Pyloric atresia and Down's syndrome: prenatal double bubble false sign. / Atresia pilórica y síndrome de Down: falso signo de la doble burbuja prenatal.

Pyloric atresia is a rare malformation, with an incidence of 1:100,000 live newborns. Male to female ratio is 1/1. Typically, it is an isolated malformation, with a good prognosis, but 20-40% of cases present epidermolysis bullosa, and to a lesser extent, multiple intestinal atresias. We present the case of a pre-term newborn prenatally diagnosed with polyhydramnios, duodenal atresia with "double bubble" sign, and suspected Down's syndrome, who eventually had pyloric atresia.

La atresia pilórica es una malformación rara, presenta una incidencia de 1:100.000 recién nacidos vivos y la ratio hombre/mujer es de 1/1. Generalmente es una malformación aislada, con buen pronóstico, pero entre el 20-40% de los casos se asocia a epidermólisis bullosa y en menor frecuencia a otras atresias intestinales múltiples. Presentamos un caso de recién nacido pretérmino con atresia pilórica con el diagnóstico prenatal de polihidramnios, atresia duodenal con signo de 'doble burbuja' y sospecha de síndrome de Down.

Atresia pilórica y síndrome de Down: falso signo de la doble burbuja prenatal / Pyloric atresia and Down’s syndrome: prenatal double bubble false sign

La atresia pilórica es una malformación rara, presenta una incidenciade 1:100.000 recién nacidos vivos y la ratio hombre/mujer es de 1/1.Generalmente es una malformación aislada, con buen pronóstico, peroentre el 20-40% de los casos se asocia a epidermólisis bullosa y enmenor frecuencia a otras atresias intestinales múltiples.Presentamos un caso de recién nacido pretérmino con atresia piló-rica con el diagnóstico prenatal de polihidramnios, atresia duodenal consigno de ‘doble burbuja’ y sospecha de síndrome de Down.(AU)

Objective. Pyloric atresia is a rare malformation, with an incidenceof 1:100,000 live newborns. Male to female ratio is 1/1. Typically, itis an isolated malformation, with a good prognosis, but 20-40% ofcases present epidermolysis bullosa, and to a lesser extent, multipleintestinal atresias.We present the case of a pre-term newborn prenatally diagnosedwith polyhydramnios, duodenal atresia with “double bubble” sign, andsuspected Down’s syndrome, who eventually had pyloric atresia.(AU)

[Videothorascocopic: approach in thoracic inlet of neurogenic mediastinal tumor in pediatric age]. / Videotoracoscopia asistida: abordaje de tumor neurogénico en la encrucijada cervicotorácica en la edad pediátrica.

We present a 4 year-old patient with precedent of pneumonia and showing an increase of X-ray density in the superior mediastinal. After a year of follow-up and the presence of irritating cough, it is decided to perform studies of image (CT), being diagnosed of a mass in the region of the thoracic inlet which affects up to D4. Video-assisted thoracic surgery is decided and the mass is entirely removed, being the result of the pathological study to ganglioneuroma. Eight months later from, the patient is asymptomatic and without relapse according to the image methods which were carried out.

Videotoracoscopia asistida: abordaje de tumor neurogénico en la encrucijada cervicotorácica en la edad pediátrica / Videothorascocopic: approach in thoracic inlet of neurogenic mediastinal tumor in pediatric age

Presentamos un paciente de 4 años de edad con antecedente de neumonía con el diagnóstico, por radiología convencional, de un aumento de densidad del mediastino superior. Tras un año de seguimiento y aparición de tos irritativa se decide realizar estudios de imagen, diagnosticándose mediante TAC un tumor en la región de la encrucijada cervicotorácica que afecta hasta D4.Se decide realizar planteamiento quirúrgico mediante videotoracoscopia asistida. El tumor es extirpado en su totalidad, siendo el diagnóstico anatomo-patológico de ganglio neuroma. El paciente a los 8meses de la intervención se encuentra asintomático y sin recidiva según los métodos de imagen realizados (AU)

We present a 4 year-old patient with precedent of pneumonia and showing an increase of X-ray density in the superior mediastinal. After a year of follow-up and the presence of irritating cough, it is decided to perform studies of image (CT), being diagnosed of a mass in the region of the thoracic inlet which affects up to D4.Video-assisted thoracic surgery is decided and the mass is entirely removed, being the result of the pathological study to ganglioneuroma. Eight months later from, the patient is asymptomatic and without relapse according to the image methods which were carried out (AU)

[New concepts in the natural history of multicystic dysplastic kidney]. / Nuevos conceptos en la historia natural de la displasia renal multiquística.

