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".

Experiencia en el abordaje de pacientes con complejo extrofia-epispadias en un centro de alto nivel de complejidad en Colombia, 10 años / 10-years experience in the management of bladder exstrophy-epispadias complex in a tertiary care health centre in Colombia

Objetivos El complejo extrofia-epispadias (CEE) se considera una de las malformaciones más severas de la línea media de compromiso multisistémico. La extrofia vesical es la presentación más frecuente en el espectro del complejo. Esta patología tiene un alto impacto en la calidad de vida. A pesar de la relación entre un cierre primario temprano y mejores resultados, en nuestro medio la remisión es tardía y la experiencia es escasa. El objetivo del siguiente estudio es mostrar la experiencia en el abordaje de CEE en los últimos 10 años en una institución de alto nivel de complejidad y remisión en Colombia. Materiales y métodos Se realiza un estudio observacional descriptivo, con una serie de casos del 2006 al 2016. Resultados En 10 años, se presentaron 5 casos de CEE en un centro de alta complejidad y remisión en Colombia. La mayoría de los pacientes han tenido múltiples intervenciones; la edad del primer procedimiento fue 829 días en promedio (27,6 meses). Se ha tenido un seguimiento postoperatorio promedio de 2,8 años. No se han presentado neoplasias en el seguimiento. Las comorbilidades más frecuentes son infección y litiasis. Ninguno de los pacientes contactados reportó inicio de vida sexual. La escala International Consultation on Incontinence Questionnaire-Urinary Incontinence-Short Form (ICIQ-UI-SF) tuvo un promedio de 9 puntos. Existen factores sociales asociados en nuestro medio. Conclusión El CEE requiere un abordaje temprano y multidisciplinario en instituciones con experiencia; los resultados en continencia urinaria, función sexual, desarrollo psicosocial y calidad de vida están sujetos a tratamiento oportuno de la patología.

Objective Exstrophy-epispadias complex (ECC) is considered one of the most severe midline abnormalities. Exstrophy of the bladder is the most frequent presentation of the exstrophy-epispadias spectrum. The disease has high impact on the quality of life. In developing countries, patient referral to experienced centres is often delayed. The experience in the disease is generally poor. The aim of this study is to describe the experience in the approach to EEC in the past 10 years in an institution of high level of complexity in Colombia. Materials and methods An observational descriptive study of a case series. Results A total of 5 cases of ECC were identified in the last 10 years in an institution of high level of complexity in Colombia. All the patients had multiple interventions. The mean follow-up time was 2.8 years. No neoplasms were recognised. The most common comorbidities were lithiasis and urinary tract infections. None of the patients contacted reported starting a sex life. The International Consultation on Incontinence Questionnaire-Urinary Incontinence-Short Form (ICIQ-UC-SF) gave a mean of 9 points. Conclusion The EEC requires an early and cross-disciplinary assessment in experienced centres. There are additional factors associated with the prognosis in our area.

Late secondary urological reconstruction of separated ischiopagus twins with exstrophic bladder and urinary incontinence / Reconstrução urológica tardia de gêmeos isquiópagos com extrofia de bexiga e incontinência urinária

ABSTRACT We report a case of secondary urinary reconstruction of previously separated conjoined twins with exstrophic bladder and urinary incontinence. Patients were male and aged 13-year-old. Twin one had a history of failed enterocystoplasty that extruded and was visible like an exstrophic neobladder. He underwent a procedure to close bladder neck and reconfigure abdominal wall. After the procedure the patient developed a fistula that was treated, but it persisted and, for this reason, a catheterizable pouch was constructed and native bladder was discarded. Twin two required the immediately construction of catheterizable pouch using the Macedo's technique. Currently, both patients are continent at 4 hour intervals. The mean follow-up was 8 months. Modern continent urinary diversion techniques offer new perspectives and hope for such complex population.

RESUMO Relata-se caso de reconstrução urinária em gêmeos siameses previamente separados com apresentação clínica de bexiga extrófica e incontinência urinária. Os dois pacientes eram do sexo masculino com idade de 13 anos. O primeiro gêmeo apresentava falha da enterocistoplastia com extrusão e visualização da neobexiga extrófica, tendo sido submetido ao fechamento do colo vesical e à reconfiguração da parede abdominal. Após o procedimento, o paciente desenvolveu fístula, que foi tratada, mas persistiu. Posteriormente, optamos por bolsa cateterizável, descartando a bexiga nativa. O segundo gêmeo foi submetido à construção imediata de bolsa cateterizável, por meio da técnica de Macedo. Atualmente, ambos os pacientes estão continentes em intervalos de 4 horas. O seguimento médio foi de 8 meses. As atuais técnicas de derivação urinária oferecem novas perspectivas e esperança para esta população complexa.

