Renal transplantation in augmented bladders.

Not many years ago, children with congenital abnormalities of the lower urinary tract or with bladder dysfunction were denied renal transplantation because they were considered very high-risk recipients. However, in the past few decades, we learned that in children with poorly compliant, low-capacity bladders, augmentation cystoplasty (AC) can create a compliant, low-pressure reservoir that helps preserve the kidney graft. Although the incidence of symptomatic urinary tract infection (UTI) may be greater in pediatric transplant recipients with an AC than in those without, UTI is related more to noncompliance with clean intermittent catheterization or vesicoureteral reflux to the native kidney or graft than to the AC itself, and usually does not lead to impairment of graft function. Today, children with a bladder reconstruction may undergo transplantation with the same outcome (graft survival and function) as those with normal bladders, although there is some possibility of malignant transformation in the intestinal segment used to augment the bladder in these patients.

Does bladder augmentation negatively affect renal transplant outcome in posterior urethral valve patients?

OBJECTIVE: Although renal transplant (RT) is a safe and effective treatment for end-stage renal disease, the outcome of RT has been mixed for posterior urethral valve (PUV) patients. In addition, some PUV patients need an augmentation cystoplasty (AC), which may negatively affect their RT outcome. The aim of this study is to compare RT outcome between PUV children with and without AC. MATERIALS AND METHODS: Between 1985 and 2012 a total of 309 children received 369 RTs at our institution. Among these patients, 36 were had classified as having PUV. Of these, 12 underwent an AC before RT (AC group) and 24 did not (controls). Data, including age at transplant, allograft source, urological complications, urinary tract infection (UTI) incidence, the presence of vesicoureteral reflux (VUR), and patient and graft survival, were compared between groups. RESULTS: Mean age at RT and mean follow-up were 7.6 versus 7.9 years and 8.9 versus 7.9 years in the AC group and in the control group, respectively (not significant [NS]). Allografts were from living donors in 50% of the AC group and in 41.6% of the controls (NS). The rate of UTI was 0.02 UTI/patient/year and 0.004 UTI/patient/year in the AC and control group, respectively (p = 0.001). Of the nine patients with UTI in the augmented group, five (55.5%) had VUR, while 5/8 (62.5%) patients in the control group with UTI had VUR. All patients with VUR in either group had UTIs previously. Of the five AC patients with more than three UTIs, two (40%) were non-compliant with clean intermittent catheterization (CIC), and UTI incidence was not associated with either a Mitrofanoff conduit or the urethra being used for CIC. Graft function at the end of study was 87.8 ± 40.5 ml/min/m(2) in the AC group and 88.17 ± 28.20 ml/min/m(2) in the control group (NS). The 10-year graft survival rate was 100% in AC group and 84.8% in controls. Two patients in the AC group lost their grafts (mean follow-up 13.3 ± 0.8 years) and five in the control group (mean follow-up 7.1 ± 4.7 years). CONCLUSIONS: Bladder augmentation does not negatively affect renal outcome in PUV patients undergoing transplantation. However, recurrent UTIs are more frequent in transplanted PUV patients with an AC than in those without AC, and they are generally related to non-compliance with CIC or the presence of VUR but, mostly, they will not result in impaired graft function.

Cambios en la vejiga después de varias modalidades de cobertura en el modelo de mielomeningocele inducido quirúrgicamente en corderos / Bladder Changes After Several Coverage Modalities in the Surgically Induced Model of Myelomeningocele in Lambs

