Classic Bladder Exstrophy Research Articles

EZH2 specifically regulates ISL1 during embryonic urinary tract formation

Isl1 has been described as an embryonic master control gene expressed in the pericloacal mesenchyme. Deletion of Isl1 from the genital mesenchyme in mice leads to an ectopic urethral opening and epispadias-like phenotype. Using genome wide association methods, we identified ISL1 as the key susceptibility gene for classic bladder exstrophy (CBE), comprising epispadias and exstrophy of the urinary bladder. The most significant marker (rs6874700) identified in our recent GWAS meta-analysis achieved a p value of 1.48 × 10− 24 within the ISL1 region. In silico analysis of rs6874700 and all other genome-wide significant markers in Linkage Disequilibrium (LD) with rs6874700 (D’ = 1.0; R2 > 0.90) revealed marker rs2303751 (p value 8.12 × 10− 20) as the marker with the highest regulatory effect predicted. Here, we describe a novel 1.2 kb intragenic promoter residing between 6.2 and 7.4 kb downstream of the ISL1 transcription starting site, which is located in the reverse DNA strand and harbors a binding site for EZH2 at the exact region of marker rs2303751. We show, that EZH2 silencing in HEK cells reduces ISL1 expression. We show that ezh2−/− knockout (KO) zebrafish larvae display tissues specificity of ISL1 regulation with reduced expression of Isl1 in the pronephric region of zebrafish larvae. In addition, a shorter and malformed nephric duct is observed in ezh2−/− ko zebrafish Tg(wt1ß:eGFP) reporter lines. Our study shows, that Ezh2 is a key regulator of Isl1 during urinary tract formation and suggests tissue specific ISL1 dysregulation as an underlying mechanism for CBE formation.

End-stage and chronic kidney disease in classic bladder exstrophy: A retrospective muti-institutional cohort study

IntroductionWhile most children with classic bladder exstrophy (CBE) are born with normal kidneys, some experience renal deterioration in adulthood. Little is known about the incidence of end-stage and chronic kidney disease (ESKD and CKD, respectively) in this population. Our group has recently published on surgical outcomes in a multi-institutional cohort of 216 people with CBE. Our aim was to describe the incidence of ESKD and prevalence of CKD in this cohort of people with CBE. MethodsWe retrospectively reviewed records of patients with CBE followed at five tertiary care centers described previously. The primary outcome was incidence of ESKD, defined as permanent peritoneal/hemodialysis or renal transplantation. The secondary outcome was prevalence of CKD stage 3 or higher (CKD3+, estimated glomerular filtration rate [eGFR]<60ml/min/1.73m2) at the last appointment. Creatinine-based eGFRs were calculated using the CKD-EPI Creatinine Equation (adults) and the Schwartz formula (children). Survival analysis and Fisher’s exact test were used. ResultsA total of 201 patients (93% of the original cohort) had renal function data available (63% male). Four patients who had a primary urinary diversion remained diverted at a median follow-up of 20.1 years. None developed ESKD and one developed CKD3+. The remaining 197 patients had a primary bladder closure. At a median follow-up of 18.8 years old, 12 were diverted, 108 were augmented and 77 were neither. Three patients developed ESKD (1.5%) at a median age of 23.4 years (1 hemodialysis, 2 transplantation). On survival analysis, the risk of ESKD was 0% at 10 years, 1% at 20 years and 5% at 30 years (Figure). This was higher than the risk of 0.003% at 21 years of age in the general population (p<0.001). The median age of 141 individuals with eGFR data was 21.6 years old (65% male). No children, 4% of adolescents and 8% of adults had CKD3+ (p=0.45). On exploratory analyses, prevalence of CKD3+ did not differ by center or birth year (p>=0.99). ConclusionsThe risk of ESKD and CKD among patients with CBE is not insignificant and appears to be more common than the general population. The potential role of modifiable contributing factors, such as increased bladder outlet resistance, warrants further investigation. Reliable long-term follow up is needed in this population to monitor for ESKD and CKD.

