[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.

[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.

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.

[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.

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)

[Nostril retainers in the primary cleft rhinoplasty]. / Conformadores nasales en la rinoplastia primaria del paciente fisurado.

INTRODUCTION: The nasal cleft cartilage tends to depression after primary rhinoplasty. Nasal stents are probe to be useful in the management of these patients in order to maintain the new morphology. AIM: The purpose of this paper is to present our experience with the use of postoperative nasal splinting in the management of cleft lip nasal deformity. PATIENTS AND METHODS: A nostril retainer was placed in 18 cleft patients (4 bilateral, 14 unilateral) during primary repair of the cleft lip nasal deformity from 2001 to 2004. The nasal morphology in the postoperative period was compared with that of 10 control patients who were operated on without nasal stenting in a previous period. Nostril retainers were left in place after the rhinoplasty using 4/0 poliglecaprone absorbable. Those sutures fixed the stent in place during three weeks after surgery. Results. Average follow-up was 18.5 months. Retention of the splint was 3.5 months mean time (range 4 weeks to 6 months). The methods employed for retention were multiple based upon parental preferences. Photogrammetric analysis showed relevant asymmetry of the nostrils in 10% of the splinted group as compared with 48% for controls (p<0.001). CONCLUSIONS: The use of nasal stents has been found effective postoperatively after cleft primary rhinoplasty, avoiding relapse of the nasal reconstruction and drop of the nasal ala. Retainers also allows airway patent and avoid surgical adhesions due to nasal secretions and scarring. Parental collaboration is needed.

[Management of choledochal cyst: laparotomy or endoscopic]. / Tratamiento del quiste de colédoco: cirugía abierta o endoscópica?

The most generally accepted therapy of choledochal cyst is cystectomy and biliar derivation by laparotomy. Last years, endoscopic papilotomy by ERCP has been a valuable therapeutic alternative, no only a diagnostic method. In this study, we reviewed five pediatric patients operated in our Deparment in last five years for choledochal cyst. The initial therapy was laparotomy (n=4) and endoscopic papilotomy by ERCP (n=1) This one was made in other Hospital. Follow-up has been between one and five years. All patients are living. Four patients who were operated by laparotomy are asyntomatic. Patient who was treated by ERCP needed a new ERCP in first posoperative month. Five years ago, she had a seriuos acute pancreatitis and we decided laparotomy and biliar derivation. Since laparotomy, she had two new episodes of acute pancreatitis and she has needed a new endoscopic dilatation with ballon by ERCP. She has been asyntomatic for four months. In conclusion, we think laparotomy with biliar derivation is safer than ERCP in management of children with choledochal cyst. ERCP must be reserved to emergency situations before laparotomy or after postoperative complications, never as exclusive therapy.

[Congenital cystic adenomatoid malformation associated with pulmonary sequestration]. / Malformación adenomatoidea quística congénita asociada a secuestro pulmonar. Presentación de dos casos clínicos.

The association of congenital cystic adenomatoid malformation with pulmonary sequestration is infrequent. Has been reported less than 60 cases. We describe two patients with this association. One of this with extralobar pulmonary sequestration, and the other with an intralobar one.

Importancia del reflujo vesicoureteral en Pediatría. Seguimiento conjunto del paciente entre Nefrología y Cirugía Pediátrica / Importance of vesicoureteral reflux in Pediatrics. Joint patient follow-up between Pediatric Nephrology and Pediatric Surgery

