Oeis complex (omphalocele-exstrophy-imperforate anus-spinal defects): a confusing syndrome. case report

ABSTRACT Introduction: The OEIS complex is a group of polymorphic defects with low incidence and prevalence worldwide. It is associated with epigenetic and genetic causes that occur in early blastogenesis, resulting in 4 classic malformations consisting of omphalocele, bladder/cloaca exstrophy, imperforate anus, and spinal cord injuries. Spina bifida, symphysis pubis diastasis and limb abnormalities may also be observed. Case presentation: 7-month-old female patient (at the time of writing this report). The mother was from a rural region of Colombia, and this was her third pregnancy, which was at high risk of obstetric complications. The infant was prenatally diagnosed with a caudal folding defect in the abdominal wall and a lipomeningocele. During birth, bladder exstrophy, imperforate anus and spinal dysraphism were observed, leading to a diagnosis of OEIS complex. Relevant interdisciplinary management was initiated. Conclusions: The OEIS complex is a fetal polymorphic malformation with characteristic signs and defects. Knowledge on its etiopathogenesis, pre- and postnatal diagnosis, genetic counseling and therapeutic approaches are essential to favor the early treatment of different comorbidities, alleviate acute symptoms, reduce multiple comorbidities and improve the patient's quality of life.

RESUMEN Introducción. El complejo OEIS es un conjunto de defectos polimalformativos con baja incidencia y prevalencia mundial que suele estar asociado a causas epigenéticas y genéticas que ocasionan alteración al final de la blastogénesis, dando como resultado la asociación de cuatro malformaciones clásicas: onfalocele, extrofia vesical, ano imperforado y lesiones de la médula espinal. En ocasiones también se presenta espina bffida, diástasis de la sínfisis púbica y anormalidades en las extremidades. Presentación del caso. Paciente femenina de 7 meses de edad (al momento de la elaboración del presente reporte), procedente de un área rural colombiana, producto de una tercera gestación con alto riesgo obstétrico y diagnosticada prenatalmente con un defecto en el plegamiento caudal de la pared abdominal y un lipomeningocele. Durante el nacimiento se evidenció extrofia vesical, ano imperforado y disrafismo espinal, lo que permitió plantear el diagnóstico de complejo OEIS e iniciar manejo interdisciplinario pertinente. Conclusiones. El complejo OEIS es una poli-malformación fetal con signos y anomalías características, en donde los conocimientos sobre la etiopatogenia, el diagnóstico pre y postnatal, el asesoramiento genético y las propuestas terapéuticas son primordiales para favorecer el manejo precoz de las diferentes comorbilidades, aliviar la sintomatologia aguda, reducir múltiples comorbilidades y mejorar la calidad de vida del paciente.

Preoperative care of Polypoid exposed mucosal template in bladder exstrophy: the role of high-barrier plastic wraps in reducing inflammation and polyp size

ABSTRACT Objective To assess the role of high-barrier plastic wrap in reducing the number and size of polyps, as well as decreasing the inflammation and allergic reactions in exstro- phy cases, and to compare the results with the application of low-barrier wrap. Materials and Methods Eight patients with bladder exstrophy-epispadias complex (BEEC) that had used a low density polyethylene (LDPE) wrap for coverage of the exposed polypoid bladder in preoperative care management were referred. The main complaint of their parents was increase in size and number of polyps. After a period of 2 months using the same wrap and observing the increasing pattern in size of polyps, these patients were recommended to use a high-barrier wrap which is made of polyvinylidene chloride (PVdC), until closure. Patients were monitored for the number and size of polyps before and after the change of barriers. The incidence of para-exstrophy skin infection/inflammation and skin allergy were assessed. Biopsies were taken from the polyps to identify histopathological characteristics of the exposed polyps. Results The high barrier wrap was applied for a mean ± SD duration of 12±2.1 months. Polyps' size and number decreased after 12 months. No allergic reaction was detected in patients after the usage of PVdC; three patients suffered from low-grade skin allergy when LDPE was applied. Also, pre-malignant changes were observed in none of the patients in histopathological examination after the application of PVdC. Conclusion Polyps' size and number and skin allergy may significantly decrease with the use of a high-barrier wrap. Certain PVdC wraps with more integrity and less evaporative permeability may be more "exstrophy-friendly".

