Vaginoplasty in the female exstrophy population: Outcomes and complications

ObjectiveVaginal stenosis is a common sequela in adolescents who have undergone reconstruction for classic bladder exstrophy in infancy. We sought to determine the incidence of vaginal stenosis in our patient population and the outcome of treatment in the first three decades of life.Patients and MethodsAn institutional review board approved bladder exstrophy database was used to identify and retrospectively review classic female bladder exstrophy patients aged 12–30 years treated at the authors' institution. Patients who underwent vaginoplasty were identified and the following outcomes were measured: age at surgery, method used for the reconstruction, complications and incidence of re-stenosis.ResultsNinety-one female classic bladder exstrophy patients were identified. Twenty-nine patients (31.8%) underwent vaginoplasty because of vaginal stenosis at a mean (SD) age of 15 (3) years. Twenty-four patients underwent perineal flap vaginoplasty, three posterior cut-back vaginoplasty and two YV vaginoplasty. One patient developed wound infection and dehiscence which required reoperation (3.4%). No patient experienced vaginal re-stenosis.Conclusionsvaginal stenosis is common after reconstruction of female classic bladder exstrophy. Vaginoplasty is highly successful in the exstrophy population when performed in the second or third decade of life with a low risk of complications.     

Outcome of bladder exstrophy repair based on age and technique. Single center experience

PurposeReconstruction of bladder exstrophy remains a challenge. We evaluated our experience with different techniques in different age groups.Material and MethodsA retrospective data review was conducted of bladder exstrophy patients presenting at our institution between May 2000 and September 2007. 61 patients (21 females and 40 males) with classic bladder exstrophy were included. Age of presentation ranged from 24 hours to 14 months. 51 patients underwent complete primary repair and 11 patients had staged repairs. Mean follow up was 3 years (1 month to 7 years). Mortality involved one male & one female (3.2%). 29 patients underwent anterior innominate osteotomy and 9 underwent posterior osteotomy. Patients were evaluated for continence, upper tract dilatation and cosmetic result.ResultsIn the primary repair group, 8 (15.6%) had failed closures and 5 (9.8%) had fistulas. In the staged repair group, 1 (0.9%) had a failed closure and 1 had an epispadic fistula. Evaluation of continence excluded 9 patients recently operated on and 17 patients not followed up at our center. The primary repair group had 15 of 25 dry patients (11 females and 4 males). In the staged repair group, 4 of 10 patients were continent. 4 of our staged cases became dry following augmentation cystoplasty. Upper tract changes were mild during our study (44% of complete repair group and 12.5% in the staged group) with all patients having normal serum creatinine.ConclusionsPatients may require more than one procedure for reconstruction. Continence was better in females than males with primary or staged repairs.     

Shortened pubic bones in bladder exstrophy: A congenital or acquired phenomenon?

ObjectiveIt is accepted that the length of the anterior segment of the pelvis in classic bladder exstrophy is shorter than that of controls. However, studies performed involve children with a wide range of ages. By studying children with classic bladder exstrophy under the age of 1 year, the authors aim to ascertain if there is a congenital pubic bone length discrepancy associated with bladder exstrophy.Material and methodsFourteen classic bladder exstrophy patients under 1 year of age and 14 age-matched controls were identified. Three-dimensional (3D) computerized tomography was used to measure the length of the superior pubic ramus bilaterally. In the exstrophy group, scans were performed before bladder closure. The Mann–Whitney U test was used and p < 0.05 was considered significant.ResultsThere was no significant difference (p = 0.76) in the median age in cases (72 days) and controls (64 days). The median superior pubic ramus length was 19.1 mm in the exstrophy group and 20 mm in the control group (p = 0.99).ConclusionsSuperior pubic ramus length in children under 1 year of age with classic bladder exstrophy is not statistically different from that in control subjects. Therefore, the authors hypothesize that the previously described shortening of the pubic bones is an acquired phenomenon, which may develop after the first year of life.     

