Rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation (ROHHAD syndrome): A case report and literature review. / Síndrome ROHHAD (obesidad de rápida progresión, disfunción hipotalámica, hipoventilación y disregulación autonómica). Presentación de un caso y revisión de la literatura.

INTRODUCTION: ROHHAD syndrome (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation) is a rare and complex disease, presenting in previously healthy children at the age of 2-4 years. Up to 40% of cases are associated with neural crest tumours. DEVELOPMENT: We present the case of a 2-year-old girl with symptoms of rapidly progressing obesity, who a few months later developed hypothalamic dysfunction with severe electrolyte imbalance, behaviour disorder, hypoventilation, and severe autonomic dysregulation, among other symptoms. Although the pathophysiology of this syndrome remains unclear, an autoimmune hypothesis has been proposed for ROHHAD. Therefore, after obtaining a limited response to intravenous immunoglobulins, we decided to test the response to a high dose cyclophosphamide (low dose was not effective either). Unfortunately our patient experienced many severe complications (among them central pontine myelinolysis, from which the patient recovered, and failure to wean from the ventilator requiring tracheostomy and long term ventilation) that required a prolonged ICU stay. Although her behaviour improved, our patient unfortunately died suddenly at home at the age of 5 due to respiratory pathology. CONCLUSIONS: ROHHAD syndrome is a rare and little-known disease which requires a multidisciplinary approach because it involves complex symptoms and multiple organ system involvement. Alveolar hypoventilation should be identified early and appropriate treatment should be started promptly for the best possible outcome. Immunomodulatory treatment with immunoglobulins, cyclophosphamide, or rituximab has previously resulted in symptom improvement in some cases. Because of the low incidence of the syndrome, multi-centre studies must be carried out in order to gather more accurate information about ROHHAD pathophysiology and design an appropriate therapeutic approach.

[Prevention of the wound infection: little changes, huge results]. / Resultados de la modificación de la actitud ante la asepsia en cirugía neonatal: pequeños cambios, grandes resultados.

INTRODUCTION: Neonatal surgical wound infection occurs in almost 50% of neonatal procedures. It increases the rates of morbimortality in neonatal units. There is no guidelines about prevention of wound infection in neonatal surgery. We present our results after changing our behaviour in neonatal surgery. MATERIALS AND METHODS: Comparative study between 2 groups. In order to decrease wound infection at the end of the procedure gloves, covertures and surgical instruments were changed and saline and antiseptic solutions were used during laparotomy closing. Group P included procedures with these recommendations and Group NP without them. Age, weight, surgery, infection, length of stay, and mortality were analized between groups through a logistic regression model. RESULTS: Group P included 55 procedures in 32 patients, median weight 1,300 g (1,000-2,100), 19 median days of life (6-40), 5 postoperative wound problems (9%). Group NP included 26 procedures in 14 neonates, median weight 1,700 g (700-2,500), 20 median days of life (3-33), 14 wound problems (53.8%). We decrease the wound problems in our patients in 44.8% (p < 0.0001). Additionally, the protection provided by our recommendations was maintained after adjustment by weight, age and type of pathology (0.07) p= 0.000. CONCLUSIONS: Simple changes in during the procedures in neonatal surgery can reduce the appearance of wound infection and morbidity.

OBJETIVOS: La infección de herida quirúrgica neonatal tiene una incidencia de hasta el 50% y produce un aumento de morbimortalidad. No existen recomendaciones preventivas consensuadas en la literatura. Presentamos los resultados tras la implantación de un protocolo creado en nuestro Servicio para cierre de laparotomía en cirugía neonatal contaminada y sucia. MATERIAL Y METODOS: Estudio ambispectivo de las laparotomías neonatales realizadas durante 32 meses comparando los resultados con un grupo de pacientes intervenidos durante un período previo similar. El protocolo incluye cambio de guantes, de campo y material quirúrgico, lavado por planos con antiséptico y sutura antibacteriana en el cierre. Se analizan edad, peso, tipo de intervención, infección, estancia hospitalaria y mortalidad y se compara con un grupo similar de pacientes intervenidos de forma previa a la creación del protocolo (NP) y se analiza la influencia del protocolo en la aparición de infección mediante regresión logística. RESULTADOS: El grupo P incluye 55 laparotomías en 32 neonatos con mediana de peso 1.300 g (1.000-2.100), mediana de edad 19 días (6-40) con 3 infecciones de herida (2 cultivos positivos) y 2 dehiscencias leves (9%). El grupo NP incluyó 26 intervenciones en 14 neonatos, mediana de peso 1.700 g (700-2.500), mediana de edad 20 días (3-33), 14 infecciones (53,8%), 8 cultivos positivos y 2 muertes. Se redujo un 44,8% la aparición de infección (p < 0,0001) y el efecto protector del protocolo se mantuvo después del ajuste por peso, edad y tipo de patología (0,07) p= 0,000. CONCLUSIONES: La sencilla modificación de la asepsia y técnica de cierre ha contribuido a disminuir considerablemente la tasa de infección y morbimortalidad en nuestros pacientes y consideramos que es necesario hacer conciencia de ello.

