Management of Bladder Exstrophy: A Case Report in the Urological Ward at Saint Elizabeth General Catholic Hospital SHISONG (2025)

Four cases of bladder exstrophy in two families

Journal of Medical Genetics, 1994

Case Report: Long Term Follow Up of Bladder Exstrophy Repaired in Stages with Complete Urinary Continence

Journal of King Abdulaziz University - Medical Sciences, 2016

Bladder exstrophy is a rare congenital anomaly displaying symptoms such as the urinary bladder turned inside out, an exposed dorsal urethra, foreshortened penis, anteriorly displaced anus and widely separated symphysis pubis. This abnormality is managed by many methods, one of which includes a functional reconstruction. Themajor goals of surgical intervention are: Preservation of the renal function, achieving urinary continence with adequate sexual function and acceptable cosmetic appearance. In this report we present a long term follow up (25 years) of a male patient with classical type bladder exstrophy, managed at our hospital in stages, he is fully continent and voids spontaneously without clean intermittent self-catheterization, with normal renal function, adequate penile erection and has ejaculation.

Management of bladder exstrophy in an adolescent girl: a case report

Medical Journal of Indonesia

Bladder exstrophy (BE) is a very rare congenital abnormality, especially in girls. We reported a 17-year-old girl with protruded bladder and urinary incontinence. Her vaginal orifice appeared stenotic and shifted anteriorly. She had an incomplete labia minora, short perineum, and anterior displacement of the anus. The pelvic radiography showed a pubic diastasis with a manta ray sign, and the ultrasonography examination showed a bicornuate uterus. She had undergone a functional reconstruction surgery with cystectomy, ileal conduit, appendectomy, and vaginoplasty along with the advancement of abdominal flap reconstruction with multiple Z-plasty. Functionally, she was able to urinate through the ileal conduit comfortably, had a regular menstrual cycle with minimal pain, had a wider vaginal canal, and had a smooth blood flow. Aesthetically, she had a good lower abdomen appearance, remained dry, and had an odorless urine. In clinical practice, this rare case report can provide additional...

Perioperative management of classic bladder exstrophy

Research and Reports in Urology, 2013

The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall muscles, and pelvic structures. Modern surgical repairs focus on reconstruction of the bladder and its adjacent structures, with the goal of achieving urinary continence, a satisfactory cosmetic result, and a high quality of life. Complex surgery in neonates and young children, as well as a prolonged postoperative course require close collaboration between surgeons, anesthesiologists, intensivists, pediatricians, and an experienced nursing staff. This article will review the spectrum of bladder exstrophy anomalies, the surgical repair, and the perioperative interdisciplinary management.

Bladder Exstrophy: A rare case report

The bladder exstrophy (BE) represents an anterior midline defect with variable expression comprising a spectrum of anomalies involving the abdominal wall, pelvis, urinary tract, genitalia, and occasionally the spine and anus. The vast majority of BE cases are classified as non-syndromic and the etiology of this malformation is still unknown. We are presenting a case report on BE. A 11 year old patient presented with continuous dribbling of the urine from the lateral side of a soft tissue mass present in the lower abdominal wall with inability to hold and pass urine. The lesion was present since birth. On examination a soft tissue mass was present in lower abdominal wall with two opening on the either sides. Penis was short with unremarkable scrotum. Testis was not palpated in the scrotum. Combined investigation-IVP, CECT and USG revealed pubic diastasis with externally placed bladder with deficient anterior bladder wall, bilateral ectopic ureteral opening and chronic cystitis with undescendant testis.

COMPLETE REPAIR OF BLADDER EXSTROPHY: PRELIMINARY EXPERIENCE WITH NEONATES AND CHILDREN WITH FAILED INITIAL CLOSURE

The Journal of Urology, 2001

Purpose: The surgical repair of bladder exstrophy remains challenging for the pediatric urologist. We present our preliminary experience with complete primary repair of exstrophy in neonates and children with failed initial closure. Materials and Methods: Between November 1998 and April 1999, 9 boys and 2 girls with bladder exstrophy underwent complete repair. This procedure was performed in the first 72 hours of life in 4 boys and at age 3 months in 1 girl. Complete repair with osteotomy was performed after failed initial closure in 5 boys and 1 girl at a mean age of 28 months (range 15 to 36). The bladder and urethra were closed in continuity and epispadias was repaired by total penile disassembly. All patients were kept in a spica cast for 3 weeks. Ureteral stents and suprapubic tube were removed 10 and 14 days, respectively, after surgery. Ultrasound was performed preoperatively and every 3 months postoperatively, voiding cystourethrography was done 6 to 12 months after surgery.

