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.

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