[Use of ultrasound for placement of central venous catheters in pediatrics: results of a national survey]. / Uso de la ecografía en la colocación de catéteres venosos centrales en pediatría: resultados de una encuesta nacional.

OBJECTIVES: To study the variability of techniques used for vascular access of central venous devices, totally implanted and external tunneled, as well as polling the use of ultrasound by pediatric surgeons in Spain. MATERIAL AND METHODS: Descriptive study of a survey results, conducted by phone, email and online, about 20 items related to the placement of these devices in children and the use of ultrasound in this procedure. RESULTS: We analyzed 71 surveys from 31 national hospitals. The response rate was 54%. In 66% of the cases the pediatric surgeon is the one who carries out the vascular access. 75% of the respondents place less than 25 catheters/year. Only 28% have attended to an ultrasound-guided vascular puncture course. The vein used more often is the internal jugular (55%), followed by the subclavian (17.4%), with preference for the right side in most of the cases (87%). 64% perform the ultrasound-guided technique, 29% the venous cutdown and 7% the landmark approach. There is no significant association between the technique chosen (landmark vs ultrasound-guided) and the vein used (p= 0,062). The majority of the respondents does not modify the usual approach in case of coagulopathy. We describe the complications associated with the three techniques. CONCLUSIONS: The preferred via for the vascular access by pediatric surgeons is the internal jugular vein, being this access ultrasound-guided in most of the cases. 91.5% consider the use of ultrasound decreases the number of associated complications. Nevertheless, just a minority has attended to a training course.

OBJETIVOS: Estudiar la variabilidad de técnicas en el acceso vascular de catéteres intravenosos centrales totalmente implantados y tunelizados externos, así como indagar sobre la utilización de la ecografía por parte de los cirujanos pediátricos en España. MATERIAL Y METODOS: Estudio descriptivo de los resultados de una encuesta realizada por vía telefónica, correo electrónico y on-line, acerca de 20 ítems relacionados con la colocación de estos dispositivos en niños y el uso de la ecografía. RESULTADOS: Se analizaron 71 encuestas de 31 hospitales españoles. La tasa de respuesta fue del 54%. En el 66% de los casos es el cirujano el que realiza el acceso vascular. El 75% de los encuestados coloca menos de 25 catéteres/año. Solo el 28% realizó algún curso de punción ecoguiada. La vena más utilizada es la yugular interna (55%), seguida de la subclavia (17,4%), prefiriendo la mayoría el lado derecho (87%). Un 64% realiza punción ecoguiada, un 29% venotomía y un 7% punción por referencias anatómicas, sin asociación significativa entre la técnica de punción elegida (referencias anatómicas vs ecoguiada) y la vena utilizada (p= 0,062). La mayoría no modifica la técnica habitual ante la presencia de coagulopatía. Se describen las complicaciones asociadas a las tres técnicas. CONCLUSIONES: La vía preferida por los cirujanos pediátricos encuestados para el acceso vascular es la vena yugular interna, siendo ecoguiado en la mayoría de los casos. Un 91,5% opina que la ecografía disminuye el número de complicaciones asociadas, sin embargo solo una minoría ha realizado algún curso de formación.

[Management protocol in gastroschisis]. / Protocolo de manejo en las gastrosquisis.

