Bladder Exstrophy-epispadias Complex Research Articles

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.

Multi-Institutional Bladder Exstrophy Consortium After 8 Years: The Short- and Intermediate-Term Outcomes.

Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real-time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long-term protection of the upper tracts. This study reports on our short-term complications and intermediate-term continence outcomes. A single prospective database for all patients undergoing surgery with a BE epispadias complex diagnosis at 3 institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies, and immobilization techniques, and subsequent surgeries. Data on short-term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate-term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound, presence of vesicoureteral reflux, cortical defects on nuclear scintigraphy, and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization, and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded. CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (interquartile range) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 patients who did not have an osteotomy), 6 in modified Bryant's traction, and 2 in external fixation with Buck's traction. Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short-term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate-term outcomes were available for 40 of the 46 patients, who have between 4 and 8 years of follow-up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals. Cumulative efforts of prospective data collection have provided granular data for evaluation. Short-term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate-term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow-up are voiding with dry intervals of > 1 hour.

Bilateral Obturator Osteotomy: A Novel Osteotomy for Bladder Exstrophy Closure

BackgroundAbdominal and pelvic closure remains a challenge during bladder exstrophy initial repair. We aimed to report on the feasibility and results of a novel technique of bilateral obturator osteotomy. MethodsRetrospective study of prospective collected data of children who underwent single-stage delayed bladder exstrophy closure combined with RSTM (Radical Soft Tissue Mobilization) for BEEC (Bladder Exstrophy Epispadias Complex) by the same team at different institutions between December 2017 and May 2021. When pubic approximation was not feasible at the end of the procedure, bilateral obturator osteotomy was performed through the same approach, consisting in bilateral divisions of the ilio-pubic rami, ischio-pubic rami, obturator membrane, and detachment of the internal obturator muscle. Pubic bone fragments were approximated together on the midline. Immobilization in a thermoformed posterior splint was indicated for 3 weeks. The main outcome criterion was the bladder dehiscence rate at 6 months, assessed by physical inspection. Secondary outcome criteria included neurovascular obturator pedicle injury, analyzed during orthopedic physical examination, wound or bone infections, gait acquisition, reported by parents and evaluated during medical examination, and vascular penile impairment, judged by penile and glans coloration. Results17 children (11 males, 6 females) were included, at a median age of 2 months [1–33]; and representing 29% (17/58) of the children with bladder exstrophy who underwent the same surgical approach during the time of study. There was no postoperative bladder dehiscence with a median follow-up of 34 months [6–47]. No complication was observed. Pelvic X-rays showed bilateral normal ossification process. Neither gait abnormality, nor clinical indication of obturator nerve deficiency was observed during follow-up. ConclusionWhen pubic bones approximation is not possible, bilateral obturator osteotomy is a useful adjunct in bladder exstrophy closure, feasible by the pediatric urologist through the same approach, and not requiring external fixator. Level of EvidenceIV.

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.

The hidden burden of Pediatric urology in Sub-Saharan Africa: an analysis of hospital admission data from three East African Health Centres.

Specialist facilities for children are still unavailable in some Sub-Saharan African contexts. It is the case of pediatric urology, whose recent advances are still largely unshared. Prenatal diagnosis of urinary abnormalities (CAKUT) is largely unknown. Early recognition and referral of Undescended testis (UDT), Hypospadia, bladder exstrophy epispadias complex, ambiguous genitalia, stone disease, and tumours are uncommon in rural areas. Missed diagnosis is not uncommon and delayed management is associated with poor outcomes. We present a cross-sectional, descriptive study about the epidemiology of Pediatric urological admissions to three sub-Saharan East African Hospitals. All the urological cases between 0-18 years referred to three distinct East African Hospitals over 124 weeks were considered. Prevalence of different groups of diseases, age, and mode of presentation were reported. We found 351 cases (M/F 127/24) out of 2543 surgical referrals (13%). Seventy percent of cases were Hypospadias and UDT. Fifty percent of UDT were beyond 6, and most Hypospadias were between 4 and 7 yrs. CAKUT had a very low prevalence (4.84%), and about 50% of Wilms Tumours came too late to be resectable. In many African contexts, urology is still a tiny portion of the pediatric surgical workload compared to the 25% of European and American reports. There are also differences in the epidemiology of genitourinary conditions. A hidden burden of diseases may be presumed, remaining undiagnosed due to the shortage of specialist facilities.