Complejo extrofia vesical-epispadias. Reporte de un caso / Bladder exstrophy-epispadias complex. A case report

RESUMEN La extrofia vesical es una grave anomalía del desarrollo embriológico del seno urogenital y del sistema esquelético vecino. Se caracteriza por una falla de la pared anterior del abdomen y de la vejiga, cuya cara posterior-inferior, evertida hacia afuera, protruye en forma de hernia y exterioriza por completo el trígono, con los meatos ureterales eyaculando directamente hacia la pared abdominal; la asociación con epispadias es la regla. Se describió el caso de una paciente pediátrica con diagnóstico del complejo extrofia vesical-epispadias, entidad poco frecuente en nuestro medio, la cual evolucionó de manera favorable y se encuentra en espera de un segundo tiempo quirúrgico como parte de su tratamiento (AU).

ABSTRACT The bladder exstrophy is a serious anomaly of theurogenital sinus´ embryologic development and the close skeletal system, characterized by a failure of the abdomen anterior wall and bladder, whose posterior inferior face, turned outside out, protrudes like an hernia and completely exteriorizes the trigone, with the ureteral meatuses ejaculating right down to the abdominal wall; the association with epispadias is the rule. It was described the case of a pediatric patient with diagnosis of exstrophy- epispadias complex, infrequent entity in our country, who has evolved with great results and is waiting a second surgical time to finish her treatment (AU).

Combined application of prenatal ultrasound and magnetic resonance imaging in diagnosis of fetal bladder exstrophy / 中华医学超声杂志（电子版）

Objective To summarized the ultrasound and MRI characteristics of fetuses with bladder exstrophy. Methods Four cases of bladder exstrophy (3 cases of single birth were conceived naturally, 1 twins were artificial pregnated) were diagnosed by ultrasound combined MRI which were confirmed by autopsy in Hubei Maternal and Child Heath Hospital, from February 2013 to December 2017, We summarized the ultrasonographic features of the four cases. Results The common abnormal ultrasound images of the 4 cases: the bladder in the pelvic cavity was not shown, the umbilical cordinsertion was low, the symphysis pubis was separated, the gender was difficult to be determined, and the amniotic water was normal. In one case, one of the twins combined with the absence of the right kidney and abdominal wall mass below the umbilical cord insertion, and 1 case was suspected to tethered cord syndrome. Four cases of fetal antenatal MRI showed the same abnormal imaging findings as ultrasonography, but none of the 4 cases of pubic symphysis separation were showed, excluding the ultrasonographic diagnosis of tethered cord syndrome. In comparison with the X-ray and pathological examination results after the labor induction, the prenatal MRI was supplemented with 4 cases of abdominal wall defects, and 3 cases more than ultrasound. Ultrasonography and MRI combined examination were performed to correctly diagnose 4 cases of fetal bladder exstrophy. No bladder showed in pelvic nor the abdominal cavity, the abdominal wall defect with bulging content, the umbilical cord insertion was low, pubic symphysis separation, genital malformation, normal kidney andamniotic fluid were the main clues for ultrasound and MRI diagnosis of bladder exstrophy. Conclusion The combination of US and MRI can further improve the diagnostic accuracy and avoid missed diagnosis of fetal BE.

Aplicación de las plaquetas en la extrofia vesical / Use of platelet in bladder exstrophy

La extrofia vesical es una anomalía congénita grave del tracto urinario inferior que afecta la vejiga, los huesos pelvianos, la pared abdominal, los genitales externos, el perineo y, en algunos casos, el intestino. Los tratamientos convencionales están basados en técnicas quirúrgicas, para lograr reconstruir la vejiga, los genitales y cerrar el defecto de la pared. Se presenta el caso de una niña de 3 años de edad, en la que el tratamiento quirúrgico se había aplicado en 6 ocasiones sin resultados. Se decidió combinar el tratamiento quirúrgico con la aplicación de medicina regenerativa. Se le aplicó lisado de plaquetas en la pared de la vejiga y los bordes de la pared abdominal a razón de 1 mL semanal, durante 4 semanas. Se logró la regeneración de los tejidos que permitió afrontar los bordes de dichas estructuras y realizar la técnica quirúrgica convencional. Como resultado se logró cierre total de vejiga, uretra y pared abdominal, sin fístulas entre estas estructuras ni al exterior. La utilización del lisado de plaquetas favorece el crecimiento y regeneración de los tejidos que componen el tracto urinario. La cirugía puede ser una solución definitiva, después de haber aplicado los factores de crecimiento plaquetarios, que preparan el tejido en cuanto a calidad y cantidad, favoreciendo el afrontamiento de los bordes, la cicatrización y disminuyendo las complicaciones posquirúrgicas(AU)

