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

PURPOSE: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Diagnóstico prenatal del complejo onfalocele, extrofia cloacal, ano imperforado y defecto de la columna vertebral (complejo OEIS) / Prenatal diagnosis of omphalocele, cloacal exstrophy, imperforate anus and spinal anomalies complex (OEIS complex)

RESUMEN El complejo onfalocele, extrofia de la cloaca, ano imperforado y anomalías de la columna vertebral (Complejo OEIS) es una combinación de malformaciones congénitas severas y extremadamente raras. Su incidencia es estimada en 1 por cada 200.000 - 400.000 nacidos vivos. La aparición de los casos es esporádica y no tiene una etiología conocida. Algunos han sido asociados a exposiciones ambientales, causas genéticas y procedimientos de fertilización in vitro. El mecanismo de desarrollo parece asociado a alteraciones de la blastogénesis temprana o defecto de la migración mesodérmica durante el período embrionario. El diagnóstico prenatal puede realizarse a las 16 semanas de gestación, aunque en ocasiones es posible un diagnóstico más temprano. Su diagnóstico definitivo se realiza con los hallazgos de la necropsia. La mayoría de los recién nacidos supervivientes necesitan múltiples cirugías con complicaciones potenciales y no siempre se alcanza los resultados deseados. Se presenta un caso de diagnóstico prenatal de onfalocele, extrofia de la cloaca, ano imperforado y anomalías de la columna vertebral (complejo OEIS).

ABSTRACT Omphalocele, cloacal exstrophy, imperforate anus and spinal anomalies complex (OEIS complex) is a combination of severe and extremely rare congenital malformations. Its incidence is estimated at 1 per 200,000 - 400,000 live births. The occurrence of cases is sporadic and has no known etiology. Some have been associated with environmental exposures, genetic causes, and in vitro fertilization procedures. The mechanism of development appears to be associated with alterations in early blastogenesis or defect in mesodermal migration during the embryonic period. Prenatal diagnosis can be made at 16 weeks of gestation, although earlier diagnosis is sometimes possible. Definitive diagnosis is made necropsy findings. Most surviving newborns require multiple surgeries with potential complications and the desired results are not always achieved. A case of prenatal diagnosis of omphalocele, cloacal exstrophy, imperforate anus and spinal anomalies (OEIS complex) is presented.

Bladder and urethral duplication and a bladder exstrophy plate with omphalocele in a female child.

The Exstrophy - Epispadias Complex (EEC) is a spectrum of rare congenital malformations involving the urinary, genital and musculoskeletal systems. We present an atypical or variant case of EEC in which a bladder plate is found involving a small omphalocele, separated pubic bones and bladder and urethral duplication. The treatment had a favorable outcome, with bladder control and the child voiding normally. Perhaps the best accepted embryonic theory to explain exstrophy and its variants should be reviewed as it fails to satisfactorily explain the alterations we found.

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.

The search for continence in bladder exstrophy: Bladder neck transection and Macedo catheterizable reservoir to augment the nativebladder.

INTRODUCTION: Bladder exstrophy remains one of the most challenging abnormalities in pediatric urology. We propose bladder neck transection and bladder augmentation with a catheterizeable reservoir technique to achieve continence after previous anatomic reconstruction in stages. METHODS: At the age of 5-6 years, we offer the transection of bladder neck and enterocystoplasty to achieve continence. We report on a 6-year-old boy that underwent this procedure. We perform the reservoir from ileum according to Macedo-technique that constructs a catheterizeable channel from the same bowel segment. The continence mechanism of the efferent tube is based on angulation and a serous lined tunnel created with 3.0 prolene sutures. The stoma is placed in the midline. RESULTS: Patient had an uneventful evolution and is continent performing CIC every 4 h with 9 months of follow up. DISCUSSION: In spite of continuous development of bladder exstrophy surgery, the urethral continence and voluntary micturition is still not possible in the majority of patients. We discuss with our patients honestly and offer this method as a viable alternative to achieve continence. CONCLUSION: In our experience, most patients accept urethral transection and suprapubic CIC when educated about results with other alternatives of bladder neck plasty.

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).

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".

Female epispadias: Single-stage approach - A technique to achieve continence.

This video provides a case report of a 3 year old girl with epispadia and the highlights of the surgery. A cystoscopic guided bladder neck plication was performed to achieve continence. Key points include: (1) Skin incision planning; (2) Cutaneous flap liberation to create a new urethra; (3) Complete bladder neck release to allow a controlled plication; (4) Use of cystoscopy to achieve the ideal bladder neck closure; (5) Bladder neck manipulation to achieve continence.