Isolated Male Epispadias with Continence in a 40 Year Old: A Case Report

Epispadias is part of the bladder exstrophy-epispadias complex; it has a defective dorsal wall of the urethra with potential incompetence of the urinary continence mechanism. Isolated male epispadias is due to failure of the urethral plate to tubularize on the dorsum of the penis. It is rare with an incidence rate of 1 in 117,000 live births in males. The severity depends on the position of the urethral opening and ranges from peno-pubic to penile and glandular. Epispadias is usually repaired within the first year of life though some patients have presented in their second decade. Epispadias is classically associated with bladder exstrophy in over 90% of the cases while isolated epispadias with continence is very rare constituting less than 10% of cases. Separation of pelvic bones is seen in 70% of peno-pubic epispadias affecting the bladder neck and external sphincter leading to incontinence and stress urinary dribbling. The goals of repair include; achieving a cosmetically acceptable and functional penis, which is straight and adequate in length, enabling penetrative sexual intercourse and urinary continence. There have been reports of isolated epispadias in adults but none was as old as 40 years. This patient has been married for 20 years and has been unable to impregnate his spouse. He had modified Cantwell-Ransley procedure after a detailed clinical and psychological evaluation to achieve the goals of repair. Adults presenting with isolated continent peno-pubic epispadias are rare. Presenting at this age and after having been married for two decades could have a derogatory effect on body image, self-confidence, psycho-sexual and reproductive life. Surgical correction of this anomaly as we did has the potential to lead to acceptable outcomes as demonstrated in this index case.

De novo variants identified by trio whole exome sequencing of bladder exstrophy epispadias complex.

Bladder exstrophy epispadias complex (BEEC) encompasses a spectrum of conditions ranging from mild epispadias to the most severe form: omphalocele-bladder exstrophy-imperforate anus-spinal defects (OEIS). BEEC involves abnormalities related to anatomical structures that are proposed to have a similar underlying etiology and pathogenesis. In general, BEEC, is considered to arise from a sequence of events in embryonic development and is believed to be a multi-etiological disease with contributions from genetic and environmental factors. Several genes have been implicated and mouse models have been generated, including a knockout model of p63, which is involved in the synthesis of stratified epithelium. Mice lacking p63 have undifferentiated ventral urothelium. MNX1 has also been implicated. In addition, cigarette smoking, diazepam and clomid have been implied as environmental factors due to their relative association. By in large, the etiology and pathogenesis of human BEEC is unknown. We performed de novo analysis of whole exome sequencing (WES) of germline samples from 31 unrelated trios where the probands have a diagnosis of BEEC syndrome. We also evaluated the DECIPHER database to identify copy number variants (CNVs) in genes in individuals with the search terms "bladder exstrophy" in an attempt to identify additional candidate genes within these regions. Several de novo variants were identified; however, a candidate gene is still unclear. This data further supports the multi-etiological nature of BEEC.

Sexual function in adolescents and young adults (AYA) with Bladder exstrophy-epispadias complex: Influence of the incontinence

