Primary Bladder Exstrophy Closure Research Articles

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.

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.

Development of bladder plate calculus on missed sutured surgical sponge following primary bladder exstrophy repair

Bladder Exstrophy (BE) has been considered as a rare congenital malformation of the genitourinary system, with an estimated incidence of approximately 1 per 50,000 live births [1]. The management of this anomaly is complex, involving several surgical reconstructions with numerous challenges to achieve anatomically, functionally, and cosmetically satisfactory outcomes [2]. Furthermore, the management must be early in the first months of life for better functional and cosmetic results [3,4]. Wound dehiscence, urethral outlet obstruction, bladder prolapsed, renal and bladder calculi are among the main complications following failed primary BE closure [5]. Urinary stone formation is estimated to be around 15% following bladder closure, in repaired BE patients, especially in cases living in poor or rural regions [6]. Bladder stone formation may occur following infra-vesical obstruction, neurogenic voiding dysfunction, urinary tract infection, foreign bodies, and metabolic risk factors years after reconstructive surgery [7]. Here, we report a 6-yearold boy with failed BE repair at an early age that was complicated by late surgery outcomes and secondary stone formation on missed sutured surgical sponge post-operatively.

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.

Extraordinary Bulging Mass in the Foetus - A Case Report of Bladder Exstrophy

Abstract INTRODUCTION: Prenatal diagnosis of bladder exstrophy is extremley rare and difficult. BACKGROUND: Due to abnormal development of the cloacal membrane there is an incomplete closure of the lower abdominal wall, absence of the anterior wall of the bladder and external exposition of the posterior wall. The pubic bones are usually separated, the umbilical cord low inserted and there is abnormal external genitalia development. CASE REPORT: At 21st week of gestation of 39-year-old multigravida multipara referred by a primary care obstetrician to high-specialised centre for a detailed ultrasound examination with a suspicion of bladder absence and inferior umbilical localisation. At 29 weeks of gestation presence of bulging mass of 2 cm, between the umbilical outlet and labia was detected. At 31 weeks of gestation previously detected structure among thighs had 3 cm diameter with lateral umbilical outlet. Major labia were prominent and minor labia were within normal limits. Between two umbilical arteries with an appropriate intraabdominal course there were no transsonic area corresponding to the urinary bladder. The newborn baby was born at term in a good condition, but with an exposed bladder of 4 cm in diameter. The urethral outlet was not visualised and the female genitals were abnormal. After a month the girl underwent primary bladder exstrophy closure. Although she suffers from recurring urinary tract infections, she is in a good general condition. CONCLUSIONS: Due to prenatal diagnostics it was possible to detect and make an initial diagnosis of severe malformation. Early diagnosis allowed to prepare parents for a newborn with a defect and teach them how to take care of the baby.

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.

Endoscopic management of massive stone load in a patient with repaired bladder exstrophy

Bladder exstrophy is a rare congenital malformation of the genitourinary system. Patients with exstrophy of the bladder who have undergone augmentation cystoplasty with Mitrofanoff procedure have a tendency of recurrent calculi formation. A 23-year-old female, with bladder exstrophy, underwent Mitrofanoff procedure and developed recurrent vesical calculi thereafter. She presented to us with a very massive stone load, which was managed successfully by endoscopic technique. She was also taught preventive measures thereafter and advised regular follow-up. The importance of the report is that it highlights urolithiasis as a recurrent complication of primary bladder exstrophy closure, which can be managed effectively and safely by modern endoscopic techniques in a hostile abdomen with reconstructed tract and inaccessible urethra.

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

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. 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. 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. 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.

Cloacal Exstrophy Repair with Primary Closure of Bladder Exstrophy: A Case Report and Review of Literature.

Cloacal exstrophy is the most complex congenital, ventral, abdominal wall defect. Traditionally surgery consists of a staged approach to repair which takes place on many separate theatre visits. In this case a primary approach was undertaken resulting in a relatively short inpatient stay and a reduced risk from multiple surgical procedures under general anaesthesia.

Ureteral Reimplantation Before Bladder Neck Reconstruction in Modern Staged Repair of Exstrophy Patients: Indications and Outcomes