El reflujo vesicoureteral (RVU) es la malformación nefrourológica más frecuente del recién nacido, pudiendo aparecer de forma secundaria en otras patologías malformativas, como en el caso de las valvas de uretra posterior, o ser secundario a una disfunción de la unión ureterovesical. De esta manera se distinguen dos fenotipos de pacientes, por un lado los diagnosticados en época prenatal o neonatal, generalmente varones, con afección anatómica y/o funcional de la unión ureterovesical, lo que se conoce con el nombre de "RVU primario", frente a formas postnatales en el escolar mayor, generalmente mujeres con disfunción vesical y de la unión ureterovesical, conocidas como "RVU secundario". Estas formas clínicas presentan distinta evo-lución clínica y pronóstico, con desarrollo de enfermedad renal crónica (ERC) debida a un mal desarrollo nefrourológico asociado o no a infecciones urinarias recurrentes. La técnica gold standard para diagnosticar daño renal es la gammagrafía renal DMSA Tc99 mientras que la prueba diagnóstica de RVU es la cistouretrografía miccional seriada (CUMS). El tratamiento inicial debe ser conservador optimizando medidas higiénicas, dada la posibilidad de resolución espontánea del mismo con el tiempo, principalmente de las formas leves de RVU, reservando el tratamiento quirúrgico correctivo en las formas severas y con mala evolución clínica, por el probable desarrollo de ERC que puede conducir al paciente a una enfermedad renal terminal con necesidad de técnicas de depuración extrarrenal o incluso trasplante renal. Dicho tratamiento quirúrgico será preferentemente endoscópico. Aún existe controversia en el uso de profilaxis antibiótica, recomendándose en casos concretos. Un manejo integral multidisciplinar del paciente mejorará su pronóstico renal y vital, así como su calidad de vida y la de su familia

Vesicoureteral reflux (VUR) is the most frequent nephrourological malformation of the newborn, and may appear secondary in other malformative pathologies, such as in the case of the posterior urethral leaflets, or be secondary to a dysfunction of the ureterovesical junction. In this way, two phenotypes of patients are distinguished, on the one hand those diagnosed in the prenatal or neonatal period, generally males, with anatomical and/or functional affection of the ureterovesical junction, which is known as the "primary VUR", compared to postnatal forms in the older schoolchild, generally women with bladder and ureterovesical junction dysfunction, known as "secondary VUR". These clinical forms present different clinical and prognostic evolution, with development of chronic kidney disease (CKD) due to poor nephrourological development frequently associated with recurrent urinary infections. The gold standard technique for diagnosing kidney damage is nuclear renal scanning with dimercaptosuccinic acid (DMSA), while the diagnostic test for VUR is voiding cystourethrography (VCUG). Initial treatment should be conservative, optimizing hygienic measures, given the possibility of spontaneous resolution of it over time, mainly in mild forms of VUR, reserving corrective surgical treatment in severe forms and with poor clinical evolution, due to the probable development of CKD that can lead the patient to end-stage kidney disease with the need for extrarenal clearance techniques or even kidney transplantation. Surgical treatment will preferably be endoscopic. There is still controversy in the use of antibiotic prophylaxis, being recommended in specificcases. A comprehensive multidisciplinary management of the patient will improve their renal and vital prognosis, as well as their quality of life and that of their family

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

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 in-terposicional de colágeno. Material y métodos. 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

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

Actualización en anomalías renales y de vías urinarias altas en pediatría / Update in renal and upper urinary tract anomalies in pediatrics

La urología pediátrica comprende una extensa serie de patologías de complejidad variable, que afectan al sistema urogenital, y que suponen aproximadamente el 50% de las intervenciones quirúrgicas en cirugía pediátrica. describiremos aquellas de mayor impacto y repercusión clínica desde el punto de vista quirúrgico, destacando las denominadas CaKUt o anomalías congénitas del riñón y del tracto urinario. estas últimas tienen su origen en la alteración de alguno de los procesos que conforman el desarrollo renal: a) Malformación del parénquima renal: hipoplasia, displasia, agenesia renal y riñón multiquístico (drMQ). b) anomalías relacionadas con la migración de los riñones: ectopia renal y anomalías de fusión. c) anomalías en el desarrollo del sistema colector: estenosis pieloureteral (epU), duplicidades ureterales, megauréter primario, uréter ectópico, ureterocele y válvulas de uretra posterior (VUp). El objetivo de este artículo es determinar en qué casos es necesaria la cirugía para resolver un problema urológico en el niño y cuándo es el mejor momento para realizar la intervención

