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)

[Meconium periorchitis prenatal diagnosis: review of the literature, about 3 cases]. / Periorquitis meconial diagnóstico prenatal: revisión de la literatura, a propósito de 3 casos.

OBJECTIVE: To assess the importance of prenatal ultrasound diagnosis of the fetus carrying meconium periorchitis and its predictive relevance for fetal monitoring and prognosis in the context of acute fetal intestinal disease. MATERIAL AND METHODS: Three male fetuses have been diagnosed of meconium periorchitis in our Unit of Fetal Medicine in the last 5 years. Their prenatal ultrasound diagnoses were: testicular tumor (n=1); Meconium periorchitis with acute fetal intestinal perforation (n=2). Gestational age at diagnosis was 33, 34 and 35 weeks. Ultrasound signs at diagnosis were: Increased size of scrotal zone, with hyperechogenic lesions inside and permanence of peritoneum-vaginal canal; at abdominal zone, echographic signs of intestinal disease with or without meconium peritonitis were found (hyperechogenic lesions, edema of intestinal loops and ascites). All three neonates were assessed postnatally by ultrasound and therapeutic indication. RESULTS: Fetal ultrasound findings influenced both evolution and termination of pregnancy. The diagnosis of meconium periorchitis was confirmed postnatally in all cases: in the 1st case, delivered at term, scrotal tumoral pathology was ruled out and did not require abdominal surgery; the other 2 patients were delivered at the same week of prenatal diagnosis and an inguinal-scrotal surgery with intestinal approach because of meconium peritonitis was performed. No patient underwent orchiectomy, maintaining the teste-epididymal binomial intact. CONCLUSION: Prenatal ultrasound diagnosis of meconium periorchitis requires a strict ultrasound follow-up of the fetus as it is a specific marker of intestinal perforation, which can lead to the termination of pregnancy and avoid appearance of complicated meconium peritonitis.

OBJETIVO: Evaluar la importancia del diagnóstico ecográfico prenatal del feto portador de periorquitis meconial y su relevancia predictiva del seguimiento y pronóstico fetal en el contexto de una enfermedad intestinal fetal aguda. MATERIAL Y METODOS: En los últimos 5 años en la Unidad de Medicina Fetal se han diagnosticado tres fetos varones de periorquitis meconial cuyos diagnósticos ecográficos prenatales fueron: tumor testicular (n=1); y periorquitis meconial con perforación intestinal aguda fetal (n=2). La edad gestacional al diagnóstico fue de 33, 34 y 35 semanas. Los signos ecográficos al diagnóstico fueron: a nivel escrotal, aumento del tamaño, lesiones hiperecogénicas y permanencia del conducto peritoneo-vaginal; a nivel abdominal pueden existir signos ecográficos de enfermedad intestinal con o sin peritonitis meconial (lesiones hiperecogénicas, edemas de asas y ascitis). Los tres neonatos fueron evaluados postnatalmente mediante ecografía comparativa de los hallazgos prenatales e indicación terapéutica. RESULTADOS: Los hallazgos ecográficos fetales influyeron en la evolución y finalización de la gestación. El diagnóstico de periorquitis meconial fue confirmado postnatalmente en los tres casos: en el 1er caso a término, se descartó patología tumoral escrotal y no requirió cirugía abdominal; en los otros dos pacientes se indicó finalizar la gestación tras el diagnóstico prenatal y se realizó cirugía inguino-escrotal y abordaje intestinal por la peritonitis meconial. CONCLUSION: El diagnóstico ecográfico prenatal de periorquitis meconial obliga a un seguimiento ecográfico estricto del feto al ser un marcador específico de perforación intestinal, que puede conllevar la finalización de la gestación y evitar la aparición de una peritonitis meconial complicada.

Periorquitis meconial diagnóstico prenatal: revisión de la literatura, a propósito de 3 casos / Meconium periorchitis prenatal diagnosis: review of the literature, about 3 cases