Bladder capacity as a predictor of voided continence after failed exstrophy closure

ObjectiveTo evaluate potential predictors of voided continence among bladder exstrophy patients with a history of a failed closure.Patients and methodsThe authors reviewed all patients who underwent a bladder neck reconstruction (BNR) with a history of one or more failed exstrophy closures between 1979 and 2007. The following data were collected for each patient: number of failures, site of surgery, mode of failure, presence of osteotomy, bladder capacity, need for additional procedures, and continence status.ResultsAmong patients who underwent successful reclosure following one or more failed closures, 52 patients underwent BNR, and 24 (46%) were continent at last follow-up. Bladder capacity was the only variable predictive of voided continence. The median bladder capacity at the time of BNR differed between those who achieved continence (100 mL) and those who did not (65 mL) (p = 0.005). ROC analysis showed an optimal pre-BNR bladder capacity cutoff for predicting future BNR success of between 80 and 100 mL.ConclusionAs previously shown in patients with successful primary closure of exstrophy, these data suggest that bladder capacity also has predictive value in the success of BNR after failed exstrophy closure.     

The modern staged repair of classic bladder exstrophy: a detailed postoperative management strategy for primary bladder closure

PurposeSuccessful primary bladder closure of classic bladder exstrophy sets the stage for development of adequate bladder capacity and eventual voided continence. The postoperative pathway following primary bladder closure at the authors’ institution is quantitatively and qualitatively detailed.Materials and methodsSixty-five consecutive newborns (47 male) undergoing primary closure of classic bladder exstrophy were identified and data were extracted relating to immediate postoperative care. Overall success rate was utilized to validate the pathway.ResultsMean age at time of primary closure was 4.6 days and mean hospital stay was 35.8 days. Osteotomy was performed in 19 patients (mean age 8.8 days), and was not required in 39 infants (mean age 2.9 days). All patients were immobilized for 4 weeks. Tunneled epidural analgesia was employed in 61/65 patients. All patients had ureteral catheters and a suprapubic tube, along with a comprehensive antibiotic regimen. Postoperative total parenteral nutrition was commonly administered, and enteral feedings started around day 4.6. Our success rate of primary closure was 95.4%.ConclusionsA detailed and regimented plan for bladder drainage, immobilization, pain control, nutrition, antimicrobial prophylaxis, and adequate healing time is a cornerstone for the postoperative management of the primary closure of bladder exstrophy.     

Management of bladder exstrophy in adulthood: Report of 5 cases

ObjectiveTo report our experience in the management of adult classic bladder exstrophy.Patients and methodsDuring 1977‒2006 we treated five adult males presenting with classic exstrophy–epispadias complex. Patient age at presentation ranged from 17 to 30 with a mean age of 23 years. Four patients had received no previous treatment and one underwent previous ureterosigmoidostomy. Work-up included evaluation of upper tract and bladder biopsy. Bladder patch condition was variable. Surgery involved bladder preservation in the three patients who underwent primary repair, including bladder closure, bladder neck reconstruction and epispadias repair; two of them also had augmentation ileocystoplasty. The remaining two patients underwent ureterosigmoidostomy, cystectomy and epispadias repair. Abdominal wall closure was by fasciocutaneous M-plasty. Osteotomy was not done in any case.ResultsIn patients with bladder preservation, one patient was continent (>3 h) and voided normally whereas the other two showed day and night continence (2–3 h) with mild stress incontinence. Patients were satisfied with functional outcome. Ultrasound and intravenous pyelography showed preservation of upper tract. Follow-up period ranged from 1 to 8 years.ConclusionPatients with bladder exstrophy presenting in adulthood should not be denied the opportunity of primary reconstruction with bladder preservation in the absence of significant histological changes in the bladder mucosa.     

