116 patients had a primary bladder closure during 1993-2017. Seventy-three patients were closed in the neonatal period, and 43 were delayed closures. In total, 64 (55%) patients received perioperative transfusions. No transfusion reactions were observed. Twenty-five transfusions were in the neonatal closure group, yielding a transfusion rate of 34%. In comparison, 39 patients were transfused in the delayed closure group, giving a transfusion rate of 91%. Pelvic osteotomy, delayed bladder closure, higher estimated blood loss (EBL), larger pubic diastasis, and longer operative time were all associated with blood transfusion. In multivariable logistic regression, pelvic osteotomy (OR 5.4; 95% CI 1.3-22.8; P<0.001), higher EBL-to-weight ratio (OR 1.3; 95% CI 1.1-1.6; P=0.029), and more recent years of primary closure (OR 1.1; 95% CI 1.0-1.2; P=0.018) remained independent predictors of receiving a transfusion (Summary Table). No adverse transfusion reactions or complications were observed. This was the first study from a single high-volume exstrophy center to explore factors that contribute to perioperative blood transfusions. Pelvic osteotomy as a risk factor was unsurprising, as the osteotomy may bleed both during and immediately after closure. However, it is important to use osteotomy for successful closure, despite the increased transfusion risk. The risks accompanying contemporary transfusions are minimal and osteotomies are imperative for successful bladder closure. More than half of CBE patients undergoing primary closure at a single institution received perioperative blood transfusions. While there was an association between transfusions and osteotomy, delayed primary closure, larger diastasis, increased operative time, and increased length of stay, only the use of pelvic osteotomy, higher EBL-to-weight ratio, and recent year of closure independently increased the odds of receiving a transfusion on multivariate analysis.

473 - Pelvic osteotomy in the newborn classic bladder exstrophy closure: Complications and outcomes

473 - Pelvic osteotomy in the newborn classic bladder exstrophy closure: Complications and outcomes

Reply by Authors

No AccessJournal of UrologyPediatric Urology1 Apr 2017Reply by Authors View All Author Informationhttps://doi.org/10.1016/j.juro.2016.09.157AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "Reply by Authors." The Journal of Urology, 197(4), p. 1143 References 1 Friedlander D, Sullivan B, Gearhart JP et al: Unpublished data. Google Scholar 2 Sullivan B, Friedlander D, Di Carlo H et al: Pelvic osteotomy in the newborn classic bladder exstrophy closure: complications and outcomes. Presented at European Association of Urology Congress, London, United Kingdom, March 26, 2017. Google Scholar © 2017 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 197Issue 4April 2017Page: 1143 Advertisement Copyright & Permissions© 2017 by American Urological Association Education and Research, Inc.MetricsAuthor Information Expand All Advertisement PDF downloadLoading ...

Peri-operative transfusion risk in classic bladder exstrophy closure: Results from a national database review

Classic bladder exstrophy (CBE) is one of the most complex pediatric urologic conditions, with patients often requiring multiple procedures throughout their lives. Patients undergoing these complex surgeries may require blood transfusion, exposing them to the risks of transfusion including antibody reactions, transmission of infectious diseases, and transfusion-related immunomodulation. We sought to determine the prevalence of and risk factors for peri-operative transfusion in patients undergoing closure for CBE. Because of the complexity of CBE management, we hypothesized that a significant number of patients undergoing closure of CBE would require peri-operative transfusion. Patients undergoing CBE closure between 2012 and 2014 were retrospectively identified by Current Procedure Terminology codes from The National Surgical Quality Improvement Program Pediatric database, which includes data from 64 participating hospitals. Patient demographics, pre-operative characteristics including comorbidities, intra-operative characteristics, and post-operative outcomes were analyzed for associations with transfusion requirement intra-operatively or in the first 72h post-operatively. Seventy-eight patients met our criteria, of whom 45 (57.7%) underwent transfusion. Patient characteristics and outcomes are noted in the Table. There was no difference between the groups in terms of age or gender. Patients who underwent transfusion were more likely to be over 3 days of age than those who did not (93.3% versus 75.8%; p=0.046). Transfused patients were also more likely to have undergone osteotomy(82.2% versus 48.5%; p=0.002), had an external fixation (46.7% versus 6.1%; p<0.001), had longer median operative times (447 versus 295min; p<0.001), and had longer median post-operative lengths of stay (LOS) (35 versus 17 days; p=0.003). There was no difference between the groups in terms of pre-operative risk factors or post-operative complications. A significant number of patients undergoing CBE closure required transfusion in the peri-operative period (57.7%). Patient characteristics found to have a higher rate of transfusion included osteotomy, external fixation, increased operative times, and longer post-operative LOS. In children undergoing closure for CBE, a large number require transfusion. The rate of transfusion is greater in older children and children undergoing osteotomy. Although osteotomy has a potentially important clinical role, especially in older patients, this study emphasizes the need for proper family counseling to include the increased likelihood of a blood transfusion and the risks associated with blood transfusion if osteotomies are performed.