Objetivo: Evaluar la importancia del diagnóstico ecográfico prenatal del feto portador de periorquitis meconial y su relevancia predictiva del seguimiento y pronóstico fetal en el contexto de una enfermedad intestinal fetal aguda. Material y métodos: En los últimos 5 años en la Unidad de Medicina Fetal se han diagnosticado tres fetos varones de periorquitis meconial cuyos diagnósticos ecográficos prenatales fueron: tumor testicular (n=1); y periorquitis meconial con perforación intestinal aguda fetal (n=2). La edad gestacional al diagnóstico fue de 33, 34 y 35 semanas. Los signos ecográficos al diagnóstico fueron: a nivel escrotal, aumento del tamaño, lesiones hiperecogénicas y permanencia del conducto peritoneo-vaginal; a nivel abdominal pueden existir signos ecográficos de enfermedad intestinal con o sin peritonitis meconial (lesiones hiperecogénicas, edemas de asas y ascitis). Los tres neonatos fueron evaluados postnatalmente mediante ecografía comparativa de los hallazgos prenatales e indicación terapéutica. Resultados: Los hallazgos ecográficos fetales influyeron en la evolución y finalización de la gestación. El diagnóstico de periorquitis meconial fue confirmado postnatalmente en los tres casos: en el 1er caso a término, se descartó patología tumoral escrotal y no requirió cirugía abdominal; en los otros dos pacientes se indicó finalizar la gestación tras el diagnóstico prenatal y se realizó cirugía inguino-escrotal y abordaje intestinal por la peritonitis meconial. Conclusión: El diagnóstico ecográfico prenatal de periorquitis meconial obliga a un seguimiento ecográfico estricto del feto al ser un marcador específico de perforación intestinal, que puede conllevar la finalización de la gestación y evitar la aparición de una peritonitis meconial complicada

Objective: To assess the importance of prenatal ultrasound diagnosis of the fetus carrying meconium periorchitis and its predictive relevance for fetal monitoring and prognosis in the context of acute fetal intestinal disease. Material and methods: Three male fetuses have been diagnosed of meconium periorchitis in our Unit of Fetal Medicine in the last 5 years. Their prenatal ultrasound diagnoses were: testicular tumor (n=1); Meconium periorchitis with acute fetal intestinal perforation (n=2). Gestational age at diagnosis was 33, 34 and 35 weeks. Ultrasound signs at diagnosis were: Increased size of scrotal zone, with hyperechogenic lesions inside and permanence of peritoneum-vaginal canal; at abdominal zone, echographic signs of intestinal disease with or without meconium peritonitis were found (hyperechogenic lesions, edema of intestinal loops and ascites). All three neonates were assessed postnatally by ultrasound and therapeutic indication. Results: Fetal ultrasound findings influenced both evolution and termination of pregnancy. The diagnosis of meconium periorchitis was confirmed postnatally in all cases: in the 1st case, delivered at term, scrotal tumoral pathology was ruled out and did not require abdominal surgery; the other 2 patients were delivered at the same week of prenatal diagnosis and an inguinal-scrotal surgery with intestinal approach because of meconium peritonitis was performed. No patient underwent orchiectomy, maintaining the teste-epididymal binomial intact. Conclusion: Prenatal ultrasound diagnosis of meconium periorchitis requires a strict ultrasound follow-up of the fetus as it is a specific marker of intestinal perforation, which can lead to the termination of pregnancy and avoid appearance of complicated meconium peritonitis

Urodinámica en el paciente urológico pediátrico / No disponible

No disponible

[Button vesicostomy in newborn: an alternative of classical cutaneous vesicostomy]. / Vesicostomía de botón en el recién nacido: alternativa a la vesicostomía clásica.

PURPOUSE: In newborn and infant with functional and/or anatomical lower urinary tract obstruction, the goals of vesicostomy are to achieve a low pressure bladder, an effective bladder drainage and to prevent urinary tract infection and sepsis. Nonetheless, classical vesicostomy is not free from complications. The aim is to describe a surgical technique of button vesicostomy as an alternative of cutaneous vesicostomy. MATERIAL AND METHODS: 13 newborn and infant, age between 14 and 60 days (median 20 days), twelve male and one female, underwent button vesicostomy. Twelve patients were neurologically normal and one with neurogenic bladder. Surgical technique of button vesicostomy differs from the classical one; it does not require bladder cupula mobilization, the botton is inserted into the bladder at the urachus insertion without suturing the bladder mucosa to the skin, creating a vesico-cutaneous fistula. RESULTS: Button vesicostomy was found to be safe and effective in 96%. Stabilization of upper urinary tracts was achieved in all cases. Botton vesicostomy has made it possible to undergo intermittent catheterization through the button before closure of the vesicostomy, as well as performing standard urodynamic studies through the urethra to evaluate bladder function during follow-up and providing objective data for proper undiversion. There were no complications. Vesicostomy was closed at a median age of twenty months, combined with reconstructive procedures, when necessary. CONCLUSIONS: Button vesicostomy has improved the outcome of the classical vesicostomy. It provides an efficient way for lowering the bladder pressure in the absence of complications, making possible to perform urodynamic studies; the evaluation of bladder function is essential prior to the undiversion.

