Repair Of Classic Bladder Exstrophy Research Articles

Redo surgery to improve urinary function, sexual function and cosmesis in male patients with exstrophy–epispadias complex complications. Technical principles and pearls based on case scenarios

The bladder exstrophy-epispadias complex includes some of the most challenging conditions treated by pediatric urologists. They are associated with the need for multiple intricate reconstructive procedures, aimed at restoring the anatomy and function of the bladder, urethra and external genitalia. These patients often endure multiple redo reconstructive procedures to improve urinary function, sexual function and cosmesis throughout the first two decades of life. In this article, we present the 30-year experience of a single surgeon performing redo surgery for males born with epispadias and bladder exstrophy. Through detailed documentation of 6 clinical cases, we highlight technical aspects that may contribute to a successful surgical reconstruction in these patients. The article is focused specifically on patients undergoing redo epispadias repair with or without concomitant continence procedures. We make the case for complete penile disassembly with external rotation of the corpora to correct recurrent dorsal curvature; this approach also allows the surgeon to have access to the proximal urethra and bladder neck after opening the intersymphiseal scar/band. This is useful when additional procedures on the bladder, such as bladder neck tailoring, are necessary. We also highlight the importance of avoiding reverse Byars' flaps when performing skin closure, due to the resulting midline scar. Besides being associated with a poor cosmetic outcome, it can also contribute to recurrent dorsal curvature. The authors advocate for rotational skin flaps to cover the penile shaft. Correction of dorsal curvature and improved cosmesis obtained with complete penile disassembly sometimes comes at the expense of the urethra being left as a hypospadias (figure). This will require further surgeries (usually a 2-stage buccal mucosa graft), much like the treatment of proximal hypospadias. Redo epispadias surgery in males remains a challenge. The systematic approach offered by the case scenarios may help guide surgeons dealing with this difficult condition. Patient with complications after repair of classic bladder exstrophy. A) Stone retrieved from posterior urethra after complete penile disassembly. B) After opening the inter-symphiseal scar, the bladder has been opened and the bladder neck tailored. C) Complete penile disassembly has been completed with corporal bodies and urethra individualized. D,E,F) Final appearance of the repair; abdominal wall was closed with anterior rectus sheath flaps, penile skin was closed with rotational flaps and urethra ended up as a hypospadias.

The use of a modified tanagho flap in the repair of posterior urethral stricture after primary exstrophy closure

In patients with classic bladder exstrophy (CBE), posterior urethral strictures after primary bladder closure can be difficult to manage and therefore necessitate alternative techniques in reconstruction of the proximal urethra. There is a paucity of literature describing treatment and management of posterior urethral stricture arising after repair of classic bladder exstrophy. To describe the technique of a bladder neck reconstruction (BNR) with a modified Tanagho anterior detrusor flap in the treatment of posterior urethral stricture arising after repair of classic bladder exstrophy. A prospectively maintained, IRB-approved database of 1401 exstrophy-epispadias patients was reviewed for patients who underwent bladder neck or proximal urethral reconstruction with modified Tanagho flap for proximal urethral stricture disease from the time period of 1975-2021. A total of 5 patients (1 female and 4 males) with classic bladder exstrophy who underwent proximal urethral reconstruction with modified Tanagho flap were identified. Of these 5 patients, the 1 female patient was treated for ongoing incontinence, not posterior urethral stricture, and was thus excluded from this report. Outcomes measured included post operative bladder capacity, post-operative continence status, upper urinary tract status, and stricture recurrence. Four male patients with CBE were treated for posterior urethral strictures and are reviewed here. Three patients underwent successful primary closure (1 via modern staged repair of exstrophy (MSRE) and 2 via complete primary repair of exstrophy (CPRE)), and 1 patient (MSRE) required repeat closure at the time of stricture repair. Mean age at BNR Tanagho flap was 3.8 (range 2.3-5.0) years. All patients remained stricture free with post-operative urinary incontinence; 2 patients underwent subsequent elective bladder neck transection to achieve continence. Mean follow up 9.9 (range 2.1-15.6) years. All 4 patients had excellent long term success rates, comparable to results reported in several adult studies utilizing a modified Tanagho flap in women with post-traumatic bladder neck contractures. In addition, our technique described here shares similarities with both the originally described Tanagho flap as well as with a dorsal onlay graft urethroplasty. In this limited series, modified Tanagho flap reconstruction is effective in treating posterior urethral stricture disease in CBE. This technique adds a valuable adjunct to the armamentarium of surgical options for this difficult to manage condition.