Pediatric Urology comprises an extensive series of pathologies of variable complexity, which affect the urogenital system, and account for approximately 50% of surgical interventions in pediatric surgery. We will describe those ones of greater impact and clinical repercussion from the surgical point of view, highlighting the so-called CaKUt or congenital anomalies of the kidney and urinary tract. the latter have their origin in the alteration of some of the processes during the renal development: a) Malformation of the renal parenchyma: hypoplasia, dysplasia, renal agenesis and multicystic kidney (MCdK). b) abnormalities related to the migration of the kidneys: renal ectopia and fusion anomalies. c) abnormalities in the development of the collecting system: pyelo-ureteral stenosis, ureteral duplicities, primary megaureter, ectopic ureter, ureterocele and posterior uretral valves. R The objective of this article is to inform about the cases in which surgery is necessary to solve a urological problem in the child, and the best time to perform the intervention

Oncología quirúrgica pediátrica / Pediatric surgical oncology

Las neoplasias suponen la primera causa de muerte en niños mayores de 1 año en países desarrollados. la cirugía pediátrica juega un importante papel terapéutico en el cáncer infantil a distintos niveles: extirpación tumoral, implantación de accesos vasculares, manejo de metástasis y complicaciones. La oncología pediátrica es una especialidad muy dinámica con actualizaciones y cambios en protocolos constantemente. el objetivo de este artículo es la revisión actualizada de los tumores sólidos más frecuentes y con características específicas en relación con la cirugía pediátrica: neuroblastoma, tumor de Wilms, hepatoblastoma, tumores ováricos, testiculares y de partes blandas. El neuroblastoma es el tumor sólido extracraneal más común en la infancia. tanto el neuroblastoma como el tumor de Wilms suelen presentarse como una masa abdominal asintomática. la combinación del grupo de riesgo, edad, factores biológicos y resultados histológicos permite asignar a cada paciente un estadio de riesgo con valor pronóstico, y establecer una estrategia terapéutica específica en cada caso de neuroblastoma. el nefroblastoma o tumor de Wilms es el tumor renal maligno más frecuente en niños. en nuestro medio el nuevo protocolo establecido por la Siop se conoce como UMBrella que se basa en la quimioterapia preoperatoria con el objetivo de reducir masa tumoral seguido de cirugía. En los casos de hepatoblastoma es importante la clasificación preteXt (Pretreatment Extent of Disease) porque define la extensión de parénquima hepático afectada y enfermedad extrahepática dando información sobre la resecabilidad del tumor y su respuesta a la quimioterapia. Ante una masa de tejido blando debe plantearse en primer lugar que se trate de procesos reactivos y tumores benignos, los tumores malignos de partes blandas son raros. dentro de estos, el rabdomiosarcoma se caracteriza por su buena respuesta a quimioterapia, por lo que está en desuso la cirugía con resecciones agresivas o mutilantes. Respecto a los tumores gonadales en la infancia, a diferencia de los adultos, predominan los germinales, en concreto el teratoma. la cirugia tiende a ser conservadora para respetar la fertilidad futura. en las últimas décadas, los avances en tratamientos oncológicos han logrado un aumento de la supervivencia en la mayoría de tumores infantiles, en parte gracias al enfoque multidisciplinar necesario desde el diagnóstico de cada caso