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

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 exstrophy 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".

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.

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

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: Eleven patients were referred to us after previous surgery. Sixteen procedures were performed; one patient had complete wound dehiscence and needed another reconstruction (6.7%). Mean follow up was 10.3±4.5 years. No patient has had a loss of renal function. Postoperative complications: four patients (26.6%) presented small fistulas, one presented penile rotation. Eleven patients (73.3%) patients underwent bladder-neck surgery. Five (33.3%) required bladder augmentation. Three cases (20%) needed subsequent treatment of VUR. At the time of our review nine (60%) patients achieved UC, two (13.3 %) patient without additional procedure. A mean of 3±1.1 procedures (2-5) was accomplished per children. 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.

Quality of life in young adult patients treated for bladder exstrophy.

INTRODUCTION: Bladder exstrophy (BE) is a rare condition that requires complex surgical corrections to achieve the goals of bladder functionality, normal sexual function, continence, and finally cosmesis. The purpose of this study was to identify clinical parameters that predict better quality of life (QOL) scores using a validated questionnaire (SF-36) with young adults after completing surgical reconstruction. MATERIAL AND METHODS: Forty-three young adults (mean age 22.35 years, 29 men and 14 women) treated for BE were evaluated using the Short Form 36 general health questionnaire (SF-36). Clinical assessment involved evaluation of the actual condition regarding continence, sexual function, genital satisfaction and overall cosmesis. RESULTS: Both genders presented similar QOL scores (p = 0.36). The QOL was not age-related (p = 0.63). Neither genders did not present any differences in the number of procedures (p = 0.27). Although no significant gender difference was found, clinical impairments - such as urinary fistula, incontinence, penile length and infertility - were associated with worse QOL scores and were male-related (p <0.01). The most common complaint after complete surgical repair was about penile length (26/29 patients, 89.6%). CONCLUSIONS: Age and gender were not predictors of better QOL scores. Any clinical impairment, such as urinary leakage due to incontinence or fistula, penile length and infertility, tended to significantly decrease the overall QOL in male patients with BE. The male genitalia seems to be the most troublesome aspect post-adolescence in treated male patients with exstrophy-epispadias. It has an important impact on the overall QOL, mainly having a social affect on those patients.

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.

When hormone defects cannot explain it: malformative disorders of sex development.

The birth of a baby with malformations of the genitalia urges medical action. Even in cases where the condition is not life-threatening, the identification of the external genitalia as male or female is emotionally essential for the family, and genital malformations represent one of the most stressful situations around a newborn. The female or male configuration of the genitalia normally evolves during fetal life according to the genetic, gonadal, and hormonal sex. Disorders of sex development occur when male hormone (androgens and anti-Müllerian hormone) secretion or action is insufficient in the 46,XY fetus or when there is an androgen excess in the 46,XX fetus. However, sex hormone defects during fetal development cannot explain all congenital malformations of the reproductive tract. This review is focused on those congenital conditions in which gonadal function and sex hormone target organ sensitivity are normal and, therefore, not responsible for the genital malformation. Furthermore, because the reproductive and urinary systems share many common pathways in embryo-fetal development, conditions associating urogenital malformations are discussed.

Duplicación rectal extrofiada asociada a malformación anorrectal y transposición pene-escrotal con hipospadias perineal: Reporte de un caso clínico / Exstrophy of rectal duplication associated with anorectal malformation and penoscrotal transposition with perineal hypospadias: A case report

Se presenta el caso de un paciente masculino quien requirió tratamiento por agenesia anorrectal con fístula rectouretral y transposición pene-escrotal con hipospadias perineal, acompañados de una masa perineal. La tumoración perineal se encontró íntimamente adherida y en continuidad al recto, lo que la hace compatible con una duplicación rectal extrofiada. La reconstrucción quirúrgica de la anomalía se realizó en etapas hasta lograr resultados funcionales y estéticos aceptables.

We present the case of a male patient who required treaatment due to anorectal agenesis with recto urethral fistula and penoscrotal transposition with perineal hypospadias, associated with a perineal tumor. The perineal tumor was found strongly adhered and contiguous to the rectum which makes it compatible with an exstrophy of rectal duplication. Surgical reconstruction of the birth defect was performed in stages until acceptable biological function and esthetic results were obtained.

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.