Bladder exstrophy: Modern staged repair experience in our institution.

Introduction:Bladder exstrophy is a major malformation in paediatric urology. The treatment results are not still completely satisfactory, and their management is an enormous problem in Sub-Saharan Africa. While outlining our challenges, we report our management experience to improve our results.Subjects and Methods:We retrospectively reviewed the records of patients undergoing surgical repair of classic bladder exstrophy at our department between January 2010 and December 2019 (10 years). Epidemiological, clinical, therapeutic and evolution data were analysed.Results:Twenty-five children with classic bladder exstrophy were treated. Our series included 16 boys and 9 girls with a sex ratio of 1.7. Age ranged from 0 day to 6 years. Twenty-five bladder closures were performed, associated to pelvic osteotomy in 11 cases. Epispadias repair was performed on nine boys. Eight cases of bladder neck reconstruction and three cases of bladder enlargement were performed. We observed six bladder fistulas, four wound dehiscence, of which three partial, two parietal suppurations and six cases of urinary tract infection. Eight children had a continence of 1–2 h.Conclusion:The treatment of bladder exstrophy in our context is still limited because of financial difficulties encountered by the population and the insufficient technical platform in our country.

Assessment of the anterior osteotomy role in the restoration of normal pelvic floor anatomy for bladder exstrophy patients using pre and postoperative pelvic floor MRI

Our aim was to evaluate the effectiveness of anterior osteotomy in the restoration of normal pelvic floor anatomy in classic bladder exstrophy repair using pelvic floor MRI as an imaging tool for evaluation. This study is a pilot prospective randomized controlled study that included 20 infants with classic bladder exstrophy older than three months with no history of previous surgical operations. All patients underwent complete primary repair and were randomized into two groups, with or without osteotomy. Both groups were assessed preoperatively & postoperatively after three months with pelvic floor MRI and compared to age & gender-matched control group infants with normal pelvic anatomy infants who underwent pelvic MRI for other medical conditions. There was not any significant statistical difference between osteotomy and non-osteotomy groups in pre-operative demographic data and all pelvic floor MRI measurements, emphasizing that both groups were equal in all characters at the start point of the study before the operation. The mean change difference between pre-operative and post-operative pelvic floor MRI measurements were compared in both groups, and there was not any significant statistical difference in all pelvic floor MRI measurements. Both groups showed the same statistical significance when compared with the control group in all pelvic floor MRI measurements except the posterior bladder neck distance, which was in favor of the osteotomy group, closer to the normal pelvic floor anatomy. Anterior osteotomy did not have a significant difference in the restoration of the normal pelvic floor anatomy when combined with complete primary repair of classic bladder exstrophy in newly diagnosed neonates older than three months, except for a single measurement, posterior bladder neck distance. Future studies needed to determine the effect of osteotomy on continence.

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.

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

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

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.

Re: Use of Human Acellular Dermal Matrix during Classic Bladder Exstrophy Repair.

Re: Use of Human Acellular Dermal Matrix during Classic Bladder Exstrophy Repair.

Commentary to “Use of human acellular dermal matrix during classic bladder exstrophy repair”

Commentary to “Use of human acellular dermal matrix during classic bladder exstrophy repair”