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.

Translational Research for Pediatric Lower Urinary Tract Dysfunction / 대한배뇨장애요실금학회지

This review provides a comprehensive view of translational research aimed at elucidating the pathophysiology of pediatric lower urinary tract dysfunction (LUTD). A web search was conducted according to combinations of keywords, and the significance of each article was defined by the author. The dramatic evolution of the mass analysis method of genomes, transcripts, and proteins has enabled a comprehensive analysis of molecular events underlying diseases, and these methodologies have also been applied to pediatric LUTD. In genetic analyses of syndromes underlying daytime incontinence, urofacial (Ochoa) syndrome may be creating a prototype of a new research approach. Nocturnal enuresis has long been studied genetically, and several candidate loci have been reported. However, the pursuit for enuresis genes has been abandoned partly because genetic association and enuresis phenotype (bladder or renal type) could not be linked. Enuresis associated with diabetes insipidus has provided new insights into the etiology of the diseases. A chronobiological approach may shed new light on this area. Posterior urethral valves and neurogenic bladders have attracted the interest of pediatric urologists to the smooth muscle biology of the bladder. Bladder exstrophy and cloacal anomalies are rare but major anomalies caused by defective urorectal development and have recently been studied from a genetic standpoint. Translational studies for pediatric LUTD may be extended to adult bladder disease, or to application of precision medicine for diseased children.

Translational Research for Pediatric Lower Urinary Tract Dysfunction / 대한배뇨장애요실금학회지

This review provides a comprehensive view of translational research aimed at elucidating the pathophysiology of pediatric lower urinary tract dysfunction (LUTD). A web search was conducted according to combinations of keywords, and the significance of each article was defined by the author. The dramatic evolution of the mass analysis method of genomes, transcripts, and proteins has enabled a comprehensive analysis of molecular events underlying diseases, and these methodologies have also been applied to pediatric LUTD. In genetic analyses of syndromes underlying daytime incontinence, urofacial (Ochoa) syndrome may be creating a prototype of a new research approach. Nocturnal enuresis has long been studied genetically, and several candidate loci have been reported. However, the pursuit for enuresis genes has been abandoned partly because genetic association and enuresis phenotype (bladder or renal type) could not be linked. Enuresis associated with diabetes insipidus has provided new insights into the etiology of the diseases. A chronobiological approach may shed new light on this area. Posterior urethral valves and neurogenic bladders have attracted the interest of pediatric urologists to the smooth muscle biology of the bladder. Bladder exstrophy and cloacal anomalies are rare but major anomalies caused by defective urorectal development and have recently been studied from a genetic standpoint. Translational studies for pediatric LUTD may be extended to adult bladder disease, or to application of precision medicine for diseased children.

Quality of life of mothers whose children work on the streets of São Paulo, Brazil / Qualidade de vida de mães de crianças trabalhadoras de rua da cidade de São Paulo, Brasil / La calidad de vida de las madres de niños trabajadores en las calles de São Paulo, Brasil

The present study evaluated the perceived quality of life of the mothers of street children and investigated the association with their history of childhood violence, the occurrence of current domestic violence, their current mental states and that of their children, and family functioning. The applied instruments were as follows: Strengths and Difficulties Questionnaire, WorldSAFECore Questionnaire, Instrument for the Assessment of Quality of Life of the WHO, Global Assessment of Relational Functioning Scale, Childhood Trauma Questionnaire and a socio-demographic questionnaire. The sample of convenience consisted of 79 low-income mothers who raised their children alone, and most of whom had a positive screening for mental illness. The multiple regression analysis showed that the perception of quality of life of these women was associated with the presence of psychopathology either in themselves or their children and family dysfunction. Thus any program aimed at improving the quality of life of such mothers should consider addressing their mental problems as well as those of their children, besides offering educational and psychotherapeutic approaches to these families to improve the social environment.