Outcome of complete primary bladder exstrophy repair: Single-center experience

ObjectiveReconstruction of bladder exstrophy remains a challenge. We evaluated our experience with complete primary repair in classic bladder exstrophy.MethodsA retrospective data review was conducted of bladder exstrophy patients presenting at our institution between May 2000 and September 2007. Fifty-one patients (21 females and 30 males) with classic bladder exstrophy were included. Age of presentation ranged from 24 h to 14 months. Mean follow up was 3 years (1 month–7 years). Patients were evaluated for continence, upper tract dilatation and cosmetic result.ResultsEight patients (15.6%) had failed closures and six (11.7%) had fistulae. Evaluation of continence excluded 16 patients not followed up at our center. Thirty-seven percent were continent on clean intermittent catheterization after the age of 5 years. Patients became dry only after augmentation cystoplasty. Upper tract changes were mild during our study with all patients having normal serum creatinine.ConclusionPatients may require more than one procedure for reconstruction. In our series, augmentation was required to achieve acceptable dryness. Early promising results with dry intervals in young patients did not translate to continence in older patients.     

The modern staged repair of bladder exstrophy in the female: a contemporary series

ObjectiveMany changes have occurred in the treatment of bladder exstrophy over the last few years and several repairs are now offered, but there is a lack of long-term follow-up data. The purpose of this study was to evaluate long-term outcomes in a select group of female patients in whom modern staged repair was undertaken.Patients and methodsFrom an institutionally approved database 41 patients were identified. All had undergone primary bladder exstrophy closure in 1988–2005, at a mean age of 2 months (range 4 h to 3 months), with or without an osteotomy by a single surgeon, and all were followed up for a minimum of 5 years. Twelve patients underwent osteotomy at the time of primary closure. Eight had a classic transverse innominate and vertical iliac osteotomy, and four a transverse innominate only. Mean age at the time of bladder neck repair (BNR) was 4.2 years (39–65 months). Mean measured bladder capacity under gravity cystograms at the time of repair was 109 cc (80–179 cc).ResultsThirty patients (74%) were continent day and night, and voiding per urethra without augmentation or intermittent catheterization. Social continence, defined as dry for more than 3 h during the day but damp at night, was found in a further four cases (10%). Seven patients are completely incontinent with dry intervals of less than 1 h day and night. The mean time to daytime continence was 12 months (4–16 months) and to night-time continence was 19 months (10–28 months). Patients with a mean capacity greater than 100 cc had better outcomes. Six of the 30 patients achieved dryness after primary closure only, and all six had transverse innominate and vertical iliac osteotomy at the time of primary closure.ConclusionsFemale classic exstrophy patients with a good template who develop adequate capacity after a successful primary closure can achieve acceptable continence without bladder augmentation and intermittent catheterization. A select group will develop continence with closure alone without the need for bladder neck repair.     

Complications of radical soft-tissue mobilization procedure as a primary closure of exstrophy

PurposeThe radical soft-tissue mobilization procedure was developed as a component of the staged closure of classical bladder exstrophy to improve continency rates without having to perform pelvic osteotomies. The authors describe complications following this procedure and discuss possible etiologies and subsequent management.Materials and methodsWe extracted from an institutionally approved exstrophy database the records of patients evaluated for complications following radical soft-tissue mobilization repair from 1999 to 2002.ResultsFour patients were referred to our institution following closure of exstrophy with the radical soft-tissue mobilization technique; two boys and two girls. Complications included ischemic penile injuries in both males, failed exstrophy closure in one female, incontinence with need for bladder neck transection and diversion in two patients, and upper tract deterioration in two patients of whom one required cystectomy and incontinent diversion.ConclusionsOmission of osteotomies when employing the radical soft-tissue mobilization repair appears to result in complications that could otherwise be prevented. Additionally, the complex dissection of the pelvic musculature, innervation and vasculature performed during radical mobilization has great potential to injure the pelvic structures and genitalia, as has been seen with the cases presented herein.     

Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects (OEIS) complex: Experience with 80 patients

ObjectiveTo review the urological management and outcomes of patients with the OEIS (omphalocele, exstrophy of the bladder, imperforate anus, spinal abnormalities) complex.Patients and methods80 patients with the OEIS complex managed at a single institution between 1974 and 2009 were reviewed.Results37 had initial closure at our institution (2 failed – 5%); 22 with successful closure were referred for incontinence; 15 failed closure at an outside institution (2 of whom are awaiting closure); 6 are skin-covered variants. Osteotomy was performed in 39/43 (91%) with successful closure versus 8/17 (47%) who failed initial bladder closure. 40 were dry (56%), but most needed additional urinary reconstruction: 2 had small bowel neobladders; 32 (84%) had augmentation cystoplasty; 30 (79%) had a continent catheterizable channel; only 9 (24%) were continent with an intact urethra. Bladder neck reconstruction allowed dryness in 7 (18%). 45 patients had XY genotype – 19 had female gender assignment at birth. All patients with XX genotype had female gender assignment.ConclusionsOsteotomy improves success of initial bladder closure. A bladder neck procedure, catheterizable channel, and augmentation cystoplasty will be required in the majority of patients to attain urinary dryness.     

Safety and efficacy of staged pelvic osteotomies in the modern treatment of cloacal exstrophy

Introduction and objectiveStaged pelvic osteotomy (SPO) prior to bladder closure has been shown to be a safe and effective method for achieving pubic approximation in cloacal exstrophy (CE) patients with extreme diastasis. However, SPO outcomes have never been compared to those for combined pelvic osteotomy (CPO) at the time of closure in CE patients.MethodsA prospectively maintained database of 1208 exstrophy–epispadias complex patients was reviewed for CE patients treated with pelvic osteotomies. Inclusion criteria were osteotomy at the authors' institution and closure within two months of osteotomy. After inclusion, patients were separated into four groups depending on osteotomy procedure (SPO vs. CPO) and whether their osteotomy occurred with primary closure or re-closure. Patient demographics, closure history, pre-operative diastasis measurement, most recent post-operative diastasis measurement, and outcomes were recorded and compared by chi-squared tests and ANOVA.ResultsAmong 116 CE patients reviewed, 46 met inclusion criteria. With primary closure or re-closure, 27 had SPO and 19 had CPO. No SPO re-closure patients had previous osteotomy; 4 CPO re-closure patients had a previous osteotomy with closure. Median time between osteotomy and closure in SPO patients was 14 days. Median follow-up after SPO and CPO were 4 and 11 years, respectively. SPO significantly reduced the pre-operative diastasis compared to CPO on most recent diastasis measurement (3.5 cm vs. 0.4 cm, p = 0.003). There were no significant differences in the overall complication rate, or the rates of each specific complication, between the SPO and CPO groups. No patients had wound dehiscence or prolapse. One CPO patient was able to intermittently catheterize per urethra while all other patients required continent urinary diversion to achieve continence.ConclusionsTo the authors' knowledge, this is the first study comparing SPO and CPO outcomes in CE patients. SPO reduces pre-operative diastasis more than CPO, and does not appear to incur increased rates of complication, closure failure, or incontinence. Due to its apparent safety and greater efficacy, SPO should be considered in all CE patients with extreme diastases undergoing primary closure or re-closure.     

Urologic complications of major genitourinary reconstruction in the exstrophy–epispadias complex