AIM: There are two classical managements in gastroschisis: primary or delayed closure. From 2007 on, a multidisciplinary approach protocol based in preterm caesarean delivery at 34-35th gestational age (in order to minimize peel injury) plus primary closure is followed in our institution. Prior to this, term delivery, either by caesarean section or not, was the rule. METHODS: Retrospective study of gastroschisis's patients before (1978-2006) and after (2007-2016) protocol approval. Complex gastroschisis frequency, peel's presence, primary or delayed closure, complications, re-interventions, neonatal intensive care unit (NICU) length of stay, age at onset of enteral nutrition, total parenteral nutrition's length and length of stay were compared. MAIN RESULTS: Study groups: before (12 patients) and after (13 newborns) the protocol implantation (pre/post). Nine complex gastroschisis were observed in the pre-protocol period, compared to one in the post-protocol era (p= 0.001). The re-interventions also decreased: 58.33% in the pre-protocol vs. 15.38% in the post-protocol group (p= 0.025). Complications rate were 66.67% before protocol vs. 23.07% after protocol (p= 0.028). NICU length of stay decreased from 40 ± 32 to 17 ± 12 days (p= 0.025). Parenteral total nutrition length was 61 ± 36 in the pre-protocol vs. 15 ± 6 in the port-protocol eras, respectively (p= 0.043). The age at onset of enteral nutrition was 34 ± 26 vs. 9 ± 5 days (p= 0.004) and hospitalization length was 98 ± 81 days pre vs. 35 ± 15 days post protocol implantation (p= 0.011). CONCLUSIONS: The protocol implantation has allowed primary closure, decreased hospitalization; reduced complications and mortality rate as well.

OBJETIVOS: Clásicamente existen dos manejos de las gastrosquisis: cierre directo o diferido. Nosotros apostamos por el cierre directo aplicando un protocolo de actuación en el que se programa cesárea a las 34-35 semanas de gestación para minimizar el "peel". MATERIAL Y METODO: Estudio retrospectivo de los pacientes con gastrosquisis en los períodos pre (1978-2006) y post (2007-2016) implantación del protocolo, comparando frecuencia de gastrosquisis complejas, la presencia o ausencia de peel, cierre directo o diferido, complicaciones, reintervenciones, días de ingreso en unidad de cuidados intensivos, edad al inicio de nutrición enteral, nutrición parenteral total y días de hospitalización. RESULTADOS: Grupo de estudio: previo (12) y posterior (13) al protocolo (previo/post). La frecuencia de gastrosquisis complejas en el período previo fue de 9 neonatos, en el período post solo 1 (p= 0,001). El número de reintervenciones fue de 58,33% previo vs. 15,38% post (p= 0,025). Las complicaciones postoperatorias fueron 66,67% previo frente a 23,07% post (p= 0,028) y existió una disminución de los días de estancia en la unidad de cuidados intensivos de 40 ± 32 vs. 17 ± 12 (p= 0,025) y de los días de nutrición parenteral (61 ± 36 frente a 15 ± 6; p= 0,043). La edad al inicio de la nutrición enteral fue de 34 ± 26 vs. 9 ± 5 días (p= 0,004) y los días de hospitalización fueron de 98 ± 81 días previo frente a 35 ± 15 días post protocolo (p= 0,011). CONCLUSIONES: La implantación del protocolo ha permitido el cierre directo, la disminución de la estancia hospitalaria, de las complicaciones y de la mortalidad.

[Utility of ultrasonography in the placement of long-term venous catheters in newborns and infants]. / Utilidad de la ecografía en la colocación de catéteres venosos de larga duración en neonatos y lactantes.

OBJETIVES: The implantation of long duration intravenous catheters in pediatric population constitutes a challenge due to the size of vascular structures. Because of that, ultrasound is an important tool for vascular accesses in newborns and infants. The objective is to analyze our experience in ultrasound-guided implantation of reservoir type and tunneled catheters, as well as to compare it between both age groups. MATERIAL AND METHODS: Review of the ultrasound-guided implantable catheters placed from October 2010 to December 2014 in children under 18 months. RESULTS: 32 devices were placed in 4 neonates and 28 infants. Mean age was of 252 days and mean weight of 6,7 kg. 23 of them were reservoir type devices and 9 tunneled. No statistically significant differences were found for mean surgical time (55 versus 52 minutes). In tunneled catheters implantation there was no difference in surgical time between neonates and infants, but there was difference for reservoir type catheters (72 minutes in neonates vs 53 minutes in infants). In 78,5% of infants it was possible to place the catheter in the first puncture, versus 50% of neonates, although it was not statistically significant. As complications, one hematoma and one postoperative infection were described. CONCLUSIONS: We found that implantation of reservoir type catheters in infants constituted less surgical time compared with neonates, and puncture on left axillary vein was the most effective. Axillary vein ultrasound-guided approach is a safe and reproducible procedure that needs a process of formation and training.

