Urethral duplication type influences on the complications rate and number of surgical procedures

ABSTRACT Introduction: Urethral duplication is rare. Characterized by the presence of two urethral channels. This anomaly presents a great variety of clinical findings that depend on the type of duplication that often is associated with other anomalies. Material and Methods: We report thirteen boys with urethral duplication managed in our institution between 1988-2015. Clinical findings, associated anomalies, treatment of urethral duplication and our results are described. Patients were classified according to Effmann classification. Results: Mean patient's age was 38.3±34.7 months (3-136 months). Mean follow-up was 7.7±3.4 years (3y8m-14y2m). Type II A2 was the most common pattern (8/13 patients, 61.5%), followed by type IA (3/13 patients, 23%) and IIA1 (2/13 patients, 15.3%). The most frequent clinical manifestations were urinary tract infections (UTI) observed in 11/13 patients (84.6%) and anal urinary leakage, found in 7/13 patients (53.8%). Associated anomalies were found in 9/13 patients (69.2%). Required surgeries were 3.53±2.84 procedures per patient. Considering groups: Type IIA2 4.25±3.28, type IIA1 4±1.41 and type IA 1.33±0.57 needed procedures per patient. Complications rate were 0% for type IA, 50% for type IIA1 and 75% for type IIA2. Conclusions: Patients with incomplete duplication (type I A or I B) can totally be asymptomatic, with no need of surgical correction. Type IIA2 is the most complex form of duplication to correct and multiple procedures might be required because of the very hypoplastic orthotopic dorsal urethral tissue. Surgical treatment should be individualized and parents should be advised on complications and need of multiple surgeries according to urethral duplication type.

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

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

A rare case of perineal hamartoma associated with cryptorchidism and imperforate anus: case report / Caso raro de hamartoma perineal associado a criptorquidia e ânus imperfurado: relato de caso

A full-term male neonate with anorectal anomaly and external perineal anomalies was referred to our service. Physical examination showed an epithelized perineal mass with cutaneous orifices, which had urine fistulization, hipotrofic perineal musculature, bilateral congenital clubfoot, hipospadic urethra, criptorquidy bilateral with nonpalpable testis and imperforate anus. A colostomy was constructed immediately after birth. The child underwent excision of perineal mass, bilateral orchidopexy, Duplay neourethroplasty and coloanal anastomosis at 3 months of age. The histopathological examination of the perineal mass revealed a hamartoma.

Recém-nascido a termo do sexo masculino encaminhado ao nosso serviço por anomalia anorretal e anomalias perineais externas. O exame físico revelou massa perineal epitelizada, com orifícios cutâneos que apresentavam saída de urina, musculatura perineal hipotrófica, pé torto congênito bilateral, uretra hipospádica, criptorquidia bilateral com testículos não palpáveis e ânus imperfurado. Logo após o nascimento, o paciente foi submetido à colostomia. Aos 3 meses de idade, a criança foi submetida à excisão da massa perineal, orquidopexia bilateral, neouretroplastia a Duplay e anastomose coloanal. A análise anatomopatológica da massa perineal indicou hamartoma.

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.

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.

Laparoscopic renal surgery in infants and children: is it a feasible and safe procedure for all pediatric age groups?

PURPOSE: Although laparoscopy is considered the mainstay for most renal procedures in adults, its role in the pediatric population is still controversial, especially for smaller children. We reviewed our experience in pediatric renal laparoscopic surgery in three pediatric age groups in an attempt to identify if age has an impact on feasibility and surgical outcomes. MATERIALS AND METHODS: From November 1995 to May 2006, 144 pediatric laparoscopic renal procedures were performed at our institution. The charts of these patients were reviewed for demographic data, urologic pathology and surgical procedure, as well as perioperative complications and post-operative outcomes. The findings were stratified into 3 groups, according to patient age (A: < 1 year, B: 1 to 5 years and C: 6-18 years). RESULTS: Median age of the patients was 4.2 years (42 days - 18 years). We performed 54 nephrectomies, 33 nephroureterectomies, 19 upper pole nephrectomies, 11 radical nephrectomies, 22 pyeloplasties and 4 miscellaneous procedures. The 3 age groups were comparable in terms of the procedures performed. Conversion rates were 0 percent, 1.4 percent and 1.9 percent for groups A, B and C, respectively (p = 0.72). Incidence of perioperative complications was 5 percent, 8.2 percent and 7.8 percent for age groups A to C, respectively (p = 0.88). CONCLUSIONS: Most renal procedures can be performed safely by laparoscopy in the pediatric population, with excellent aesthetic and functional outcomes. The morbidity related to the procedure was minimal irrespective of the age group.

Laparoscopic diagnosis and treatment of nonpalpable testis

INTRODUCTION: Treatment of the cryptorchid testicle is justified due to the increased risk of infertility and malignancy as well as the risk of testicular trauma and psychological stigma on patients and their parents. Approximately 20 percent of cryptorchid testicles are nonpalpable. In these cases, the videolaparoscopic technique is a useful alternative method for diagnosis and treatment. MATERIALS AND METHODS: We present data concerning 90 patients submitted to diagnostic laparoscopy for impalpable testicles. Forty-six patients (51.1 percent) had intra-abdominal gonads. In 25 testicles of 19 patients, we performed a two stage laparoscopic Fowler-Stephens orchiopexy. The other 27 patients underwent primary laparoscopic orchiopexy, in a total of 29 testicles. RESULTS: We obtained an overall 88 percent success rate with the 2 stage Fowler-Stephens approach and only 33 percent rate success using one stage Fowler-Stephens surgery with primary vascular ligature. There was no intraoperative complication in our group of patients. In the laparoscopic procedures, the cosmetic aspect is remarkably more favorable as compared to open surgeries. Hospital stay and convalescence were brief. CONCLUSIONS: In pediatric age group, the laparoscopic approach is safe and feasible. Furthermore, the laparoscopic orchiopexy presents excellent results in terms of diagnosis and therapy of the impalpable testis, which is why this technique has been routinely incorporated in our Department.