Long Term Follow Up of Complete Bladder Exstrophy Repair. A Case Report

Folia Medica, 2014

ABSTRACTOBJECTIVE: Bladder exstrophy is a congenital anomaly which is not always successfully managed by surgery. Major goals of surgical intervention in such cases are preservation of normal renal function, development of adequate bladder function and urinary continence and avoidance of future urinary tract infections. We present 5-year data on a patient who underwent complete repair of the bladder exstrophy. CASE REPORT: We describe a full term female infant who presented at birth with complete bladder exstrophy. Complete repair of the condition was performed 3 days after birth (Ransley technique). During hospitalization the patient had a positive urinary culture with Candida lusitaniae, enterococcus and septicemia with Klebsiella pneumoniae ESBL. The patient had no complications until the age of 20 months when she developed an episode of pyelonephritis and five further episodes of cystitis with E. coli. Radiographic testing showed small bladder capacity (23 ml at the age of 3 yea...

Bladder exstrophy: An epidemiologic study from the International Clearinghouse for Birth Defects Surveillance and Research, and an overview of the literature

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 2011

Bladder exstrophy (BE) is a complex congenital anomaly characterized by a defect in the closure of the lower abdominal wall and bladder. We aimed to provide an overview of the literature and conduct an epidemiologic study to describe the prevalence, and maternal and case characteristics of BE. We used data from 22 participating member programs of the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR). All cases were reviewed and classified as isolated, syndrome, and multiple congenital anomalies. We estimated the total prevalence of BE and calculated the frequency and odds ratios for various maternal and case characteristics. A total of 546 cases with BE were identified among 26,355,094 births. The total prevalence of BE was 2.07 per 100,000 births (95% CI: 1.90-2.25) and varied between 0.52 and 4.63 among surveillance programs participating in the study. BE was nearly twice as common among male as among female cases. The proportion of isolated cases was 71%. Prevalence appeared to increase with increasing categories of maternal age, particularly

Early Outcome Following Complete Primary Repair of Bladder Exstrophy in the Newborn

The Journal of Urology, 2005

Purpose: Complete primary repair of bladder exstrophy (CPRE) represents a paradigm shift from the staged approach for surgical management. We present early clinical outcomes in our patients following CPRE.

Challenges in salvaging urinary continence following failed bladder exstrophy repair in a developing country

Journal of pediatric urology, 2017

The management of urinary incontinence following failed bladder exstrophy repairs is not well defined, some surgeons recommend urinary diversion, others would pursue reconstructive surgery. Herein we review our experience with various strategies for management of urinary incontinence and their outcome in 61 patients born with bladder exstrophy who failed their initial repairs. We also examine the impact of diverse factors in making the surgical decision for patients with limited resources in a developing country. Between 1981 and 2014, 61 incontinent patients (age 3-18 years) born with bladder exstrophy were referred for secondary or tertiary repair. In nine children the bladder was exposed following wound dehiscence and 52 had had one or more attempted repairs resulting in contracted bladders. The primary treatment included: re-closure and bilateral iliac osteotomy in five patients (group 1); urinary diversion in Mainz II pouch in 15 patients (group 2); and 41 patients (group 3) un...

Contemporary issues relating to transitional care in bladder exstrophy

Canadian Urological Association Journal

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

European Urology, 2005

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 AE 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 AE 7 and 7 AE 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.

Bladder Exstrophy from Childhood into Adult Life

Journal of the Royal Society of Medicine

Exstrophy of the bladder is rare and the incidence of bladder exstrophy is calculated to be from 1 per 30 000 to 50 000 live births with male to female ratio ranging from 1.5-5 to 1 1-4 It was found that persistence or overgrowth of the cloacal membrane on the lower anterior abdominal area, prevents normal mesenchymal ingrowth. This causes divergence of the lower abdominal muscular structures and forces the genital ridges to fuse caudal to the cloacal membrane. The stage of ingrowth of the urorectal septum at the time of rupture determines whether one will produce an exstrophic urinary tract alone (classic bladder exstrophy or epispadias) or cloacal exstrophy with the hindgut interposed between the hemibladders5,6.