Bladder exstrophy is a severe congenital anomaly of lower urinary tract that affects the bladder, pelvic bones, abdominal wall, external genitalia, perineum and in some cases intestine. Conventional treatments are based on surgical techniques, in order to reconstruct bladder, genitals and close wall defect. We present a case of a 3 year old girl, in which surgical treatment was applied 6 times without results. It was decided to combine the surgical treatment with application of regenerative medicine. It was applied platelet lysate in the bladder wall and the edges of the abdominal wall at the rate of 1 mL weekly for 4 weeks, achieving tissue regeneration. It enabled to face the edges of those structures and perform conventional surgical technique. As a result, we achieved a total closure of bladder, urethra and abdominal wall, without fistulas either between these structures or outside. Therefore the use of platelet lysate promotes growth and tissue regeneration comprising the urinary tract, decreasing number of interventions, time exposition structures of the abdominal cavity, and post surgical complications such as fistulas(AU)

One - staged reconstruction of bladder exstrophy in male patients: long - term follow-up outcomes

ABSTRACT Introduction The surgical correction of bladder exstrophy remains challenging. In our institution, the repair has evolved from a staged repair to one-stage reconstruction. The one-stage reconstruction includes; bladder closure, Cantwell-Ransley neourethroplasty and abdominoplasty using groin flaps, without the need of pelvic ostheotomies. Repair of urinary continence (UC) and vesicoureteral reflux (VUR) is done after development of the infant. Objective To present our experience of our modified one-stage reconstruction of bladder exstrophy in male patients. Materials and Methods Medical records of male patients submitted to one-stage reconstruction of bladder exstrophy were analyzed retrospectively. Fifteen exstrophy bladder patients with mean age 4.2±7 years were treated at our institution between 1999-2013. Results 2 5 Conclusions One-stage reconstruction minimizes the number of surgical procedures required to achieve UC and potentiates bladder-neck function. The advantages of using groin flaps over current techniques for complete repair are the small risk for penile tissue loss and the avoidance of ostheotomies.

A Rare Case of an Adult with Untreated Bladder Exstrophy Presenting with Adenocarcinoma of Urinary Bladder.

Untreated bladder exstrophy in an adult is rare, as the defect is obvious and primary reconstruction is usually done in infancy. There are less than 90 reported cases of primary adenocarcinoma in an untreated bladder exstrophy in literature and only two such case reports from India. We report the case of adenocarcinoma in a 29 year old male with untreated bladder exstrophy to highlight the extreme rarity, yet distinct possibility and challenges faced in surgical management of such cases.

Bladder exstrophy / Extrofia vesical

SUMMARY Bladder exstrophy is a rare congenital anomaly resulting from failure of fusion of the middle of the pelvis line tissues during embryogenesis. It is characterized by malformation of the lower abdominal wall involving the genitourinary tract and the musculoskeletal system. Its incidence is estimated at 1:30,000 to 1:50,000 live births, and it is 2 or 3 times more frequent in males. The child’s age is important and the best results are obtained when treatment is performed shortly after birth.

RESUMO A extrofia de bexiga é uma anomalia congênita rara decorrente de falha da fusão dos tecidos da linha média da pelve durante a embriogênese e caracteriza-se por má-formação da região inferior da parede abdominal, envolvendo o trato geniturinário e o sistema musculoesquelético. Apresenta incidência estimada de 1:30.000 a 1:50.000 nascidos vivos, sendo 2 a 3 vezes mais frequente no sexo masculino. A idade da criança é importante e os melhores resultados são obtidos quando o tratamento é realizado logo após o nascimento.