OBJETIVOS: La colocación de catéteres intravenosos de larga duración en Pediatría constituye un reto debido al tamaño de las estructuras vasculares. Por ello, la ecografía es una herramienta importante para los accesos vasculares en recién nacidos y lactantes. El objetivo es analizar nuestra experiencia en la colocación guiada por ecografía de los catéteres tipo reservorio y tunelizados así como compararla en estos dos grupos etarios. MATERIAL Y METODOS: Revisión de los catéteres colocados mediante punción ecoguiada desde octubre de 2010 a diciembre de 2014 en niños menores de 18 meses. RESULTADOS: Se colocaron 32 dispositivos en 4 neonatos y 28 lactantes. La media de edad fue 252 días y el peso medio, 6,7 kg. 23 fueron dispositivos tipo reservorio y 9 tunelizados. No se encontraron diferencias estadísticamente significativas para el tiempo quirúrgico medio empleado en ambos dispositivos (55 vs 52 minutos). En la colocación de catéteres tunelizados no hubo diferencia en el tiempo quirúrgico entre neonatos y lactantes, pero sí para los reservorios (72 minutos en neonatos vs 53 minutos en lactantes). En el 78,5% de los lactantes se logró canalizar en el primer intento frente al 50% en los neonatos, aunque esto no fue estadísticamente significativo. Como complicaciones se describieron un hematoma y una infección postoperatoria. CONCLUSIONES: Encontramos que la colocación de reservorios en lactantes supuso un menor tiempo quirúrgico comparado con los neonatos, y la punción sobre vena axilar izquierda fue más efectiva. El abordaje ecoguiado de la vena axilar es un procedimiento seguro y reproducible, que requiere un proceso de formación y entrenamiento.

[Neonatal surgical site infection: multicentric survey on prophylactic measures]. / Infección de herida quirúrgica neonatal: encuesta multicéntrica sobre medidas profilácticas.

OBJECTIVES: Surgical site infection (SSI) has a considerable impact on neonatal morbidity. There are known risk factors such type of surgery (clean/contaminated), prematurity, surgical length, hypoalbuminemia, previous infection, prolonged mechanical ventilation, and so on. Many perioperative factors have not been studied, opposite to adults. We have developed a survey on intraoperative attitudes and measures, as surgical wound management in Neonates among pediatric surgeons, to seek for a wider consense. METHODS: Multi-response survey with 22 items, based on the Surgical Infection Society NIH 2008 clinical guideline. Each item poses a question on perioperative attitudes, surgical aspects and wound management. Each question is subdivided in two categories, depending on urgency and type of surgery (clean/contaminated). RESULTS: 159 surveys were sent. Among those, we received back 51 (32%). 69% of the interviewed surgeons use clorhexidin to prepare surgical field, 25% use Iodine solutions. 69% never use diathermy to incise skin. There was no agreement on the use of sterile plastic adhesive drapes, intra-cavity lavage, changing surgical gloves/material, or wound irrigation during closure. 82% never use cyanocrilate dressing. Intracuticular skin suture and simple stitches were used indistinctly. Wound management and dressings were not uniform and depended on each pediatric unit. CONCLUSIONS: The survey reflects the lack of consensus regarding prophylactic measures and wound management among pediatric surgeons who care after surgical neonates.

OBJETIVOS: La infección de herida quirúrgica (IHQ) es una causa frecuente de morbimortalidad en Neonatología. Existen factores de riesgo conocidos: tipo de cirugía (sucia/contaminada/limpia), prematuridad, duración de intervención, hipoalbuminemia, infección previa, ventilación mecánica prolongada y contaminación de vía central. Otros factores perioperatorios no han sido estudiados en neonatos, sí en adultos. Desarrollamos una encuesta sobre las actitudes y las medidas, intraoperatorias y de manejo de herida. Pretendemos dilucidar si existe consenso sobre la profilaxis de IHQ neonatal. METODOS: Encuesta con 22 ítems, basada en la guía de prevención de la IHQ en adultos (Surgical Infection Society, 2008). Cada ítem cuestiona temas sobre hábitos perioperatorios, intra y postoperatorios, relacionados con el manejo de la herida quirúrgica en el neonato, y consta de dos preguntas, según sea cirugía sucia/urgente o limpia/contaminada. Las opciones de respuesta son 4: siempre, con frecuencia, raramente y nunca. Enviamos la encuesta a cirujanos pediátricos españoles mediante correo electrónico en el primer trimestre del 2012. RESULTADOS: Se enviaron 159 encuestas. 51 (32%) respondieron. 69% emplean clorhexidina para preparar campo, 25% usan povidona iodada. Solo el 51% refirió dejar actuar siempre un minuto el antiséptico. 69% nunca usan bisturí eléctrico en piel para ampliar herida. No hubo respuestas unánimes en cuanto al uso de paños adhesivos protectores del campo, la irrigación de planos con suero o antisépticos durante el cierre, el empleo de drenajes, cambio de guantes y/o material al iniciar el cierre (cirugía sucia). El 72% y el 82% nunca emplean cianocrilato como cierre o apósito en neonatos, respectivamente. En cirugía sucia, 43% suturan piel con intradérmica y 49% con puntos sueltos. CONCLUSIONES: La distribución de respuestas refleja la falta de consenso sobre los aspectos de la técnica quirúrgica y el manejo perioperatorio que podrían estar relacionados con la profilaxis de IHQ neonatal.