Objetivo: Determinar las anomalías vesicales precoces en un modelo animal de mielomeningocele (MMC) con y sin corrección quirúrgica intraútero. Método: Creamos una lesión similar al MMC en 18 fetos de cordero entre los días 60 y 80 de gestación. Ocho de ellos no se repararon prenatalmente (grupo A), 3 se intervinieron mediante cierre abierto en 2 planos (grupo B), 3 se cerraron con pegamento biológico (grupo C ) y 4 por fetoscopia (grupo D). Al final de la gestación las vejigas se estudiaron macroscópica e histológicamente usando tinción de hematoxilina-eosina y tricrómico de Masson. Resultados: Cinco animales del grupo A (5/8, 62%), 2 en el grupo B (2/3, 66%), uno en el grupo C (1/3, 33%) y uno en el grupo D (1/4, 25%) sobrevivieron. Macroscópicamente las vejigas del grupo A estaban muy dilatadas y sus paredes eran muy finas. Microscópicamente mostraban una delgada capa de colágeno (capa azul [CA]) inmediatamente por debajo del urotelio; las capas musculares estaban muy adelgazadas. En el grupo no corregido también encontramos vejigas de baja acomodación, con paredes engrosadas y capacidad disminuida. El grupo B y el control mostraban preservación de las capas musculares y ausencia de CA. Los grupos C y D presentaban CA y preservación de las capas musculares. Conclusión: Los cambios vesicales en el modelo quirúrgico de MMC en corderos pueden describirse mediante datos histopatológicos. Ambos extremos del espectro pueden darse en dicho modelo. Estos cambios pueden prevenirse por completo mediante cirugía fetal abierta y parcialmente a través de otras formas de cobertura

Objective: To assess the presence of early bladder abnormalities in a prenatally corrected and uncorrected animal model of Myelomeningocele (MMC). Method: A MMC-like lesion was surgically created in 18 fetal lambs between the 60th and the 80th day of gestation. Eight of them did not undergo fetal repair (group A), three were repaired with an open two-layer closure (group B), three using BioGlue(R) (group C) and four fetoscopically (group D). At term, bladders were examined macroscopically and histopathological changes were assessed using H-E and Masson Trichrome. Results: Five animals in group A (5/8, 62%), two in group B (2/3, 66%), one in group C (1/3, 33%) and one in group D (1/4, 25%) survived. Macroscopically bladders in group A were severely dilated and showed thinner walls. Microscopically they showed a thin layer of colagenous tissue (Blue layer. BL) lying immediately subjacent to the urothelium. The muscular layers were thinner. Non compliant pattern with thick wall and low capacity was also found in the non corrected model. Group B and the control showed preservation of muscular layers and absence of BL. Groups C and D presented BL but also preservation of muscular layers. Conclusion: Bladder changes in a surgically induced model of MMC can be described using histopathological data. Both extremes of bladder changes can be observed in the model. These changes were completely prevented with open fetal surgery and partially with other coverage modalities

Bladder changes after several coverage modalities in the surgically induced model of myelomeningocele in lambs.

OBJECTIVE: To assess the presence of early bladder abnormalities in a prenatally corrected and uncorrected animal model of Myelomeningocele (MMC). METHOD: A MMC-like lesion was surgically created in 18 fetal lambs between the 60th and the 80th day of gestation. Eight of them did not undergo fetal repair (group A), three were repaired with an open two-layer closure (group B), three using BioGlue® (groupC) and four fetoscopically (group D). At term, bladders were examined macroscopically and histopathological changes were assessed using H-E and Masson Trichrome. RESULTS: Five animals in group A (5/8, 62%), two in group B (2/3, 66%), one in group C (1/3, 33%) and one in group D (1/4, 25%) survived. Macroscopically bladders in group A were severely dilated and showed thinner walls. Microscopically they showed a thin layer of colagenous tissue (Blue layer. BL) lying immediately subjacent to the urothelium. The muscular layers were thinner. Non compliant pattern with thick wall and low capacity was also found in the non corrected model. Group B and the control showed preservation of muscular layers and absence of BL. Groups C and D presented BL but also preservation of muscular layers. CONCLUSION: Bladder changes in a surgically-induced model of MMC can be described using histopathological data. Both extremes of bladder changes can be observed in the model. These changes were completely prevented with open fetal surgery and partially with other coverage modalities.

Long-term consequences of posterior urethral valves.