Anestesia y Analgesia para corrección de extroña vesical. Reporte de tres casos / Anaesthesia and analgesia for bladder exstrophy correction. Case reports

Introduction: Neonatal bladder exstrophy repairs imply correcting the genitourinary malformation, and closing and stabilizing the pelvic girdle with external fixation and traction. Successful results are achieved in terms of reduced urinary incontinence, adequate aesthetic appearance, improved quality of life, reduction of the risk associated with neonatal surgery and minimization of the number of procedures associated with multistage repairs. In such procedures, prolonged perioperative anaesthesia is key for the patient to tolerate the traction and external fixation, to help osteotomy healing, and to reduce tension in the surgical wound. Patients' age and weight have an effect on the risk of toxicity from local anaesthetics and respiratory depression from opioid analgesics. The prolonged use of caudal catheters in the management of these cases is associated with infection at the insertion site. Case presentation: The article presents the cases of three infants between 7 months and 1 year of age taken to bladder exstrophy repair and pelvic osteotomy with tunnelled caudal catheter and continuous local anaesthetic infusion as perioperative anaesthetic management technique. The use of these techniques was aimed at reducing the risk of infection at the insertion site and the risks associated with prolonged pain management in infants. Conclusion: The cases suggest that tunnelled caudal catheter placement and continuous local anaesthetic infusion are safe techniques in the management of prolonged anaesthesia in infants, decreasing the risk of insertion site infection.

Introducción: La corrección de la extrofia vesical en el lactante menor implica la reparación de la malformación genitourinaria y el cierre y estabilización del anillo pélvico utilizando un tutor externo y tracción. Se obtienen resultados exitosos en la continencia urinaria del paciente, adecuado aspecto estético y buena calidad de vida reduciendo el riesgo que implica la cirugía neonatal y minimizando el número de procedimientos. La analgesia postoperatoria prolongada es fundamental para tolerar el tutor y/o la tracción, permitir la cicatrización de las osteotomías y reducir la tensión sobre la herida quirúrgica. La edad y el peso de estos pacientes aumentan el riesgo de toxicidad por anestésico local y de depresión respiratoria con el uso de opioides, al igual que el uso prolongado de catéteres caudales se asocia a infección del sitio de inserción. Presentación de caso: Se describen tres casos de lactantes de 7 meses a 1 ano de edad llevados a corrección de extrofia vesical y osteotomía pélvica mas fijación con tutor externo donde el manejo analgésico postoperatorio se realizó mediante la infusión de anestésico local por catéter caudal tunelizado para prolongar el tiempo de analgesia y reducir el riesgo de infección. Conclusión: Se señala el uso de la tunelización de los catéteres caudales y la infusión continua de anestésico local como técnica segura para el manejo analgésico prologado en el paciente lactante con reducción del riesgo de infección del sitio de inserción.

Modified Cantwell-Ransley procedure for epispadias repair in 37 patients / 中华泌尿外科杂志

Objective To present the outcomes of modified Cantwell-Ransley procedure in 37 patients with isolated epispadias and exstrophy complex.Methods A total of 37 patients underwent modified Cantwell-Ransley epispadias repair.The median age at surgery was 6.6 years (range 1-32).Of the 37 patients,13 patients had isolated penopubic epispadias and 24 had bladder exstrophy with no previous attempts at epispadias repair.Nine patients with exstmphy complex underwent complete primary repair,while other 15 cases received urinary diversion.Results The patients were followed up for 6 months to 3 years (mean,12 months).All patients had normal conical symmetric glans/corpora and an orthotopic meatus.The complications included glans/corporal ischemia (1 case),urethral fistula (3 cases),and foreskin infection (1 case).Conclusions Modified Cantwell-Ransley procedure is safe and effective for epispadias repair.Cosmetic appearance and functional outcomes of the penis are encouraging.

Spontaneous Pregnancy after Cystectomy and Urinary Diversion by Indiana Pouch Method for Congenital Bladder Exstrophy: A case Report.