BackgroundBladder exstrophy-epispadias complex (BEEC) is the most severe congenital anomaly involving the urinary tract. Currently, there is a growing interest in the long-term outcomes and their impact on quality of life (QoL). MethodsOur aim was to determine the incidence of urinary incontinence and whether it affects sexual function. The study population included BEEC patients >18 years treated at a specialized third-level hospital. We contacted them via telephone and emailed a voluntary online questionnaire which was anonymously self-completed. The online test included validated questionnaires to assess the following parameters: incontinence (ICIQ-SF), LUTS (ICIQ-FLUTS/IPSS) and sexual function (FSH/FSM2). Answers of the questionnaires were collected into a database for further evaluation. Statistical significance was defined by p < .05. ResultsAmong the thirteen patients (7 men and 6 women) who answered the questionnaire the median score on ICIQ-SF was 6/21. Based on that questionnaire only 4 patients (30.8 %) were classified as continent (ICIQ-SF = 0). However, when directly asked if they were able to stay dry in their day-to-day life, 61.5 % responded affirmatively, a number that doubled those who were considered continent according to the ICIQ-SF test. Incontinence was associated with an increased sexual dysfunction, having a correlation with low satisfaction in men and diminished libido and lubrication in women. ConclusionsIn the present study, we found a high incontinence rate, which is discrepant with the self-perception of day-to-day dryness. In addition, our data showed that incontinence is linked to a diminished sexual function.

Exome Survey and Candidate Gene Re-Sequencing Identifies Novel Exstrophy Candidate Genes and Implicates LZTR1 in Disease Formation.

The bladder exstrophy-epispadias complex (BEEC) is a spectrum of congenital abnormalities that involves the abdominal wall, the bony pelvis, the urinary tract, the external genitalia, and, in severe cases, the gastrointestinal tract as well. Herein, we performed an exome analysis of case-parent trios with cloacal exstrophy (CE), the most severe form of the BEEC. Furthermore, we surveyed the exome of a sib-pair presenting with classic bladder exstrophy (CBE) and epispadias (E) only. Moreover, we performed large-scale re-sequencing of CBE individuals for novel candidate genes that were derived from the current exome analysis, as well as for previously reported candidate genes within the CBE phenocritical region, 22q11.2. The exome survey in the CE case-parent trios identified two candidate genes harboring de novo variants (NR1H2, GKAP1), four candidate genes with autosomal-recessive biallelic variants (AKR1B10, CLSTN3, NDST4, PLEKHB1) and one candidate gene with suggestive uniparental disomy (SVEP1). However, re-sequencing did not identify any additional variant carriers in these candidate genes. Analysis of the affected sib-pair revealed no candidate gene. Re-sequencing of the genes within the 22q11.2 CBE phenocritical region identified two highly conserved frameshift variants that led to early termination in two independent CBE males, in LZTR1 (c.978_985del, p.Ser327fster6) and in SLC7A4 (c.1087delC, p.Arg363fster68). According to previous studies, our study further implicates LZTR1 in CBE formation. Exome analysis-derived candidate genes from CE individuals may not represent a frequent indicator for other BEEC phenotypes and warrant molecular analysis before their involvement in disease formation can be assumed.

Ensuring our exstrophy-epispadias complex patients and families thrive.

Individuals with bladder exstrophy-epispadias complex (EEC) need long-term integrated medical/surgical and psychosocial care. These individuals are at risk for medical and surgical complications and experience social and psychological obstacles related to their genitourinary anomaly. This care needs to be accessible, comprehensive, and coordinated. Multiple surgical interventions, reoccurring hospitalizations, urinary and fecal incontinence, extensive treatment regimens for continent diversions, genital differences, and sexual health implications affect the quality of life for the EEC patient. Interventions must include psychosocial support, medical literacy initiatives, behavioral health services, school and educational consultation, peer-to-peer opportunities, referrals to disease-specific camps, mitigation of adverse childhood events (ACEs), formal transition of care to adult providers, family and teen advisory opportunities, and clinical care coordination. The priority of long-term kidney health will necessitate strong collaboration among urology and nephrology teams. Given the rarity of these conditions, multi-center and global efforts are paramount in the trajectory of improving care for the EEC population. To achieve the highest standards of care and ensure that individuals with EEC can thrive in their environment, multidisciplinary and integrated medical/surgical and psychosocial services are imperative.

EDITORIAL COMMENT

The authors are to be commended in their maintenance of a database of their bladder exstrophy-epispadias complex patients which has served as a valuable tool for assessing outcomes and the evolution of care of exstrophy patients over time. The authors have a large referral population, but because most patients who seek care at a referral center following closure elsewhere do so because of complications or being dissatisfied with their care, the outcomes data of patients treated initially at an outside institution tend to be biased. Thus, the authors have carefully omitted any comparison between outcomes at their institution vs those done at an outside facility. The real strength and impact of this study comes from looking at how trends at the authors institution, a high-volume center of excellence, have changed over time.