Avaliamos a percepção da qualidade de vida de mães de crianças em situação de rua e investigamos a sua possível associação com os antecedentes maternos de abuso e violência na infância e vitimização atual, sintomas emocionais delas e de seus filhos e com o funcionamento familiar. Foram aplicados os seguintes instrumentos: Instrumento para a Avaliação da Qualidade de Vida da OMS - versão abreviada, Questionário de Capacidades e Dificuldades, WorldSAFECore Questionnaire, Escala de Avaliação Global de Funcionamento nas Relações Familiares, Childhood Trauma Questionnaire e um questionário sociodemográfico. Nossa amostra de conveniência foi constituída por 79 mães, a maioria delas com rastreamento positivo para doença mental. Aplicamos um modelo de regressão múltipla que encontrou associação entre a percepção de pior qualidade de vida com a presença de psicopatologia nelas e em seus filhos e com pior funcionamento familiar. Dessa forma, qualquer programa voltado para a melhora da qualidade de vida de mães nessa condição deve considerar a abordagem de problemas mentais nelas e em seus filhos, além de oferecer recursos educacionais e psicoterapêuticos para as famílias, visando à melhora das condições socioambientais.

Se presenta aquí una evaluación de la calidad de vida percibida por madres de niños en las calles, y se investigó la posible asociación con antecedentes maternos de abuso y violencia en la infancia y victimización actual, los síntomas emocionales de ellos y de sus hijos y el funcionamiento familiar. Se aplicaron los Instrumentos para la Evaluación de la Calidad de Vida de la OMS - versión abreviada, Cuestionario de Capacidades y Dificultades, WorldSAFECore Questionnaire, Escala de Evaluación Global de Funcionamiento en las Relaciones Familiares, Childhood Trauma Questionnaire y un cuestionario sociodemográfico. La muestra incluyó a 79 madres, la mayoría de ellas con una detección positiva para la enfermedad mental. La percepción de calidad de vida se asoció con la presencia de psicopatología en ellos y sus hijos y el funcionamiento familiar. Por lo tanto, cualquier programa destinado a mejorar la calidad de vida de las madres en esta condición debe considerar el enfoque de los problemas mentales en ellos y sus hijos y ofrecer trabajo educativo y terapéutico a esas familias.

Cloacal Exstrophy: a complex disease

Introduction Cloacal exstrophy is a rare occurrence with an incidence of 1:200,000 to 1:400,000 live births. It represents one of the most challenging reconstructive endeavors faced by pediatric surgeons and urologists. Aside from the genitourinary defects, there are other associated anomalies of the gastrointestinal, musculoskeletal and neurological systems that require a multidisciplinary approach when counseling anxious parents. Material and Methods We present a video of a patient with cloacal exstrophy treated with 21 days of life. Surgery consisted of separation and tubularization of the cecal plate from the exstrophied bladder halves and colostomy construction. The bladder was closed primarily and umbilical scar reconstructed and used for ureteral and cistostomy drainage. A urethral catheter was used to guide bladder neck tubularization. A final epispadic penis was obtained and planned for further repair in a second step. Results The patient had an initial uneventful postoperative course and immediate outcome was excellent. The bladder healed nicely but patient presented with abdominal distension in the 5th day of postoperative setting requiring parenteral nutrition. The distal colon persisted with lower diameter although non obstructive, but causing difficulty for fecal progression. Continuous colostomy dilatation and irrigation were required. Conclusions Approximating the bladder halves in the midline at birth and primary bladder closure is a viable option, intestinal transit may be a issue of concern in the early postoperative follow-up. .

Y chromosome aberration in a patient with cloacal-bladder exstrophy-epispadias complex: an unusual finding / Aberração cromossômica do Y em uma paciente com extrofia de bexiga e de cloaca e epispadia: um achado raro

Chromosome aberrations or genetic syndromes associated with cloacal-bladder exstrophy complex have rarely been reported. The aim of this report is to describe a 14 year-old female Brazilian patient with a complex urogenital malformation, short stature, lack of secondary se­xual characteristics and Y chromosome aberration. A girl with cloacal bladder exstrophy complex was referred for evaluation of short stature and absence of secondary sexual characteristics. Pre-pubertal levels of gonadotropins and sex steroids were observed at the beginning of monitoring, but follow-up showed a progressive increase in testosterone levels. The patient underwent gonadectomy and testicular tissue was identified without dysgenetic characteristics. She had a 46,X,inv(Y)(p11.1q11.2) karyotype, normal SRY sequence, and no Y deletions. The pericentric inversion of Y chromosome apparently did not contribute to the development of the complex urogenital malformation in this patient. Currently, no teratogenic agent, environmental factor, or defective genes have been recognized as etiologic factors for this type of urogenital malformation.