A litotripsia extracorpórea no tratamento de cálculos urinários em crianças / Extracorporeal lithotripsy for the treatment of urolithiasis in children

Objetivo: cálculos urinários atingem de 5 a 10por cento da população em alguma fase da vida. Destes casos, somente 2 a 3por cento são crianças.O tratamento da litiase urinária na faixa pediátrica, com o uso de métodos minimamente invasivos, representa uma alternativa cada vez mais atraente. O presente trabalho objetiva apresentar resultados de litotripsia extracorpórea como tratamento minimamente invasivo de litiase urinária na infância.Métodos: no periodo de setembro de 1991 a setembro de 2000, foram tratadas e acompanhadas 87 crianças, sendo que seis delas tinham dois cálculos; portanto foram tratados 93 cálculos urinários.Foi utilizado o equipamento de litotripsia extracorpórea por ondas de choque, da Dornier-Philips.Resultados: os cálculos piélicos, caliciais superiores e médios foram fragmentados e eliminados na porcentagem de 87,7por cento e 77,8por cento, respectivamente. Os cálculos localizados no cálice inferior foram eliminados em 64,7por cento. Dos três casos de cálculo coraliforme, somente um ficou livre do cálculo (33,3por cento). Os dez pacientes com cálculo ureteral ficaram livres de litiase (100por cento). Os cálculos vesicais foram tratados e eliminados em 60por cento dos casos.Conclusão: a litotripsia extracorpórea demonstrou ser uma forma eficiente de tratamento de cálculos piélicos, caliciais, ureterais e vesicais...

Retalho epigástrico inferior: estudo experimental / Inferior epigastric flap: an experimental study

Neste trabalho os autores se propöem a estudar a artéria epigástrica superficial inferior (AESI) desde suas origens, seu trajeto e a rede anastomótica estabelecida por suas tributárias. Assim, em cadáveres frescos e em pacientes portadoras de abdome volumoso submetidas a dermolipectomias abdominais, identificaram sempre a AESI. Nas preparaçöes anatômicas ficou constatado que a AESI tinha origem num tronco comum à artéria circunflexa ilíaca superficial, ramo da artéria femoral. A custa de radiografia contrastada, identificaram a rede anastomótica estabelecida pelas tributárias da AESI com: ramos da artéria epigástrica superior; ramos perfurantes do músculo oblíquo externo; ramos da epigástrica profunda inferior; ramos das artérias circunflexas ilíacas superficial e profunda. Através das dermolipectomias atestaram que a AESI cruza o ligamento inguinal e transita sobre a fascia superficialis. A constância desta distribuiçäo permite a obtençäo de longo retalho, de um dos lados do abdome, que vai desde a prega abdominal até a reborda costal. Este retalho, como já foi demonstrado por outros autores, tem amplo arco de rotaçäo. Dado seu eixo na AESI, os autores sugerem denominá-lo retalho epigástrico inferior e propöem aplicá-lo na reconstruçäo de pênis, no tratamento da extrofia de bexiga, na reparaçäo de grandes lábios e no tratamento das úlceras trocanterianas

Ureterocele ortotópica prolapsada: relato de um caso / Orthotopic prolapsed ureterocele

Os autores relatam um caso de paciente feminino com 30 anos de idade, com diagnóstico de ureterocele ortotópica prolapsada, manifestada clinicamente como tumoraçäo vulvar; discutem as várias modalidades de tratamento da uretrocele na literatura

Correçäo de fístula uretrocutânea através de interposiçäo de retalho desepitelizado de pele / Correction of urethrocutaneous fistula through the interposition of de-epithelized skin flap

Quinze crianças portadoras de fístula uretrocutânea pós-neouretroplastia foram submetidas a correçäo cirúrgica, através de interposiçäo de retalho de pele desepitelizado. Obteve-se cura em 15 de 17 fístulas operadas (88%); somente em 7 crianças utilizou-se derivaçäo urinária

Diagnóstico pré-natal e perinatal de anomalias congênitas do trato urinário e seu tratamento neonatal / Pre and perinatal diagnosis of congenital anomalies of the urinary tract and neonatal treatment

Apresentam-se 15 casos de malformaçöes congênitas do trato urinário com diagnóstico pré-natal através do exame ultra-sonográfico, a sua abordagem e tratamento perinatal

Correçäo cirúrgica de hipospádia médio-peniana, peniana proximal e peno-escrotal utilizando duplo retalho ilhado de prepúcio (double island flap) / Surgical repair of middle penile, proximal penile and penile-scrotal hypospadias using double island flap of prepuce

A correçäo cirúrgica de hipospádia pela técnica do duplo retalho cutâneo ilhado representa uma nova alternativa da correçäo em um só tempo de hipospádias médio-penianas, penianas proximais e peno-escrotais. Apresenta-se uma experiência inicial de treze casos, em que cinco pacientes tiveram a reparaçäo da hipospádica em apenas uma operaçäo. Até o momento, dez crianças tiveram a correçäo do seu defeito em uma ou duas operaçöes, tendo sido a segunda operaçäo, um procedimento simples. Três crianças aguardam serem operadas de fístula uretrocutânea. Trata-se de uma técnica convincente, em que o aproveitamento do excesso de pele dorsal para se fazer a neouretra e para recobrir o defeito cutâneo é feito utlizando-se tecidos virgens e bem vascularizados através de pedículo peniano dorsal superficial comum