Clasificación, diagnóstico y tratamiento de los trastornos miccionales en la infancia / Classification, diagnosis and treatment of urinary disorders in childhood

La disfunción vesical o disfunción del tracto urinario inferior(DTUI), traducción literal del inglés de Lower urinary Tract Disfunction (LUTD) puede ser causa del 40% de las consultas en pediatría. Estos trastornos suponen una alteración en cualquiera de las fases del ciclo miccional y pueden responder a causas neurológicas, anatómicas o funcionales. Es muy importante realizar una adecuada historia clínica que nos permita conocer al paciente de manera global de manera que podamos lograr un correcto diagnóstico de la causa de la disfunción y así poder realizar un tratamiento dirigido. La evaluación de la mayoría de los niños con disfunción vesical puede limitarse a una anamnesis exhaustiva, un examen físico y pruebas no invasivas, como un análisis de orina y un urocultivo. En niños seleccionados, una evaluación más extensa incluye estudios de imagen urológicos, mediciones del flujo urinario y determinación del residuo postmiccional. En cuanto a las herramientas terapéuticas, partiremos de pautas higiénico-dietéticas básicas y de aplicación global a las que podremos asociar terapia conductual, farmacológica, fisioterápica o quirúrgica, siempre de manera individualizada.(AU)

Vesical disfunction or Lower urinary Tract Dysfunction (LUTD), can be the cause of 40% of pediatric consultations. Dysfunctions of the lower urinary tract involve an alteration in any of the phases of the voiding cycle and may respond to neurological, anatomical or functional causes. It is very important to take an adequate clinical history that allows us to know the patient globally so that we can achieve a correct diagnosis of the cause of the lower urinary tract dysfunction and thus be able to carry out targeted treatments. The evaluation for most children with bladder dysfunction can be limited to a thorough history, physical examination, and noninvasive testing, such as a urinalysis and urine culture. In selected children, more extensive evaluation includes urologic imaging studies, measurements of urinary flow, and post-void residual determination. As for therapeutic tools, we will start from basic hygienic-dietary guidelines of global application to which we can associate behavioral, pharmacological, physiotherapy or surgical therapy, always in an individualized manner.(AU)

Carotid glomus in childhood: presentation of a clinical case. / Glomus carotídeo en la infancia. Presentación de un caso clínico.

INTRODUCTION: Carotid glomus is an exceptional extra-adrenal paraganglioma in childhood originating at the carotid body. Only 3% of paragangliomas occur in the head and the neck. Familial forms, associated with Succinate Dehydrogenase (SDH) gene mutations, account for 10% of cases, the proportion being higher in childhood. They are benign in 95% of patients, but they can extend to both carotids. Treatment is surgical with or without previous embolization. Metastasis is rare and associated with malignant cases, which are limited. CLINICAL CASE: 8-year-old patient with a cervical mass originating 4 months ago and normal serum levels. Regarding family history, she had an aunt who underwent cervical surgery. Ultrasound examination demonstrated a greatly vascularized hypoechoic mass most likely related to carotid glomus. Full surgical resection without embolization was decided upon, which proved uneventful. The genetic study was positive for SDH gene mutation. CONCLUSION: Carotid glomus in childhood should be considered as a differential diagnosis in cervical masses. Surgical treatment without previous embolization represents a safe therapeutic option in selected cases.

INTRODUCCION: El glomus carotídeo es un paraganglioma extraadrenal, excepcional en la infancia, cuyo origen es el cuerpo carotídeo. Solo el 3% de los paragangliomas se presentan en cabeza y cuello. Existe un 10% de formas familiares asociadas a mutaciones en el gen de la succinato deshidrogenasa (SDH), porcentaje que es mayor en la infancia. Son tumores benignos en un 95% de los pacientes, pero pueden afectar por extensión a ambas carótidas. Su tratamiento es quirúrgico con o sin embolización previa. Las metástasis son raras y están asociadas a los escasos casos de malignidad. CASO CLINICO: Paciente de 8 años de edad con masa cervical de 4 meses de evolución, serologías normales. Como antecedente destaca una tía sometida a cirugía cervical. Ecográficamente se objetiva masa hipoecoica muy vascularizada en probable relación con glomus carotídeo. Se decide resección quirúrgica sin embolización que resulta completa y sin incidencias. Estudio genético positivo para la mutación en el gen de la SDH. CONCLUSION: El glomus carotídeo en la infancia ha de ser tenido en cuenta como diagnóstico diferencial en masas cervicales. El tratamiento quirúrgico sin embolización previa es una opción terapéutica segura en casos seleccionados.