OBJECTIVE: Retroperitoneoscopy has shown that US involution is not synonymous with complete regression of the dysplastic renal parenchyma in US-involuted multicystic dysplastic kidney (MCDK). The objective is to analyze the meaning of this results. PATIENTS AND METHODS: 16 patients (nine girls and seven boys) with unilateral MCDK that showed complete involution on ultrasonography, underwent prospectively a retroperitoneoscopic approach. US showed complete cysts involution at the mean age of 10 months (ranging from five to 22 months). All patients underwent a retroperitoneoscopic approach after US involution of the MCDK. The mean age of retroperitoneoscopy was 36 months (ranging from eight to 56 months). RESULTS: The retroperitoneoscopic approach revealed persistence of dysplastic renal tissue in 100% of the patients. The mean lenght of the renal renmant was 2 cm (ranging from 1 and 3.5 cm). All patients had a mean length of stay of less than 24 hours. Anatomo-pathological study of the samples showed a wide spectrum of dysplastic renal tissue and the absence of preneoplastic cells. CONCLUSIONS: Ultrasonography is our method of choice to follow up MCDK until cyst involution takes place. The presence of a dysplastic renal remnant which is not visible on US, requires an appropriate long-term follow up to screen for the growth of tumors. In our experience, retroperitoneoscopy allows the diagnosis and treatment of the displastic renal renmant in the same minimally invasive ambulatory procedure, avoiding long-term development of tumors. Overall, it is our responsibility to sufficiently inform to the family about the persistence of dysplastic renal remnant to facilitate their decision about the best treatment for their child.

Nuevos conceptos en la historia natural de la displasia renal multiquística / New concepts in the natural history of multicystic dysplastic kidney

Objetivo. La retroperitoneoscopia ha demostrado que en la DRM involucionada ecográficamente, los quistes desaparecen pero el tejido renal displásico permanece. El objetivo de este trabajo es analizar la repercusión que estos hallazgos tienen sobre el tratamiento y seguimiento a largo plazo de los niños portadores de DRM involucionada. Material y métodos. Los hallazgos están basados en el estudio prospectivo de 16 pacientes, 9 niñas y 7 niños, portadores de DRM unilateral, 11 de afectación izquierda y 5 derecha, que habían sufrido completa involución de la DRM en el estudio ecográfico seriado. La edad media de desaparición de los quistes renales fue de 10 meses (rango entre 5 y 22 meses). En todos los pacientes se realizó un abordaje retroperitoneoscópico para confirmar la persistencia o no de un remanente displásico renal. La edad media del abordaje retroperitoneoscópico fue de 36 meses (rango entre 8 y 56 meses). Resultados. La retroperitoneoscopia detectó persistencia de tejido renal displásico en el 100% de los casos. El tamaño medio del remanente renal fue de 2 cm (rango entre 1 y 3,5 cm). En todos los casos se realizó la extirpación del remanente renal displásico (nefrectomía retroperitoneoscópica). Los hallazgos anatomopatológicos en todas las muestras confirmaron la existencia de displasia renal. La estancia media hospitalaria fue inferior a 24 horas en todos los casos. Todos los niños fueron dados de alta definitiva al mes postoperatorio, no siendo necesario el seguimiento a largo plazo. Conclusiones. La ecografía no es útil en el seguimiento de la DRM involucionada. La persistencia del remanente displásico no visible en la ecografía obliga al despistaje de tumores a largo plazo mediante ecografías seriadas frecuentes. Por ello, cuando los quistes desaparecen, consideramos a la retroperitoneoscopia el método de elección, mínimamente invasivo, para el diagnóstico del remanente displásico renal en la DRM involucionada, que permite su tratamiento en el mismo acto quirúrgico sin alargar la estancia hospitalaria. Este abordaje evita la necesidad de seguimiento a largo plazo para el despistaje de tumores, reduciendo la ansiedad de la familia y la necesidad de revisión del niño por el especialista (AU)

OBJECTIVE: Retroperitoneoscopy has shown that US involution is not synonymous with complete regression of the dysplastic renal parenchyma in US-involuted multicystic dysplastic kidney (MCDK).The objective is to analyze the meaning of this results. PATIENTS AND METHODS: 16 patients (nine girls and seven boys) with unilateral MCDK that showed complete involution on ultrasonography, underwent prospectively a retroperitoneoscopic approach. US showed complete cysts involution at the mean age of 10 months (ranging from five to 22 months). All patients underwent a retroperitoneoscopic approach after US involution of the MCDK. The mean age of retroperitoneoscopy was 36 months (ranging from eight to 56 months). RESULTS: The retroperitoneoscopic approach revealed persistence of dysplastic renal tissue in 100% of the patients. The mean lenght of the renal renmant was 2 cm (ranging from 1 and 3.5 cm). All patients had a mean length of stay of less than 24 hours. Anatomo-pathological study of the samples showed a wide spectrum of dysplastic renal tissue and the absence of preneoplastic cells. CONCLUSIONS: Ultrasonography is our method of choice to follow up MCDK until cyst involution takes place. The presence of a dysplastic renal remnant which is not visible on US, requires an appropriate long-term follow up to screen for the growth of tumors. In our experience, retroperitoneoscopy allows the diagnosis and treatment of the displastic renal renmant in the same minimally invasive ambulatory procedure, avoiding long-term development of tumors. Overall, it is our responsibility to sufficiently inform to the family about the persistence of dysplastic renal remnant to facilitate their decision about the best treatment for their child (AU)

Does the multicystic dysplastic kidney really involute? The role of the retroperitoneoscopic approach.