Stomal Stenosis After Continent Urinary Diversion in Bladder Exstrophy: Risk Factors and Management

OBJECTIVETo identify risk factors for stenosis and compare management strategies for stenosis etiology and to examine the efficacy of each approach. Patients with classic bladder exstrophy (CBE), a rare genitourinary malformation, may require construction of a continent urinary stoma (CUS) if incontinence persists. Stomal stenosis is a challenging complication as it is common, progressive and recurrent. METHODSCBE patients who underwent CUS were retrospectively reviewed for risk factors for stenosis including stoma type, prior midline laparotomy number, and umbilicoplasty suture material. Stenosis etiology and management strategies were further reviewed. RESULTSA total of 260 CBE patients underwent CUS creation. Stenosis developed in 65 patients (25.0%) at a median interval of 1.9 years. Etiology included scar contracture (n=41), keloid (n=17), and hypertrophic scar (n=7). Multifilament suture was the only variable associated with an increased risk of stenosis compared to monofilament suture (p=0.009). Almost all patients required surgical intervention. Most scar contractures underwent stomal incision with success in 100%. Hypertrophic scars and keloids responded best to excision with local tissue rearrangement (66.7%). At last follow-up, all patients achieved success. CONCLUSIONStomal stenosis is common and challenging for the reconstructive surgeon. Strategies to prevent and effectively manage this are greatly desired. Use of multifilament suture for the umbilicoplasty increased stenosis perhaps from a greater inflammatory response and scarring, while monofilament suture may reduce its incidence. Stomal incision for treating scar contractures, and excision with local tissue rearrangement for hypertrophic scars and keloids may improve successful primary surgical intervention.

Adult patients treated for bladder exstrophy at a young age What are their current demands?

Bladder exstrophy-epispadias complex is a rare condition that necessitates numerous surgical procedures during a patient's youth to achieve adequate urine storage and continence. This study aimed to identify the specific needs and functional challenges faced by adults who underwent pediatric bladder exstrophy reconstructions and assess the management of these issues in an adult population. A retrospective chart review was conducted for all bladder exstrophy complex patients who underwent surgery at a young age and were subsequently referred to our center between 2005 and 2020. Inclusion criteria included patients with cloacal or classical bladder exstrophy older than 18 years. We recorded the reasons for referral, management of contemporary complaints, types of past and present urinary reconstructions, and their current functional status. The study included 38 patients. The primary reasons for referral were incontinence (39%) and catheterization difficulties (24%). Management typically involved partial or complete surgical revision of their urinary reservoir, occasionally combined with a bladder neck procedure. Ultimately, only three patients continued to experience incontinence, while none reported catheterization issues after undergoing treatment at our center. Long-term exstrophy-related reconstruction complications included urinary tract infections (39%), stones (29%), stenosis (24%), fistulas (13%), chronic renal disease (16%), metabolic abnormalities (3%), and cancer (3%). Adults who have previously undergone bladder exstrophy reconstruction exhibit a wide range of urologic reconstructions. Their needs often revolve around continence and catheterization concerns. Most patients with satisfactory functional outcomes perform self-catheterization through a continent cutaneous channel and have either a continent pouch or an augmented bladder.

Classic bladder exstrophy – the timing of initial closure and technical highlights

Classic bladder exstrophy – the timing of initial closure and technical highlights

Classic bladder exstrophy: patient and operating theatre preparation for primary bladder closure

Classic bladder exstrophy: patient and operating theatre preparation for primary bladder closure

Orthopaedic management and considerations for classic bladder exstrophy

Orthopaedic management and considerations for classic bladder exstrophy

Editorial comment on “Long-term psychosexual adjustment of adults born with classic bladder exstrophy”

Editorial comment on “Long-term psychosexual adjustment of adults born with classic bladder exstrophy”