Glomus carotídeo en la infancia. Presentación de un caso clínico / Carotid glomus in childhood: presentation of a clinical case

Introducción: El glomus carotídeo es un paraganglioma extraadre-nal, excepcional en la infancia, cuyo origen es el cuerpo carotídeo. Soloel 3% de los paragangliomas se presentan en cabeza y cuello. Existeun 10% de formas familiares asociadas a mutaciones en el gen de lasuccinato deshidrogenasa (SDH), porcentaje que es mayor en la infancia.Son tumores benignos en un 95% de los pacientes, pero pueden afectarpor extensión a ambas carótidas. Su tratamiento es quirúrgico con o sinembolización previa. Las metástasis son raras y están asociadas a losescasos casos de malignidad. Caso clínico: Paciente de 8 años de edad con masa cervical de 4meses de evolución, serologías normales. Como antecedente destacauna tía sometida a cirugía cervical. Ecográficamente se objetiva masahipoecoica muy vascularizada en probable relación con glomus ca-rotídeo. Se decide resección quirúrgica sin embolización que resultacompleta y sin incidencias. Estudio genético positivo para la mutaciónen el gen de la SDH. Conclusión: El glomus carotídeo en la infancia ha de ser tenido encuenta como diagnóstico diferencial en masas cervicales. El tratamientoquirúrgico sin embolización previa es una opción terapéutica segura encasos seleccionados.(AU)

Introduction: Carotid glomus is an exceptional extra-adrenal para-ganglioma in childhood originating at the carotid body. Only 3% ofparagangliomas occur in the head and the neck. Familial forms, associ-ated with Succinate Dehydrogenase (SDH) gene mutations, account for10% of cases, the proportion being higher in childhood. They are benignin 95% of patients, but they can extend to both carotids. Treatment issurgical with or without previous embolization. Metastasis is rare andassociated with malignant cases, which are limited. Clinical case. 8-year-old patient with a cervical mass originating4 months ago and normal serum levels. Regarding family history, shehad an aunt who underwent cervical surgery. Ultrasound examinationdemonstrated a greatly vascularized hypoechoic mass most likely relatedto carotid glomus. Full surgical resection without embolization wasdecided upon, which proved uneventful. The genetic study was positivefor SDH gene mutation.Conclusion: Carotid glomus in childhood should be considered as adifferential diagnosis in cervical masses. Surgical treatment without pre-vious embolization represents a safe therapeutic option in selected cases.(AU)

[Multilayer repair of palatal fistula with an interpositional collagen matrix]. / Reparación multicapa de la fístula palatina con interposición de matriz de colágeno.

OBJECTIVE: Palatal fistula after the repair of cleft palate appears in 7.7-35% of patients. We present two cases of palatal fistula, detailing a multi-layer repair with an interpositional collagen graft. MATERIAL AND METHODS: Patient 1: girl with a cleft palate operated using a Furlow technique. A reintervention was performed due to a Pittsburgh type III fistula. Patient 2: male with cleft palate operated using a Furlow technique. A reintervention was performed due to a type V fistula. RESULTS: We used a multilayer repair with a local rotational flap and the interposition of a collagen matrix between the nasal and oral layers. The suture was reinforced with a fibrin hemostatic adhesive. No recurrence of the fistula after 2 years. CONCLUSIONS: The three-layer closure is simple, safe, effective and avoids refistulizations. Interpositional grafts of a resorbable collagen membrane provide a "scaffold" for tissue growth, revascularization and epithelialization of the mucosa.

OBJETIVO: La fístula palatina tras la reparación del paladar fisurado aparece en un 7,7-35% de pacientes. Presentamos dos casos de fístula palatina, detallando la técnica de reparación multicapa con injerto interposicional de colágeno. MATERIAL Y METODOS: Paciente 1: niña con fisura de paladar blando, operada mediante técnica de Furlow. Se programa reintervención por fístula secundaria tipo III de Pittsburgh. Paciente 2: varón con fisura de paladar blando, operado mediante técnica de Furlow. Se programa reintervención por fístula secundaria tipo V. RESULTADOS: Reparación multicapa mediante flap rotacional y matriz de colágeno entre las capas nasal y oral. Refuerzo con adhesivo hemostático de fibrina. Ausencia de recidiva tras 2 años de seguimiento. CONCLUSIONES: El cierre en tres capas es sencillo y efectivo a la hora de evitar refistulizaciones. Los injertos interposicionales de membrana reabsorbible de colágeno proporcionan un "andamio" para el crecimiento de los tejidos, revascularización y epitelialización de la mucosa.

[Laparoscopic assisted percutaneous treatment of inguinal hernia in infants]. / Tratamiento percutáneo asistido por laparoscopia de la hernia inguinal infantil.