ObjectiveTo present the authors' experiences with urologic complications associated with various techniques used to create a continent stoma (CS), augmentation cystoplasty (AC), and neobladder in the exstrophy–epispadias complex (EEC) population.MethodsRetrospective review of medical records of patients who underwent CS with or without bladder augmentation were identified from an institutional review board-approved database of 1208 EEC patients. Surgical indications, tissue type, length of hospital stay, age, preoperative bladder capacity, prior genitourinary surgeries, postoperative urological complications, and continence status were reviewed.ResultsAmong the EEC patients reviewed, 133 underwent CS (80 male, 53 female). Mean follow-up time after initial continent stoma was 5.31 years (range: 6 months to 20 years). Appendix and tapered ileum were the primary bowel segments used for the continent channel and stoma in the EEC population. The most common stomal complications in this population were stenosis, incontinence, and prolapse. Seventy-nine percent of EEC CS patients underwent AC primarily done with sigmoid colon or ileum. Eleven patients (8%) underwent neobladder creation with either colon or a combination of colon and ileum. Bladder calculi, vesicocutaneous fistula, and pyelonephritis were the most common non-stomal complications. Stomal ischemia was significantly increased in Monti ileovesicostomy compared to Mitrofanoff appendicovesicostomy in classic bladder exstrophy patients (p = 0.036). Furthermore, pyelonephritis was more than twice as likely in colonic neobladder than all other reservoir tissue types in the same cohort (OR = 2.53, 95% CI: 1.762–3.301, p < 0.001).ConclusionsTo the best of the authors' knowledge, this is the largest study examining catheterizable stomas in the exstrophy population. While Mitrofanoff appendicovesicostomy is preferred to Monti ileovesicostomy because it is technically less challenging, it may also confer a lower rate of stomal ischemia. Furthermore, even though ileum or colon can be used in AC with equally low complication rates, practitioners must be wary of potential urologic complications that should be primarily managed by an experienced reconstructive surgeon.     

Role of urethral bulking agents in epispadias–exstrophy complex patients

ObjectiveInjection of urethral bulking agents (UBA) has been used to increase bladder capacity prior to bladder neck reconstruction (BNR) or as an adjuvant therapy following BNR to improve continence. The purpose of this study was to determine the effectiveness of urethral injections in the exstrophy population.Materials and methodsA review was performed of patient characteristics, bladder capacity, and continence status of all patients with the exstrophy–epispadias complex who underwent injection of UBA between 1980 and 2008.ResultsAmong 66 patients with a median follow-up of 8 years, 41 underwent injections prior to BNR, and 25 had injections after BNR. Only 24% of patients who underwent injections prior to BNR were continent on last follow-up. Among 25 patients who underwent BNR prior to injection(s), 16 were partially continent and nine were incontinent prior to first injection. Patients who were partially continent attained social continence (dry interval greater than 3 h) at a significantly higher rate than those who were incontinent (63% vs. 13%, p = 0.047). No patient with cloacal exstrophy in either group attained urethral continence.ConclusionUBAs do not appear to have a role prior to BNR. However, they may provide benefit when given adjunctively following BNR in patients who are partially continent.     

Penile ischemic injury in the exstrophy/epispadias spectrum: New insights and possible mechanisms

ObjectivePartial or complete penile loss following bladder exstrophy and/or epispadias repair has been reported in the literature progressively more frequently.Patients and methodsThe authors report new cases of penile injury following bladder exstrophy and/or epispadias repair referred to their centers and not previously published. They review the literature on this subject and offer an explanation as to the likely mechanism for the penile injury and recommendations to avoid this complication.ResultsSeven new cases of partial or complete penile loss following bladder exstrophy or epispadias repair have been recently referred to the authors’ institutions. Twenty-one patients have previously been reported in the literature. Altogether, 24 cases occurred after bladder exstrophy closure: 23 after complete primary repair of exstrophy (Mitchell repair) and one after first-stage radical soft-tissue mobilization (Kelly repair). Nineteen of 24 patients did not have a pelvic osteotomy at the time of primary closure. Four cases occurred after epispadias repair: two following the second-stage radical soft-tissue mobilization (Kelly repair) and two following penile disassembly epispadias repair (Mitchell repair).ConclusionExstrophy closure combined with epispadias repair can be followed by ischemic penile injury, particularly when osteotomy is not performed. Compression of the pudendal vessels after pubic apposition and/or direct injury to the pudendal vessels play an important role in the pathogenesis of this complication.     