To evaluate a single referral center's experience with vesicoureteral reflux (VUR) and its role in management before bladder neck reconstruction (BNR) in exstrophy patients after modern staged repair of exstrophy (MSRE). VUR after primary closure of bladder exstrophy can usually be managed conservatively until ureteral reimplantation (UR) at the time of BNR. However, VUR in these patients is occasionally associated with febrile urinary tract infection and worsening renal function necessitating UR before BNR. A total of 199 patients who underwent MSRE were identified from an institutional review board-approved database. Patient demographics, closure history, postprimary closure VUR, history of urinary tract infection, cystography outcomes, renal function, history of UR before BNR, and UR outcomes were recorded. Univariate and multivariate analyses were performed to determine factors predicting high-grade VUR and the need for pre-BNR UR. After primary closure, VUR was observed in 150 patients (75.4%) of whom 31 (15.58%) had high-grade VUR. Closure without osteotomy and postclosure outlet obstruction were independently associated with postprimary closure high-grade VUR (P<.05). Twenty-two patients (11.06%) required pre-BNR UR. Female gender and those with high-grade VUR were more likely to require UR before BNR (P<.05). Early UR did not affect the continence rate. VUR after exstrophy closure can generally be managed with conservative treatment and periodic monitoring of the upper urinary tracts. About 11% of patients undergoing MSRE will require UR before BNR. Closure without osteotomy and patients who develop outlet obstruction after closure are at increased risk for developing high-grade VUR.

Is osteotomy necessary for primary exstrophy closure?

A recent study has reported a novel approach to primary bladder exstrophy closure without osteotomy in newborns. In this study, most patients were managed on a paediatric urology ward with an epidural catheter, with similar outcomes to patients treated in an intensive care unit with ventilation and muscle relaxation.

This Month in Pediatric Urology

No AccessJournal of UrologyThis Month in Pediatric Urology1 Jan 2014This Month in Pediatric Urology Laurence S. Baskin Laurence S. BaskinLaurence S. Baskin More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2013.10.029AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "This Month in Pediatric Urology." The Journal of Urology, 191(1), pp. 3–4 References 1 : Sexual function in adult life following Passerini-Glazel feminizing genitoplasty in patients with congenital adrenal hyperplasia. J Urol2014; 191: 206. Abstract, Google Scholar 2 : Primary bladder exstrophy closure in neonates: challenging the traditions. J Urol2014; 191: 193. Link, Google Scholar 3 : Urinary NGF, TGF-β1, TIMP-2 and bladder wall thickness predict neurourological findings in children with myelodysplasia. J Urol2014; 191: 199. Abstract, Google Scholar 4 : Risk factors and predictors of severity score and complications of pediatric hemorrhagic cystitis. J Urol2014; 191: 186. Abstract, Google Scholar © 2014 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 191Issue 1January 2014Page: 3-4 Advertisement Copyright & Permissions© 2014 by American Urological Association Education and Research, Inc.MetricsAuthor Information Laurence S. Baskin More articles by this author Expand All Advertisement PDF downloadLoading ...

Primary Bladder Exstrophy Closure in Neonates: Challenging the Traditions

We describe a novel approach to neonatal bladder exstrophy closure that challenges the role of postoperative immobilization and pelvic osteotomy. We reviewed the primary management of bladder exstrophy at our institutions between 2007 and 2011. In particular we compared postoperative management in the surgical ward using epidural analgesia to muscle paralysis and ventilation in the intensive care unit. Clinical outcome measures were time to full feed, length of stay, postoperative complications and redo closure. Cost-effectiveness was also evaluated using hospital financial data. Data are expressed as median (range). Significance was explored by Fisher exact test and unpaired t-test. A total of 74 patients underwent primary closure without osteotomy. Successful closure was achieved in 70 patients (95%). A total of 48 cases (65%) were managed on the ward (group A) and 26 (35%) were transferred to the intensive care unit (group B). The 2 groups were homogeneous for gestational age (median 39 weeks, range 27 to 41) and age at closure (3 days, 1 to 152). Complications requiring surgical treatment were noted in 4 patients (8.3%) in group A and 3 (11.5%) in group B (p = 0.609). Length of stay was significantly shorter for the group managed on the ward (11 vs 18 days, p <0.0001). Median costs were $42,732 for patients admitted to the intensive care unit and $16,214 for those admitted directly to the surgical ward (p <0.0001). Primary closure of bladder exstrophy without lower limb immobilization and osteotomy is feasible. Postoperative care on the surgical ward using epidural analgesia results in shorter hospitalization.

PEDIATRIC UROLOGY Complications after primary bladder exstrophy closure – role of pelvic osteotomy

IntroductionBladder exstrophy is the most common form of the exstrophy – epispadias complex. It is observed in 1:30 000 life births, about four times more often in boys than in girls. Iliac osteotomy is used to facilitate bringing together pubic bones and to minimize the tension of fused elements. To analyze complications after primary bladder exstrophy closure with a special consideration of the role of pelvic osteotomy.Material and methodIt is a retrospective study evaluating 100 patients (chosen by chance out of 356) with bladder exstrophy (65 boys and 35 girls), treated in Pediatric Urology Department of the Children's Memorial Health Institute in Warsaw, Poland between 1982 and 2006. All children underwent primary bladder exstrophy closure, among them 32 elsewhere. Primary bladder exstrophy closure with contemporary iliac osteotomy was done in 36 children. In the rest 64 patients bladder was closed without osteotomy, regardless child's age.ResultsDehiscence after primary closure followed with bladder prolaps occurred in 31 patients, among them 13 out of 68 (19%) operated in our department and 18 out of 32 (56%) operated in another hospital. Primary bladder exstrophy closure with contemporary iliac osteotomy was done in 32 infants above 72 hours of life.ConclusionsOsteotomy performed at primary bladder exstrophy closure diminishes the risk of wound dehiscence independently of patient's age. Posterior iliac osteotomy is sufficient and safe and could be repeated if necessary.