Use of human acellular dermal matrix during classic bladder exstrophy repair

The extent of the abdominal wall defect in people with classic bladder exstrophy (CBE) varies, and can be extensive. In this study, human acellular dermis (HAD) was used to bridge the fascial gap, as an alternative to osteotomy, to support a fascial repair of the abdominal wall, and as a filler in selected cases of CBE. To demonstrate the efficacy of the employed techniques of using HAD within the bladder exstrophy population. The medical records of six males, born with CBE, and who had abdominal wall defects were reviewed. Two children, aged 6 and 8 years old, were referred from overseas with unrepaired bladder exstrophy plates and large abdominal wall defects (8 cm and 12 cm wide). Both had their bladders reconstructed, placed within the pelvis, and HAD was used to replace the absent abdominal wall (bridged repair) without the use of pelvic osteotomy. In three other patients, HAD reinforced the native fascial repair (bolster repair). In three patients, HAD also served as a filler for the abdominal depression that was present following initial staged repair. Where HAD was used for bridged or bolster repair, the edges of the allograft were extended 2-3 cm circumferentially beyond the perimeter of the abdominal wall defect. All six patients healed well, without evidence of abdominal wall hernias at 1-3 years postoperatively. Functionally, each patient regained an appropriate level of abdominal wall strength. Two children successfully underwent a secondary procedure through the bridged allograft repair, as each required bladder neck reconstruction and bilateral ureteral reimplantation through the reconstructed abdominal wall. Continence was achieved in these two patients, with one child voiding at 2-hourly intervals and the second at 3-hourly intervals. One patient developed a urethral-cutaneous fistula, distant to location of the allograft. There were no associated wound complications. In this series of patients born with classic bladder exstrophy, HAD acted as a biologic scaffold and allowed native cellular ingrowth and tissue remodeling. It served as an alternative to pelvic osteotomy in older patients with unrepaired CBE. The material reinforced a weak or potentially suboptimal fascial repair and filled a tissue gap, resulting in improved aesthetics. Given its ease of preparation and the lack of significant morbidity associated with its use, combined with the functional and esthetic results in the present series, HAD may be considered during delayed reconstruction of abdominopelvic tissues in people born with CBE.

Medicinal Leech Therapy for Glans Penis Congestion After Primary Bladder Exstrophy-Epispadias Repair in an Infant: A Case Report

Many postoperative complications have been reported after repair of classic bladder exstrophy. We present a case of medicinal leech therapy for glans penis congestion following exstrophy repair in an infant. A 2-week-old male with classic bladder exstrophy underwent complete primary repair. On postoperative day 1, he developed rapidly worsening glans penis venous congestion. Medicinal leech therapy was instituted with antibiotics and blood transfusions to maintain a hematocrit >30%. After 24 hours, venous congestion improved and therapy was discontinued. The patient's remaining hospital course was uncomplicated. Medicinal leeches are an effective therapy to relieve glans penis venous congestion.

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.

Preservation of renal function in the modern staged repair of classic bladder exstrophy

ObjectiveTo compare the estimated glomerular filtration rate (eGFR) in bladder exstrophy patients with published normative GFR estimates. Patients and methodseGFR was calculated using the Schwartz formula at three timepoints, with mean eGFR at each timepoint compared to normative values. ResultsAt primary closure (n = 53) the mean eGFR (ml/min/1.73 m2) in exstrophy patients was similar to norms at 0–7 days (exstrophy vs norm: 42.5 vs 40.6, p > 0.05) and after 2 years of age (108.8 vs 133, p > 0.05). However, the mean eGFR in exstrophy patients was significantly lower than norms between 8 days (44.8 vs 65.8, p < 0.0001) and 2 years of life (68 vs 95.7, p = 0.01). At bladder neck reconstruction (n = 13) no statistically significant difference existed between the exstrophy and normative eGFR values (137.1 vs 133, p > 0.05). Similarly, among 27 patients with at least 1 year follow-up after bladder neck reconstruction, the mean exstrophy eGFR was no worse or higher than normative values (2–12 years: 124.5 vs 133, p > 0.05; males ≥13 years 175.6 vs 140, p = 0.04; females ≥13 years 128.8 vs 126, p > 0.05). ConclusionThe staged reconstruction of exstrophy does not appear to negatively impact renal function in most patients. As eGFR detects only significant changes, surgical reconstruction may still cause more subtle renal damage.

Commentary to ‘The modern staged repair of classic bladder exstrophy: A detailed postoperative management strategy for primary bladder closure’

Commentary to ‘The modern staged repair of classic bladder exstrophy: A detailed postoperative management strategy for primary bladder 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.

Penile loss following complete primary repair of bladder exstrophy

The complete penile disassembly technique is increasingly employed during the surgical repair of classic bladder exstrophy. We describe the complication in a newborn boy of loss of the glans penis following complete primary exstrophy repair. The possible causes and prevention of this injury are discussed.

