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

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

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

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

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

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.

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.

A wet dressing for male genital surgery: A phase II clinical trial

ABSTRACT Purpose: This study was to confirm the safety and efficacy of BC dressing when used in surgical male wound healing at the urogenital area. Methods: Open, non-controlled clinical study of phase II. A total of 141 patients, among those children, adolescents and adults with hypospadias (112), epispadias (04), phymosis (13) and Peyronie's disease (12) that had a BC dressing applied over the operated area after surgery. A written informed consent was obtained from all participants. Study exclusion criteria were patients with other alternative treatment indications due to the severity, extent of the injury or the underlying disease. The outcomes evaluated were efficacy, safe and complete healing. The costs were discussed. Results: In 68% patients, the BC dressing fell off spontaneously. The BC was removed without complications in 13% of patients at the outpatient clinic during the follow-up visit and 17% not reported the time of removal. In 3% of the cases, the dressing fell off early. Complete healing was observed between 8th and 10th days after surgery. The BC dressings have shown a good tolerance by all the patients and there were no reports of serious adverse events. Conclusion: The bacterial cellulose dressings have shown efficacy, safety and that can be considered as a satisfactory alternative for postoperative wound healing in urogenital area and with low cost.

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.

Anatomy correction of penile epispadias / 中华整形外科杂志

<p><b>OBJECTIVE</b>To investigate the pathological characters and anatomic correction of penile epispadias.</p><p><b>METHODS</b>The urethra was formed by local urethra plate mucosa flaps. The contracture on dorsum of penis was released by cutting off the superficial suspensory ligament to reposition the penile and urethral sponge.</p><p><b>RESULTS</b>From Jun. 2004 to Dec. 2010, 26 cases with penile epispadias were treated. 18 cases were followed up for 6 months to 5 years. 10 cases were treated successfully with good cosmetic and functional results. Urinary incontinence happened in 8 cases, which healed after tightening operation.</p><p><b>CONCLUSIONS</b>The anatomic correction of penile epispadias can recover the normal anatomic structure, resulting good cosmetic and functional results.</p>

Proteomic analysis of the testis and differential expression of Annexin A3 in hypospadiac rats / 中华男科学杂志

<p><b>OBJECTIVE</b>To investigate the effect of in utero exposure to di-n-butyl phthalate (DBP) on the protein expression in the penile tissue of hypospadiac rats, isolate and identify differentially expressed proteins, and determine the role of the differential expression of Annexin A3 in the development of hypospadia in the rat offspring after maternal exposure to DBP.</p><p><b>METHODS</b>Twenty pregnant SD rats were randomly assigned to an experimental group, intragastrically administered DBP at 800 mg/kg, and a control group, given soybean oil at 5 ml/kg, both for 5 days. Three days after birth, the penises of the newborn rats were removed, and the total protein extracted for 2D-electrophoretic separation and image analysis. Differentially expressed protein spots were screened and identified by mass spectrometry, and the changes in the expression of Annexin A3 detected by Western blotting and immunohistochemistry.</p><p><b>RESULTS</b>Thirty-one differentially expressed protein spots were screened, of which 17 were identified by mass spectrometry and the SwissProt database, including pyruvate kinase M2, alpha-enolase, and Annexin A3. Western blot showed that Annexin A3 was mainly located in the urethral epithelia and had a lower expression in the hypospadiac rats (1.851 +/- 0.014, n = 10) than in the controls (2.603 +/- 0.012, n = 10) (P < 0.05).</p><p><b>CONCLUSION</b>A pedigree of differentially expressed proteins in the penises of DBP-induced hypospadia and normal rats was established by the proteomic method. The differential expression of Annexin A3 may play an important role in the development of hypospadia.</p>

Diphallus: report on six cases and review of the literature

Diphallus is an extremely rare anomaly. Numerous associated genitourinary, gastrointestinal and other anomalies have been described with diphallus. These patients need several investigations, and finally surgical intervention. In this report we discuss six patients with diphallus which evaluated retrospectively. Five patients had complete diphallia, and one had bifid diphallus. Meatus was normal in 3, hypospadiac in 2, and epispadiac in one patient. The most common associated anomaly was bifid scrotum [5 cases], and other common anomalies consisted of bladder duplication [3 cases], imperforate anus [2 cases], and hypospadias [2 cases]. Phalloplasty was performed for all but one. All the patients with urethral duplication have to be evaluated carefully because of the high incidence of other systemic anomalies

Bladder exstrophy.