Global surgical collaboration to treat bladder exstrophy-epispadias in India led to a larger geographical catchment area

Background A gap of care exists in low- and middle-income countries (LMICs) for patients with complex urogenital abnormalities, such as bladder exstrophy-epispadias complex (BEEC). A successful primary surgical repair is critical for optimal long-term outcomes for BEEC, but the availability of such a complex procedure is limited by the expertise needed to deliver a successful initial repair. We hypothesize that a long-term, multi-institutional collaboration based at a tertiary institution in a LMIC may improve outcomes for BEEC by encouraging earlier referrals for the first repair, rather than after failed repairs, and also increase the geographical catchment area by encouraging more distant referrals, once the availability of care becomes widely known. Methods A long-term collaboration between two US academic research centers and the Civil Hospital in Ahmedabad, Gujarat, India was formed in 2009. This collaboration expanded later to include another US and one Middle Eastern hospital system. The entire post-operative cohort of the collaboration was recalled in 2019 and 2020, when epidemiology and demographic surveys were obtained from 82 patients. Results Since 2009, the collaboration has grown overall. The geographical catchment area for referrals and distance traveled by patients to reach the collaboration site has increased to include nine states in India and surrounding countries in Southeast Asia (P = 0.044). Patients traveling from farther distances tended to be of higher socioeconomic status than local referrals (P = 0.041). Conclusions The success of the International Bladder Exstrophy Consortium to improve the care for BEEC patients in India and Southeast Asia is visible from its changing demographics. Patients, some even from families of a higher socioeconomic status, are traveling farther to receive care at a government-funded tertiary hospital. While not all positive benefits of a global surgery collaboration regarding earlier and primary referrals have been achieved, the collaboration is seeing benefits that align with a staged process of referrals. The continued commitment to creating a center of excellence is a model for sustained success for global surgery collaborations.

Exstrophic bladder duplication in the sagittal plane: Surgical management of a rare case

The bladder-Exstrophy-Epispadias complex (BEEC) contains a wide spectrum of congenital malformations. A treatment naïve refugee was referred to our center with what was identified as BEEC. A 27-year-old female patient was referred for total incontinence since birth by the general practitioner from the refugee center. An exstrophic bladder with blind ending ureteral orifices and a second non-exstrophic bladder with two orthotopic ureters was identified, demonstrating the bladder duplication in the sagittal plane. Laparotomy was performed, dissecting the exstrophic bladder plate caudally and using it as a ventral onlay to augment the non-exstrophic bladder. A Mitchell-type bladder neck reconstruction was performed with an autologous fascia sling around the bladder neck to obtain continence. As the patient had never voluntarily voided, chances of spontaneous voiding after surgery were low. Therefore creation of a continent Mitrofanoff-type vesicostomy was additionally realized and genital reconstruction was achieved. 12 months post operatively, the patient was completely continent, had a bladder capacity of 250 ml, and performed self-catheterization 5 times a day. No post-operative complications were observed. Admission of political refugees can implicate challenging surgeries for congenital malformations in adults, such as BEEC. This demonstrates the importance of multidisciplinary transitional care.

Bladder exstrophy-epispadias complex related litigation: A legal database review.