Newborn exstrophy closure without osteotomy: Is there a role?

Recent articles document successful classic bladder exstrophy (CBE) closure without osteotomy. Still, many patients require osteotomy if they have a large bladder template and pubic diastasis, or non-malleable pelvis. To understand the indications and outcomes of bladder closure with and without pelvic osteotomy in patients younger than 1 month of age. An institutional database of 1217 exstrophy-epispadias patients was reviewed for CBE patients closed at the authors' institution within the first month of life. Patient demographics, closure history, pubic diastasis distance, bladder capacity, and outcomes were recorded and compared using chi-square tests between osteotomy and non-osteotomy patients. Failure was defined as bladder dehiscence, prolapse, vesicocutaneous fistula, or bladder outlet obstruction requiring reoperation. Bladder capacity >100 mL was deemed sufficient for bladder neck reconstruction (BNR). One hundred CBE patients were included for analysis: 38 closed with osteotomy (26 male, 12 female), and 62 closed without osteotomy (42 male, 20 female). There were four failed closures in the osteotomy group (2 dehiscence, 2 prolapse) and four failed closures in the non-osteotomy group (2 dehiscence, 2 prolapse). This corresponded to statistically equivalent rates of failure between the osteotomy and non-osteotomy groups (10.5% vs. 6.5%, p = 0.466). There was no statistically significant difference between the groups' ability to achieve bladder capacity sufficient for BNR (82% vs. 71%, p = 0.234). A successful primary bladder closure, regardless of the use of osteotomy, has been shown to be the single most important predictor of eventual continence. Because of the complexity of exstrophy manifestations, a multidisciplinary team approach is of the utmost importance. Based on our institutional experience, closure without osteotomy is considered when patients are <72 h of life, have a pubic diastasis <4 cm, malleable pelvis, and pubic apposition without difficulty. Rates of successful closure and attaining sufficient capacity for BNR were both statistically equivalent across groups. This retrospective study is limited by selection bias and the significant difference in follow-up time between groups. Nevertheless, as a high-volume exstrophy center this study draws from one of the largest cohorts available. Regardless of the type of closure undertaken, there clearly is a role for newborn CBE closure without pelvic osteotomy in patients considered suitable for closure by both the pediatric urologist and orthopedic consultant. However, if there is any doubt concerning pubic diastasis width, pelvic malleability, or ease of pubic apposition, an osteotomy is highly recommended.