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

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.

Congenital prepubic sinus: a case report

Congenital prepubic sinus is an exceptional disorder of uncertain origin. These sinuses may represent a variant of epispadiac duplication or dorsal urethral duplication of the urethra.We present a case of isolated blind ending prepubic congenital sinus in a 5-year-old male child; which revealed transitional epithelium lining proximally; and squamous epithelium distally. This entity presents with either an opening in the prepubic region and/or persistent mucoid discharge. Excision leads to a permanent cure

Modified Mitchell's technique for repair epispadias with a report of 3 cases / 中华整形外科杂志

<p><b>OBJECTIVE</b>To evaluate a modified Mitchell's technique for epispadias repair.</p><p><b>METHODS</b>Three patients with epispadias were undergoing the treatment. It was performed to form a penile pedicled flap or scrotal mediastinum flap for the repair of the urethra and the urine was drained through a perined "U" shaped stent.</p><p><b>RESULTS</b>All of the patients were successfully treated in one-stage with a good appearance and a matching opening of the urethra.</p><p><b>CONCLUSION</b>The above mentioned technique could be a good method for epispadias repair.</p>

Isolated torsion of the penis: technique and review of the literature

Torsion of the penis is a rather rare condition in which the penis is rotated to one side with different degrees. It is a common condition when associated with other congenital defects of the penis such as hypospadias and epispadias and their variants. The association of chordee and bending of the penis is a common happening. This study in meant to demonstrate a new technique in the treatment of Torsion of the penis. Among the cases admitted to the Paediatric Surgical Unit of the Medical Research Institute and of the Faculty of Medicine-University of Alexandria, 18 cases of isolated torsion were included in this study. These were gathered based on consecutive admission in the period between March 2001 and February 2003. A total of 11 cases were admitted in the Faculty of Medicine Unit, while 7 cases were managed in the Medical Research Institute. The age ranged between 1 and 10 years with a mean of 4.4 years. The cases associated with hypospadias and epispadias were not included in this study. However, some deviation of the isolated cases of torsion included 1 case with chordee, 1 case of lateral bent penis and 2 cases of deficient ventral prepuce. The annual distribution was 6 cases in 2001, 9 cases of deficient ventral prepuce. The annual distribution was 6 cases in 2001, 9 cases in 2002 and 3 cases in 2003, Fifteen out of the 18 cases were showing an anti-clokwise torsion. Simple detorsion under general anaesthesia was the rule using a modification to adjust the shape of the penis after circumcision. This depends on protection of the glans and determination of the ratio between the post-coronal collar and the skin in anticipation. Adequate apposition of the median raphe to the new de-roted shaft is put in consideration. No complications were encountered except in one case, where residual torsion was reported after healing. Another go was necessary to ensure satisfactory detorston

Vesicostomía continente en pediatría: utilidad del apéndice cecal / Continent vesicostomy in children: cecal appendix use

Antecedentes: El apéndice cecal se ha utilizado con éxito en el niño para reemplazar el uréter, la uretra, para vesicostomías continentes en ciertas patologías vesicales (técnica de Mitrofanoff). Objetivo: Mostrar los resultados obtenidos con esta técnica y además abogar por la conservación del apéndice cecal. Material y métodos: Entre febrero de 1991 a octubre de 1997 se efectuaron 46 vesicostomías continentes. Se empleó la operación de Mitrofanoff utilizando el apéndice cecal en 39 casos. En los restantes, por ausencia de apéndice, se usó como tubo el uréter, íleon o colon modelados. La edad de los pacientes osciló entre los 1 y 24 años, con una media de 9 años. Se operaron 32 varones y 14 mujeres. La indicación de la vesicostomía continente fue: a) en enfermos con pobre vaciamiento vesical en cateterismo intermitente limpio, con dificultad para realizarlo por alteraciones anatómicas cérvico/uretrales (estenosis, divertículos, malformaciones, anfractuosidades) o sensibilidad uretral aumentada; b) pacientes con oclusión uretral definitiva o temporaria; c) reservorios vesicales para continencia y vaciamiento del mismo. En 41 casos la vesicostomía se asoció a otros procedimientos simultáneos (cierre del cuello vesical, ampliación vesical, tratamiento de reflujo y onfaloplastia). El tiempo de seguimiento osciló entre 6 a 82 meses con una media de 46 meses. En el período de seguimiento, 32 pacientes no presentaron complicaciones. Resultados: En 7 niños se presentó estenosis del ostoma con dificultad para el cateterismo, 5 se dilataron y 2 se corrigieron quirúrgicamente. En 5 casos (no ubicados en la cicatriz umbilical) se produjo un ectropión mucoso, que si bien no dificultó el cateterismo, por razones estéticas se resecó. Sólo un paciente con tubo realizado con un segmento ileal persiste levemen incontinente. En 2 pacientes hubo inconvenientes en el pasaje de la sonda que requirió la colocación de vesicostomía percutánea transitoria. Ningún niño mostró deterioro de su función renal atribuible al procedimiento. El vaciamiento periódico y completo redujo francamente el número de infecciones urinarias. Conclusiones: La vesicostomía continente con apéndice cecal (Técnica de Mitrofanoff) es un excelente método y de escasa morbilidad. Bien indicado facilita el vaciamiento vesical periódico y permite al niño mantenerse seco y libre de infección, mejorando su calidad de vida...