OBJECTIVE: To assess the role of video-assisted retroperitoneoscopy in the follow up of multicystic dysplastic kidney (MCDK) that has involuted--disappeared?--on serial renal ultrasonography (US). PATIENTS AND METHODS: Prospectively, we performed a retroperitoneoscopy in 14 patients, nine girls and five boys, with unilateral MCDK that had involuted on serial US. MCDK was diagnosed in utero (80%) and confirmed postnatally by US and Tc99m dimercaptosuccinic acid radionuclide scan. Follow up US examinations were performed at 1 month, 5 months and 12 months in the first year of life and every 6 months from then on. US showed complete involution at a mean age of 13 months (range 5-18 months). Retroperitoneoscopy was then indicated, at a mean age of 23 months (range 8-24 months), to confirm the disappearance of the kidney dysplastic remnant. RESULTS: Retroperitoneoscopy detected persistence of anomalous kidney tissue in 100% of cases. The mean length of the renal remnant was 2 cm (range 1-3.5 cm). Two cases showed a pelvic ectopic location that was not detected by US before involution. The remnant was removed during the same procedure. Anatomo-pathological findings were found to be compatible with dysplastic renal tissue. There were no intra- or postoperative complications. All patients had a mean length of stay of less than 24h. CONCLUSIONS: Complete resolution on US does not mean disappearance of MCDK, as US does not detect renal dysplastic remnants after cyst involution has occurred. The retroperitoneoscopic approach to the renal and pelvic area is a minimally invasive, safe and effective procedure to diagnose and treat the renal dysplastic remnant in US-involuted MCDK.

[Retroperitoneoscopic nephrectomy: our experience in short-term hospital stay]. / Nefrectomía retroperitoneoscópica. Nuestra experiencia con ingreso en hospital de corta estancia.

OBJECTIVE: To prove that the nephrectomy, in spite of being a major surgery, requires less than 48 hours stay hospital if it is performed by retro peritoneal approach. Consequently this procedure can be carry out in a short hospital stay. MATERIAL AND METHODS: From 1995, we have treat 40 patients by retroperitoneoscopic nephrectomy, 46 nephrectomies in total, being bilateral in 6 patients because of terminal renal failure (TRF), who do not take part in that study. The average age was 4.8 years range old (with a rank between 6 months and 13 years old), in relation with sex, male (n=16) and female (n=18). As for the side of the nephrectomy, it was the right in 20 cases and left in 14. Those pathologies, which indicated the nephrectomy because of kidney's functional annulations, were: obstructive hydronephrosis (n=2); obstructive megaureter (n=1); kidney destroyed by reflux (n=8); multicystic displasia (n=11); involution multicystic diyplasia (n=12). RESULTS: In all the cases, the nephrectomy was pure retroperitoneal approach. The average time of surgical duration was of 92 minutes (with a range between 60-240 minutes). It was reconversion in 1 case (4.5%) by peritoneal micro perforation (n=1). We underline absence of inherent complications to retroperitoneoscopy, intraoperative and postoperative procedures. As secondary complications to the access surgical procedure were 2 wall relaxations because of incisional injury and one serum collection of the first port site. The bleeding during the operative act was imperceptible, less than 20 cc. Drainages were not left in the operative bed. The average stay has been reduced to 27 hours, without being counted the 6 cases of TRF. In the last 18 cases the hospitality discharge was made in the first 24 h postoperative. The analgesic established by protocol was analgesic of infiltration of ports site with 1% bupivacaine and one dose of paracetamol/ibuprofen, given 12h after the intervention and repeated after hospitality discharge. Essumpcion of their day home-life and school- was earlier in 100% of cases. We underline the absence of secondary complications to pain (breathing) neither to immobilization. CONCLUSIONS: The retroperitoneoscopic nephectomy is a clearly established indication and of required fulfilment in paediatric age. It is the suitable surgical boarding to perform a nephrectomy because of its reliability, reproductibile and safety. Media hospital stayis lesser when the nephrectomy was performed by retro peritoneal approach than conventional surgery. Consequently this procedure can be carry out in a short hospital stay.

