[Concordant posterior urethral valves in male monochorionic twins with secondary prune belly syndrome]

Posterior urethral valves [PUVs]. The most common congenital cause of lower urinary tract obstruction, have been described to occur in identical and nonidentical twins. Until now, reports have been published on 15 cases of PUVs. We report a new case of concordant PUVs in one set of male monochorionic twins with secondary Prune Belly Syndrome. The twins were born by elective cesarean section at 38 weeks of gestation to a 36-year-old mother, gravida 6, para 6. On ultrasound perfomed at 18 weeks's gestation, both fetuses showed signs of PUVs. At birth, physical examination of both revealed a secondary Prune Belly Syndrome [PBS]. Postnatal renal ultrasound confirmed the diagnosis of PUV. The two infants underwent transurethral resection of the valves after a cystoscopic evaluation of the urethra. Since this procedure. their voiding has been unremarkable with stable renal function and sterile urine until their discharge. We have documented a rare association between VUP and PBS in two monochiorionic twins. More studies are needed to throw light on the significance of the present associated anomalies

Posterior urethral valves in childhoos: experience in a center with scarce facilities

Posterior urethral valves [PUV] are the most common congenital causes of lower urinary tract obstruction in male children, but few cases have been reported from Nigeria. In this study we describe our 7-year experience of management of PUV in children in order to increase the awareness of this condition in our environment. This is a report of 41 consecutive children with PUV who were managed at the Jos University Teaching Hospitals [JUTH], Jos, Nigeria, from June 2000 to April 2006. Their age at presentation ranged from 2 days to 15 years [mean: 2.5 years]. The relevant clinical, laboratory and radiological data were entered into a database and analyzed. Twenty-eight patients presented with a condition highly suspicious of PUV, while 7 patients presented with other urological conditions. Six patients presented with non-urological symptoms which caused a delay in diagnosis and institution of treatment. Voiding cystourethrography was diagnostic in all cases. In the majority of patients [n=31], management consisted of transurethral balloon avulsion of the valves yielding a satisfactory outcome in over 80% of the patients. Urethral stricture was the main complication [which responded to serial dilatation] and occurred in 3 patients. The postoperative mortality rate was 2.6%. Although this study was restricted to one hospital, there appears to be a high incidence of PUV in children in North Central Nigeria. An increased awareness of varied clinical features, a high index of suspicion and simple conservative treatment by balloon avulsion of PUV would improve the outcome

Congenital urethral anomalies in boys. Part II

In the second part of this article, congenital urethral anomalies other than posterior urethral valve were reviewed. The anomalies considered in the current review were anterior urethral valve, lacuna magna, syringocele, Cobb's collar, duplication of urethra, megalourethra, and prostatic urethral polyps. The literature was extensively reviewed concerning the presentations, diagnosis, different types of treatment modalities, morbidity, mortality, and new concepts for the above disorders. Anterior urethral valves or diverticula are the most prevalent congenital anomalies of anterior urethra. The lacuna magna is the largest depression in the dorsal aspect of the fossa navicularis. It is demonstrable on a well-performed voiding cystourethrography of the distal urethra. The dilated Cowper's gland duct is the other missed diagnosed anomaly of the urethra in boys. The congenital narrowing of the bulbar urethra with a variable clinical presentation and obstruction grade and different types of anterior urethral obstruction are the most common presentation of these anomalies. However, other symptoms or signs including, hematuria, bloody spotting on underwear, discomfort or sever pain in the vicinity of the glance, interrupted voiding, infection, bulging of anterior urethra, enuresis, and postvoiding dribbling are the only nonspecific manifestations of these disorders. All of these disorders are demonstrable on a well-performed voiding cystourethrography of the distal urethra. The urologist must be aware about these uncommon congenital anomalies and the anterior urethra should be carefully evaluated for such anomalies. Diagnosis of these entities is elusive unless the physician is looking for them. Nonspecific symptoms mentioned here besides radiographic findings can be a valuable clue for diagnosis

Neonatal infravesical obstruction in females

Neonatal infravesical obstruction in females is less common than in boys and rarely reported. In this article, we report on 3 cases of infravesical obstruction in female neonates with different etiologies. We also reviewed the literature on this topic. Including our cases, 17 cases of neonatal infravesical obstruction in girls have been reported. Neonatal infravesical obstruction in females has different underlying pathology compared to boys with congenital tumors being more common in girls. A high index of suspicion is essential for early recognition and management

