[Factors involved in the success or failure of endoscopic treatment in paediatric primary vesicoureteral reflux: A systematic review.] / Factores condicionantes de éxito o fracaso del tratamiento endoscópico en el reflujo vesicoureteral primario pediátrico: una revisión sistemática.

OBJECTIVE: The objective of this paper isidentifying the factors that may condition the success or failure of endoscopic therapy for vesicoureteral reflux in the paediatric population.MATERIAL AND METHODS: A bibliographic review was performed in the Cochrane Library and PUBMED of all the studies which the principal subject was about identifying factors involved in the success or failure of endoscopic therapy for vesicoureteral reflux in the paediatric population. RESULTS: Of a total of 1410 studies, 14 studies and17 factors potentially involved in the success or failure of endoscopic therapy were fully analysed according to the different studies reviewed. After analyzing these studies, we identified that the most widely accepted factors that determine the success or failure of endoscopic therapy are the high degree of pre-surgical reflux, the previous injection of antireflux material, the surgical experience and the presence of dysfunction of the lower urinary tract (UTD). Other factors such as the amount of bulking substance used, meatal morphology, renal damage and age at the time of injection could be potentially involved. The technique used and the bulking material do not seem to be associated with the response to therapy. Exists a great heterogeneity of the reviewed studies, both in relation to the characteristics of the patients included, criteria and surgical technique and evaluation of results. CONCLUSIONS: The degree of pre-surgical reflux, the presence of UTD, previous injections and surgical experience are the most widely accepted determining factors in the results of endoscopic therapy according to the different studies published in this topic.

OBJETIVO: Identificar los factores que puedan condicionar el éxito o fracaso de la terapia endoscópica del reflujo vesicoureteral en la población pediátrica.MATERIAL Y MÉTODOS: Se realizó una búsqueda bibliográfica en Cochrane Library y PUBMED de todos los estudios sobre factores implicados en el éxito o fracaso de la terapia endoscópica del reflujo vesicoureteral en la población pediátrica. RESULTADOS: De un total de 1410 trabajos se analizaron íntegramente 14 estudios y 17 factores potencialmente implicados en el éxito o fracaso de la terapia endoscópica según los diferentes trabajos evaluados. Tras el análisis de estos trabajos identificamos que los factores que condicionan el éxito o fracaso de la terapia endoscópica más ampliamente aceptados son, el alto grado de reflujo prequirúrgico, la inyección previa de material antirreflujo, la menor experiencia quirúrgica y la presencia de disfunción del tracto urinario inferior (DTUI). Otros factores como el volumen de sustancia de abultamiento empleada, la morfología meatal postpunción, la presencia de daño renal previo o la edad en el momento de la inyección podrían así mismo estar potencialmente asociados. La técnica empleada y el material de abultamiento no parecen asociarse al éxito de la terapia endoscópica. Destaca tras dicho análisis la gran heterogeneidad de los estudios revisados tanto en relación a características de los pacientes incluidos, criterios y técnica quirúrgica y evaluación de resultados. CONCLUSIONES: El grado de reflujo prequirúrgico, la presencia de DTUI, las inyecciones previas y la experiencia quirúrgica son los condicionantes más ampliamente aceptados en los resultados de la terapia endoscópica según los diferentes estudios publicados al respecto.

Complete penoscrotal transposition: A three-stage procedure.

Complete penoscrotal transposition (CPST) with an intact scrotum is a rare anomaly in which the scrotum is located cephalic to the penis. It is the most severe degree of malformation of a spectrum of abnormalities in scrotal development. There are few cases reported in the literature, and there are few descriptions of the technique for correction and results. We describe a new case of CPST and its sequential correction.

[Vesicoureteral reflux grades III-IV: factors involved in the efficacy of endoscopic treatment in pediatric patients]. / Reflujo vesicoureteral grados III-IV: factores implicados en la efectividad del tratamiento endoscópico en pacientes pediátricos.