Posterior urethral valves (PUV) are the most common congenital cause of bladder outlet obstruction in infancy, and it is the effect of this obstruction on the bladder and the kidneys that will decide a patient's prognosis. With the improvements in diagnosis and treatments, what was previously a poor prognosis for boys with PUV has improved, and more patients will encounter the long-term sequelae of PUV during puberty and adulthood. In these patients the long-term prognosis in terms of renal and bladder function and fertility, as well as the risk of malignancy in those whose bladders were augmented with gastrointestinal segments, is still a matter of great concern and all of these topics will be discussed in this article.

Long-term bladder function, fertility and sexual function in patients with posterior urethral valves treated in infancy.

OBJECTIVE: To address the issue of sexual function and fertility in PUV patients. PATIENTS AND METHODS: Of 47 patients (age > 18 years) treated for PUV in infancy 28 were contactable. They were sent a standard questionnaire requesting details on voiding dysfunction symptoms, and experience of erection, orgasm and ejaculation. RESULTS: Of the 28, 16 (mean age 24 years) returned the questionnaire. Voiding frequency ranged from 3 to 10 times per day (mean = 5). Two patients had occasional mild diurnal incontinence but none had symptoms of overactivity. Three patients had a weak urinary stream. Renal function was normal in 9, 4 had a glomerular filtration rate <80 ml/min/1.73 m(2), and 3 had undergone renal transplant. Erections and orgasm were experienced by the 15 patients who responded to these questions, with 4 reporting mild or medium erectile dysfunction and 1, on dialysis, reporting slow ejaculation. Post-masturbation samples of semen and urine were collected from 6 patients. One had an alkaline pH, high percentage of immotile sperm and low sperm count. Another had a high concentration of abnormal forms, and seminal fluid was present in the urine of 3 patients. CONCLUSIONS: In long-term follow-up, 44% of PUV patients develop chronic renal failure or end-stage renal disease, but bladder dysfunction symptoms are infrequent. Sexual function is mostly normal. Total semen counts and motility are compatible with paternity in most patients.

Maldescenso testicular / Undescended testicle

El mal descenso testicular o, ausencia del testículo en la bolsa escrotal, es la anomalía más frecuente del tracto genitourinario, afectando al 3% delos niños recién nacidos. El descenso testicular se produce en dos etapas bien diferenciadas: en la primera (8-15 semanas de gestación) tiene lugar la migración intraabdominal del testículo; en la segunda etapa (25-35 semanas), el testículo migra hasta el escroto. Cualquier anomalía que interrumpa alguna de las etapas puede causar un mal descenso, por lo que la etiología continúa siendo desconocida. La importancia de esta anomalía estriba en su repercusión sobre la fertilidad, la mayor incidencia de tumores testiculares y en los posible factores psicológicos. Los pilares fundamentales para el diagnóstico de los testes palpables continúan siendo la anamnesis y la exploración física, siendo la laparoscopia la técnica de elección para el diagnóstico y tratamiento de los testes no palpables. En la actualidad, la orquidopexia entre los 6 a 12 meses de edad es el tratamiento de elección (por vía inguinal para los testes palpables y por laparoscopia para los testes no palpables). Aún está por determinar si la orquidopexia precoz mejora la fertilidad y disminuye el riesgo de cáncer (AU)

Cryptorchidism is the most common anomaly of the male genitalia, affecting 3% of male newborns. Testicular descent takes place in two distinct stages: during the first (between 8-15 weeks of gestation) the intra-abdominal migration of the testicle occurs; during the second stage (25-35 weeks), the testicle migrates to the scrotum. Any anomaly that interrupts any of these stages may cause a cryptorchidism, so the etiology remains unclear. The importance of this abnormality lies in its impact on fertility, increased incidence of testicular tumors and psychological sequelae. Medical history and physical examination are the fundamental pillars for the diagnosis of palpable testes; laparoscopy remains the golden standard for the diagnosis and treatment of non-palpable testes. Currently, orchidopexy at 6 to 12 months of age is the treatment o choice (via inguinal approach for palpable testes and laparoscopy for non-palpable testes). It has yet to be determined whether early orchidopexy improves fertility and lowers the risk of cancer (AU)

[Laparoscopic resection of the prostatic utricle in children]. / Resección laparoscópica del utrículo prostático en niños.