Nefrectomía retroperitoneoscópica. Nuestra experiencia con ingreso en Hospital de Corta Estancia / Retroperitoneoscopic nephrectomy: our experience in sort hospital stay

Objetivos. Demostrar que la nefrectomía, a pesar de ser un procedimiento de cirugía mayor, requiere una estancia hospitalaria menor de 48 horas, siempre que se realice mediante abordaje retroperitoneal laparoscópico. Consecuentemente este procedimiento puede ser realizado en el Hospital de Corta Estancia. Material y métodos. Desde 1995 hemos tratado 40 pacientes mediante nefrectomía retroperitoneoscópica con un total de 46 nefrectomías. En 6 pacientes fueron bilaterales por insuficiencia renal terminal (IRT), y por lo tanto no están reflejadas en este estudio. La edad media fue de 4,8 años (rango entre 6 meses y 13 años), con relación al sexo: varones (n=16) y hembras (n=18). En cuanto al lado de la nefrectomía fue derecho en 20 e izquierdo en 14. Las patologías que indicaron nefrectomía(n=34) por anulación funcional del riñón fueron: hidronefrosis obstructiva (n=2); megauréter obstructivo (n=1); nefropatía por reflujo vesicoureteral (n=8); displasia renal multiquística (n=11); riñón multiquístico desaparecido (n=12). Resultados. En todos los casos la nefrectomía fue retroperitoneal pura. El tiempo de duración quirúrgico medio fue de 92 min (rango de 60- 240 min). Se reconvirtió en 1 caso (4,5%) por microperforación peritoneal (n=1). Destacamos la ausencia de complicaciones inherentes al procedimiento retroperitoneoscópico intraoperatorio y postoperatorio. Como complicaciones secundarias al procedimiento quirúrgico se presentaron dos relajaciones de pared en herida incisional y un seroma de puerto de entrada. El sangrado durante el acto operatorio fue inapreciable, menor de 20 cc. No se dejaron drenajes en el lecho operatorio. La estancia media ha sido reducida a 27 horas, no siendo contabilizados los 6 casos de IRT. En los 18 últimos casos, el alta hospitalaria fue dada en las primeras 24 horas postoperatorias. La analgesia establecida por protocolo ha sido infiltración de puertos con bupivacaína 1% y una dosis de paracetamol/ibuprofeno a las 12 horas de la intervención, repetida al alta hospitalaria. La incorporación a su vida cotidiana –domicilio y escuela– ha sido precoz en el 100% de los casos. Destacamos la ausencia de complicaciones secundarias al dolor (respiratorias) y a la inmovilización. Conclusiones. La nefrectomía retroperitoneoscópica es una indicación claramente establecida y de obligada realización en la edad pediátrica. Es el abordaje quirúrgico idóneo para realizar una nefrectomía por su fiabilidad, reproducibilidad y seguridad. La estancia media se ha reducido con respecto a la cirugía convencional pudiéndose realizar en Hospital de Corta Estancia (AU)