To review the nature and extent of bladder exstrophy-epispadias related malpractice litigation in the United States. Two legal databases (Nexis Uni, WestLaw) were reviewed for state and federal cases using the terms "bladder exstrophy", "cloacal exstrophy", "epispadias", in combination with "medical malpractice", or "negligence", or "medical error", or "complication", or "malpractice", or "tort". Databases were queried from 1948 to 2022 and reviewed for medical and legal details. Our search yielded 16 unique legal cases with 6 fitting established criteria for analysis. Urology and paediatric urologists were named in 50% of cases as were community medical systems. Cause for lawsuit included negligence in surgical performance (50%), primary closure of exstrophy (33%), and post-operative care (50%). Settlement agreement was reached in one case (17%). Outcomes favoured the physician in 60% of trials. Lawsuits alleging negligent surgical performance and/or post-operative care exclusively named urologists with outcomes favouring the surgeon in 66% of cases. The settlement payment (n = 1) was $500,000 and monetary damages (n = 1) equated to $1.3 million. Malpractice litigation related to BEEC treatment is rare. Trial outcomes favour the medical provider. Cases that resulted in financial liability successfully alleged avoidable negligence resulting in irreversible physical damage. The authors recommend families with BEEC seek board-certified paediatric urologists experienced in treating this complex and/or Bladder Exstrophy Centers of Excellence. Further, we recommend surgeons treating BEEC properly educate patients and families on the severity of this major birth defect including its lifelong implications and need for surgical revisions.

MP18-17 MAGNETIC RESONANCE IMAGING OF THE BLADDER, PROSTATE, PENILE VASCULATURE AND PELVIC FLOOR MUSCLES ANATOMY IN ADULT MEN AFTER CHILDHOOD BLADDER EXSTROPHY-EPISPADIAS COMPLEX REPAIR

You have accessJournal of UrologyCME1 Apr 2023MP18-17 MAGNETIC RESONANCE IMAGING OF THE BLADDER, PROSTATE, PENILE VASCULATURE AND PELVIC FLOOR MUSCLES ANATOMY IN ADULT MEN AFTER CHILDHOOD BLADDER EXSTROPHY-EPISPADIAS COMPLEX REPAIR Ahmed Elkashef, Doaa Elsayed Sharaf, Ahmed Abdelhalim, and Mohamed S. Dawaba Ahmed ElkashefAhmed Elkashef More articles by this author , Doaa Elsayed SharafDoaa Elsayed Sharaf More articles by this author , Ahmed AbdelhalimAhmed Abdelhalim More articles by this author , and Mohamed S. DawabaMohamed S. Dawaba More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003238.17AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: The musculoskeletal defects in lower abdomen and pelvis and genitourinary defects associated with bladder exstrophy-epispadias complex (BEEC) may result in variations in the anatomy of pelvic floor musculature and pelvic organs, which may affect the urinary continence and sexual function of BEEC patients following primary repair. Therefore, we evaluated the anatomy of bladder, prostate, penile vasculature and pelvic floor muscles of adult men who had experienced BEEC repair using a 3-Tesla pelvic magnetic resonance imaging (MRI). METHODS: In a retrospective study, we reviewed the database of the post pubertal male patients who underwent repair of BEEC at our tertiary center from 1990 till 2006. Those patients were invited to undergo a 3-Tesla pelvic MRI to delineate the pelvic anatomy after the multiple reconstructive surgeries that might compromise the neurovascular supply of the pelvic organs and affect the pelvic musculature. RESULTS: A total of 21 male patients with mean ages 21.7±5.2 years old, were included. Seven patients underwent repair as a primary procedure, while 14 patients had redo procedures. Coronal images of pelvic MRI showed bladder neck to erectile body distance, iliococcygeal angle, prostate diameter, corpus cavernosum diameter, inter-corporeal diameter, neurovascular bundle, penile vasculature and length, symmetry between both corporeal bodies and depth of bladder neck from anterior skin (Table 1). Axial images revealed iliac, ischial, obturator internus and puborectalis angles, posterior anal distance, bladder neck distance, anteroposterior diameter of the prostate, symphyseal and erectile diastasis distance, while sagittal images showed sacral anal and sacral bladder neck angles (Table 2). CONCLUSIONS: MRI can accurately outline the anatomy of the pelvic organs, neurovascular bundles and pelvic musculature in adult men who had undergone BEEC repair, so it may reflect the urinary continence and sexual function of those patients. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e230 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Ahmed Elkashef More articles by this author Doaa Elsayed Sharaf More articles by this author Ahmed Abdelhalim More articles by this author Mohamed S. Dawaba More articles by this author Expand All Advertisement PDF downloadLoading ...