Bladder exstrophy–epispadias complex: The effect of urotherapy on incontinence

IntroductionAchieving urinary continence is a key goal in children born with the bladder exstrophy-epispadias complex (BEEC). Unfortunately, this goal is only moderately achieved despite sometimes extensive surgical treatment. Undergoing repeated hospitalization and operations may consequently have a negative impact on quality of life. We therefore believe that other, conservative treatment options should be explored in an earlier stage of incontinence treatment in BEEC patients. As part of this, an intensive urotherapy program based on was offered to patients with persistent incontinence after reconstructive surgery for BEEC. ObjectiveThe aim of this study is to evaluate the benefits of intensive urotherapy on incontinence after reconstructive surgery in children with BEEC. Study designA retrospective chart study was performed including all children who were enrolled in an intensive urotherapy program because of persistent incontinence after reconstructive surgery for BEEC. Urotherapy consisted of a ten-day inpatient training program based on cognitive behavioral therapy, with intensive follow-up by experienced urotherapists. Main outcome measurement was continence, expressed as the percentage of children that achieved complete continence (good result; 100% dry) or 50-99% decrease of wet days a week (improved result) after treatment. ResultsData of 33 patients with a mean age of 10.6 years were analyzed. In 61% of cases (20/33) an improved or good result was reported on incontinence after urotherapy. Children with classic bladder exstrophy more often achieved a good or improved result (13/16; 81%), compared to children with epispadias (6/16; 38%). The only patient with a cloacal exstrophy completed treatment with an improved result. From the group of patients with persistent incontinence, 75% (12/16) reported that the complaints were socially acceptable at the end of follow-up. DiscussionBy following our intensive urotherapy program the majority of patients achieved complete continence or improved incontinence. In addition, our results show that the inpatient training program has a positive impact on acceptance in cases of persistent incontinence. The urotherapists offer individualized care and clear guidance, which we deem essential elements of successful treatment. Considering that repeated surgery may impede progress and offers no guarantee of continence, we recommend giving preference to conservative treatment options ConclusionOur results show that 37% (12/33) of patients with BEEC who were enrolled in our intensive urotherapy program because of persistent incontinence after reconstructive surgery, achieved complete continence after urotherapy and 63% (21/33) still experienced some degree of incontinence. 75% of patients who did not achieve complete continence, described the remaining incontinence as socially acceptable. These findings strongly support counselling patients with BEEC to consider conservative treatment before opting for further surgery.

Long-term psychosexual adjustment of adults born with classic bladder exstrophy

In literature studies exploring long-term psychosexual development and intimacy of adults living with the complex genitourinary conditions associated with classic bladder exstrophy (BE) are scarce, with small sample sizes and lacking in methodology. This study aims to examine areas of potential psychosexual distress in adults born with classic BE to develop targeted clinical interventions. The validated Sexrelation Evaluation Schedule Assessment Monitoring (SESAMO) questionnaire was administered to all BE patients aged ≥18 years operated on in our tertiary referral center during infancy. Z-scores were calculated for each area of interest, considering i) gender (female vs male); ii) committed partnership status (singles vs couples); iii) the voiding technique adopted to empty the bladder. A total of 33 (F:M 12:21; singles: couples 11:22) adults with BE were enrolled in the study at a median age of 39 (32-47) years. Overall, BE adults performed the worst regarding psychosexual identity (z-score:-1.282), pleasure (z-score:-0.915) and desire (z-score:-0.583); singles regarding relational attitude (z-score:-1.751) and imaginative eroticism (z-score:-0.806); couples regarding extramarital sexuality (z-score:-1.175) and sexual communication (z-score:-0.255). When it came to gender, females significantly performed worse than males regarding psychosexual identity (-1.645 vs -1.282; p-value:<0.0001) and areas of pleasure (-1.126 vs -0.359; p-value:<0.001). Single females performed worse than males regarding actual masturbation (-0.763 vs 0.583; p-value:<0.05) and better regarding relational attitude (-1.226 vs -1.751; p-value:<0.05). Females in stable relationships performed worse than males regarding actual masturbation (-1.645 vs 0.306; p-value:<0.05) and better regarding sexual intercourse (1.866 vs -0.565; p-value:<0.01). The voiding techniques used to empty the bladder did not show any influence on these results. Adults with BE have a greater likelihood of experiencing a wide range of psychosexual difficulties. Identifying the specific areas of psychological distress can help them cope with their medical experience and actual clinical condition and clinicians plan adequate psychological interventions.