[Gallbladder motility and long term results in cholecystolithotomy]. / Estudio de la función vesicular y resultados a largo plazo de la colecistolitotomía.

INTRODUCTION: Gallstone removal preserving the gallbladder is a therapeutic option in cholelithiasis. Our group has made use of this technique for twenty-five years in pediatric patients. The purpose of this review was to study gallstone recurrence and gallbladder motility. MATERIALS AND METHODS: Patients who had gallstones removed with this technique from 1989 until 2013 were contacted and asked about digestive or other health problems. A sonographic assessment was made in order to look for gallstone recurrence and to measure gallbladder volumes and contraction after fasting and after consuming a fatty meal. RESULTS: All patients are asymptomatic except a girl who suffered colic pain and who had a gallstone recurrence. The median of volume after fasting was 18.37 cc and after consuming the meal 7.16 cc. The median of gallbladder contraction was 57.63%. CONCLUSIONS: A gallstone relapse was identified (10%). Long-term follow up showed an excellent gallbladder function and contraction (greater than 30%) without other recurrences. Based on our experience, in patients properly selected cholecistolithotomy is an interesting option in children.

[Quality of life and long term results in Hirschsprung's disease]. / Calidad de vida y resultados a largo plazo en pacientes con enfermedad de Hirschsprung.

INTRODUCTION: The most frequent long term side effects of Hirschsprung's disease (HD) surgery are obstructive problems and fecal incontinence. In this study, we analyse long term functional results of the two most used surgical techniques (Duhamel-D- and De la Torre-dlT-) from the patient's point of view, through quality of life and functionality questionnaires. METHODS: We selected short segment HD patients ≥ 4 years) that were operated in our unit from 1996 until 2011. We employed two validated questionnaires: Bowel Function Score (BFS) and Gastrointestinal Quality of Life Index (GIQLI). Both questionnaires were also tested in two control age matched groups, each for every arm (controlD or control dlT). RESULTS: Among 28 preselected patients, 22 (78.6%) answered both questionnaires. Group D (10 patients) showed more constipation: 60% vs. 16.7% ( p = 0.01). Patients in group dlT (12 patients) showed more leaks: 58.3 vs. 10% (p = 0.03). Results of both questionnaires were higher (better) in Group D: 16 vs. 12.8 points (BFS, p = 0.007) and 74.1 vs. 69.8 (GIQLI, p = 0.17). The control group showed an overall better scores than HD patients: 17.2 vs. 14.3 (BFS p = 0.001) and 75.9 vs. 71.8 (GIQLI, p = 0.04). Separately, both groups of patients showed worse scores when compared with each control group. DISCUSSION: Our results are similar to other studies, where global scores of functional results and quality of life are worse in operated HD patients than in age matched controls. Fecal incontinence has more impact on social scores than constipation. Due to the fact that our dlT patients have more frequently fecal leaks, their scores are worse than in the D group. Nevertheless, patients in dlT group are younger and may improve their results as they get older, as it is often the case in operated HD patients.

[Hepatopulmonary hydatidosis treatment through minimally invasive surgery in our hospital: report of 4 cases]. / Tratamiento de la hidatidosis hepatopulmonar con cirugía mínimamente invasiva en nuestro hospital. A propósito de 4 casos.