Objective. To prove that the nephrectomy, in spite of being a major surgery, requires less than 48 hours stay hospital if it is performed by retro peritoneal approach. Consequently this procedure can be carry out in a short hospital stay. Material and methods. From 1995, we have treat 40 patients by retroperitoneoscopic nephrectomy, 46 nephrectomies in total, being bilateral in 6 patients because of terminal renal failure (TRF), who do not take part in that study. The average age was 4.8 years range old (with a rank between 6 months and 13 years old), in relation with sex, male (n=16) and female (n=18). As for the side of the nephrectomy, it was the right in 20 cases and left in 14. Those pathologies, which indicated the nephrectomy because of kidney’s functional annulations, were: obstructive hydronephrosis (n=2); obstructive megaureter (n=1); kidney destroyed by reflux (n=8); multicystic displasia (n=11); involution multicystic diyplasia (n=12). Results. In all the cases, the nephrectomy was pure retroperitoneal approach. The average time of surgical duration was of 92 minutes (with a range between 60-240 minutes). It was reconversion in 1 case (4.5%) by peritoneal micro perforation (n=1). We underline absence of inherent complications to retroperitoneoscopy, intraoperative and postoperative procedures. As secondary complications to the access surgical procedure were 2 wall relaxations because of incisional injury and one serum collection of the first port site. The bleeding during the operative act was imperceptible, less than 20 cc. Drainages were not left in the operative bed. The average stay has been reduced to 27 hours, without being counted the 6 cases of TRF. In the last 18 cases the hospitality discharge was made in the first 24 h postoperative. The analgesic established by protocol was analgesic of infiltration of ports site with 1% bupivacaine and one dose of paracetamol/ibuprofen, given 12h after the intervention and repeated after hospitality discharge. Essumpcion of their day home-life and school- was earlier in 100% of cases. We underline the absence of secondary complications to pain (breathing) neither to immobilization. Conclusions. The retroperitoneoscopic nephectomy is a clearly established indication and of required fulfilment in paediatric age. It is the suitable surgical boarding to perform a nephrectomy because of its reliability, reproductibile and safety. Media hospital stay is lesser when the nephrectomy was performed by retro peritoneal approach than conventional surgery. Consequently this procedure can be carry out in a short hospital stay Objective. To prove that the nephrectomy, in spite of being a major surgery, requires less than 48 hours stay hospital if it is performed by retro peritoneal approach. Consequently this procedure can be carry out in a short hospital stay. Material and methods. From 1995, we have treat 40 patients by retroperitoneoscopic nephrectomy, 46 nephrectomies in total, being bilateral in 6 patients because of terminal renal failure (TRF), who do not take part in that study. The average age was 4.8 years range old (with a rank between 6 months and 13 years old), in relation with sex, male (n=16) and female (n=18). As for the side of the nephrectomy, it was the right in 20 cases and left in 14. Those pathologies, which indicated the nephrectomy because of kidney’s functional annulations, were: obstructive hydronephrosis (n=2); obstructive megaureter (n=1); kidney destroyed by reflux (n=8); multicystic displasia (n=11); involution multicystic diyplasia (n=12). Results. In all the cases, the nephrectomy was pure retroperitoneal approach. The average time of surgical duration was of 92 minutes (with a range between 60-240 minutes). It was reconversion in 1 case (4.5%) by peritoneal micro perforation (n=1). We underline absence of inherent complications to retroperitoneoscopy, intraoperative and postoperative procedures. As secondary complications to the access surgical procedure were 2 wall relaxations because of incisional injury and one serum collection of the first port site. The bleeding during the operative act was imperceptible, less than 20 cc. Drainages were not left in the operative bed. The average stay has been reduced to 27 hours, without being counted the 6 cases of TRF. In the last 18 cases the hospitality discharge was made in the first 24 h postoperative. The analgesic established by protocol was analgesic of infiltration of ports site with 1% bupivacaine and one dose of paracetamol/ibuprofen, given 12h after the intervention and repeated after hospitality discharge. Essumpcion of their day home-life and school- was earlier in 100% of cases. We underline the absence of secondary complications to pain (breathing) neither to immobilization. Conclusions. The retroperitoneoscopic nephectomy is a clearly established indication and of required fulfilment in paediatric age. It is the suitable surgical boarding to perform a nephrectomy because of its reliability, reproductibile and safety. Media hospital stay is lesser when the nephrectomy was performed by retro peritoneal approach than conventionalsurgery. Consequently this procedure can be carry out in a short hospital stay (AU)

[Use of oral anticholinergic therapy in children under 1 years of age with high risk bladder]. / Utilización de anticolinérgicos orales en menores de 1 año portadores de vejiga de alto riesgo.

THE AIM: To assess the effectiveness and safety of the treatment with oral anticholinergic agents (Oxybutin clorure) in patients under 1 year old, and who aree carriers of high risk bladder secondary to neurological illness as well as no neurological one. MATERIAL AND METHODS: Since 1989, we have indicated treatment with anticholinergic agents to 16 patients: 9 patients had neurogenic bladder secondary to: myelomeningocele (n=7) and sacrocoxigeal teratoma (n=2). Others 7 patients had non neurogenic bladder secondary to: posterior urethral valvulas (n=1), valvula-like syndrome (n=4), post-surgery of neonatal giant bladder diverticulum (n=1) and Prune-Belly syndrome (n=1). The urodinamic study was performed during the first six months of life, being "high risk bladder" defined according to the parameters of compliance vesical and pressure of leak at point (PER). Five of the patients showed neonatal cronic renal failure (CRF), who were treated by cutaneus temporary derivation. All patients at treatment with anticholinergic agents at a 0.2 mg/kg/day dose was established; other early adjunctive treatment prior to the closure of the urinary derivation in children with CRF(n=5); or as a part of the conservative treatment (n=3), alone or associate to intermittent bladder catheterization (IBC) (n=8). During the treatment with anticholinergic agents, the cardiac frequency was controlled by EKG registration in 6 patients, being the rest of the children clinicaly controled (skin colour, mouth dryness, cardiac frequency and intestinal function). RESULTS: In all the cases, the minimum duration of the treatment was one year, until the functional stabilization of the urinary tract. It is to underline the absence of secondary complications which would have caused the suspension or the reduction of the treatment at long term. Conventional studies of urologicals image and urodinamic studies, showed the stabilization of the urinary tract and also the preservation of kidney function and not only was demonstrated in those patients with oral anticholinergic agent as their unique treatment, but also in those patients who had previously been derivated. The previous vesical stabilization allowed the optimum result of subsequent corrective surgeries in five patients (ureteroneocystostomy and vesiscotomy clousure. In cases with previous derivation, the treatment with oral anticholinergic agents made easier vesicostomic clousure in the forth quarter of the first year old wih the maintenance of the vesical stability. CONCLUSIONS: The use of oral anticholinergic agents at a dose of 0.2 mg/kg/day, has resulted to be safe as well as effective in the treatment of high risk bladder in infancy those with less than one year of life. In those patients with serious dysplasia by reflux, it contributes to the preservation of kidney function, it makes easier the early desderivation and it also stabilizes in a functional way the bladder as a previous step to subsequent reconstructive surgeries.