Malignant neoplasms constitute the first cause of death in children over 1 year of age in developed countries. Pediatric surgery plays an important therapeutic role in childhood cancer on different sides: removing tumors, placing vascular access devices, metastasis management and complications. Pediatric oncology is a dinamyc specialty with constant updates and changes in protocols. the principal aim of this report is an updated review of more common solid tumors and their specific surgical aspects: neuroblastoma, Wilms' tumor, hepatoblastoma, ovarian tumor, testicular tumor and rhabdomyosarcoma. Neuroblastoma is the most common extracranial solid tumor in chidhood. Wilms' tumor and neuroblastoma usually appears as an asyntomatic abdominal mass. the risk group, age, biological factors and histologic analysis allow to assign a risk stage to each patient, that has prognostic value and determines the specific treatment for each case of neuroblastoma. Nephroblastoma or Wilms' tumor is the most frequent malignant kidney tumor in children. UMBrella is the new protocol of Siop based on preoperative chemotherapy to reduce the size of the tumor and surgery after. The classification PRETEXT (Pretreatment Extent of Disease) is important in cases of hepatoblastoma because defines the extent of hepatic infiltrated parenchyma, so we can know about its resectability and the response to chemotherapy. A soft-tissue mass is probably a reactive process or a benign tumor, since malignant soft-tissue tumors are rare. rhabdomyosarcoma has a good response to chemotherapy, so wide resections and radical surgery are not current techniques. With regard to gonadal tumors in childhood, unlike adults, germ tumors predominate, in particular teratoma. Sparing surgery is the current treatment to preserve future fertility. over the last several decades, the advances in cancer treatment have achieved an increased survival in most of childhood tumors, thanks to multidisciplinary approach from diagnosis

Tratamiento quirúrgico del reflujo vesicoureteral en la infancia. Actualización / Surgical treatment of vesicoureteral reflux in childhood. Updat

El tratamiento endoscópico (ET) y quirúrgico, es una de las opciones fundamentales para alcanzar la curación del Reflujo vesicoureteral (RVU) en el paciente pediátrico. La tasa de éxito del tratamiento quirúrgico del RVU, es superior al 90%. Existe controversia a la hora de elegir la modalidad de tratamiento óptimo, e individualizada por cada paciente. El objetivo de esta revisión es actualizar conocimientos, conocer las nuevas modalidades diagnósticas y terapéuticas, y obtener un algoritmo de manejo individualizado según las características propias de cada paciente. El tratamiento quirúrgico es el pilar fundamental y el más efectivo, para un tratamiento exitoso del RVU en pacientes pediátricos

Endoscopic and surgical treatment is one of the fundamental options to achieve cure of Vesicoureteral Reflux (VUR) in the pediatric patient. The success rate of surgical treatment of RVU is greater than 90%. There is controversy when choosing the optimal treatment modality, and individualized by each patient. The objective of this review is to update knowledge, learn about new diagnostic and therapeutic modalities, and obtain an individualized management algorithm according to the characteristics of each patient. Surgical treatment is the fundamental and most effective pillar for successful treatment of RVU in pediatric patients

Patología quirúrgica hepatobiliar en la infancia / Pediatric hepatobiliary surgery pathology

La patología quirúrgica hepatobiliar en la infancia posee una gran relevancia debido a la repercusión que presenta en los niños afectados, razón por la que el correcto conocimiento de la misma se correlaciona con un diagnóstico temprano y un manejo oportuno que redunda en beneficio para nuestros pacientes. la presente revisión es una actualización centrada en las cuatro principales patologías hepatobiliares pediátricas. en primer lugar, se considera la atresia de vías biliares (aVB), que es la causa quirúrgica más común de enfermedad colestásica neonatal y la primera indicación de trasplante hepático infantil. es de resaltar la evolución en el concepto de la aVB en la última década, que recientemente se ha definido como un fenotipo caracterizado por la obliteración o ausencia de las vías biliares extrahepáticas, asociado a una alteración en la formación de los conductos biliares intrahepáticos, que puede deberse a múltiples etiologías. en segundo lugar, se revisa la dilatación de la vía biliar extrahepática y/o intrahepática, que afecta con mayor frecuencia al colédoco, conocida como quiste de colédoco. Se hace énfasis en su clasificación anatómica y etiológica, en el papel relevante de la colangiorresonancia en la evaluación preoperatoria de la lesión y en el tratamiento oportuno en función del tipo de dilatación y de la condición clínica del niño. en tercer lugar, se analiza el incremento de la presión del sistema venoso portal, denominado como hipertensión portal. dicho aumento de presión puede encontrar su origen en una obstrucción venosa a nivel prehepático, hepático y posthepático. Se destaca la singularidad de la hipertensión portal en la infancia que, a diferencia de los adultos, muestran una gran proporción de pacientes con una causa prehepática, los cuales desarrollan hiperesplenismo y hemorragia secundaria a várices esofagogástricas con mayor frecuencia, pero con una baja mortalidad atribuible a dicha hemorragia y ausencia de progresión a cirrosis hepática. Finalmente, se habla de la litiasis biliar en la infancia, destacando el amplio espectro clínico que presentan los niños afectados y la variedad en la composición de los cálculos biliares en pediatría, remarcando las estrategias terapéuticas a seguir en cada caso