INTRODUCTION: Thanks to the generalization of minimally invasive surgery in pediatric patients some classic techniques are being replaced. Inguinal hernia repair may be an example. MATERIAL AND METHODS: We present the technique and our first results in 10 patients treated by percutaneous laparoscopic assisted herniotomy. For this purpose we used an umbilical port, for a 5 mm camera and an optional 2 mm grasper. By laparoscopic guidance we make two inguinal punctures with a 20G needle that introduces a non absorbable suture that crosses the whole inguinal defect and allows it closure by extracorporeal knot tying. RESULTS: The ages ranged between 1 month and 8 years with a median age of 10 months. 50% of the patients were boys. 50% of the patients had previous diagnosis of inguinal bilateral hernia. 30% of patients presented a postoperatory diagnosis different from the previous one. The average time of intervention was 32 minutes in bilateral hernias, and 19 in the unilateral ones. Follow-up time was 6 months without complications. The aesthetic result was ideal. CONCLUSION: Percutaneous laparoscopic assisted herniotomy is an effective, simple and feasible option for inguinal hernia in chidren without a high rate of complications.

Tratamiento percutáneo asistido por laparoscopia de la hernia inguinal infantil / Laparoscopic assisted percutaneous treatment of inguinal hernia in infants

Introducción. Gracias a la generalización de la cirugía mínimamente invasiva en pacientes pediátricos se están sustituyendo algunas técnicas clásicas, como el tratamiento de la hernia inguinal, por nuevos abordajes asistidos por laparoscopia. Material y métodos. Presentamos nuestra técnica y nuestros primeros resultados de 10 pacientes tratados mediante herniotomía inguinal percutánea asistida por laparoscopia. Para ello utilizamos un puerto umbilical, por el que introducimos una óptica de 5 mm y una pinza opcional de 2 mm. Mediante dos punciones a nivel inguinal con una aguja de epidural de 20 G se introduce un hilo de sutura de 3/0 irreabsorbible que recorre todo el trayecto del orifico y permite que, al traccionar de este es de la piel, se cierre el orifico inguinal, quedando el nudo de sutura bajo una incisión subcutánea de 2 mm. Resultados. Las edades de los pacientes varían entre 1 mes y 8 años con una mediana de 10 meses. El 50% de los pacientes fueron niños. El 50% de los pacientes tenían diagnóstico previo de hernia inguinalbilateral y el resto, unilateral, 3 de los 10 pacientes presentaron un diagnóstico postoperatorio distinto al previo. El tiempo de intervención medio fue de 32 minutos en hernias bilaterales, mayor en varones, y 19 (..) (AU)

Introduction. Thanks to the generalization of minimally invasivesurgery in pediatric patients some classic techniques are being replaced. Inguinal hernia repair may be an example. Material and methods. We present the technique and our first results in 10 patients treated by percutaneous laparoscopic assisted herniotomy. For this purpose we used an umbilical port, for a 5 mm camera and an optional2 mm grasper. By laparoscopic guidance we make two inguinal punctures with a 20G needle that introduces a non absorbable suture that crosses the whole inguinal defect and allows it closure by extracorporeal knot tying. Results. The ages ranged between 1 month and 8 years with a median age of 10 months. 50% of the patients were boys. 50% of the patients had previous diagnosis of inguinal bilateral hernia. 30% of patients presented a postoperatory diagnosis different from the previous one. The average time of intervention was 32 minutes in bilateral hernias, and 19 in the unilateral ones. Follow-up time was 6 months without complications. The aesthetic result was ideal. Conclusion. Percutaneous laparoscopic assisted herniotomy isan effective, simple and feasible option for inguinal hernia in chidren without a high rate of complications (..) (AU)

Nefroureterectomía laparoscópica en el tumor de Wilms / Laparoscopic nephroureterectomy in wilms tumor

Introducción. La cirugía mínimamente invasiva (CMI) en el tratamiento de tumores malignos en la infancia comienza a tener un importante papel con resultados similares a la cirugía abierta en casos seleccionados. Presentamos nuestros dos primeros casos de tumor de Wilms tratados por laparoscopia. Material y métodos. Caso 1: niña de tres años de edad que acude or hematuria macroscópica. En el diagnóstico por imagen se identifi ca masa de 8 cm en riñón izquierdo en probable relación con tumor de Wilms. Tras 4 semanas de quimioterapia se realiza nefroureterectomía laparoscópica. El resultado anatomopatológico es tumor de Wilms estadio I de alto riesgo anaplásico. Se completa el tratamiento quimioterápico7 meses más. Caso 2: paciente de 5 años, diagnosticada de tumoración renal derecha de 6,5 cm en una ecografía realizada por abdominalgia en probable relación con tumor de Wilms. Después de 4semanas de quimioterapia se realiza nefroureterectomía laparoscópica. El resultado anatomopatológico es tumor de Wilms estadio II de alto riesgo (blastematoso). Se completa el tratamiento con quimioterapia ostoperatoria. Resultados. Después de 1 año de seguimiento ambas pacientes se encuentran libres de enfermedad. Conclusión. La CMI en casos seleccionados de tumor de Wilms cumple con éxito los objetivos del tratamiento quirúrgico (AU)