Repeat pelvic osteotomy in cloacal exstrophy: applications and outcomes

PurposePelvic osteotomies have been shown to enhance success rates for classic exstrophy patients when closed primarily or secondarily after initial failure. Primary closure of cloacal exstrophy also benefits from osteotomy but this has yet to be shown for re-closure of cloacal exstrophy failures. This study looks at the applications, complications, and long-term success rates in this very select group of patients.MethodsWe extracted from an institutionally approved exstrophy database 15 patients who had undergone repeat pelvic osteotomy and analyzed patient history, complications and orthopedic outcomes.ResultsAll patients who underwent reclosure at our institution remain closed. Major complications were seen in two patients and minor complications in four patients. Urinary continence was achieved in 10 patients with augmentation and continent stoma formation, urinary diversion was performed in two patients and three patients await a continence procedure.ConclusionsRepeat pelvic osteotomy in cloacal exstrophy is successful and the complication rate is low. Pelvic osteotomy is associated with enhanced success rates of primary and secondary closure with better cosmesis of the abdominal wall and genitalia. Intrasymphyseal plates along with gradual reduction of the extreme diastasis utitlizing an external fixation device can be beneficial prior to further genitourinary surgery.     

Indiana pouch in children: A 15-year experience

ObjectiveWe report our experience with the Indiana pouch (continent urinary reservoir) in 12 consecutive children over the last 15 years and report their follow-up.Material and methodsTwelve consecutive children, who underwent the continent urinary reservoir procedure in the form of an Indiana pouch, were prospectively enrolled in the study. All consecutive children who were referred to our service with multiple failed surgeries for exstrophy–epispadias repair, cloacal exstrophy, genitourinary rhabdomyosarcoma with residual disease in the trigonal area not amenable to partial cystectomy, and neuropathic bladder with nephrogenic metaplasia were included over the period 1997–2012. All these children were offered the same form of bladder substitution (Indiana pouch) as described by the Indiana group many years ago. Postoperative care was on a fixed protocol, and follow-up details recorded over the years. They were followed up for dry interval with clean intermittent catheterisation, social acceptance, and early and late complications.ResultsOut of these 12 patients (7 males and 5 females), eight patients had exstrophy–epispadias with multiple failed operations carried out elsewhere, cloacal exstrophy (2), residual rhabdomyosarcoma in the trigonal area with incontinence following chemotherapy (1), and neuropathic bladder with recurrent diffuse neoplastic polyposis (1). In the follow-up period of 1–15 years (median 24 months) all the patients had a dry interval of 4 h or more with clean intermittent catheterisation. One patient had wound dehiscence during the postoperative period and another required stomal revision 1 year after initial surgery.ConclusionsThe Indiana pouch is a reliable, safe, and effective form of bladder substitution. It can be reconstructed in a wide range of lower urinary tract disorders. In the vast majority of children with multiple failed surgical procedures for exstrophy–epispadias, the Indiana pouch is a safe, reliable, and reproducible procedure to provide a socially acceptable quality of life with a dry interval of 4 h.     

Factors responsible for successful primary closure in bladder exstrophy

 To assess the important factors for successful primary closure in staged reconstruction of bladder exstrophy, 25 patients (18 males, 7 females) underwent primary bladder closure during the years 1993–1997. Twenty-one were more than 72 h old; all of these underwent bilateral posterior iliac osteotomies followed by primary bladder closure during the same anesthetic. Bladder closure was done in a double layer. The ureteric catheters were removed after 2 weeks and the bladder catheter after 3.5–4 weeks. Only 1 patient had a bladder dehiscence on the 10th postoperative day due to infection; 3 had partial wound dehiscences but no bladder dehiscence. One had a partial bladder prolapse. The osteotomies needed no drainage, and no complications occurred. One patient needed a urinary diversion 3 years after surgery as the bladder capacity did not increase. Eleven important factors play a pivotal role for successful primary bladder closure: (1) Proper patient selection; (2) A staged approach; (3) Anterior approximation of the pubic bones with placement of the bladder and urethra in the true pelvis; (4) Posterior bilateral iliac osteotomies in all indicated cases; (5) Double-layered closure of the bladder; (6) Two weeks' proper ureteric catheter drainage; (7) Prevention of infection; (8) Prolonged and proper postoperative immobilization; (9) Prompt treatment of bladder prolapse; (10) Prevention of abdominal distension postoperatively; and (11) Ruling out bladder-outlet obstruction before removing the bladder catheter. Accepted: 12 July 1999     