OBJECTIVES: The aim of this review is to present our experience in minimally invasive surgical procedures as treatment of hepatic and pulmonary hydatidosis in the last five years. MATERIAL AND METHODS: Retrospective review of patients with pulmonary and hepatic hydatid cysts treated in our hospital between 2008-2012 with minimally invasive surgery. RESULTS: We identified 4 cases, all of them Moroccan origin, the mean age was 6.7 years. Only one patient with hepatic cyst and three with hepatopulmonary cysts. All of them were treated with albendazole before surgery. Laparoscopy and/or thoracoscopy was performed and, in one case a transdiaphragmatic approach was used, as a way to reach a posterior hepatic injury unapproachable by laparoscopy. The technique used was the sterilization of the contents of the cyst with hypertonic saline solution, cyst opening and cystic membrane removal. We didn't do capitonage or pericystectomy. The resolution rate of residual cavities of the cysts was 100% and there was no recurrence or intraoperative complications. CONCLUSIONS: In children surgical treatment can be performed using endoscopic techniques with the same safety and efficacy as open surgery. Treatment with albendazole before surgery is helpful in preventing recurrences and as unique treatment in small lesions. Surgery must be as sparing as possible avoiding unnecessary liver or lung resections. Treatment of the residual cavity seems unnecessary. The thoracoscopic transdiaphragmatic approach should be considered as an alternative treatment, for those liver lesions in posterior segments, which are intractable with laparoscopy.

[Functional results of Hirschsprung's disease patients after Duhamel and De la Torre procedures]. / Resultados funcionales en pacientes con enfermedad de Hirschsprung intervenidos mediante las técnicas de Duhamel y De la Torre.

INTRODUCTION: Long term results of different surgical techniques in Hirschsprung's Disease (HD) are contradictory. There are still no long term large or multicentric reports about functional results of De la Torre technique. We have studied the mid term functional results of the patients operated on Duhamel (D) and De la Torre (dlT) pull-through procedures. PATIENTS AND METHODS: We collected data from medical records and telephone interviews of the HD patients operated in our unit in the last 16 years. RESULTS: 38 patients were found. Ages ranged from 1.5 to 21 years. Mean age was 7.7 years. Median follow up was 5.9 years. 33 (86.8%) had rectosigmoid disease and 5 (13.2%) had long segment disease. D procedure was performed in 17 (44.7%), Soave in 1 (2.6%), Duhamel-Lester-Martin in 4 (10.5%) and dlT pull-through in 16(42%). In the last visit record, 12 (31.6%), had constipation, and fecal leaks were noted in 11 (33.3%) of the 33 patients > or = 4 years old.. 10 patients (29.4% of the > or = 4 years old group) referred encopresis along the follow-up. Patients from the D group referred higher rates of constipation than those in the dlT group (53.3% vs 20% p=0.048). dlT patients referred more frequency of leaks (46,1% vs 13,3%, p=0,05) Children with very short resections (< or = 10 cm) were more prone to constipation than children with longer resections (66,6% vs 17.4% p=0.007), and less prone to present leaks (12.5% vs 47.3% p=0.08). Encopresis was similar in all groups. DISCUSSION: Both techniques show similar functional results in the mid term, although children in the D group were more prone to constipation and those in the dlT group presented more fecal leaks. All patients with EH need long follow-ups.

[Management of penile trauma from compressed air gun in a child]. / Manejo del traumatismo peneano por arma de aire comprimido en un niño.

We report on a 6-year old child with a penile injury resulting in the inclusion of a pellet in the cavernous body, by a fortuitous shot. Conservative approach with elective extraction of the bullet and cavernous body repair led to a satisfactory result, in better anatomic conditions. The patient did not have any perioperative complication. Accidents by air weapons are particularly important in children, because these weapons are erroneously considered as toys. Air gun injuries to head, neck and thorax have been often published, but these injuries are rare in the chidren genitourinary area.

[Stephens-Fowler in two times: results 14 years later]. / Stephens-Fowler en 2 tiempos: resultados 14 años después.

The management of intra-abdominal testis can be challenging, as evidenced by the multiple methods suggested for its assessment and treatment. In this paper, we report our long-term results for our 14-year surgical experience with laparoscopic management. Methods. Retrospective study of laparoscopic interventions performed at our department for nonpalpable testes between 1996 and 2009. In 174 procedures, we found 72 intra-abdominal testes (5 bilateral) and 25 direct orchiopexies (3 bilateral) were performed. One-stage Fowler-Stephens orchiopexies (FSO) were performed in 5 children. 40 boys underwent the two-stage FSO technique (2 bilateral) in those that reached the contralateral inguinal ring. The second stage was performed in 4 cases with a laparoscopic approach, the rest of them underwent an inguinal procedure exclusively. Mean age for the first intervention was 3.7 yrs, and the second stage was performed on average after 10.9 months. Of 42 undescended testes, 22 were on the right side and 20 on the left side. Median followup was 24 months. Results. Of 42 two-stage FSOs performed, 27 surgeries were very successful, with a testis size equivalent to the contralateral mate; 7 had good results, with a smaller testis (volume up to 50% of contralateral); and 7 resulted in atrophic testis. No complications were encountered during surgery. Conclusions. There is no doubt about the usefulness of laparoscopy for intra-abdominal testes with short spermatic vessels, and the two-stage Fowler-Stephens technique should be the procedure of choice given its high success rate.