An Analysis of the Efficacy of Multilayered Repair and Reconstruction Using Combined Tissue Pedicle Flaps for Abdominal Wall Defects in Adult Bladder Exstrophy Patients.

In this study, we conducted a retrospective analysis of cases involving adult classic bladder exstrophy (CBE) accompanied by the absence of the abdominal wall. Specifically, we focused on the utilization of multilayer flaps for reconstructive purposes. In addition, we aimed to share our clinical treatment experience pertaining to similar challenges, thereby providing valuable insights to complement the surgical management of this rare disease. We conducted a retrospective analysis of 12 adult patients diagnosed with CBE who underwent initial treatment between June 2013 and January 2020. All patients underwent multilayer reconstruction to address their abdominal wall defects. This involved utilizing shallow flaps derived from the superficial fascia of the abdomen and incorporating myofascial flaps composed of the anterior sheath of the rectus abdominis and aponeurosis of the external oblique muscle. The flap sizes ranged from 9 × 11 cm to 13 × 15 cm. Abdominal wall reconstruction in the 12 patients with CBE resulted in an absence of wound dehiscence recurrence, urinary obstruction, or urinary tract infection. All patients expressed satisfaction with the aesthetic outcome of their abdominal wall based on self-evaluation. They reported a successful resumption of normal life and work activities without experiencing any restrictions. The married patients expressed contentment with their sexual function. The utilization of a multilayered reconstruction technique involving multiple flaps in adults with congenital CBE allows for successful restoration of urinary function, as well as the attainment of sufficient abdominal wall strength to support daily life and work activities, while preserving sexual function. However, it is important to approach the evaluation of surgical outcomes with caution because of the rarity of this condition and the lack of objective assessment measures.

Innervation pattern of the unclosed detrusor muscle in classic bladder exstrophy: a study of patients with urothelial overexpression of nerve growth factor

PurposeAn overexpression of nerve growth factor (NGF) in the urothelium is discussed to lead to neuronal hyperinnervation of the bladder detrusor. The aim was to assess the sensory and sympathetic innervation of the detrusor in unclosed exstrophic bladders patients with known overexpression of NGF in the urothelium.MethodsFull-thickness bladder biopsies were prospectively obtained from 34 infants at delayed primary bladder closure between 01/2015 and 04/2020. The bladder biopsies were immunohistochemically stained with antibodies against S100, calcitonin gene-related peptide (anti-CGRP), Neurofilament 200 (anti-NF200), and tyrosine-hydroxylase (anti-TH). Specimens from 6 children with congenital vesicoureterorenal reflux (VUR) served as controls.ResultsThere was no statistically significant difference in nerve fiber density in any of the immunohistochemical assessments (anti-S100 [p = 0.210], anti-CGRP [p = 0.897], anti-NF200 [p = 0.897]), and anti-TH [p = 0.956]) between patients with BE and patients with VUR. However, we observed a trend toward lower nerve fiber densities in exstrophic detrusor.ConclusionOverall our results showed an unharmed innervation pattern in this cohort but a lower density of nerve fibers in the detrusor compared to controls. Further studies in patients after successful primary closure are needed to clarify the potential impact of the urothelial overexpression of NGF modulating the innervation pattern in exstrophic bladders.

Comment to “Optimizing prenatal diagnosis and referral of classic bladder exstrophy: lessons from a single-institution experience”

Comment to “Optimizing prenatal diagnosis and referral of classic bladder exstrophy: lessons from a single-institution experience”

Adult patients treated for bladder exstrophy at a young age: What are their current demands?