OBJECTIVES: Vesicoureteral reflux (VUR) is detected in 30-50% of pediatric patients diagnosed of urinary tract infection (UTI). Multiple clinical trials have compared the results of conservative management against surgical therapy. The introduction of a third alternative in 1984 (endoscopic approach) has dramatically modified the management of VUR. The objective of our work is to analyze the factors influencing the favourable outcome of the endoscopic treatment of VUR. METHODS: Retrospective and prospective cohort study analyzing 90 patients with grades III and IV VUR treated endoscopically over the last eight years. 184 patients undergoing endoscopic management were selected from a group of 550 patients (735 VUR renal units) with the diagnosis of VUR over the study period. All patients underwent VCUG or galactose-based sonocystography to obtain the diagnosis. DMSA scintigram was used to evaluate renal damage. Data about gender, age, UTI, bilateralism, renal damage, voiding dysfunction, outcomes, complications, and recurrences were registered. A new factor was defined: ureteral dilation independent from the international classification. This factor was evaluated by a single pediatric urologist blinded for the international classification results. Data analysis was performed using the chi square test and Fischer exact test; a multiple logistic regression analysis was also performed. Statistical significance was established at p < 0.05. SPSS 11.0 software was employed for data analysis. RESULTS: Boys/girls ratio was 2.91. Bilateral VUR appeared in 44.4% of the cases. UTI was the presenting diagnosis in 64 cases; the diagnosis was prenatal in 21 cases (with a male predominance 5.2:1). We found a strong correlation between renal scarring at the time of diagnosis and voiding dysfunction (p< 0.01). Cure rate after first injection was 64%. Although univariate analysis showed a strong correlation between cure and first injection, biloterality, voiding dysfunction, initial nephropathy, and ureteral dilation grade, on multivariate logistic regression the only significant factor to predict treatment response was the existence of severe ureteral dilation in comparison with mild dilation (p < 0.001; odds ratio 0.045, 95% confidence interval 0.01-0.16). Renal damage progression was specially detected in those patients with voiding dysfunction (p = 0.053), bilaterality (p = 0.034) and severe ureteral dilation (p < 0.001). CONCLUSIONS: A direct relation between cure rate after endoscopic treatment of grade III and IV reflux and severity of ureteral dilation was demonstrated. Other relevant factors implied in the endoscopic resolution of VUR include the existence of voiding dysfunction, bilateral reflux and renal damage determined by DMSA at the time of initial diagnosis. Gender, age, and number of infections during follow up have no influence in the probability of cure of grade III and IV VU reflux treated with subureteral injection of biocompatible substances.

Ectopic adrenal tissue in the spermatic cord in pediatric patients: surgical implications.

OBJECTIVE: To study the incidence and relevance of ectopic adrenal tissue in pediatric patients who underwent groin surgical explorations. MATERIALS AND METHODS: We studied 1120 patients with groin surgical explorations during a period of 8 consecutive years. Patients' clinical data and histological findings were analyzed. RESULTS: We found ectopic adrenal tissue in 13 patients in 1120 groin surgical exploration (1.16%). Of the 13 cases, 5 were diagnosed as having undescended testes, 6 inguinal hernia and 2 communicating hydrocele. Median age at diagnosis was 5.6 years. Histological sections showed adrenal cortical tissue with no medulla present. CONCLUSION: Based on the clinical implications of those adrenal rests it is mandatory the removal of this ectopic tissue whenever encountered during surgical interventions in the groin region in children.

Ectopic adrenal tissue in the spermatic cord in pediatric patients: surgical implications

OBJECTIVE: To study the incidence and relevance of ectopic adrenal tissue in pediatric patients who underwent groin surgical explorations. MATERIALS AND METHODS: We studied 1120 patients with groin surgical explorations during a period of 8 consecutive years. PatientsÆ clinical data and histological findings were analyzed. RESULTS: We found ectopic adrenal tissue in 13 patients in 1120 groin surgical exploration (1.16 percent). Of the 13 cases, 5 were diagnosed as having undescended testes, 6 inguinal hernia and 2 communicating hydrocele. Median age at diagnosis was 5.6 years. Histological sections showed adrenal cortical tissue with no medulla present. CONCLUSION: Based on the clinical implications of those adrenal rests it is mandatory the removal of this ectopic tissue whenever encountered during surgical interventions in the groin region in children.