Secondary Reclosure in Classic Bladder Exstrophy: Challenges and Outcomes

To analyze the outcomes of failed classic bladder exstrophy (CBE) reclosure with and without pelvic osteotomy. Each failed CBE closure decreases the chance of eventual continence. To minimize secondary failures, most institutions use pelvic osteotomy with reclosure. Reclosure with and without osteotomy can still fail. An institutional database of 1210 exstrophy-epispadias complex patients was reviewed for CBE patients who had 2 prior failed closures with the third closure at the authors' institution. Patient demographics, closure history, diastasis distance, bladder capacity, and outcomes were examined by chi-square tests comparing osteotomy status with first reclosure. Of 848 CBE patients, 17 met inclusion criteria: 12 with osteotomy at reclosure (group 1) and 5 without (group 2). Median time between initial closure and reclosure in the 2 groups were 6.5 months (range, 0-42 months) and 3 months (range, 0-59 months), respectively. There was no significant difference in the rate of attaining sufficient bladder capacity for bladder neck reconstruction (BNR; 100 cc) between groups 1 and 2 (42% vs 40%; P = .490). Within group 1, patients receiving proper immobilization with external fixation (n = 5) demonstrated a significantly greater rate of attaining sufficient bladder capacity for BNR compared with patients who did not (80% vs 14%; P = .023). There were no differences in the rates of attaining dryness per urethra. CBE outcomes worsen with each successive failed closure. Reclosure should be performed with osteotomy and proper immobilization to maximize the chance of sufficient capacity for BNR or augmentation cystoplasty.

Padded Self-Adhesive Strap Immobilization Following Newborn Bladder Exstrophy Closure: The Utah Straps

Several methods have been described for immobilization of the pelvis following bladder exstrophy closure, which can be challenging to manage. We hypothesized that immobilization can be significantly simplified using a modified mermaid wrap with padded Velcro® straps around the thigh and lower leg. We retrospectively reviewed all patients who underwent bladder exstrophy closure in the newborn period at our institution from 1990 through 2010. Patients with cloacal exstrophy and those who underwent delayed closure due to other medical conditions were excluded. We collected data on closure technique, length of stay and complications of the primary closure as outcomes. A total of 20 boys and 7 girls underwent closure of classic bladder exstrophy. Followup ranged from 2 to 22 years. Seven boys underwent complete primary repair and 13 underwent staged repair. All patients had the legs stabilized with a modified wrap technique using 2 lengths of Velcro straps lined with self-adhering open cell foam pads for 3 weeks. Complications of exstrophy closure included bladder dehiscence in 1 patient (4%) and incisional hernia in 2(7%). Following complete primary repair urethrocutaneous fistula developed in 2 patients and urethral stricture in 2. Average length of stay for patients without significant prematurity was 15 days. Padded Velcro strap immobilization simplifies postoperative care, provides secure fixation, decreases length of stay, and enables parents to hold and bond with the child shortly after repair. We advocate this simplified technique, which can be applied with a rate of complications that is comparable to other procedures.

Evaluation of Pelvic Floor Muscular Redistribution After Primary Closure of Classic Bladder Exstrophy by 3-Dimensional Magnetic Resonance Imaging

We used 3-dimensional magnetic resonance imaging reconstruction to generate models of the pelvic floor musculature in classic bladder exstrophy, allowing for statistical analysis of changes seen in the anatomy after primary closure. Patients with classic bladder exstrophy underwent pelvic magnetic resonance imaging before and after primary closure. Contours of the levator ani were mapped and measured in 3-dimensional space. In addition, 2-dimensional angles and measurements were used to make a quantitative and qualitative analysis of the pelvic floor before and after closure. A total of 19 cases of classic bladder exstrophy were included in the study, with 12 closed as newborns without osteotomy and 7 closed later with osteotomy. In both groups the pre-closure exstrophy pelvic floor in the axial plane was box-like and after closure it had a more inward rotation. The steepness and angulation of the levator ani muscle remained relatively unchanged in both groups. The levator ani muscle group, with and without osteotomy, was redistributed into the anterior compartment of the pelvis after closure. Postoperatively a successfully closed exstrophy had the bladder positioned deeply within the pelvis. After closure the levator ani muscle regained the expected smooth contoured shape. Primary closure of bladder exstrophy 1) reshapes the pelvis from a box-like configuration to a more inwardly rotated hammock, 2) redistributes a significant portion of the levator ani muscle into the anterior compartment and 3) facilitates a smooth uniform contouring to the pelvic floor. Closing the bony pelvic ring by pubic reapproximation in the newborn or by osteotomy in an infant produces similar changes in the pelvic floor.