INTRODUCTION: The prostatic utricle is an embryological remnant from Müllerian duct tissue. Most prostatic utricles are asympomatic, but they may manifest as a urinary tract infection or an incontinence. MATERIAL AND METHOD: Surgery consists in a cysto-urethroscopy and cannulation of the prostatic utricle with a ureteral catheter (Fogarty ner 4) and the cystoscopy left in situ to facilitate identification and mobilization. A 10 mm port through a umbilical incision and two more 5 mm working ports were inserted. After mobilization, the ureteral defect was closed by an absorbable suture and ultrasonic coagulation. RESULTS: Five boys with symptomatic prostatic utricles underwent surgery at a mean (range) age of 7 (2-11) years. The laparoscopic excision was successful in all but one due to a bleeding. The mean (range) operative duration was 165 (120-240) min. CONCLUSION: Laparoscopic excision under cystoscopic guidance offers a good surgical view and allowing easy dissection.

Nefrectomía laparoscópica de donante vivo: Repercusión funcional en el receptor pediátrico / Laparoscopic living donor nephrectomy: Functional repercussion in the pediatric recipient

Objetivo. Analizar el impacto que la nefrectomía laparoscópica(NL) puede tener en la función renal y supervivencia del injerto en el receptor pediátrico. Material y métodos. Estudiamos retrospectivamente 63 receptores de injerto de donante vivo entre 1994 y 2007. Dieciséis pacientes obtuvieron el injerto por NL y 47 por nefrectomía abierta (NA). La edad media del receptor fue de 9,3 ± 5 años y la edad media del donante fue 40,8 ± 7 años. Estudiamos la incidencia del retraso en la función del injerto, el rechazo agudo y la función a medio plazo del injerto en ambos grupos. Resultados. Los datos demográficos del donante y del receptor y (.. )(AU)

Aim. The impact of laparoscopic (LDN) vs. open nephrectomy(ODN) on early graft function and survival in pediatric kidney recipient remains unclear. Material and method. We retrospectively review the records of63 pediatric recipient of living donor renal trasplant from 1994 to 2007.We compared those who recieved allograft recovered by LDN (n: 16)with those by ODN (n: 47). The mean recipient age was 9.3±5 years and the mean donor age was 40.8±7 years. Outcomes of interest included the incidence of delayer graft function, acute rejection and long-termgraft function. Results. Donor, recipient, demographic data and the total time of cold ischemia (1.9±0.3 hours) were similar in both groups. The mean of kidney warm ischemia was (195 LDN vs. 20 ODN seconds). There were not any significant differences between the two groups, not even in the patient survival after 36 months (100% LDN vs. 98% ODN), neither in (..) (AU)

Resección laparoscópica del utrículo prostático en niños / Laparoscopic resection of the prostatic utricle in children

Introducción. La mayor parte de los niños intervenidos de malformaciones anorectales (MAR) presentan algún tipo de disfunción intestinal. Un adecuado seguimiento de este aspecto marcará su calidad de vida. Material y métodos. Seleccionamos una muestra de 20 niños intervenidos de MAR mediante anorectoplastia sagital posterior (ARSP) y cuyo seguimiento no pudo realizarse adecuadamente. Tras contactar telefónicamente con ellos, son citados, entrevistados y explorados en nuestra consulta. Analizamos las variables relacionadas con la continencia y evaluamos la calidad de vida de los pacientes.Resultados. Reunimos 12 niñas y 8 niños. Rango de edad entre (..) (AU)

Introduction. The prostatic utricle is an embryological remnant from Müllerian duct tissue. Most prostatic utricles are a sympomatic ,but they may manifest as a urinary tract infection or an incontinence. Material and method. Surgery consists in a cysto-urethroscopy and can nulation of the prostatic utricle with a ureteral catheter (Fogartyner 4) and the cystoscopy left in situ to facilitate identification and mobilization. A 10 mm port through a umbilical incision and two more (..) (AU)

[Laparoscopic living donor nephrectomy: functional repercussion in the pediatric recipient]. / Nefrectomía laparoscópica de donante vivo: repercusión funcional en el receptor pediátrico.