Reflujo vesicoureteral grados III-IV: factores implicados en la efectividad del tratamiento endoscópico en pacientes pediátricos / Vesicoureteral reflux grades III-IV: factors involved in the efficacy of endoscopic treatment in pediatric patients

OBJETIVO: El Reflujo Vesicoureteral (RVU) es una de las anomalías congénitas urológicas más frecuentesy se detecta hasta en el 30-50% de los pacientespediátricos diagnosticados de Infección del Tracto Urinario (ITU). Su tratamiento es controvertido. Existen múltiples estudios clínicos que comparan los resultados de la terapéutica médica antibiótica frente a la reimplantación quirúrgica, pero desde la introducción de una tercera alternativa en el año 1984 (tratamiento endoscópico), las recomendaciones y protocolos de tratamiento del RVU han cambiado dramáticamente. El objetivo de nuestro estudio es el de describir y analizar los factores que influyen en la curación del reflujo mediante tratamiento endoscópico en sistemas con RVU grados III y IV según la Clasificación Internacional.MÉTODOS: Estudio de cohortes retrospectivo y prospectivoque analiza los 90 pacientes con RVU grados III y IV tratados endoscópicamente entre marzo de 1998 y diciembre de 2004. Sobre un total de 550 pacientes (735 unidades renales refluyentes) diagnosticados de RVU en el Servicio de Cirugía Pediátrica de A Coruña en el período de estudio, se seleccionaron sólo 184 tratadosendoscópicamente. El grupo final de estudio de 90 pacientes (130 unidades) se constituyó tras excluir los casos de reflujo secundario (vejiga neurógena, ureterocele) y aquellos con seguimiento incompleto. Todos los pacientes fueron sometidos a cistografía miccional convencional o bien a Sonocistografía con galactosa para realizar el diagnóstico, gammagrafía con DMSA para evaluar la nefropatía, ecografía renal y vesical y exploración clínica del patrón miccional. Se registraron datos sobre sexo, edad, ITU, bilateralidad, daño renal, disfunción miccional, tasa de curación, complicaciones y recurrencias. Definimos un nuevo factor: grado de dilatación ureteral independiente de la Clasificación Internacional. Este factor fue valorado por un único urólogo pediátrico sin conocer el grado de la Clasificación Internacional. Los datos fueron analizados empleando el test estadístico del chi-cuadrado y el test exacto de Fisher además de una regresión logística múltiple. Se estableció significación estadística con valores de p<0.05. Empleamos el paquete estadístico SPSS 11.0 para el estudio de los datos. RESULTADOS: La relación niños/niñas fue de 2.91. El RVU fue bilateral en 44.4%. UTI se diagnostico como forma presentación en 64 casos y el diagnóstico fue antenatal en 21 (con predominio de varones 5.2:1). Encontramos una relación estrecha entre la incidencia de daño renal en el momento del diagnóstico y existencia de disfunción miccional (p<0.01). La tasa de curación con la primera inyección fue del 64%. Mientras que en análisis univariable mostró una fuerte relación entre la curación con la 1ª inyección y la bilateralidad, disfunción miccional, nefropatía inicial y grado de dilatación ureteral, la regresión logístico multivariante el único factor realmente significativo a la hora de predecir la respuesta al tratamiento fue la existencia de una dilatación ureteral severa frente a una dilatación leve (p<0.001; odds ratio 0.045, IC 95% 0.01-0.16). La progresión del daño renal fue detectada especialmente en aquellos pacientes con disfunción miccional (p=0.053), bilateralidad (p=0.034) y dilatación ureteral severa (p<0.001). CONCLUSIONES: Se demuestra la relación directa entre la tasa de curaciones con el tratamiento endoscópico del los reflujos grados III y IV y el grado de dilatación ureteral. Otros factores relevantes implicados en la resolución endoscópica del RVU son la existencia de disfunción miccional, bilateralidad y existencia de daño renal medido por DMSA en el momento del diagnóstico inicial. El sexo, edad y número de infecciones durante el seguimiento no tienen influencia en el pronóstico de curación del reflujo de grados III y IV tratado mediante el uso de sustancias biocompatibles a nivel subureteral