[Transanal irrigations in patients with constipation and fecal incontinence: results, indications and follow up in our center]. / Irrigación transanal en pacientes con estreñimiento e incontinencia orgánica refractaria. Resultados, indicaciones y seguimiento en nuestro centro.

AIM OF THE STUDY: To present our results with transanal irrigation (TAI) for management of fecal incontinence and fecal constipation without response to other treatments. METHODS: Retrospective study of patients with fecal constipation and/or fecal incontinence treated with TAI between 2013 and 2017. A Rintala questionnaire(1) was carried out comparing pre and post-treatment results. This study was approved by the ethical committee. MAIN RESULTS: Twenty-five patients were included with a median age of 13 years old (range 6-44 years). Nineteen patients had spinal pathology (76%), four colorectal surgery (16%) and two functional constipation (8%). They presented fecal incontinence in 20% of cases, 12% of fecal constipation and 68% both conditions. After a mean follow-up of 1.5 years (1 month-4 years), 52% of the patients abandoned the treatment. The mean Rintala score was 6.8±4 before treatment, and after, resulted to 11.42±2.75 (p=0.001). The main complications throughout the treatment were pain (68%) and balloon leaks (28%). The patients declared as cause of treatment cessation: reduced mobility (15%), fear or misinformation (32%) and pain (76%). All patients with reduced mobility (n=3) left treatment, versus 45% (n=12) of the patients that had full mobility (p=0.17, OR 8.3 [IC95% 0.3-38]). Complementary treatments such as laxatives, enemas or digital extraction were abandoned in 55% of the patients. CONCLUSIONS: Using TAI seems to improve quality of life in patients with fecal constipation and fecal incontinence refractory compared to other treatments. Our abandonment rate was higher than expected, so we believe it is necessary to create a support group to improve follow-ups.

OBJETIVOS: Presentar nuestros resultados con el uso del irrigador transanal (IT) en el manejo intestinal de pacientes con incontinencia fecal o estreñimiento sin respuesta a otros tratamientos. METODOS: Estudio retrospectivo de pacientes con estreñimiento y/o incontinencia fecal refractaria tratados con IT (2013-2017) con el sistema Peristeen® (Coloplast). Se realizó cuestionario Rintala(1), comparando resultados pre y postratamiento. La realización del estudio fue aprobada por el comité de investigación clínica. RESULTADOS: Fueron incluidos en el estudio veinticinco pacientes, mediana de edad 13 años (6-44 años), cuatro no localizables. Diecinueve presentaban patología medular (76%), cuatro cirugías colorrectales (16%) y dos estreñimiento funcional (8%). El 20% aquejaba incontinencia, 12% estreñimiento y 68% ambas condiciones. Tras una mediana de seguimiento de 1,5 años (1 mes-4 años), el 52% abandonó el tratamiento. La puntuación media pretratamiento fue 6,8±4, frente a 11,42±2,75 tras su empleo (p=0,001), disminuyendo el número de escapes con repercusión positiva en actividades diarias. Las complicaciones principales fueron dolor (68%) y expulsión del balón (28%). Refirieron como causa de abandono: dificultad de uso por movilidad reducida (15%), miedo o desinformación (32%) y dolor (76%). El 100% de pacientes con movilidad reducida (n=3) abandonó el tratamiento, frente al 45% (n=12) en el resto (p=0,17, OR 8.3 [IC95% 0,3-38]). El 55% de los pacientes dejaron de utilizar laxantes, enemas o extracción digital. CONCLUSIONES: El uso de IT parece mejorar la calidad de vida en pacientes con estreñimiento e incontinencia de causa orgánica refractaria. La tasa de abandono fue mayor de la esperada, por lo que creemos necesaria la creación de un grupo de apoyo que mejore el seguimiento.

[Long-term renal function in Wilms tumor survivors]. / Función renal a largo plazo en supervivientes de tumor de Wilms.