Utilización de anticolinérgicos orales en menores de 1 año portadores de vejiga de alto riesgo / Use os oral anticholinergic therapy in children under 1 years of age with high risk bladder

Evaluar la eficacia y seguridad del tratamiento con anticolinérgicos orales (cloruro de oxibutinina) en pacientes menores de 1 año de edad, portadores de vejiga de alto riesgo secundaria a enfermedad neurológica y no neurológica. Material y Métodos. Desde 1989 hemos indicado tratamiento con anticolinérgicos en 16 pacientes: 9 con vejiga neurógena secundaria a mielomeningocele (n=7) y teratoma sacrococxígeo (n=2), y 7 con vejiga no neurogénica secundaria a válvulas de uretra posterior (n=1), micción no coordinada fetal (n=4), poscirugía de divertículo vesical gigante neonatal (n=1) y síndrome de Prune-Belly (n=1). El estudio urodinámico se realizó durante el primer semestre de vida, definiéndose "vejiga de alto riesgo" según los parámetros de compliance vesical y presión de escape en reposo (PER). Cinco de los pacientes presentaron insuficiencia renal (IR) neonatal que fueron tratados mediante derivación urinaria temporal. En todos los pacientes se instauró tratamiento con anticolinérgicos a dosis de 0,2 mg/kg/día, bien como tratamiento coadyuvante previo al cierre de la derivación urinaria en los niños con IR (n=5), o bien como parte del tratamiento conservador (n=11), sólo o asociado a cateterización vesical intermitente (CVI) (n=8). Durante el tratamiento con los anticolinérgicos se monitorizó la frecuencia cardíaca mediante registro electrocardiográfico en 6 pacientes siendo el resto de los niños controlados clínicamente (coloración de la piel, sequedad de boca, frecuencia cardíaca y función intestinal). Resultados. En todos los casos, la duración mínima del tratamiento fue de una año, hasta la estabilización funcional del tracto urinario. Es de destacar la ausencia de complicaciones secundarias al uso de anticolinérgicos orales que hayan provocado la suspensión o reducción del tratamiento a largo plazo. Los estudios convencionales de imagen urológicos así como los estudios urodinámicos, han demostrado estabilización del tracto urinario y preservación de la función renal tanto en los pacientes en los que instauró el anticolinérgico oral como único tratamiento, como en los pacientes que previamente habían derivado. En 5 pacientes la estabilización vesical previa ha favorecido el resultado óptimo de cirugías correctoras posteriores (ureteroneocistostomía y cierre de vesicostomía). En los casos con derivación previa, el tratamiento con anticolinérgicos orales facilitó la desderivación precoz en el cuarto trimestre de vida con mantenimiento de la estabilidad vesical tras el cierre de la derivación. Conclusiones. Los anticolinérgicos orales a dosis de 0,2 mg/kg/día han resultado seguros y eficaces en el tratamiento de la vejiga de alto riesgo en el lactante menor de un año de vida. En los lactantes con nefropatía grave, contribuyen a la preservación de la función renal, facilitan la desderivación precoz y estabilizan funcionalmente la vejiga como paso previo a cirugías reconstructivas posteriores (AU)