OBJECTIVES: Vesicoureteral reflux (VUR) is detected in 30-50% of pediatric patients diagnosed of urinary tract infection (UTI). Multiple clinical trials have compared the results of conservative management against surgical therapy. The introduction of a third alternative in 1984 (endoscopic approach) has dramatically modified the management of VUR. The objective of our work is to analyze the factors influencing the favourable outcome of the endoscopic treatment of VUR. METHODS: Retrospective and prospective cohort study analyzing 90 patients with grades III and IV VUR treated endoscopically over the last eight years. 184 patients undergoing endoscopic management were selected from a group of 550 patients (735 VUR renal units) with the diagnosis of VUR over the study period, . All patients underwent VCUG or galactose-based sonocystography to obtain the diagnosis. DMSA scintigram was used to evaluate renal damage. Data about gender, age, UTI, bilateralism, renal damage, voiding dysfunction, outcomes, complications, and recurrences were registered. A new factor was defined: ureteral dilation independent from the international classification. This factor was evaluated by a single pediatric urologist blinded for the international classification results. Data analysis was performed using the chi square test and Fischer exact test; a multiple logistic regression analysis was also performed. Statistical significance was established at p < 0.05. SPSS 11.0 software was employed for data analysis. RESULTS: Boys/girls ratio was 2.91. Bilateral VUR appeared in 44.4% of the cases. UTI was the presenting diagnosis in 64 cases; the diagnosis was prenatal in 21 cases (with a male predominance 5.2: 1). We found a strong correlation between renal scarring at the time of diagnosis and voiding dysfunction (p< 0.01). Cure rate after first injection was 64%. Although univariate analysis showed a strong correlation between cure and first injection, bilaterality, voiding dysfunction, initial nephropathy, and ureteral dilation grade, on multivariate logistic regression the only significant factor to predict treatment response was the existence of severe ureteral dilation in comparison with mild dilation (p < 0.001; odds ratio 0.045, 95% confidence interval 0.01-0.16). Renal damage progression was specially detected in those patients with voiding dysfunction (p = 0.053), bilaterality (p = 0.034) and severe ureteral dilation (p < 0.001). CONCLUSIONS: A direct relation between cure rate after endoscopic treatment of grade III and IV reflux and severity of ureteral dilation was demonstrated. Other relevant factors implied in the endoscopic resolution of VUR include the existence of voiding dysfunction, bilateral reflux and renal damage determined by DMSA at the time of initial diagnosis. Gender, age, and number of infections during follow up have no influence in the probability of cure of grade III and IV VU reflux treated with subureteral injection of biocompatible substances

Predictive value of clinical factors for successful endoscopic correction of primary vesicoureteral reflux grades III-IV.