Failed exstrophy closure: Management and outcome

ObjectiveIn a series of failed exstrophy closures, to identify determinants of successful repeat closure and the impact of failed closure on the fate of the lower urinary tract and continence status.Patients and methodsWe performed a retrospective review of operative notes and medical records of patients with a history of one or more failed exstrophy closures in 1978–2007. The primary surgical endpoints were failure rate of repeat closure attempts, mode of continence surgery and continence outcome. Continence was defined as achieving a dry interval of >3 h and voiding through the urethra.ResultsWe identified 122 patients (85 male/37 female) who had undergone repeat closure following failure. The success rate of repeat closure attempts at our institution was 98%.Of the 94 patients who had undergone successful repeat closure, definitive continence management and had their dryness evaluated, 38 were candidates for bladder neck reconstruction and 17(18%) were continent. Of the remaining patients, 90% were able to attain dryness, but at the expense of clean intermittent catheterization and continent urinary diversion.ConclusionA failed exstrophy closure has significant implications for long-term surgical outcome. Reclosure can be accomplished in the majority of cases. In comparison to patients with successful primary closure, the rates of urethral continence following successful repeat closure were lower.     

Our initial experience with the technique of complete primary repair for bladder exstrophy

ObjectiveWe reviewed our initial results with complete primary repair of exstrophy in regard to continence status and the need for subsequent continence procedures.Patients and methodsWe performed a retrospective review of our surgical records from 1996 to 2008 to identify all patients with bladder exstrophy managed at our center.ResultsSixteen children were closed successfully. Six patients (37.5%) experienced complications: umbilical hernias in two, transient penopubic fistula in three, and subcoronal fistula due to meatal stenosis in one. Of the 12 males, seven (58.3%) were left with a hypospadias at the time of primary closure. Two (22.2%) children required a formal bladder neck reconstruction to achieve continence. Bladder augmentation and continent catheterizable stoma was performed in four cases (44.4%), and bladder neck injection in one case (11.1%). Bladder neck closure was also performed in another child following primary closure. Three of these children are continent and void spontaneously (33.3%). The remaining six require clean intermittent catheterization four to six times a day, resulting in four (44.4%) being continent. The number of continence procedures and mean number per patient were 15 and 1.66, respectively.ConclusionOur early experience with this technique has been encouraging, with few major complications, a highly successful closure rate and a cosmetically normal result.     

The search for continence in bladder exstrophy: Bladder neck transection and Macedo catheterizable reservoir to augment the nativebladder

IntroductionBladder exstrophy remains one of the most challenging abnormalities in pediatric urology. We propose bladder neck transection and bladder augmentation with a catheterizeable reservoir technique to achieve continence after previous anatomic reconstruction in stages.MethodsAt the age of 5–6 years, we offer the transection of bladder neck and enterocystoplasty to achieve continence. We report on a 6-year-old boy that underwent this procedure. We perform the reservoir from ileum according to Macedo-technique that constructs a catheterizeable channel from the same bowel segment. The continence mechanism of the efferent tube is based on angulation and a serous lined tunnel created with 3.0 prolene sutures. The stoma is placed in the midline.ResultsPatient had an uneventful evolution and is continent performing CIC every 4 h with 9 months of follow up.DiscussionIn spite of continuous development of bladder exstrophy surgery, the urethral continence and voluntary micturition is still not possible in the majority of patients. We discuss with our patients honestly and offer this method as a viable alternative to achieve continence.ConclusionIn our experience, most patients accept urethral transection and suprapubic CIC when educated about results with other alternatives of bladder neck plasty.