Síndrome de Prunne-Belly / Prunne-Belly syndrome

El síndrome de Prunne-Belly se caracterizan por una tríada constituida por deficiencia congénita de la musculatura de la pared abdominal, anormalidades del tracto urinario y criptorquidia bilateral. A ella se agregan malformaciones musculoesqueléticas y viscerales. Se considera que el afecto de este síndrome reside en la placa intermedio/lateral mesodérmica o prominencia genital. Se comunica un caso de síndrome de Prunne-Belly en un neonato que sobrevivió 6 días. Clínicamente presentó abdomen distendido con piel arrugada, megacisto, fístula vesicocutánea con emisión de orina y pie equinovario bilateral. El bebé falleció por insuficiencia renal y respiratoria. En la necropsia se demostró la ausencia de musculatura en la pared abdominal, atresia de la unión vesicouretral, megacisto, dilatación quística de los uréteres, riñones con cambios quísticos y displásicos, criptorquidia bilateral, hipoplasia pulmonar, ciego móvil, pie equinovaro bilateral, pliegue simiano en la mano izquierda y fascies dismórfica con implantación baja de las orejas. Llamaron la atención los cambios coilocitóticos en el epitelio del glande. Fallecio por insuficiencia renal y respiratoria

Posterior urethral valves with congenital megalo-urethra.

A 3-year-old male child was admitted with complaints of dysuria and dribbling of urine since birth. There was a small plum sized swelling, on the ventral surface of penis, which increased in size during micturition and on compression of the swelling urine dribbled out. Voiding cysto-urethrogram showed posterior urethral valves with scaphoid megalo-urethra. Vesicostomy with fulguration of the valves and one year later reduction urethroplasty were performed.

Valvas de la uretra posterior (análisis de 35 casos) / Posterior urethra valves (analysis of 35 cases)

Se revisó el tratamiento efectuado en el Hospital de pediatría del Centro Médico Nacional de Occidente, I.M.S.S., Guadalajara, Jalisco, México, de 35 pacientes con valvas de uretra posterior. Los 35 pacientes se trataron dentro del periodo comprendido entre febrero de 1987 y abril de 1995. Para determinar la gravedad y el pronóstico de esta patología se utilizó el criterio por B. Churchill, que clasifica a estos casos en pacientes con pronóstico favorable cuando existen productos azoados normales, no hay reflujo vesicouretral o unilateral ni displasia, y con pronóstico desfavorable, pacientes con productos azoados elevados, reflujo vesicoureteral bilateral, ureterectasia grave y displasia renal. La edad de los pacientes varió entre el nacimiento y los 12 años, con promedio de 3.3 años. La principal manifestación fue la infección de vías urinarias en 85 por ciento de los pacientes, seguida por alteraciones en la micción en 6 por ciento, y retención aguda de orina en 3 por ciento. El diagnóstico se estableció con cistoureterograma muccional en 100 por ciento de los casos e informó acerca del reflujo. Ecosonograma, urograma y el gamagrama renal establecieron el estado de las vías urinarias superiores. Diez y nueve pacientes (54 por ciento tuvieron pronóstico favorable y su tratamiento consistió en resección primaria de valvas en 13 y vesicostomía en seis. Sólo dos pacientes requirieron reimplantación subsecuente, y los 19 pacientes se encontraron estables cuando se redactaba este informe. Tuvieron pronóstico desfavorable 16 pacientes (46 por ciento). De ellos 14 desarrollaron reflujo, ocho bilateral y seis unilateral. Seis pacientes tuvieron megauréter, ocho bilateral y seis unilateral. Seis pacientes tuvieron megauréter obstructivo y en dos de ellos fue bilateral. Este grupo se trató con derivación urinaria (14 pacientes) o resección primaria de valvas (dos pacientes). Estos pacientes requirieron varios procedimientos reconstructivos: reimplantación vesicoureteral cinco, transureteroureteroanastomosis dos, transureteroureteroanastomosis más aumento vesical dos, y nefroureterectomía de riñones unilaterales no funcionales los últimos siete pacientes (20 por ciento). De estos 16 pacientes, ocho se encuentran en insuficiencia renal crónica progresiva y, probablemente, requerirán transplante renal a largo plazo. Cabe concluir que las valvas de uretra posterior son un defecto congénito con una forma de presentación clínica. En los casos leves o moderados...

Infecçäo urinária na criança: aspectos urológicos / Primary tract infection in children

O trabalho analisa as implicaçöes urológicas da infecçäo urinária na criança, estudando, em particular, a hidronefrose e obstruçäo da junçäo uretro-piélica; o refluxo víseico-reteral; e as válvulas de uretra posterior. Em cada um desses capítulos säo revistos a importância do problema, o quadro clínico, exames complementares indicados para seu diagnóstico e o tratamento, especialmente quanto aos procedimentos cirúrgicos, seguimento pós-operatório e prevençäo das possíveis complicaçöes

Válvula da uretra posterior em adultos / Posterior urethra valvula in adults

Os autores descrevem três casos de válvula congênita da uretra posterior em adultos, com sintomatologias diversas e variadas. Em dois dos pacientes apresentados, a fertilidade estava presente, pois eram pais. Aspectos sobre o diagnóstico e tratamento säo considerados, dada a divergência dos autores, quando ao modo de observar a válvula e o colo vesical, quando hipertrofiado