Aims: To share our experience in the management of a patient of congenital bladder exstrophy, who conceived spontaneously following Indiana pouch surgery for urinary diversion. surgery for congenital bladder exstrophy, reported for antenatal care. In the first year of her life, an ineffective operation was performed for closure of her abdominal wall defect. At 10 years of age she underwent cystectomy, trygonocuteneostomy, diastasis of pubic bones were fixed and urinary diversion operation was performed by Indiana pouch method. She was followed up at the antenatal clinic of Dicle University until 38th week of pregnancy. Thereafter, an elective caesarean section was performed and a live healthy female baby was delivered. Discussion and Conclusion: Bladder exstrophy is an extremely rare congenital abnormality. Woman with bladder exstrophy and lower urinary tract reconstruction surgery may conceive spontaneously. However they have a high risk pregnancy and an elective caesarian section is advocated for delivery. Review of literature and our experience is presented.

Squamous Cell Carcinoma in Exstrophy of the Bladder

Exstrophy of the bladder is a rare congenital anomaly with an incidence of about 1 per 50,000 newborns. The malignant potential of the exstrophied bladder mucosa is well known; 95% are adenocarcinomas, and 3% to 5% are squamous cell carcinomas. Most of the malignant tumors (60%) associated with an exstrophy of the bladder occur during the fourth and fifth decades of life. Of the remaining, about 20% each occur after 60 years and before 40 years. Here we present a case in which squamous cell carcinoma developed in an unrepaired exstrophy of the bladder. We present the management of the case and a brief review of the literature.

Reconstrução tardia da região abdominal inferior e vulvar como tratamento complementar para sequelas pós-correção de extrofia vesical / Delayed reconstruction of the vulvar and lower abdominal regions as a complementary treatment for postsurgical sequelae of bladder exstrophy correction

Patients with malformations of the exstrophy-epispadias complex, including bladder exstrophy, may present for correction of deformities and sequelae in abdominal area, after primary treatment of urogenital malformations, performed early in life and in multiple stages. The secondary correction with aesthetic and functional goals is usually performed after growth and definition of hairy and fat distribution as well as after completion of urological treatment. Psychological aspects should also be considered. We report three female patients, with a history of bladder exstrophy correction in the neonatal period, presenting multiple deformities in the abdominal and vulvar areas, treated at our institution...

Os portadores de malformações do complexo extrofia-epispádia, incluindo a extrofia vesical, podem se apresentar para correções de deformidadese sequelas abdominais após o tratamento primário das malformações urogenitais, realizado nos primeiros anos de vida e em múltiplos estágios. A correção secundária, com objetivos estéticos e funcionais, é normalmente realizada após o crescimento e definição da distribuição pilosae adiposa, bem como após a finalização do tratamento urológico. Os aspectos psicológicos também devem ser considerados. Relatamos uma série de três casos de pacientes do sexo feminino, com antecedente decorreção de extrofia vesical no período neonatal, apresentando múltiplas deformidades na região abdominal e vulvar, submetidas à reconstrução em nosso serviço...

Bladder exstrophy: reconstructed female patients achieving normal pregnancy and delivering normal babies

PURPOSE: Bladder exstrophy (BE) is an anterior midline defect that causes a series of genitourinary and muscular malformations, which demands surgical intervention for correction. Women with BE are fertile and able to have children without this disease. The purpose of this study is to assess the sexual function and quality of life of women treated for BE. MATERIALS AND METHODS: All patients in our institution treated for BE from 1987 to 2007 were recruited to answer a questionnaire about their quality of life and pregnancies. RESULTS: Fourteen women were submitted to surgical treatment for BE and had 22 pregnancies during the studied period. From those, 17 pregnancies (77.2 percent) resulted in healthy babies, while four patients (18.1 percent) had a spontaneous abortion due to genital prolapse, and there was one case (4.7 percent) of death due to a pneumopathy one week after delivery. There was also one case (5.8 percent) of premature birth without greater repercussions. During pregnancy, three patients (21.4 percent) had urinary tract infections and one patient (7.14 percent) presented urinary retention. After delivery, three patients (21.4 percent) presented temporary urinary incontinence; one patient (7.14 percent) had a vesicocutaneous fistula and seven patients (50 percent) had genital prolapsed. All patients confirmed to have achieved urinary continence, a regular sexual life and normal pregnancies. All patients got married and pregnant older than the general population. CONCLUSIONS: BE is a severe condition that demands medical and family assistance. Nevertheless, it is possible for the bearers of this condition to have a satisfactory and productive lifestyle.