AIM: The impact of laparoscopic (LDN) vs. open nephrectomy (ODN) on early graft function and survival in pediatric kidney recipient remains unclear. MATERIAL AND METHOD: We retrospectively review the records of 63 pediatric recipient of living donor renal trasplant from 1994 to 2007. We compared those who recieved allograft recovered by LDN (n: 16) with those by ODN (n: 47). The mean recipient age was 9.3 +/- 5 years and the mean donor age was 40.8 +/- 7 years. Outcomes of interest included the incidence of delayer graft function, acute rejection and long-term graft function. RESULTS: Donor, recipient, demographic data and the total time of cold ischemia (1.9 +/- 0.3 hours) were similar in both groups. The mean of kidney warm ischemia was (195 LDN vs. 20 ODN seconds). There were not any significant differences between the two groups, not even in the patient survival after 36 months (100% LDN vs. 98% ODN), neither in the graft survival with proportional hazards Cox analysis. The LDN group needed more days (9.56 +/- 2.3 vs. 4.72 +/- 0.57 ODN) to reach the minimum serum creatinine, but the GF (ml/min/1.73 m2) was similar at 6 months (122 +/- 12 LDN vs. 87 +/- 17 ODN), one year (139 +/- 45 LDN vs. 88 +/- 27 ODN), and two years (110 +/- 64 LDN vs. 82 +/- 30 ODN) after transplant. CONCLUSIONS: LDN delays the recovery of the graft function in pediatric recipient. Pediatric LDN recipient have graft outcomes comparable to those of ODN.

[Strategy for high cavoatrial tumor thrombus in children with nephroblastoma]. / Tratamiento del nefroblastoma con trombosis tumoral cavoatrial en niños.

INTRODUCTION: Wilms tumor surgery with intracaval-atrial extension is a challenge. Modern imaging techniques, precise preoperative thrombus location, and multidisciplinary surgical approach is mandatory. We aim to evaluate the outcome of our patients. PATIENTS AND METHODS: Between 1992 and 2005, 52 patients with nephroblastomas underwent surgery in our institution. Nine of them had renal-caval (RC) or cavo-atrial(CA) thrombus extension. Four patients presenting short RC intravascular extension were excluded. RESULTS: All cases were treated with pre-postoperative chemotherapy SIOP protocols. The level of the extension was retrohepatic in 2 cases, atrial in 3 patients and it even reached the ventricle in 2 of them. A multidisciplinary team was necessary to plan surgery and in all patients thrombus and tumor could be removed under cardiopulmonary by-pass in 3 cases (CPBP). One pulmonary tamponade due to thrombus migration (CAV) occurred and was solved by CPBP. Three cases were stage III, one stage IV and 1 stage II. Conclusions. Tumor size can be significantly reduced by preoperative chemotherapy. In case of CA extension, CPBP and right liver displacement to gain access to retro-hepatic cava are mandatory in order to reduce surgical complications.

Tratamiento del nefroblastoma con trombosis tumoral cavoatrial en niños / Strategy for high cavoatrial tumor thrombus in children with nephroblastoma