OBJETIVOS: La vesicostomía clásica tiene como objetivos en el recién nacido-lactante con obstrucción funcional/anatómica del tracto urinario inferior (TUI): conseguir un TUI de baja presión, drenaje efectivo del TUI y disminución de la infección/sepsis urinaria; pero no está exenta de complicaciones (prolapso/estenosis). El objetivo es describir la técnica quirúrgica de la vesicostomía-de-botón y sus resultados como alternativa a la vesicostomía cutánea clásica. MATERIAL Y METODOS: Hemos realizado 13 vesicostomías-de-botón, en pacientes recién nacidos-lactantes; 12 neurológicamente normales y 1 vejiga neurógena. La distribución por edad-sexo: 14-60 días (mediana 20 días); 12 varones y 1 niña. La técnica quirúrgica de la-vesicostomía-de-botón difiere de la clásica en que no precisa movilización de la cúpula vesical, se realiza en la inserción del uraco y evita la fijación de la mucosa vesical a piel, creando una fístula-vésico-cutánea. RESULTADOS: La vesicostomía-de-botón ha sido eficaz y segura en el 96,4%. Ha facilitado la estabilidad vesical en el 100%, así como la realización de estudios urodinámicos estándar-por uretra- durante el evolutivo y como dato objetivo de indicación de desderivación-urinaria. Favoreció la realización de cateterización vesical intermitente, previo al cierre de la vesicostomía. Destacamos la ausencia de complicaciones. El cierre de la vesicostomía se realizó a una media de edad de 20 meses, concomitante con la reconstrucción del TUI, cuando fue necesaria. CONCLUSIONES: La vesicostomía-de-botón ha cambiado el pronóstico evolutivo de la vesicostomía clásica, siendo resolutiva en la normalización de la alta presión del TUI, en ausencia de complicaciones y facilitando la realización de estudios urodinámicos; clave para la desderivación.

Vesicostomía de botón en el recién nacido: alternativa a la vesicostomía clásica / Button vesicostomy in newborn: an alternative of classical cutaneous vesicostomy

Objetivos. La vesicostomía clásica tiene como objetivos en el recién nacido-lactante con obstrucción funcional/anatómica del tracto urinario inferior (TUI): conseguir un TUI de baja presión, drenaje efectivo del TUI y disminución de la infección/sepsis urinaria; pero no está exenta de complicaciones (prolapso/estenosis). El objetivo es describir la técnica quirúrgica de la vesicostomía-de-botón y sus resultados como alternativa a la vesicostomía cutánea clásica. Material y métodos. Hemos realizado 13 vesicostomías-de-botón, en pacientes recién nacidos-lactantes; 12 neurológicamente normales y 1 vejiga neurógena. La distribución por edad-sexo: 14-60 días (mediana 20 días); 12 varones y 1 niña. La técnica quirúrgica de la-vesicostomíade-botón difiere de la clásica en que no precisa movilización de la cúpula vesical, se realiza en la inserción del uraco y evita la fijación de la mucosa vesical a piel, creando una fístula-vésico-cutánea. Resultados. La vesicostomía-de-botón ha sido eficaz y segura en el 96,4%. Ha facilitado la estabilidad vesical en el 100%, así como la realización de estudios urodinámicos estándar-por uretra- durante el evolutivo y como dato objetivo de indicación de desderivación-urinaria. Favoreció la realización de cateterización vesical intermitente, previo al cierre de la vesicostomía. Destacamos la ausencia de complicaciones. El cierre de la vesicostomía se realizó a una media de edad de 20 meses, concomitante con la reconstrucción del TUI, cuando fue necesaria. Conclusiones. La vesicostomía-de-botón ha cambiado el pronóstico evolutivo de la vesicostomía clásica, siendo resolutiva en la normalización de la alta presión del TUI, en ausencia de complicaciones y facilitando la realización de estudios urodinámicos; clave para la desderivación (AU)