Bladder exstrophy-epispadias complex is a rare condition that necessitates numerous surgical procedures during a patient's youth to achieve adequate urine storage and continence. This study aimed to identify the specific needs and functional challenges faced by adults who underwent pediatric bladder exstrophy reconstructions and assessing the management of these issues in an adult population. A retrospective chart review was conducted for all bladder exstrophy complex patients who underwent surgery at a young age and were subsequently referred to our center between 2005 and 2020. Inclusion criteria included patients with cloacal or classical bladder exstrophy older than 18 years. We recorded the reasons for referral, management of contemporary complaints, types of past and present urinary reconstructions, and their current functional status. The study included 38 patients. The primary reasons for referral were incontinence (39%) and catheterization difficulties (24%). Management typically involved partial or complete surgical revision of their urinary reservoir, occasionally combined with a bladder neck procedure. Ultimately, only three patients continued to experience incontinence, while none reported catheterization issues after they underwent treatment at our center. Long-term exstrophy-related reconstruction complications included urinary tract infections (39%), stones (29%), stenosis (24%), fistulas (13%), chronic renal disease (16%), metabolic abnormalities (3%), and cancer (3%). Adults who previously underwent bladder exstrophy reconstruction exhibit a wide range of urologic reconstructions. Their needs often revolve around continence and catheterization concerns. Most patients with satisfactory functional outcomes perform selfcatheterization through a continent cutaneous channel and have either a continent pouch or an augmented bladder.

Optimizing prenatal diagnosis and referral of classic bladder exstrophy: Lessons from a single-institution experience

Classic bladder exstrophy (CBE) is a malformation of the genitourinary system that occurs due to failure of abdominal wall closure. Unlike other malformations of similar incidence, prenatal diagnosis of CBE relies on suggested, rather than formal, diagnostic criteria. This report describes prenatal diagnosis of CBE in the largest single-institutional cohort to date and delineates key sonographic findings and protocols for specialist referral. A single-institutional database was reviewed for CBE patients born since 2000. Data on screening ultrasound use, gestational age at ultrasound, and abnormal findings were extracted. Where possible, time of prenatal diagnosis (pre- or postnatal and gestational age), ultrasound findings and other imaging data, specialist referral, institution of birth and closure, and outcome of primary closure attempt were compared. Of 557 patients born with CBE between 2000 and 2022, 284 met inclusion criteria and complete data were available for 280 (229 born domestically and 51 born internationally) who were included for analysis. Abnormal sonography suggestive of CBE was present for 48% (n=134) of patients, for whom absent bladder was the most common abnormal finding (76% [102/134]). Of domestic patients, 46% (n=106) were diagnosed prenatally at a median gestational age of 22 weeks (inter-quartile range [IQR]: 20-24), and 14% (n=32) underwent confirmatory fetal magnetic resonance imaging. Of domestic patients with abnormal prenatal findings, 75% (n=80/106) consulted with maternal-fetal medicine and 58% (n=62/106) consulted with pediatric urology. On univariate analysis, prenatal diagnosis was positively associated with primary repair at Association for the Bladder Exstrophy Community-recognized centers of excellence (54% vs. 38%, p=0.02) and negatively associated with osteotomy at primary closure (41% vs 59%, p=0.003) but not success of primary closure (74% vs. 82%, p=0.07). Rates of prenatal diagnosis in this cohort were similar to previous reports of smaller cohorts. Diagnosis allows for comprehensive pre- and postnatal follow-up with a pediatric urologist, with implications on birth planning and decisions on termination of pregnancy. Because of the previously-reported association between exstrophy and invitro fertilization, these pregnancies should undergo detailed sonography. Any nonvisualization of the fetal bladder should prompt a detailed exam, and any finding characteristic of bladder exstrophy warrants referral to pediatric urology. Although CBE is a rare disorder, it is underdiagnosed during pregnancy. Sonographers and obstetricians should be aware of characteristic findings and best practices following diagnosis. Early referral to pediatric urology and maternal-fetal medicine is important for counseling and postnatal planning.