Pediatric hepatobiliary surgery pathology is of great relevance due to its repercussion in the affected children. For this reason, correct knowledge about it is correlated with an early diagnosis and pertinent management that results in a benefit for our patients. This review is an update focused on the four main pediatric hepatobiliary pathologies. Bile duct atresia (BVA) is considered in the first place, this being the most common surgical cause of neonatal cholestatic disease and the first indication of child liver transplant. the evolution in the concept of BVa in the last decade should be emphasized, which has recently been defined as a phenotype characterized by the obliteration or absence of the extrahepatic bile ducts, associated to an alteration in the formation of the intrahepatic bile ducts, which can be due to multiple etiologies. in the second place, dilatation of the extrahepatic and/or intrahepatic bile ducts, that with greater frequency affects the choledoch, known as choledochal cyst, is reviewed. emphasis is placed on their anatomical and etiological classification, on the relevant role of the cholangioresonance in the pre-operative evaluation of the lesion and on the pertinent treatment based on the type of dilatation and on the clinical condition of the child. in the third place, an analysis is made of the increase of the pressure of the portal venous system, called portal hypertension. Said increase in pressure can originate in a venous obstruction on the prehepatic, hepatic or post-hepatic level. the singularity of portal hypertension in children stands out which, on the contrary to in adults, shows a large proportion of patients with a prehepatic cause, these developing hypersplenism and hemorrhaging secondary to esophagogastric varices more frequently. However, they have a low mortality attributable to said hemorrhaging and absence of progression to hepatic cirrhosis. Finally, mention is made of gallstones in children, stressing the wide clinical spectrum that the affected children have and the variety in the composition of the gallstones in pediatrics, emphasizing the therapeutic strategies to follow in each case

Descenso vaginal vía perineal asistido por laparoscopia en paciente con atresia vaginal distal / Laparoscopically assisted pull-through surgery in a patient with distal vaginal atresia

La atresia vaginal distal es una anomalía infrecuente que deriva de la falta de desarrollo de los 2/3 distales de la vagina a partir del seno urogenital. Presentamos una paciente de 14 años con amenorrea primaria, abdominalgia, masa palpable en hipogastrio y ausencia de orificio vaginal. La ecografía abdominal y la RMN evidenciaron hematometrocolpos secundario a agenesia vaginal distal, con una distancia al periné de 5cm, sin otras malformaciones. Se realiza un drenaje vaginal transuretral y descenso vía perineal asistido por laparoscopia. Evolución favorable con calibre vaginal adecuado y normalización de la menstruación a los 6 meses

Distal vaginal atresia is an uncommon disorder that results from the lack of development of the distal 2/3 of the vagina from the urogenital sinus. The case is presented on a 14 year-old patient with primary amenorrhoea, abdominal pain, a palpable mass in the hypogastrium, and an absent vaginal opening. The abdominal ultrasound and magnetic resonance imaging showed haematometrocolpos secondary to distal vaginal agenesis, with a distance of 5cm to the perineum, with no other malformations. A transurethral vaginal drainage and laparoscopic assisted pull-through were performed. Her follow-up was favourable with an adequate vaginal calibre and normal menstruation after 6 months

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.

Hidronefrosis: manejo prenatal y postnatal / Hydronephrosis: perinatal and postnatal management

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