Purpose. In newborn and infant with functional and/or anatomical lower urinary tract obstruction, the goals of vesicostomy are to achieve a low pressure bladder, an effective bladder drainage and to prevent urinary tract infection and sepsis. Nonetheless, classical vesicostomy is not free from complications. The aim is to describe a surgical technique of button vesicostomy as an alternative of cutaneous vesicostomy. Materials and methods. 13 newborn and infant, age between 14 and 60 days (median 20 days), twelve male and one female, underwent button vesicostomy. Twelve patients were neurologically normal and one with neurogenic bladder. Surgical technique of button vesicostomy differs from the classical one; it does not require bladder cupula mobilization, the botton is inserted into the bladder at the urachus insertion without suturing the bladder mucosa to the skin, creating a vesicocutaneous fistula. Results. Button vesicostomy was found to be safe and effective in 96%. Stabilization of upper urinary tracts was achieved in all cases. Botton vesicostomy has made it possible to undergo intermittent catheterization through the button before closure of the vesicostomy, as well as performing standard urodynamic studies through the urethra to evaluate bladder function during follow-up and providing objective data for proper undiversion. There were no complications. Vesicostomy was closed at a median age of twenty months, combined with reconstructive procedures, when necessary. Conclusions. Button vesicostomy has improved the outcome of the classical vesicostomy. It provides an efficient way for lowering the bladder pressure in the absence of complications, making possible to perform urodynamic studies; the evaluation of bladder function is essential prior to the undiversion (AU)

[Hyaluronic acid scrotoplasty: minimally-invasive procedure to enhance the volume of scrotum until puberty]. / Escrotoplastia de relleno con acido hialurónico como alternativa mínimamente invasiva para aumentar el volumen escrotal hasta la pubertad.

PURPOSE: There is a controversy concerning infant testicular prosthesis. The problem is that this may necessitate further surgery to insert a larger prosthesis when the child gets older. An alternative strategy is to delay the placement of the definitive prosthesis until the child reaches adolescence. However, the underdeveloped scrotum may fail to accommodate the desired sized testicular prosthesis. We present scrotoplasty using hyaluronic acid gel injection as a minimally-invasive alternative to enhance the volume of scrotum until puberty. MATERIAL AND METHODS: A prospective report of 35 boys younger than seven years old with monorchia underwent injection of hyaluronic acid for scrotal filling. Mean follow-up of 24 months (range 12-48 months). RESULTS: The only complication was early resorption in 2 patients at 8 and 10 months after implantation, respectively. In long-term followup 100 per cent of the families rated the cosmetic appearance as good and 95 per cent were content with the decision regarding placement of a testicular implant irrespective of whether they had been retreated. CONCLUSIONS: It shows that hyaluronic acid gel scrotal injection can provide satisfactory improvement in enhance the volume of scrotum. It is associated with high family and patient satisfaction, and provides a long-lasting result. This technique makes placement of prosthetic testis a very simple procedure that can be performed at the time of diagnostic exploration or orchiectomy, increasing scrotal space until post pubertal definitive prosthesis.

Escrotoplastia de relleno con ácido hialurónico como alternativa mínimamente invasiva para aumentar el volumen escrotal hasta la pubertad / Hyaluronic acid scrotoplasty: minimally-invasive procedure to enhance the volume of scrotum until puberty

Objetivos. Existe una controversia sobre la implantación de prótesis testicular en el niño pequeño, debido a la necesidad de recambio de la prótesis en la pubertad. La alternativa es retrasar su implantación definitiva hasta la adolescencia. Sin embargo, la hipoplasia escrotal puede discultar la acomodación de la prótesis del tamaño deseado. Presentamos la escrotoplastia de relleno mediante la inyección de ácido hialurónico como una alternativa minimamente invasiva para aumentar el volumen escrotal hasta la pubertad. Material y métodos. Estudio prospectivo de 35 niños varones menores de siete años de edad con monorquia. Escrotoplastia de relleno mediante inyección intraescrotal de ácido hialurónico modificado hasta aumentar de forma simétrica el volumen del hemiescroto vacío respecto al contralateral. Seguimiento medio de 24 meses. Resultados. La única complicación ha sido la reabsorción precoz en dos pacientes a los 8 y 10 meses de la implantación, respectivamente. A largo plazo, el 100% de los padres consideran que la apariencia cos-mética es buena y el 95% están contentos con la decisión del implante, con independencia de si han sido reinyectados. Conclusiones. La escrotoplastia de relleno proporciona un aumento satisfactorio del volumen escrotal. Se asocia a un alto grado de satisfacción familiar, con buenos resultados a largo plazo. Convierte a la implantación protésica en un procedimiento simple, mínimamente invasivo, que puede realizarse durante la exploración diagnóstica o la orquiectomía. Esta técnica puede repetirse hasta la prótesis definitiva postpuberal