Male epispadias

A better anatomical and embryological knowledge has allowed a significant advance in the management of male epispadias. Surgery has the following goals: 1. reconstruction of the urethra to enable urine voiding, either spontaneously or by intermittent catheterization through a neomeatus placed at the distal portion of the glans; 2. attainment of urinary continence preserving the upper urinary tract; 3. reconstruction of the penis so that its morphological characteristics be adequate to enable sexual relations; 4. achievement of a favorable esthetical appearance. Modern techniques provide good results with a low rate of complications. At present, the best technique for male epispadias repair is the modification introduced by ransley in the technique originally described by Cantwell. Although Mitchell's technique is promising more experience is to be gained through a close follow-up of patients

Exstrophic anomalies: recent advances and long-term outlook.

Exstrophy-epispadias complex has been a difficult disease to treat. As in the minds of the public and most physicians alike, these children are crippled with life-long ailments and multiple operations. Much of the morbidity of this condition relates to failure to preserve urogenital tract function in these children. In recent years, through better understanding of the exstrophic anatomy and improved surgical techniques, complete repair of the exstrophic anomaly has become possible as soon as the child is born. Results of such closure indicate that such repair is not only feasible but mandatory for a successful outcome. Continence rate and cosmetic appearance are superior to the conventional 3-stage technique. Such repair is also socially acceptable as the parents will bring home a normal-looking baby with much fewer operations expected in the future. It is anticipated that complete one-stage exstrophy closure in the newborn period will revolutionize the outcome of all exstrophy patients.

RECONSTRUCTION OF URETHRAL DEFECT DUE TO PERINEAL SORE USING PEDICLED SCROTAL SEPTAL FLAP

Reconstruction of the male urethra is required for a multitude of congenital anomalies, such as hypospadias and epispadias, as well as fort acquired lesions such as stricture disease and trauma. No method is appropriate for all types of repair of the urethra. A successful surgical reconstruction of the urethra is the result of a proper operative selection that depends on the site and length of the disrupted urethra and also depends on, as well as the experience of the surgeon. Reconstruction of the urethra has been performed by the use of homografts, silicone tubes, grafts taken from the bladder mucosa. But serious postoperative problems, such as urinary fistula, urethrostenosis, stricture of the site of urethral anastomosis, and diverticulum formation, are not uncommon. Especially, reconstruction of Proximal urethra is very difficult because of limitations of operation field exposure and flap transfer. However, the hairless median scrotal skin flap with the scrotal septum attached, rich in neurovascular plexus, is ideal material for creating a new urethra without interference to its blood supply and other complications. We experienced a successful reconstruction of the proximal urethral defect in about 8cm length and perineal sore with pedicled scrotal septal flap and gracilis musculocutaneous flap, respectively.

A Case of Female Epispadias / 대한비뇨기과학회지

Female epispadias is an uncommon congenital anomaly in genitourinary tract. We experienced a case of female epispadias with total urinary incontinence which was improved with periurethral injection of Glutaraldehyde Cross-Linked Collagen(GAX-Collagen) into the area of the bladder neck. The procedure was simple to perform and without significant complications. Herein we report a case of female epispadias in 29-year old female.