Exstrophy of the bladder

The Journal of Pediatrics, 1945

Bladder Exstrophy

Bladder exstrophy, more properly, the exstrophy-epispadias complex is a rare congenital anomaly occurring once every 10,000-50,000 live births with a 2.3:1 as male & female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior bony pelvis and external genitalia. It occurs due to failure of the abdominal wall to close during fetal development and results in protrusion of the posterior bladder wall through the lower abdominal wall. We report a case of bladder exstrophy managed by us.

COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY: INITIAL EXPERIENCE WITH 33 CASES

The Journal of Urology, 2004

Purpose: We evaluated our initial experience with complete primary repair of bladder exstrophy in 33 children. Materials and Methods: Between 1998 and, 33 children with classic bladder exstrophy were treated with 1-stage primary repair for the first time in all except 4, who had undergone previous failed initial bladder closure. Our series included 26 boys and 7 girls with a mean age of 2 months (range 3 weeks to 14 months). The bladder was closed in continuity with the urethra and complete penile disassembly was used for epispadias repair. Anterior transverse innominate osteotomy was performed in all cases. Combined general and caudal anaesthesia were applied in all cases with an indwelling epidural caudal catheter in 7.

Congenital Renal Anomalies in Patients With Classic Bladder Exstrophy

Urology, 2012

OBJECTIVE To determine the proportion and type of renal anomalies that occur in children born with classic bladder exstrophy. MATERIAL AND METHODS All patients in a database of 1044 patients with the bladder exstrophy-epispadias-cloacal exstrophy complex were reviewed. Those with classic bladder exstrophy had their complete medical records reviewed and children with radiographic evidence of congenital renal anomalies were identified and characterized. RESULTS A total of 674 patients were identified with classic bladder exstrophy, of whom 462 had renal ultrasounds at the authors' institution. In this population, 13/462 (2.8%) had concomitant renal anomalies. The most common malformation was a duplicated collecting system in 6 patients. Hypoplastic or absent kidneys were present in 3 patients, pelvic kidney in 2, ureteropelvic junction obstruction in 1, and multicystic dysplastic kidney in 1 patient. CONCLUSION Isolated renal anomalies occur at a rate of 2.8% in children born with classic bladder exstrophy. Although uncommon in this rare birth defect, anatomic understanding of the entire genitourinary system is critical for operative planning and long-term follow-up of patients with bladder exstrophy.

Results of Surgical Treatment in Children with Bladder Exstrophy

British Journal of Urology, 1992

Methods We performed a retrospective analysis of 11 patients diagnosed with CM-1 who were treated in our center in the years 2007 to 2016. There were 6 female and 5 male individuals. Short-term evaluation of the outcome was based on comparison of the presenting symptoms and radiological images before and after the surgical treatment. Long-term follow-up was carried out using survey questionnaires based on the Chicago Chiari Outcome Scale (CCOS) devised originally by Aliaga et al. Results Patients, based on their CCOS score were divided into three groups marked as Bimproved,^Bunchanged,^and Bworse,d epending on a range of CCOS score: 13-16, 9-12, 4-8, respectively. The outcome of patients was as follows: 6 patients (55%) were evaluated as improved, and 5 (45%) as unchanged. No patient was classified as worse after surgery. Significant negative Spearman's correlation was found between the CCOS score and patients' age at the time of surgery (R = − 0.85, p = 0.0009). Conclusions The decision of whether to operate pediatric patients with CM-1 should be considered very carefully. In our department, the main indication for surgery was the occurrence of clinical symptoms. Our study revealed that in the symptomatic patients, surgery improves their quality of life measured with CCOS.

Early and Late Complications of Bladder Exstrophy Surgery at Al-Ribat University Paediaric Surgery Center 2006 - 2012

Sudan Medical Journal, 2013

Modern management of bladder exstrophy repair

Current urology reports, 2013

The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall musculature, and pelvic bones. Historically, surgical outcomes in patients with classic bladder exstrophy, the most common presentation of the exstrophy-epispadias complex, were poor. However, modern techniques have increased the success of achieving urinary continence, satisfactory cosmesis, and improved quality of life. Still, recent studies recognize complications that may occur during management of these patients. This review provides readers with an overview of the exstrophy-epispadias complex, the modern management of bladder exstrophy, and potential surgical complications.