THE AIM: To assess the effectiveness and safety of the treatment with oral anticholinergic agents (Oxybutin clorure) in patients under 1 year old, and who aree carriers of high risk bladder secondary to neurological illness as well as no neurological one. MATERIAL AND METHODS: Since 1989, we have indicated treatment with anticholinergic agents to 16 patients: 9 patients had neurogenic bladder secondary to: myelomeningocele (n=7) and sacrocoxigeal teratoma (n=2). Others 7 patients had non neurogenic bladder secondary to: posterior urethral valvulas (n=1), valvula-like syndrome (n=4), post-surgery of neonatal giant bladder diverticulum (n=1) and Prune-Belly syndrome (n=1). The urodinamic study was performed during the first six months of life, being "high risk bladder" defined according to the parameters of compliance vesical and pressure of leak at point (PER). Five of the patients showed neonatal cronic renal failure (CRF), who were treated by cutaneus temporary derivation. All patients at treatment with anticholinergic agents at a 0.2 mg/kg/day dose was established; other early adjunctive treatment prior to the closure of the urinary derivation in children with CRF(n=5); or as a part of the conservative treatment (n=3), alone or associate to intermittent bladder catheterization (IBC) (n=8). During the treatment with anticholinergic agents, the cardiac frequency was controlled by EKG registration in 6 patients, being the rest of the children clinicaly controled (skin colour, mouth dryness, cardiac frequency and intestinal function). RESULTS: In all the cases, the minimum duration of the treatment was one year, until the functional stabilization of the urinary tract. It is to underline the absence of secondary complications which would have caused the suspension or the reduction of the treatment at long term. Conventional studies of urologicals image and urodinamic studies, showed the stabilization of the urinary tract and also the preservation of kidney function and not only was demonstrated in those patients with oral anticholinergic agent as their unique treatment, but also in those patients who had previously been derivated. The previous vesical stabilization allowed the optimum result of subsequent corrective surgeries in five patients (ureteroneocystostomy and vesiscotomy clousure. In cases with previous derivation, the treatment with oral anticholinergic agents made easier vesicostomic clousure in the forth quarter of the first year old wih the maintenance of the vesical stability. CONCLUSIONS: The use of oral anticholinergic agents at a dose of 0.2 mg/kg/day, has resulted to be safe as well as effective in the treatment of high risk bladder in infancy those with less than one year of life. In those patients with serious dysplasia by reflux, it contributes to the preservation of kidney function, it makes easier the early desderivation and it also stabilizes in a functional way the bladder as a previous step to subsequent reconstructive surgeries (AU)

[In which patients is the oxybutynin-desmopressin combination therapy indicated?]. / En qué pacientes está indicada la terapia combinada oxibutinina-desmopresina?

PURPOSE: We assume that desmopressin decreases nocturnal urine output of the enuretic child. The aim of this study is to evaluate the efficacy of desmopressin plus oxybutynin and to define urodynamic pattern in children with bladder hyperactivity partially-responders to oxybutynin alone (in which nocturnal enuresis persists in spite of bladder stability). METHODS: 48 enuretic patients with urodynamically confirmed bladder hyperactivity were enrolled in the prospective study. All patients were treated with oxybutynin, 0.3-05 mg/Kg/day orally, and bladder therapy, for six months. At the end of this period, patients with excellent response (cessation of nocturnal and diurnal symptoms) was designed as group I (28 children, 58%); patients with partial response (persistence of nocturnal enuresis) as group II (19 children, 40%); and patients non-responders as group III (1 patient, excluded because of no completion of treatment). After six months patients were assessed by standardized urodynamic study. Group II patients were treated with combined therapy, desmopressin-20 mcg/day-plus oxybutynin, for six months. Group I patients served as controls. RESULTS: The differences between mean bladder accommodation and mean voiding pressure in patients with excellent response and partially response to oxybutynin were statistically significant. After combined therapy 18 of 19 patients (95%) of group II became group I. CONCLUSIONS: The efficacy of desmopressin plus oxybutynin is confirmed in patients with low final accommodation (< 3 ml/cm H2O) and high voiding pressure (> 70 cm H2O). Combined therapy can be a good choice of treatment in children with bladder hyperactivity with partial response to oxybutynin alone.

[Indications from the laparoscopic approach in pediatric urological surgery: our experience in 49 patients]. / Indicaciones del abordaje laparoscópico en cirugía urológica pediátrica: nuestra experiencia en 49 pacientes.

OBJECTIVES: To analyse the diagnostic and therapeutic potential of video-assisted laparoscopy in the urology paediatric pathology. We had analysed its indications and results, to measure in disminution of morbidity and hospital stay. PATIENTS AND METHODS: We are treated in our section of Paediatric Urology 49 patients during the period of June 1995 and December 1998, performing 51 laparoscopics procedures. The mean age was 8 years (2-16 years ago). The laparoscopic indications were diagnostic in 33 patients: intra-abdominal testes (n = 16), renal biopsy (n = 17), and terapeuthics in 16 patients (17 laparoscopics procedures): varicoceltomy (n = 7), orchiectomy (n = 1), closure peritoneal-vaginal duct (n = 2), retroperitoneal nephrectomy (n = 6), marsupilazation and omentoplastic in giant lymphocel renal post-transplantation (n = 2). RESULTS: The laparoscopic approach were effective in 100% of diagnostic procedures and 93.4% of therapeuticm procedures. Conversion in 2 cases were necessary to make renal biopsy (n = 1). We hadn't have postoperatives complications and the indices of intraoperative morbidity was of 2.4%. The mean hospital stay was 1.4 days. CONCLUSIONS: We believe that exist indications clinically stabilised of laparoscopic approach--diagnostic and therapeutic--in paediatric urology. There are others indications that its are consider anecdotal in the present but in immediately future these indications will be valid.

