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".

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.

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. .

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...

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

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.

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.

Salvage reconstructive surgery in an adult patient with failed previous repair of an extrophy-epispadias complex: an operation with a functional and aesthetic purpose

Salvage surgical procedures after failed reconstruction for an extrophy-epispadias complex are extremely challenging. The goals are to restore continence and improve aesthetic appearance in order to provide quality of life and an improved body image to the patient. We describe the surgical steps in an adult patient who presented anal urinary incontinence and a poor body image due to the absence of an umbilicus and the presence of hypertrophic scars. He underwent a modified Mainz II reconstruction of the lower urinary tract at childhood for an extrophy-epispadias complex. Restoration of continence was achieved by the construction of a modified Mainz I pouch with a continent stoma in a neo-umbilicus. Body image improved dramatically by the construction of a neo-umbilicus, a surgical revision of the hypertrophic abdominal scars and an abdominoplasty. It is mandatory that such demanding surgery should only be attempted as a combined multidisciplinary effort with urologists and plastic/reconstructive surgeons.

Extrofia de bexiga: reconstruçäo vesical ou derivaçäo primária? Resultados do Pouch Sigmóide-Reto (Mainz Pouch II) / Bladder exstrophy: reconstruction of the bladder neck or primary diversion? Results of the sigma-rectum pouch (Mains Pouch II)

Os autores discutem as opçöes para o tratamento cirúrgico da extrofia da bexiga, confrontando os procedimentos de reconstruçäo vesical com a derivaçäo urinária primária. Apresentam a seguir resumidamente a técnica operatória do pouch sigmóide-reto (Mainz Pouch II) e os resultados de 17 pacientes com extrofia de bexiga submetidos a esta cirurgia. Ausência de complicaçöes perioperatórias e índices de continência de 92,8 por cento foram obtidos como resultados

Una nueva técnica quirúrgica para aumentar la resistencia cérvico uretral / A new a technique for bladder neck resistance augmentation

Se diseñó y experimentó en 12 perros una técnica de plástica endovesical del cuello, para aumentar la resistencia cérvico uretral.La vesicotomía se realizó desde uno de los laterales hasta el otro pasando por la cúpula vesical,dejando dividida la vejiga en una valva anterior y otra posterior.Se disecó un colgajo mucoso con base en el orificio cervical por la cara anterior de la vejiga, el mismo se llevó a la cara posterior suturando sus dos lateralesen espejo.A continuación se cerró el defecto de la cara anterior y se cubrió la plástica dejándola en posición submucosa.Los resultados obtenidos llevaron a aplicar esta técnica en cuatro pacientes, dos varones y dos niñas.Sus edades oscilaron entre 4f y 17 años.Tres de ellos presentaban como patología de base, una extrofia vesical y una niña una vejiga neurogénica por trauma obstétrico.Todos los pacientes tenían antecedentes de cirugías vesicales. En tres de ellos se realizaron ampliaciones vesicales y un ostoma de vaciamiento en forma simultánea a la plástica de cuello. Las indicaciones de ampliación vesical y Mitrofanoff fueron independientes a la construcción de la plástica cervical. El seguimiento fue de 6meses, 1 año, 1 año y dos años respectivamente. Todos los pacientes se mantienen actualmente secos por más de 4 horas, evacuando a través del ostoma continente.Esta nueva operación permite evitar el cierre del cuello y es fácil de revertir.Ningún paciente necesitó disección extravesical. La plástica de cuello no fue la indicación para ampliar la vejiga de ningún paciente

Gastrocistoplastia: una alternativa para la reconstrucción de las vías urinarias infantiles / Gastrocystoplasty: a reconstructive alternative of pediatric urinary tract

Las alteraciones urológicas complejas en los niños frecuentemente alteran la urodinamía vesical: producen disminución de la capacidad vesical y obstrucción urinaria con reflujo vesicoureteral y posible lesión renal terminal. El estómago es un órgano secretor con mínima producción de moco, y que ofrece buena capacidad de distensión. Estas características permiten usarlos como una buena alternativa en la ampliación vesical. En este trabajo se presentan tres casos de reconstrucción de las vías urinarias inferiores, en los que se utilizó un segmento de estómago para la ampliación vesical. Los resultados obtenidos en los tres casos se consideran excelentes

Colgajos de fascia lata en la cirugía reconstructiva de la pared abdominal inferior / Fascia lata myocutaneos flaps in reconstruction of inferior abdominal wall

Los grandes defectos de la pared abdominal inferior por anomalías congénitas, o por secuelas de traumas o infecciones con esfacelos, se pueden reconstruir con facilidad y excelentes resultados aportando tejidos vascularizados de los muslos. Los colgajos miofasciales y miofasciocutáneos del músculo tensor de la facia lata, han sido usados con éxito y sin complicaciones con este fin. Se presentan tres casos clínicos cuya buena evolución a largo plazo, nos permite recomendar este recurso en situaciones similares

Extrofia vesical: tratamiento de casos complicados por operaciones previas / Vesical extrophy: treatment of complicated cases by previous surgical procedures

En el tratamiento de la extrofia vesical ocurren resultados poco satisfactorios; en forma invariable, complicaciones como reproducción extrófica, prolapso de la mucosa vesical e incontinencia urinaria, que resultan al tratar de recontruir esta malformación congénita. Em la actualidad, la extrofia vesical representa un gran reto para el cirujano y para el urólogo pediátra. De marzo a septiembre de 1992 se reconstruyeron quirúrgicamente con muy buen éxito 10 casos de extrofia vesical, que ya antes habían sido operados, basados en algunos principio de osteotomía anterior para facilitar: a) cierre de la pared abdominal anterior, b) plastía de genitales; c) cierre del anillo pélvico, y d) capacidad vesical adecuada, mediante alguna forma de cistoplastía de aumento

Seven years' experience of ureterosigmoidostomy in surgically failed exstrophy of the bladder.

Reconstruction of a typical exstrophy of bladder and to achieve normal or nearly normal functional activity is still in investigating stage. In spite of efforts of many surgeons satisfactory results are too far to be obtained and it is almost impossible to obtain a near normal bladder capacity with full continence, free of infection and non-obstructive to the upper urinary tracts. Six cases of exstrophy of bladder where reconstruction has failed several times were subsequently referred to the urology department of Calcutta National Medical College, Calcutta, for urinary diversion; on one case previous attempt at repair resulted in small thick walled bladder with recurrent calculus formation. This study was made on those 6 patients, their age ranging from 3 to 14 years, in whom ureterosigmoidostomy was carried out during the year 1979 to 1986. In all patients careful bowel preparation was carried out by low residue diet, laxatives, enemas and antibiotics. Two patients developed hyperchloraemia and acidosis, 2 pyelonephritis and 2 remained asymptomatic till December 1986. None of those patients died so far. Ureterosigmoidostomy is not ideal but it can be adequate and sometimes is the preferred form of urinary diversion within limited resources. None of the methods of urinary diversion are free from complications but considering all, ureterosigmoidostomy as a method of urinary diversion is non-hazardous simple procedure with practically no mortality.(ABSTRACT TRUNCATED AT 250 WORDS)