OBJECTIVE: To evaluate the clinical factors that influence the outcome of endoscopically treated primary grade III and IV vesicoureteral reflux (VUR). MATERIALS AND METHODS: Excluding secondary VUR and patients with incomplete follow up, a retrospective and prospective cohort observational study was performed on 90 children (130 renal units) with primary grade III or IV reflux treated endoscopically with Polytef or Macroplastique from March 1997 to January 2004. All patients underwent voiding cystourethrography (or galactose-based sonocystourethrography), dimercaptosuccinic acid (DMSA) scan, renal ultrasound and clinical exploration of micturition. Data were collected on age, gender, urinary tract infection (UTI), bilaterality, renal scarring, voiding dysfunction, and success rate. A new factor was defined, ureteral dilatation of the renal unit (not related to grade according to findings of pelvi-calyceal images), and graded by a single pediatric urologist blinded to its International Classification grade. RESULTS: The male-to-female ratio of patients was 2.91. Reflux was bilateral in 44.4%. There was UTI at presentation in 64 cases, and 21 newborns had been prenatally diagnosed (ratio M/F 5.2:1). We found a strong relation between the incidence of renal scarring at presentation and voiding dysfunction (P<0.01). Cure rate at first injection was 64%. While univariate analysis appeared to show a strong association between resolution at first injection and bilaterality, voiding dysfunction, nephropathy and ureteral dilatation grade, with simultaneous logistic regression, the only statistically significant predictor was the presence of high-grade dilatation vs low ureteral dilatation (P<0.001; OR 0.045; 95% CI 0.01-0.16). Progression of renal damage was noted especially in patients with voiding dysfunction (P=0.053), bilaterality (P=0.034), and severe ureteral dilatation (P<0.001). CONCLUSIONS: The relationship between success rate of endoscopic treatment and grade of ureteral dilatation is demonstrated. Other factors significantly involved in the resolution of VUR are voiding dysfunctional syndrome, bilateralism of reflux, and scarring on initial DMSA. Gender, age, number of infections during follow up and prenatal diagnosis do not influence the outcome of endoscopic treatment.

Kaposiform hemangioendothelioma associated with Milroy's disease (primary hereditary lymphedema).

Kaposiform infantile hemangioendothelioma (KHE) is a rare recently characterized, locally aggressive, endothelial-derived neoplasm that occurs exclusively in the pediatric age group. Milroy-Nonne disease (primary hereditary lymphedema) is an uncommon congenital entity with familiar history of lower limb edema as typical clinical features. An 8-year-old boy developed a hard painless mass in the right leg 7 years after the diagnosis of congenital primary lymphedema of the right lower extremity. Histopathological analysis of the tumor showed the typical findings of the KHE. To our knowledge this is the first reported case of a KHE engrafting on this infrequent benign lymphatic anomaly.

Treatment of monomorphic B-cell lymphoma with rituximab after liver transplantation in a child.

Rituximab, a monoclonal antibody directed against the B-cell specific CD20 antigen has been used with success in post-transplant lymphoproliferative disorder (PTLD) of B-cell phenotype. However, the use of such drug in children with liver transplantation and PTLD is very limited. We report a 2-yr-old liver transplant recipient with monomorphic non-Hodgkin lymphoma of B-cell origin. The lymphoma did not respond to immunosuppression withdrawal, with a subsequent allograft rejection. Despite resumption of immunosuppression and rejection treatment, the lymphoma was successfully treated with rituximab.

Heterotopic brain tissue in the upper lip of a neonate mimicking bilateral cleft lip features.

Heterotopic brain tissue is an extremely rare developmental anomaly frequently diagnosed in the newborn period. This entity has been described in various sites of the head and neck, most commonly in the nasal area. Computed tomography and magnetic resonance imaging are necessary for exclude associated cranial communications. Heterotopic brain tissue is an extremely rare developmental anomaly frequently diagnosed in the newborn period. This entity has been described in various sites of the head and neck, most commonly in the nasal area. Computed tomography and magnetic resonance imaging are necessary for exclude associated cranial communications.

Lengthening the urethral plate with a double flap technique: a new procedure for correction of primary hypospadias with chordee.

PURPOSE: We describe a new 1-stage technique for the surgical treatment of primary hypospadias with chordee dividing the urethral plate into 2 flaps. MATERIAL AND METHODS: The urethral plate is divided into 2 flaps in an oblique fashion. Penis straightening is achieved with resection of the fibrous ventral tissue after mobilization of the plate. The ventral side of the neo-urethra is harvested from the preputium penis with an onlay island flap or penile skin with the Mathieu "flip-flap" procedure. RESULTS: The new 1-stage procedure has been used in 6 patients. After 10-month followup a fistula developed in only 1 patient who required a new surgical procedure for correction. No meatal stenosis or urethral stricture was noticed. No dorsal plication sutures were required. All 6 patients who underwent a successful procedure had excellent cosmetic results. CONCLUSIONS: This technique is a valid surgical option in patients with hypospadias with severe chordee when we believe that the short plate tethers the penis, avoiding adequate straightening.