Introducción. El tratamiento quirúrgico del tumor de Wilms con extensión cavo-atrial es aún un reto para el cirujano. Es fundamental el empleo de las técnicas de imagen actuales, la localización preoperatoria exacta del trombo y un equipo quirúrgico multidisciplinar. El objetivo de nuestra revisión es evaluar el resultado en estos pacientes. Pacientes y métodos. Entre 1992 y 2005 se intervinieron en nuestro servicio 52 pacientes con nefroblastomas. Nueve de ellos tenían extensión tumoral cavo-renal o cavo-atrial. Excluimos en nuestro estudio a 4 pacientes con extensión extravascular mínima a vena renal. Resultados. Todos fueron tratados con quimioterapia pre y postoperatoria según el protocolo de la SIOP. La extensión intravascular afectaba vena cava en 2 casos, aurícula en 3 pacientes e incluso llegaba a ventrículo en 2 de ellos. En la planificación quirúrgica intervino un equipo multidisciplinar y se consiguió la extirpación del tumor y el trombo en todos, mediante circulación extracorpórea en 3 de ellos. En un paciente se produjo una embolia pulmonar por migración del trombo, que se resolvió mediante circulación extracorpórea. Tres casos fueron estadio III, 1 estadio IV y 1 estadio II. Conclusión. El tamaño tumoral puede reducirse significativamente mediante quimioterapia preoperatoria. En caso de extensión cavoatrial, la circulación extracorpórea y el desplazamiento del lóbulo hepático derecho que permita acceder a la cava retro-hepática, son necesarios para reducir las complicaciones quirúrgicas (AU)

Introduction. Wilms tumour surgery with intracavalatrial extension is a challenge. Modern imaging techniques, precise preoperative thrombus location, and multidisciplinary surgical approach is mandatory. We aim to evaluate the outcome of our patients. Patients and methods. Between 1992 and 2005, 52 patients with nephroblastomas underwent surgery in our institution. Nine of them hadrenal-caval (RC) or cavoatrial (CA) thrombus extension. Four patients presenting short RC intravascular extension were excluded. Results. All cases were treated with pre-postoperative chemotherapy SIOP protocols. The level of the extension was retrohepatic in 2 cases, atrial in 3 patients and it even reached the ventricle in 2of them. A multidisciplinary team was necessary to plan surgery and in all patients thrombus and tumour could be removed under cardiopulmonary by-pass in 3 cases (CPBP). One pulmonary tamponed due to thrombus migration (CAV) occurred and was solved by CPBP. Three cases were stage III, one stage IV and 1 stage II. Conclusions. Tumor size can be significantly reduced by preoperative chemotherapy. In case of CA extension, CPBP and right liver displacement to gain access to retro-hepatic cava are mandatory in order to reduce surgical complications (AU)

Sarcomas de partes blandas 1991-2004 / Soft tissue sarcomas (1991-2004)

: Introducción. El objetivo de este estudio es revisar los resultados del tratamiento de los sarcomas de partes blandas (SPB) en nuestro centro en los últimos trece años. Material y métodos. Tratamos 57 niños, 39 con rabdomiosarcomas (RMS) (67%) y 18 con otros tipos de sarcomas (33%). Dentro del primer grupo, excluimos 13 tumores orbitarios, 4 parameníngeos y 2 faciales tratados por otros servicios. Analizamos la edad, sexo, localización, histología, estadio inicial, tratamiento médico-quirúrgico y resultados. Evaluamos cada uno de estos datos como factor pronóstico mediante curvas de sobrevivencia actuarial y pruebas de log-rank. Resultados. La mediana de edad al diagnóstico fue de 2,3 años en los RMS (rango 6 m-16 a), y 10,9 años (0-16 a) en los otros SPB. La relación varón/mujer fue de 2,8:1 y 2:1 respectivamente. Doce RMS fueron urogenitales, 3 torácicos, 3 abdominales, 1 de extremidades y 1 cervical. Trece tumores (65%) eran embrionarios, 5 (25%) botrioides, 1 alveolar y otro fusiforme. Al diagnóstico, 74% se hallaban en estadio 1 o 2, y 26% en 3 o 4. En 5 niños (25 %) se realizó punción por aspiración de aguja fina (PAAF), que dio un diagnóstico impreciso o erróneo. En 12 (60%) se realizó biopsia antes de la cirugía definitiva y en 8 (40%) el diagnóstico se obtuvo tras analizar la pieza quirúrgica, aunque en 3 casos este diagnóstico inicial fue erróneo (neuroblastoma, tumor neuroectodérmico primitivo y leiomiosarcoma) y no se obtuvo el definitivo hasta la recidiva. Todos recibieron quimioterapia, el 60% antes de la extirpación quirúrgica, los restantes después. Sólo 2 pacientes (..) (AU)