Purpose. There is a controversy concerning infant testicular prosthesis. The problem is that this may necessitate further surgery to insert a larger prosthesis when the child gets older. An alternative strategy is to delay the placement of the definitive prosthesis until the child reaches adolescence. However, the underdeveloped scrotum may fail to accommodate the desired sized testicular prosthesis. We present scrotoplasty using hyaluronic acid gel injection as a minimally-invasive alternative to enhance the volume of scrotum until puberty. Material and methods. A prospective report of 35 boys younger than seven years old with monorchia underwent injection of hyaluronic acid for scrotal filling. Mean follow-up of 24 months (range 12-48 months). Results. The only complication was early resorption in 2 patients at 8 and 10 months after implantation, respectively. In long-term follow-up 100 per cent of the families rated the cosmetic appearance as good and 95 per cent were content with the decision regarding placement of a testicular implant irrespective of whether they had been retreated. Conclusions. It shows that hyaluronic acid gel scrotal injection can provide satisfactory improvement in enhance the volume of scrotum. It is associated with high family and patient satisfaction, and provides a long-lasting result. This technique makes placement of prosthetic testis a very simple procedure that can be performed at the time of diagnostic exploration or orchiectomy, increasing scrotal space until post pubertal definitive prosthesis

[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.

[Retroperitoneoscopic assisted pyeloplasty for ureteropelvic junction obstruction in children]. / Pieloplastia asistida por retroperitoneoscopia: nuestra experiencia en 30 pacientes.

OBJECTIVES: Description of the retroperitoneoscopic approach in the conventional pyeloplasty for ureteropelvic junction obstruction in children. MATERIAL AND METHODS: From 1998 pyeloplasty assisted by retroperitoneoscopic approach (PARA) was performed in 30 patients. TECHNIQUE: Position in latero-dorsal decubitus and incision of 1 cm in angle costolumbar. We made retroperitoneoscopic space by ball dissection technique and 11 mm Hg pressure. The ureteropelvic junction was extracted through the incision of the port. The UPJ was resected in all patients and Anderson-Hynes pyeloplasty with double PDS 6/0 continuous sutures was performed. In all patients a drainage type Penrose in perirenal space was used. In the last 18 patients a 4F double-J stent was placed. The mean follow-up time was 42 months (range between 6 and 84 months). Operative time, hospital stay, handling of postoperative pain and the postoperative studies have been revised. RESULTS: In all the cases the retroperitoneoscopic approach was good for the identification and dissection of the ureteropelvic junction facilitating the extraction and reconstruction (pyeloplasty) through the mini-incision of the entrance port. The mean operative time was 90 min. (range between 65 and 128 min). We highlight the absence of intraoperative complications. The only postoperative complication has been a pyohydronephrosis in a patient not having internal drainage that was solved by percutaneous pyelostomy and didn't need reintervention. The postoperative handling of the pain was good by means of caudal locorregional anesthesia or by infiltration of the wound with local anesthesic and a dose of Ibuprofeno previous to leave the hospital. The mean hospital stay was 2 days (1-3 days) excluding the complicated case. Postoperative diuretic renograms at the 6 and 18 have shown absence of obstruction in all cases. In the long term follow-up, in 1 case nephrectomy was performed. CONCLUSIONS: The PARA for UPJ obstruction is a safe and effective procedure with the advantage of a minimal invasive approach that facilitates the reconstruction of the ureteropielic junction. Reduces operative time and hospital stay, with appropriate postoperative results. In our experience PARA constitutes an alternative to the conventional pyeloplasty and laparoscopic pyeloplasty in the pediatric age.