Economic Impact of Failed or Delayed Primary Repair of Bladder Exstrophy: Differences in Cost of Hospitalization

Patients with bladder exstrophy and failed primary newborn closure or who undergo delayed primary repair have suboptimal functional outcomes. We sought to determine whether these patients also have costlier, more resource intensive hospitalizations compared to patients who undergo neonatal primary closure. We reviewed hospital coding records to identify patients who underwent surgical repair of classic bladder exstrophy at The Johns Hopkins Hospital between 1997 and 2006, and obtained charge records for each hospitalization. Total hospital charges (excluding professional fees) were inflation adjusted to year 2005 dollars. Cases were identified as newborn primary repair, delayed primary repair or reclosure of failed prior repair. Results of classic exstrophy repair were analyzed in 80 patients. A total of 34 procedures were newborn primary repairs, 15 were delayed primary repairs and 31 were reclosures of failed prior repair. All of the patients undergoing delayed primary repairs and reclosures underwent osteotomy, compared to only 21% of those undergoing newborn primary repair. Overall mean inflation adjusted hospitalization charge was $66,348 +/- $26,625 (range $29,689 to $179,403). Newborn closures were significantly less costly (mean charge $53,188 +/- $15,086) than either reclosure ($71,621 +/- $19,366) or delayed primary closure ($85,278 +/- $42,354, p <0.0001). Controlling for multiple variables in a regression model showed that the primary factors associated with charges were operative time, days in intensive care unit and length of stay. Length of stay and operative times were significantly shorter in the newborn surgical group, likely accounting for the lower costs in this group (despite higher intensive care unit use). Mean hospital charges and mean length of stay increased during the study period. Primary newborn exstrophy repair is associated with lower surgical hospitalization costs compared to delayed primary repair and reclosure. Combined with previous data on clinical outcomes, these data reiterate the paramount importance of achieving a successful initial newborn closure whenever possible.

Complete Repair of Bladder Exstrophy in Boys: Can Hypospadias Be Avoided?

Introduction: Complete repair of classic bladder exstrophy in male newborns has been successful with minimal morbidity. However, the technique may create hypospadias in some cases. We have recently adopted a modification to obtain an orthotopic meatus in bladder exstrophy boys. Materials and methods: Between November 1998 and December 2002 complete repair of classic bladder exstrophy was carried out in 27 boys. Complete penile disassembly was performed in 22 boys including 4 newborns and 18 older children; mean age 3 ± 2 years old. Modified disassembly was used in the last 5 boys including 4 newborns and a 9-month-old boy. During repair of epispadias, the dissection starts on the ventral aspect of the penis as usual. The urethral plate is separated from both corpora cavernosa to allow ventral transposition of the plate. In the complete disassembly technique, the urethral plate is completely separated from both hemiglans. In this modification, while the urethral plate is completely separated from both corpora cavernosa, the extreme distal end of the urethral plate remains attached to the distal ends of both hemiglans. Thus, when the plate is tubularized with fine interrupted sutures the meatus ends up at the tip of the glans penis. The symphysis pubis is re-approximated as usual and corpora cavernosa are approximated dorsally in the midline. Results: There was no major complication. Mean follow-up was 43 ± 7 and 7 ± 4 months for complete and modified disassembly groups respectively. Of the 22 boys, who underwent complete disassembly, 15 (68%) ended up with hypospadias and 7 (32%) had an orthotopic meatus. Modified disassembly has resulted in an excellent cosmetic appearance and orthotopic meatus in the 5 boys. Penile length was not shorter than boys who underwent complete disassembly. Parents of the 5 boys noticed normal straight morning erection. Conclusion: Although complete penile disassembly allows ventral placement of the urethra, hypospadias is created in approximately two thirds of the cases. When the extreme distal end of the urethral plate remains attached to the distal ends of both hemiglans (modified disassembly), orthotopic meatus can be obtained in all boys. With modified disassembly, posterior mobilization of the bladder and urethra does not result in corporal angulations or shortening because of the proportionate inward movement of the corpora that accompanies symphyseal approximation. The modification is feasible in newborns and infants. These short-term results may obviate the need for later penile reconstructive procedure.