Background.The aim of this study is to review the results of the treatment of soft tissue sarcomas (STS) in our Department during the last 13 years. Material and methods. Fifty-seven children (39 rhabdomyosarcomas (RMS) and 18 other types of sarcomas) have been treated. Nineteen RMS were excluded because they were treated by oher departments. The charts of 39 chidren were analysed evaluating several parameters (age, sex, location, histology, initial stage, clinical and surgical treatment and results) as prognostic factors using actuarial survival analyses and log-rank tests. Results. 1. RMS: Median age at diagnosis was 2.3 years (range 6 m- 16y).Twelve were genitourinary, 3 thoracic, 3 abdominal, 1 was located in limb and 1 in the neck. Histologically, 13 were embryonal, 5 botryoid, 1 alveolar and 1 fusiform. At diagnosis, 74% were in stages I or II. Fine needle aspiration biopsy (FNAB) was made in 5 children and the result was always imprecise or mistaken. Surgical biopsy was made before the definitive surgery in 12 cases. In the remaining 8 children the diagnosis was made only after surgical resection. With an mean follow-up of 70 ± 43 moths, 6 children died. The prognostic factors associated with poor outcomes were genitourinary (..) (AU)

[Soft tissue sarcomas (1991-2004)]. / Sarcomas de partes blandas 1991-2004.

BACKGROUND: The aim of this study is to review the results of the treatment of soft tissue sarcomas (STS) in our Department during the last 13 years. MATERIAL AND METHODS: Fifty-seven children (39 rhabdomyosarcomas (RMS) and 18 other types of sarcomas) have been treated. Nineteen RMS were excluded because they were treated by oher departments. The charts of 39 chidren were analysed evaluating several parameters (age, sex, location, histology, initial stage, clinical and surgical treatment and results) as prognostic factors using actuarial survival analyses and log-rank tests. RESULTS: 1. RMS: Median age at diagnosis was 2.3 years (range 6 m-16y). Twelve were genitourinary, 3 thoracic, 3 abdominal, 1 was located in limb and 1 in the neck. Histologically, 13 were embryonal, 5 botryoid, 1 alveolar and 1 fusiform. At diagnosis, 74% were in stages I or II. Fine needle aspiration biopsy (FNAB) was made in 5 children and the result was always imprecise or mistaken. Surgical biopsy was made before the definitive surgery in 12 cases. In the remaining 8 children the diagnosis was made only after surgical resection. With an mean follow-up of 70 +/- 43 moths, 6 children died. The prognostic factors associated with poor outcomes were genitourinary location, non radical excission, the presence of distant metastases at onset and alveolar histology. 2. Other sarcomas: Median age at diagnosis was 10.9 years (range 4 days-15 years). Among this group, there were 6 fibrosarcomas, 4 indifferentiated sarcomas, 3 synovial sarcomas, 2 abdominal desmoplastic small round cell tumours, 2 neurofibrosarcomas and 1 leiomyosarcoma. Only 9 received chemotherapy and one radiotherapy. All but one were operated. Five out ot the 19 died. CONCLUSIONS: Although the role of surgery is crucial, it is necessary to refine the initial histological diagnosis, because neither the PAAF or the biopsy have always been correct. The negative prognostic factors in our series were metastases present at diagnosis, genitourinary location and alveolar (RMS), desmoplastic or indifferenciated histology.

Can urodynamic studies predict bladder behaviour changes after artificial urinary sphincter implantation?