Author's replay

Looking for bladder exstrophy as a diagnosis, we found 356 cases in our database, which makes us one of the biggest centers of exstrophy-epispadias complex treatment in the world. Some patients are treated at our institution from the beginning while others are coming for further treatment after primary bladder exstrophy closure or because of complications and poor results of surgery performed at another institution. From the technical point of view it was not possible to include all 356 patients into our study, because we felt that a group of 100 cases was large enough to function as a representative group. All of the cases were chosen randomly from the database. Bladder exstrophy is treated with staged reconstruction, which begins with primary closure, followed by urethral and penile reconstruction, and finally bladder neck creation. The primary closure is crucial because it involves not only bladder closure but also the necessity to rearrange the pelvic bones, pelvic floor muscles, and anterior abdominal wall layers. In newborns up to the third day of life, the primary bladder closure could be performed without osteotomy. At this age, the pelvic bones are elastic and it is possible to connect the pubic symphysis without osteotomy. However, we learned, that in case of a diastasis of 4 cm or more, it is better to perform osteotomy even if the baby is less than three days old. In older children, however, osteotomy is recommended independently of the distance of the pubic bones. Unfortunately, there are doctors who do not understand the role of osteotomy and they still perform exstrophy closure without this procedure. As a consequence of the wide diastasis of the symphysis, the results of primary closure are bad. Most often wound dehiscence is observed with re-exstrophy of the bladder. In other cases, the cicatricial hernia develops or extensive scarring occurs. Moreover, wide standing pubic bones make the proper reconstruction of external genitalia impossible and predispose to vaginal prolapse in adulthood. That is why we decided to call attention to primary bladder exstrophy closure and the value of osteotomy in this procedure. Pelvic osteotomy combined with bladder exstrophy closure has only one aim – to connect the pubic bones. The reconstruction of the symphysis allows the pelvic floor muscles and abdominal rectus muscles to close up. Approximation of the pubic bones is achieved with iliac osteotomy independently of the method of osteotomy. The most popular is posterior iliac osteotomy, which was described for the first time in 1958 by Schulz and practically unchanged until today. In some cases with big diastasis of the symphysis, even if the osteotomy is performed, the pubic bones are connected with tension, which predisposes to wound dehiscence and bladder prolapse. That is why re-exstrophy may occur in all centers even those doing osteotomy in all patients. However, closure without osteotomy always gives tension. The discrepancy between the results achieved in our center and in the others (failure rate 19% to 56%, respectively), derives evidently from the osteotomy that was, or was not, performed together with bladder closure (Tables 2, 3, 4, & 5). Our experience and results push us to conclude that it is better to close the exstrophy together with iliac osteotomy in all age groups, even in the newborn less than three days old – and this is a new message. Furthermore, if we should perform osteotomy regardless child's age, let us postpone the surgery for about one month to let the baby stay with the mother and allow the rest of family to develop emotional relations. Although the delay of operation should not be longer because of epithelialization of the bladder mucosa.

Persistent vesicocutaneous fistula after repair of classic bladder exstrophy: A sign of failure?

ObjectiveTo describe the potential predisposing clinical characteristics at initial closure, the treatments of and outcomes associated with vesicocutaneous fistula (VCF) after primary bladder closure. Materials and methodsEighteen patients were referred for VCF after primary bladder exstrophy closure. Aspects from the primary closure such as gender, timing of diagnosis and repair, osteotomies, pelvic immobilization, layers of closure, use of tissue adjuncts, and complications in addition to details from their required treatment of VCF were retrospectively extracted from the medical record. ResultsA diagnosis of failed closure was made at the time of evaluation in 13 of 18 patients who presented with VCF. All 13 patients underwent delayed single-layer closures and had a widened pubic diastasis. Five patients with secure closures who underwent successful simple fistula repairs were all closed early in life, had multi-layered closures with pelvic osteotomies, and had minimal change in pubic diastasis. ConclusionVCF may represent a failed bladder closure. Factors which may suggest failure are a fistula tract in the lower abdominal midline between the pubic rami, a pubic diastasis increased from pre-closure measurement, and cystoscopic evidence of an anteriorly positioned bladder.