Osteotomia pélvica anterior bilateral para o fechamento de extrofia de bexiga: descrição de técnica / Bilateral anterior pelvic osteotomy for closure of bladder exstrophy: description of technique

A extrofia de bexiga e cloaca são más formações raras associadas a alterações da pelve. Os objetivos na reconstrução são: obter uma bexiga fechada, continente e com aparência cosmética aceitável. O tratamento das alterações da anatomia da pelve é parte importante para o sucesso do tratamento urológico dessas condições. O artigo visa relatar a técnica da osteotomia pélvica anterior bilateral para tratamento de extrofia de bexiga e cloaca, além da exposição de dois casos para demonstração das dificuldades e suas aplicações.

Bladder and cloacal exstrophy are rare malformations associated with abnormalities in the pelvis. The objectives in reconstruction are to obtain a closed and continent bladder, with an acceptable cosmetic appearance. Treatment for the abnormalities of pelvic anatomy is an important part of achieving successful treatment for these urological conditions. This article aims to describe the technique of bilateral anterior pelvic osteotomy for treating bladder and cloacal exstrophy, and presents two cases to demonstrate the difficulties and applications of the technique.

Tratamento da extrofia de bexiga: osteotomia posterior dos ossos ilíacos e fechamento da pelve com cinta de náilon / Treatment of bladder exstrophy: posterior osteotomy of iliac bones and closing of the pelvis with a nylon braces

Objetivo: Avaliou-se o resultado obtido em nove correções cirúrgicas de extrofia de bexiga em pacientes pediátricos. Métodos: Foi utilizada osteotomia posterior dos ossos ilíacos seguida pelo fechamento da pelve com cinta de náilon. Os procedimentos foram realizados em dois tempos cirúrgicos distintos com 48h de intervalo. Resultado: A média de idade foi de 17,6 meses. Cinco pacientes eram do sexo masculino. O seguimento médio foi de 32 meses. Conclusão: Os resultados finais foram animadores, mesmo nos pacientes que apresentaram complicações pós-operatórias como infecções e deiscência.

Objective: The authors evaluated the results obtained in nine surgical correction of bladder exstrophy in pediatric patients. Methods: We used posterior osteotomy of pelvic iliac bones followed by closing with a nylon brace. The procedures were performed in two different surgical times with intervals of 48 hours. Results: The mean age was 17.6 months. Five patients were male. The mean follow-up was 32 months. Conclusion: The final results were encouraging even in patients who had postoperative complications such as infection and dehiscence.

Primarily Closed Bladder Exstrophy in a Female Patient Complicated by a Bladder Calculus and Squamous Cell Carcinoma-A Rare Presentation.

Bladder exstrophy is a rare congenital anomaly with an estimated incidence of 1 in 10,000 and 1 in 50,000 live births. It is even rarer in females with a male-to-female ratio being 5:1 to 6:1.We report a 19yrs old female who had primary closure of exostrophy at the age of 6months and now presented with vesical calculus and squamous cell carcinoma of bladder..

Rehabilitation of urinary incontinence following delayed bladder exstrophy repair in a female child

Bladder exstrophy is relatively rare and even more rarely referred for rehabilitation. A nine-year-old girl with congenital bladder exstrophy from Samar presented with an abnormally low umbilicus, an abdominal opening below the umbilicus, and an anteriorly located anus. The patient has had no prior medical consult. She underwent primary repair of the exstrophy, anterior innominate bone osteotomy, application of external fixators, and traction. Postoperatively, the patient was referred for multidisciplinary rehabilitation management. The rehabilitation bladder program consisted of bladder training with clean intermittent catherization, use of voiding diaries, fluid restriction, timed voiding, electrical stimulation, and pelvic floor muscle exercises. Bed sore care, pulmophysiotherapy, and strengthening exercises for lower extremities were also implemented. At present there are no validated guideline in the management of urinary incontinence among female children with pelvic surgery, specifically with bladder exstrophy. This case report aims to present the rehabilitation management of female pediatric patient with delayed bladder exstrophy repair, resulting in improved outcome.

True duplicate bladder exstrophy: A rare lesion.

A two-day-old newborn male child with a rare variant of exstrophy bladder was managed in our institute. The child has true duplicate bladder exstrophy which is extremely rare and only 8 cases reported in the world literature so far. We describe another one and briefly review the literature.

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.