OBJECTIVE: To assess whether preoperative urodynamic studies can predict bladder behaviour changes that will necessitate bladder augmentation in patients with neuropathic incontinence after undergoing artificial urinary sphincter (AUS) implantation. PATIENTS AND METHODS: We analysed 17 patients, with a mean age of 14.4 years at AUS implantation, who did not undergo bladder augmentation either before or at implantation. All patients were followed at regular intervals before and after implantation. Urodynamic studies were done at least three times before implantation and yearly thereafter. Bladder capacity and compliance were evaluated preoperatively and during the follow up, in order to determine whether these urodynamic measurements could predict the eventual need for bladder augmentation. RESULTS: After a mean follow up of 7.5 years bladder behaviour was unchanged in 11 patients while six required augmentation. In these six patients, normal bladder capacity and compliance decreased from 67 to 39.6% of age expected capacity (AEC) (P=0.004) and from 31.6 to 13.5 ml/cm of water (P=0.001), respectively. Four of the six had no clinical symptoms to suggest the change in bladder behaviour. The differences in the average preoperative and follow up values for both normal bladder capacity (72.4 vs 83.5% of AEC) and compliance (30 vs 33.7 ml/cm of water) were not significant in the non-augmented patients (P=0.2 and P=0.3, respectively). Preoperative normal bladder capacity and compliance were 72% of AEC and 30 ml/cm of water, respectively, in the 11 non-augmented patients and 67% and 31 ml/cm of water in the six patients with augmentation. These differences between the two groups were not significant (P=0.06; P=0.1). CONCLUSION: Preoperative urodynamic studies do not predict bladder function outcome after AUS implantation. These patients require long-term careful observation to detect any asymptomatic change in detrusor behaviour.

Initial and long-term management of posterior urethral valves.

Posterior urethral valves are the most common cause of congenital obstructive uropathy, resulting in renal failure in childhood. Nowadays, in most cases, diagnosis is suggested by antenatal ultrasound. However, antenatal intervention has not resulted in a significantly improved outcome. Endoscopic valve ablation is the initial treatment in most of these neonates, but others procedures, like vesicostomy or ureterostomy, can also be justified in some particular cases in order to improve renal function prognosis. Different factors like bladder dysfunction, VUR, polyuria and proteinuria, can be responsible for the slow and progressive deterioration in renal function that some of these patients show over the years. By treating them all, we may prevent or delay the onset of end stage renal disease.

[Pyeloplasty follow-up. How and how long?]. / Seguimiento de la ureteropieloplastia: Cómo y cuánto tiempo?

INTRODUCTION: Some controversies still persist about the pueloplasty follow-up and this study aims to determine which test should be done in these patients during the follow-up and for how long these patients should be under control. PATIENTS AND METHODS: Forty six children who underwent to pueloplasty were retrospectively reviewed and those with contralateral renal abnormalities were excluded from study. Six children were lost to follow-up and were excluded. Of 40 p (25 M, 14 F) who fulfilled the requirements of this study, 28 were diagnosed prenatally and 28 had a left UPJ obstruction. All children were diagnosed by renal ultrasound, diuretic renography and excretory urogram (except 2). Mean age at Anderson Hynes pyeloplasty was 5.1 months and mean follow-up was 6.1 years. Renal ultrasound at 3 months and every year, and a diuretic renography at 1 year were performed in all children during follow-up. In some of them diuretic renography was repeated. RESULTS: At 3 months renal ultrasound demonstrated a significant hydronephrosis decrease in all children (p < 0.0001) but not in further controls. Differential renal function remained unchanged after pyeloplasty and during follow-up. However, there was a significant UPJ drainage improvement after pyeloplasty and during follow-up (p < 0.0001, p < 0.005). Any child needed a second pyeloplasty procedure and all of them showed a normal UPJ drainage in the antegrade ureterogram performed on the 5th postoperative day, except one. CONCLUSIONS: Differential renal function did not improve after pyeloplasty in our children. If renal ultrasound 3 months after pyeloplasty demonstrated a significant hydronephrosis decrease and at 1 year diuretic renography reveals a normal UPJ drainage any change should be expected in the long-term follow-up.