OBJETIVE: To evaluate long-term renal function and morbimortality in non-syndromic Wilms tumor (WT) survivors. METHODS: Retrospective study about WT patients treated in 1993-2017, according to SIOP protocols. Mortality, glomerular filtration rate (GFR), prevalence of hypertension and requirement of dialysis and renal transplant were evaluated. Chronic kidney disease (CKD) was defined as GFR <90 ml/min/1.73 m2. RESULTS: Thirty-nine children were treated in the 25 analyzed years. Median time of follow-up was 6 years (0.5-21 years). 48% (19 patients) debuted with stage I or II. Four cases had high-grade histo-logy. Mortality rate was 10%. GFR data were found in 37 patients. Chronic kidney disease (grade I-II) turned up in 6 patients (16%). No patient required renal replacement therapy or renal transplant. 16% of patients developed CKD in both unilateral and bilateral WT, (p>0.05); OR 1.04 (IC 95% 0.09-10.9). Identical results were obtained comparing patients treated with or without radiotherapy (16%). Children with stage I-III had CKD in 11% vs. 40% of patients with stage IV (p=0.12); OR 5.3 (IC 95% 0.61-45). None of them presented hypertension in addition. CONCLUSIONS: In the current study the prevalence of CKD was low but not negligible, although no patients required renal replacement therapy or renal transplant. Bilateral renal involvement and radiotherapy were not associated with CKD development. Metastatic disease determines a higher risk of CKD.

OBJETIVOS: Evaluar la función renal y la morbimortalidad a largo plazo, en supervivientes de tumor de Wilms (TW) no sindrómico. MATERIAL Y METODOS: Estudio retrospectivo de pacientes con TW entre 1993-2017 tratados según protocolos SIOP. Evaluamos mortalidad, filtrado glomerular (FG), prevalencia de hipertensión arterial (HTA), necesidad de diálisis y trasplante renal. Se definió enfermedad renal crónica (ERC) como FG <90 ml/min/1,73 m2. RESULTADOS: En los 25 años analizados se trataron 39 pacientes con edad media diagnóstica de 3,6 años (0,3-11 años). Mediana de seguimiento 6 años (0,5-21 años). El 48% (19 pacientes) debutaron con estadio I o II. Cuatro pacientes presentaron histología de alto riesgo (10%). La mortalidad fue del 10%. El 16% (6 pacientes) desarrolló ERC (grados I-II). Ningún paciente precisó terapia renal sustitutoria (TRS) o trasplante. La presencia de ERC tanto en enfermedad unilateral como bilateral fue del 16%, p>0,05; OR 1,04 (IC 95% 0,09-10,9). Se obtuvieron idénticos resultados (16%) comparando pacientes que recibieron radioterapia frente a aquellos que no. Los pacientes en estadio I, II y III presentaron una prevalencia de ERC del 11% vs. 40% en estadio IV (p=0,12); OR 5,3 (IC 95% 0,61-45). Ningún paciente asoció HTA crónica. CONCLUSIONES: En el presente estudio la prevalencia de ERC en supervivientes de TW no sindrómico es baja pero no desdeñable, aunque ninguno precisó trasplante renal o TRS. La presencia de enfermedad bilateral y la radioterapia no se asociaron al desarrollo de ERC. La enfermedad metastásica condiciona un riesgo mayor de ERC.

[Mesenteric cysts in children]. / Quistes mesentéricos en la edad pediátrica: ¿qué son en realidad?

OBJECTIVES: Mesenteric cysts (MC) are benign cystic tumors that grow within mesentery or omentum tissue. We have reviewed the cases of MC reported and operated on in our centre. MATERIAL AND METHODS: Retrospective review of clinical records of MC cases during the period 2002-2012 RESULTS: A total of 7 patients were found. Mean age was 5.3 years (range 3-11). Abdominal ultrasound was the diagnostic tool in all cases, except for one, which was diagnosed during laparotomy. All presented abdominal pain, 5 (71.4%) vomiting, 4 (57%) gross abdominal distension, 3 (42.8%) fever, and none presented complete abdominal obstruction, although 2 patients (28.6%) had slight sub-occlusion symptoms. All MC were pedicled or sesil, except for our last case, which extended into the retroperitoneum. All specimens were reported as Limphatic Malformation. None recurred. CONCLUSIONS: MC in children are mostly Lymphatic Malformations of mesentery or omentum origin, and clinical presentation varies from chronic abdominal pain to sudden-onset peritonitis or volvulus. About 50-60% require intestinal resection and anastomosis.

[Rhabdomyomatous mesenchymal hamartoma]. / Hamartoma mesenquimal rabdomiomatoso.

Rhabdomyomatous mesenchymal hamartoma is a rare congenital lesion which consists of randomly arranged striated muscle fibers interspersed with mesenchymal elements. We describe the clinical and histopathological features of a rhabdomyomatous mesenchymal hamartoma in a one year-old patient presenting a bilobulated lesion in the mid-cervical line. No associated congenital malformations were observed.