Introduction. Minimally invasive surgery (CMI) for the treatment of malignant tumors in children begins to have a role for selected cases and reaches similar results than open surgery. We show our first two cases of Wilms tumor treated by laparoscopy describing patients and technique. Material and methods. Three-year-old girl with macroscopic hematuriais diagnosed of 8 cm mass in the left kidney suggesting Wilms tumor. After 4 weeks of chemotherapy she went under laparoscopic nephroureterectomy. The histological result was Wilms tumor. Chemotherapy was completed seven more months. Five-year-old patient with abdominal pain is diagnosed of renal right mass suggesting Wilms tumor. After 4 weeks of chemotherapy the laparoscopic nephroureterectomy was performed. The histological result was Wilms tumor. Treatment was completes with postoperatory chemotherapy .Results. After 1 year follow-up both patients have clinical and radiological absence of disease. Conclusion. The CMI in selected cases of Wilms tumor fulfi llssuccessfully the aims of the surgical treatment (AU)

[Laparoscopic nephroureterectomy in Wilms tumor]. / Nefroureterectomía laparoscópica en el tumor de Wilms.

INTRODUCTION: Minimally invasive surgery (CMI) for the treatment of malignant tumors in children begins to have a role for selected cases and reaches similar results than open surgery. We show our first two cases of Wilms tumor treated by laparoscopy describing patients and technique. MATERIAL AND METHODS: Three-year-old girl with macroscopic hematuria is diagnosed of 8 cm mass in the left kidney suggesting Wilms tumor. After 4 weeks of chemotherapy she went under laparoscopic nephroureterectomy. The histological result was Wilms tumor. Chemotherapy was completed seven more months. Five-year-old patient with abdominal pain is diagnosed of renal right mass suggesting Wilms tumor. After 4 weeks of chemotherapy the laparoscopic nephroureterectomy was performed. The histological result was Wilms tumor. Treatment was completes with postoperatory chemotherapy. RESULTS: After 1 year follow-up both patients have clinical and radiological absence of disease. CONCLUSION: The CMI in selected cases of Wilms tumor fulfills successfully the aims of the surgical treatment.

[Role of alpha antagonists in uncoordinated micturition syndrome in childhood]. / Papel de los alfa-antagonistas en el síndrome de micción no coordinada en la infancia.

INTRODUCTION: Dysfunctional voiding syndrome in children is characterized by a pattern of dysfunctional bladder emptying due to an active contraction of the external sphincter during micturition. Diagnosis is based on electromyographic and flowmetry results. The treatment is focused on relaxing the external sphincter during micturition where biofeedback is the treatment of choice. By the moment there are still centres without this possibility, alpha blockers are an alternative. OBJECTIVE: To determine the efficacy of alpha blockers as an alternative to biofeedback as a therapeutic possibility. MATERIAL AND METHODS: We included a total of 17 children with dysfunctional voiding syndrome and carried out a retrospective study. We registered age, symptoms at diagnosis, presence of associated urologic problems, flowmetry results pre and post-treatment, type of treatment used and its effectiveness comparing patients treated with alpha blockers and those who are starting to deal with biofeedback. RESULTS: There were 12 girls and 5 boys. The mean age at diagnosis was 4.9 years old, 88% of these children related enuresis, diurnal urinary incontinence and urgency, 57% of them had also urinary infections, 63% constipation, 36% had psychosocial problems. Ten patients were treated with alpha-antagonists: 6 with Tamsulosin and 4 with Doxazosin. They followed this treatment an average of 5.8 months, range between 2 and 12 months. Five patients were treated with biofeedback. All cases had an abnormal pelvic electromyography. Patients treated with alpha-blockers achieved a 70% of electromyographic improvement with a 70% of recurrence. In children treated with biofeedback we got improvement in 80% with no recurrence. After alpha blocker therapy, maximum flow rates and average flow values were better but not statistically significant, this difference was significant with biofeedback. A patient treated with Tamsulosin left treatment due to hypotension, 2 patients left Doxazosin because of dizziness. CONCLUSIONS: Alpha-blockers are effective in the treatment of dysfunctional voiding syndrome with a high percentage of recurrence. They can be an alternative to biofeedback but this one is the effective and definitive treatment.