Magnetic Resonance Imaging of the bladder, prostate, penile vasculature and pelvic floor muscles anatomy in adult men after childhood bladder exstrophy-epispadias complex repair

Magnetic Resonance Imaging of the bladder, prostate, penile vasculature and pelvic floor muscles anatomy in adult men after childhood bladder exstrophy-epispadias complex repair

Bladder exstrophy-epispadias complex

A 2-year-old male, with the bladder exstrophy-epispadias complex since birth presents to the department of surgical pediatrics for surgical management. Exposed, everted bladder template is clearly obvious immediately below umbilicus; left and right corpora cavernosa are visible beneath and alongside urethral plate; the scrotum is caudally displaced. A spectrum of congenital defects epispadias, typical bladder exstrophy, and cloacal exstrophy, make up the exstrophy-epispadias complex (EEC). Nelson et al. calculated the total incidence of EEC at 2.15 per 100,000 live births, with an equal male-to-female ratio. Normal invagination of the urogenital system begins at the end of the third week of pregnancy in the intermediate layer of mesoderm, while the lateral plate mesoderm contributes to the formation of the primitive gut tube. EEC develops because of disruption in this interaction, which may be caused by an overgrowth of the cloacal membrane that prevents mesenchymal tissue from migrating medially. The severity of the resulting condition depends on the point at which the interaction between the mesodermal layers is disturbed. Depending on the form and degree of the defect, different surgical procedures are required to rectify it; most neonates, however, require bladder and abdominal wall closure, epispadias repair, ureteral reimplantation, and bladder neck repair.

The Role of Bladder Neck Injection in the Quest for Continence

The Role of Bladder Neck Injection in the Quest for Continence

Preoperative and postoperative urinary NGAL levels in children with bladder exstrophy.

Urinary NGAL (neutrophil gelatinase-associated lipocalin) levels have been shown to predict renal damage in various medical conditions. The present study was conducted to study the role of urinary NGAL levels in children with bladder exstrophy-epispadias complex post single-stage total reconstruction (SSTR) as markers of early renal functionreduction. Urine samples were collected from children with bladder exstrophy before SSTR (Group A, n = 11), 5years post SSTR (Group B, n = 40) and controls (Group C, n = 41) and stored at - 20°C. NGAL levels were estimated using double antibody sandwich ELISA. Mean NGAL levels in Groups A, B and C were 1.39, 34.24 and 2.58ng/ml, respectively. Mean NGAL levels among Group B subjects with glomerular filtration rate (GFR) ≥ 80ml/min/1.73m2 body surface area (BSA) was 29.8ng/ml, while it was 31.74ng/ml in those with GFR < 80ml/min. Urine samples were also evaluated 6months post SSTR. Mean NGAL at 6months was 6.76ng/ml, while at 12months it was 30.3ng/ml, remaining > 30ng/ml at 18 and 24months. Dimercaptosuccinic acid (DMSA) scans did not show any scarring, and GFRon diethylenetriamine pentaacetate (DTPA) scans remained stable. Increasing levels of urinary NGAL following bladder-exstrophyandepispadias complex repair suggest that NGAL detects the earliest signs of renal damage even before any deterioration is observed in DMSA and/or DTPA-GFR scans. Further studies with an adequate sample size and periodic measurement of NGAL need to be performed before any definitive conclusion can be drawn.

Editorial Comment.

No AccessJournal of UrologyPediatric Article23 Dec 2022Editorial CommentThis article comments on the following:Dextranomer Endoscopic Injections for the Treatment of Urinary Incontinence in Bladder Exstrophy-epispadias Complex Luke Harper Luke HarperLuke Harper * E-mail Address: [email protected] Department of Pediatric Urology, Hôpital Pellegrin-Enfants, CHU Bordeaux, Bordeaux, France More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003086.01AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack Citations ShareFacebookLinked InTwitterEmail "Editorial Comment." The Journal of Urology, Publish Ahead of Print, pp. –