Aberrações cromossômicas ou síndromes genéticas associadas ao complexo extrofia de bexiga e de cloaca e epispadia são raramente relatadas. O objetivo é descrever uma paciente brasileira com 14 anos que apresenta uma malformação urogenital complexa, baixa estatura, ausência de características sexuais secundárias e alteração do cromossomo Y. Uma menina com extrofia de bexiga e de cloaca e epispadia foi encaminhada para avaliação de baixa estatura e ausência de desenvolvimento de características sexuais secundárias. Níveis pré-puberais de gonadotrofinas e esteroides sexuais foram observados no início da avaliação, mas durante o seguimento notou-se um aumento progressivo dos níveis de testosterona. Ela foi submetida à gonadectomia e identificou-se a presença de testículos sem características disgenéticas. O cariótipo era 46,X,inv(Y)(p11.1q11.2), com sequência normal do SRY e ausência de deleções do Y. A inversão pericêntrica do cromossomo Y, aparentemente, não contribuiu para o desenvolvimento da malformação urogenital complexa nessa paciente. Atualmente, nenhum agente teratogênico, fator ambiental ou mutações gênicas foram reconhecidos como fatores etiológicos para essa malformação urogenital.

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.

Exstrophy Anomalies: Preliminary Review of 18 Cases in Maiduguri; North-Eastern Nigeria

Background: Exstrophy anomalies; although rare; pose a tremendous challenge to both surgeons and surrogates. Management is intricate and experience with large members is restricted to a few centres worldwide. This report is a review of 18 cases of these anomalies managed in University of Maiduguri Teaching Hospital (UMTH) over 8 years; highlighting the intricacies of management; and contributes to the existing data bank on this subject matter in our environment. Materials and Methods: We reviewed the records of all cases of anomalies that were managed in the index hospital from December 2002 to December 2010. Results: Eighteen cases were studied. The youngest was a 2-day-old neonate and the oldest was 6 years at repair; with a median age of 4 months. There were 7 boys and 9 girls with bladder exstrophy; and the remaining two were girls with cloacal exstrophy; giving a male-female ratio of 7:11 for the exstrophy anomalies. Eight patients (33.5) had associated congenital anomalies; 16.7 had undescended testes (UDT); anorectal malformation (ARM) and patent processus vaginalis (PPV); and duplicate bladder occurred in 5.6 of the patients. All but one patient had bilateral posterior iliac osteotomy at bladder closure. Out of the 17 patients that had osteotomy; 76.5 had satisfactory bladder closure (no complication); 5.9 had superficial wound dehiscence; 11.8 had bladder neck dehiscence and 5.9 had complete disruption. The lone patient that did not undergo osteotomy had complete bladder dehiscence. Postoperative immobilization was done for 3 weeks for 16 cases; one was discontinued after 1 week because of complication. The longest follow-up was for 2 years and the shortest for 2 weeks. Four patients (22.2) did not turn up for follow-up. Conclusion: Exstrophy anomalies although rare remain a major challenge in paediatric surgery. Iliac osteotomy; among other technical considerations; remains a cornerstone for successful bladder closure

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...

Squamous cell carcinoma in bladder exstrophy: a rare entity

Carcinomas arising from an exstrophic urinary bladder are rare entities, and only seven such cases have been reported in the literature. We present the eighth case of advanced squamous cell carcinoma arising from an exstrophic bladder, with a pertinent review of the literature. The mean age of the patients was 54.9 years, with a male to female ratio of 3:1. The average duration of symptoms was 18.6 months. The appearance of a new growth was the most common symptom. Three patients had stage I disease, one patient each had stage II and III disease, two patients had stage IV disease, and the disease stage was not known in one patient. Five out of these eight patients underwent surgery. Four patients in the treatment group remained disease-free, with a mean survival period of 30 months. In conclusion, regular surveillance with cystoscopy is advised in all cases that had primary closure of the exstrophic bladder.

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.