Interposing Rectus and Gracilis Muscle Flaps For Pelvic Reconstruction in Bladder Exstrophy After Bladder Neck Closure.

The exstrophy-epispadias complex is a spectrum of ventral wall malformations including classic bladder exstrophy (CBE) and cloacal exstrophy (CE). Patients undergo multiple soft-tissues procedures to achieve urinary continence. If unsuccessful bladder neck closure (BNC) is performed, muscle flaps may be used to reinforce BNC or afterwards for fistula reconstruction. In this study, patients reconstructed using a rectus abdominis or gracilis muscle flap were reviewed. A retrospective cohort study of exstrophy-epispadias complex patients who underwent BNC and had a muscle fap was performed. Indication for flap use, surgical technique, risks for BNC failure including mucosal violations (MVs) were reviewed. MVs were prior bladder mucosa manipulation for exstrophy closure, repeat closure(s) and bladder neck reconstruction. Success was defined as BNC without fistula development. Thirty-four patients underwent reconstruction. Indications included during BNC (n=13), fistula closure after BNC (n=17), following BNC during open cystolithotomy (n=1) or fistula closure after open cystolithotomy (n=3). A vesicourethral fistula developed most frequently in CBE (88.9%) and vesicoperineal fistula in CE (87.5%). Thirty-three rectus flaps and 3 gracilis flap were used with success achieved in 97.1% and 66.7%, respectively. All 34 patients achieved success and 2 CE patients required a second flap. The rectus flap is preferred as it covers the antero-inferior bladder and pelvic floor to prevent urethral, cutaneous, and perineal fistula formation. The gracilis flap only reaches the pelvic floor to prevent urethral and perineal fistula development. Increased MVs, increase the risk of fistula formation and may influence the need for prophylactic flaps.

Perioperative management of primary classic bladder exstrophy: A single institutional pathway to success

Appropriate perioperative management is crucial in patients undergoing classic bladder exstrophy closure (CBE). Therefore, the authors sought to review their intra and postoperative management of patients with CBE undergoing primary closure and examine the impact of this pathway on patient outcomes. A prospectively maintained institutional approved exstrophy-epispadias complex database was reviewed for patients with CBE who had undergone primary closure between 2016 and 2022 and whose closure was performed within one year of age. Electronic medical records for eligible patients were retrospectively reviewed to examine patient demographics, use of pelvic osteotomy, immobilization status, pediatric intensive care unit (PICU) admission and management, perioperative analgesia and sedation, nutritional support, drainage tubes, blood transfusions, antibiotic coverage, hospital length of stay, postoperative complications, and closure failure. A total of 25 patients were identified, 22 with CBE and 3 with variant CBE. Closure was performed at a median age of 84 days with patients ranging in age from 9 to 351 days. All patients underwent osteotomy and immobilization with modified Buck's traction and external fixation for a median duration of 41 days. A suprapubic tube was placed in all patients for a median duration of 46.5 days. All patients underwent PICU admission following closure for a median duration of 8 days. Ventilator support was required in 68% of patients for a median of 3 days. Epidural analgesia was used in all patients and catheters were maintained for a median duration of 19 days. All patients received a blood transfusion over the course of their admission. Patient-controlled analgesia was used in most patients as an adjunct for a median duration of 38.5 days. Other commonly used analgesic adjuncts included acetaminophen, diazepam, clonidine, and dexmedetomidine. TPN was used in 80% of patients for a median of 7 days with a return of oral feeding thereafter. Overall, the closure success rate in this cohort of patients was 100%. The outcome of primary bladder closure can have inauspicious consequences that can affect a child's continence for years. The incidence of failed bladder closure can be minimized with the implementation of a detailed plan for immobilization, analgesia, and nutrition guided by an experienced multi-disciplinary team. We have identified several guiding principles for perioperative success in exstrophy patients at our center including Buck's traction with external fixation, provision of adequate postoperative analgesia and sedation, aggressive nutritional support, renal and bladder drainage, and robust antibacterial support. Our high success rate in managing this complex pathology demonstrates its validity and use as a pathway to success.