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)

Pieloplastia asistida por retroperitoneoscopia: nuestra experiencia en 30 pacientes / Retropenitoneoscopic assisted pyeloplasty for ureteropelvic junction obstruction in children

Objetivos. Descripción del abordaje retroperitoneoscópico en la pieloplastia convencional para la obstrucción de la unión pieloureteral en niños. Material y métodos. Desde 1998 hemos realizado pieloplastia asistida por retroperitoneoscopia (PAR) en 30 pacientes. Técnica. Posición en decúbito lateroposterior e incisión de 1 cm en ángulo costolumbar. Creación de espacio retroperitoneoscópico con balón de disección neumática y mantenimiento del mismo mediante retroneumoperitoneo de hasta 11 mmHg. Exteriorización de la unión pieloureteral a través de la incisión del puerto de entrada. Ureteropieloplastia mediante neoanastomosis con doble sutura continua de PDS 6/0 y magnificación. Drenaje tipo Penrose en lecho peri renal. En los últimos 18 pacientes se tutorizó la neounión mediante drenaje interno doble J. El seguimiento medio ha sido de 42 meses (rango entre 6 y 84 meses). Se ha revisado: el tiempo operatorio, la estancia hospitalaria, el manejo de dolor postoperatorio y los estudios postoperatorios de seguimiento renográfico. Resultados. En todos los casos, el abordaje retroperitoneoscópico fue óptimo para la identificación y disección de la unión pieloureteral facilitando la extracción y reconstrucción (pieloplastia) a través de la miniincisión del puerto de entrada. El tiempo medio de la cirugía ha sido de 90 min (rango entre 65 y 128 min). Destacamos la ausencia de complicaciones intraoperatorias. La única complicación postoperatoria de la serie ha sido un episodio de pielohidronefrosis, en un paciente no portador de drenaje interno, que se solucionó mediante pielostomía percutánea y que no precisó reintervención. El manejo postoperatorio del dolor fue óptimo mediante anestesia locorregional caudal o infiltración de la herida quirúrgica con anestésico local y una dosis de ibuprofeno oral intrahospitalario. La estancia media ha sido de 2 días (rango entre 1 y 3 días) excluyendo el caso complicado. Los controles postoperatorios mediante renograma diurético a los 6 y 18 meses de la cirugía han mostrado ausencia de obstrucción en todos los casos. En el seguimiento a largo plazo fue necesario realizar nefrectomía en 1 caso. Conclusiones. La pieloplastia asistida por retroperitoneoscopia ha demostrado ser una técnica de abordaje mínimamente invasiva que facilita la cirugía reconstructiva de la unión pieloureteral, que reduce el tiempo operatorio y la estancia hospitalaria, con resultados postoperatorios adecuados. La PAR constituye una alternativa a la pieloplastia convencional y a la pieloplastia laparoscópica en el tratamiento de la hidronefrosis en la edad pediátrica (AU)

OBJECTIVES: Description of the retroperitoneoscopic approach in the conventional pyeloplasty for ureteropelvic junction obstruction in children. MATERIAL AND METHODS: From 1998 pyeloplasty assisted by retroperitoneoscopic approach (PARA) was performed in 30 patients. Technique: Position in latero-dorsal decubitus and incision of 1 cm in angle costolumbar. We made retroperitoneoscopic space by ball dissection technique and 11 mm Hg pressure. The ureteropelvic junction was extracted through the incision of the port. The UPJ was resected in all patients and Anderson-Hynes pyeloplasty with double PDS 6/0 continuous sutures was performed. In all patients a drainage type Penrose in perirenal space was used. In the last 18 patients a 4F double-J stent was placed. The mean follow-up time was 42 months (range between 6 and 84 months). Operative time, hospital stay, handling of postoperative pain and the postoperative studies have been revised. RESULTS: In all the cases the retroperitoneoscopic approach was good for the identification and dissection of the ureteropelvic junction facilitating the extraction and reconstruction (pyeloplasty) through the mini-incision of the entrance port. The mean operative time was 90 min. (range between 65 and 128 min). We highlight the absence of intraoperative complications. The only postoperative complication has been a pyohydronephrosis in a patient not having internal drainage that was solved by percutaneous pyelostomy and didn't need reintervention. The postoperative handling of the pain was good by means of caudal locorregional anesthesia or by infiltration of the wound with local anesthesic and a dose of Ibuprofeno previous to leave the hospital. The mean hospital stay was 2 days (1-3 days) excluding the complicated case. Postoperative diuretic renograms at the 6 and 18 have shown absence of obstruction in all cases. In the long term follow-up, in 1 case nephrectomy was performed. CONCLUSIONS: The PARA for UPJ obstruction is a safe and effective procedure with the advantage of a minimal invasive approach that facilitates the reconstruction of the ureteropielic junction. Reduces operative time and hospital stay, with appropriate postoperative results. In our experience PARA constitutes an alternative to the conventional pyeloplasty and laparoscopic pyeloplasty in the pediatric age (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.