Quantitative nuclear cystography does not predict outcome in patients with primary vesicoureteral reflux.

PURPOSE: Quantitative nuclear cystography has been advocated as a tool for determining the prognosis in children with primary vesicoureteral reflux. We reviewed our data on this technique to assess its usefulness for predicting the outcome in this population. MATERIALS AND METHODS: We retrospectively reviewed the records of all patients with primary reflux in whom findings were positive on at least 2 nuclear cystograms at our institution between 1992 and 1997. Patients followed at least 3 years were stratified according to outcome. Unfavorable prognostic criteria included bladder volume at reflux onset 60% or less of total bladder capacity and calculated volume of reflux 2% or greater of bladder capacity. RESULTS: Of the 107 patients in our study 63 were followed for 3 years or longer, and reflux resolved in 17, was repaired in 24 and persisted in 22. Mean patient age at latest followup, duration of followup and number of cystograms did not significantly differ among groups. Intermittent reflux in 33% of the patients followed 3 years or longer was not associated with outcome or detrusor instability. Bladder and reflux volume varied and was nonpredictive in individuals. CONCLUSIONS: Quantitative nuclear cystography did not predict the outcome in patients followed for primary vesicoureteral reflux at a single institution for 3 years or longer. Intermittent reflux was common. These data suggest that nuclear cystography cannot be used to assess reliably the prognosis in individuals. Strong consideration should be given to using negative findings on 2 cystograms to confirm reflux resolution in patients at high risk.

Histomorphometric changes after small bowel transplantation.

AIM: Rejection results in destruction of the intestinal mucosa. This is the major cause of morbidity and morbility in human allografts. The aim of this study was to investigate histomorphometric changes in both the graft and recipient intestinal mucosa after experimental orthotopic small bowel transplantation. EXPERIMENTAL DESIGN: Using the pig model, 21 large-white pigs underwent: laparotomy (n = 7) (control group); orthotopic small bowel transplantation without immunosuppression (n = 7); and orthotopic small bowel transplantation with cyclosporine A (n = 7). RESULTS: 1) Significant reduction of total mucosal thickness (villi plus crypt) (p < 0.05) in both the graft and recipient mucosa; 2) Attenuation of this effect by administration of cyclosporine A, which reduced the loss of mucosal thickness mainly at the expense of crypt hipertrophy. CONCLUSIONS: These findings suggest that villi and crypt length measurements may be a reliable method for monitoring rejection after small bowel transplantation.

[Multicystic renal dysplasia: changes in the contralateral urinary tract]. / Displasia renal multiquistica: alteraciones en el tracto urinario contralateral.

We have treated seven children with multicystic dysplastic kidney during the last 15 years. Three of then presented contralateral upper urinary tract anomalies. Four cases were diagnosed during prenatal period and three during the first term of life. We did analytic kidney function evaluation, ultrasound examination, voiding cystourethrography, radionuclide scan, gammagraphy, excretory urography and urinary cultures in all cases. We have found a vesicoureteral reflux with a bifid pelvis associated in the same child and two other cases with nonobstructive hydronephrosis. The child with vesicoureteral reflux presented also ipsilateral anomalies like a bladder diverticulum and an incomplete urethral duplication. One child with hydronephrosis opposite to the multicystic dysplastic kidney, also presented a bowel malrotation and a congenital cardiopathy, as extra-urologic associated anomalies.

[Therapeutic attitude in patients with multicystic dysplastic kidneys]. / Actitud terapéutica ante la displasia renal multiquística.

We presented seven multicystic dysplastic kidney cases treated in our Hospital during the last 15 years. Four children were diagnosed prenatally and three during the first term of life. Five were girls and two boys. We found three contralateral upper urinary tract anomalies. We did five nephrectomies. In two cases because compressive abdominal mass during the neonatal period, in two cases during the first year of life because symptomatic urinary tract infections and one case at the age of 13 months with persistent multicystic dysplastic kidney followed by ultrasound. Two cases were not operated. In one patient with partial regression of the multicystic kidney the family refused operation. We have lost follow-up in the last patient.

Microsurgical treatment of varicocele in adolescents.

Between March 1990 and November 1992, 22 adolescents with varicocele, 13 unilateral left and 4 bilateral, are presented. They were all treated with microsurgical techniques by shunting the venous drainage by means of a spermatic inferior epigastric end-to-side anastomosis. The results achieved have been optimum with the clinical disappearance of varicose veins in 100% of the cases and the elimination of the spontaneous reflux of the interna spermatic vein in 100% of the patients shown by echodoppler. This technique enables the establishment of an immediate and physiological testicular venous drainage fundamental for testicular growth and development.