Modified penile reconstruction in classic bladder exstrophy: Can complete corporal covering of the urethral closure be achieved using incomplete disassembly technique?

To answer the question of whether it is possible to achieve complete corporal covering of the urethral closure using incomplete penile disassembly in classic bladder exstrophy. We hypothesize that mobilization of the corpora under Buck's fascia, their dorsal translocation through the incisions in Buck's fascia and suturing corporal convex sides above the urethra would allow extend corporal covering of the urethra, reducing the risk of urethra-cutaneous fistula formation. A prospective follow-up on all boys who underwent the modified Cantwell-Ransley primary penile reconstruction was conducted. Inclusion criteria comprised bladder exstrophy closure in our institution, ensuring a postoperative follow-up period of no less than 24 months. The key innovation of the technique lies in a deep dissection of the dependent corpora under Buck's fascia, followed by their dorsal relocation through extended dorsal incisions in Buck's fascia, and limited external corporal rotation 90 degrees only at the base of the penis. Between November 2019 and March 2022, 18 boys aged 11 to 35 months met the inclusion criteria and underwent the modified penile reconstruction. Surgical procedures and postoperative period did not include any major complications. Total corporal covering of the urethral sutures was achieved in 15 of 18 patients. No urethra-cutaneous fistulas were observed within 2 years of follow-up. All individuals demonstrated spontaneous erections, and the absence of dorsal curvature was documented. The modified technique of incomplete penile disassembly applied in a homogenous group of patients with classic bladder exstrophy allows penile shaft elongation, improved aesthetic outcomes, preserved erections, and eliminates dorsal curvature. The technique demonstrated feasibility and reliability while maintaining positive effects on tissue circulation. The absence of urethra-cutaneous fistulae is attributed to the complete corporal covering of the urethral sutures and supports the initial hypothesis.

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 true pelvic volume change with various corrective osteotomy techniques for exstrophy-epispadias complex spectrum: the value of computer-assisted virtual surgery.

Pelvic osteotomies are essential to approximate widened symphysis pubis in the exstrophy-epispadias complex, yet it is unknown which osteotomy type has the greatest effect on pelvic volume. We therefore used virtual surgery to study pelvic volume change with anterior, oblique, and posterior iliac osteotomies. Preoperative CT scans of two cloacal and one classic bladder exstrophy patients were used. Simulations were free-hand or constrained to keep minimal strain in the sacrospinous SSL and sacrotuberous STL ligaments. Changes in inter-pubic distance, pelvic volume, SSL and STL strains were measured. Mean pelvic volume decreased by 10% with free hand compared to 23% with constrained simulations ( P = 0.171) and decreased by 7% with posterior, 17% with diagonal and 26% with horizontal osteotomies ( P = 0.193). SSL and STL were strained by 20% and 26%, respectively, with free-hand simulations. A statistically significant moderate positive correlation was found between the decrease in inter-pubic distance and reduction in pelvic volume (r = 0.6, P = 0.004). Mean pelvic volume decreased 0.05, 0.37 and 0.62% for each mm of pubic symphysis approximation with posterior, diagonal and horizontal osteotomies, respectively. Differences in effect on pelvic volume were identified between the osteotomies using virtual surgery which predicted residual diastasis in actual cloacal exstrophy surgical reconstructions. Oblique osteotomies are a compromise, avoiding difficulties with posterior osteotomies and excessive pelvic volume reduction with horizontal osteotomies. Understanding how osteotomy type affects pelvic morphology with virtual surgery may be an effective adjunct to pre-operative planning in exstrophy spectrum.