Inguinal lymph nodes agenesia in a patient with Schimmelpenning-Feuerstein-Mims syndrome with proven somatic KRAS mutation.

Schimmelpenning-Feuerstein-Mims (SFM) syndrome is a neurocutaneous disorder that can affect many body systems. The principal and most characteristic anomalies are craniofacial naevus sebaceous in association with neurological, ocular and skeletal findings. The presence of vascular malformations in this condition is unusual; nevertheless, vascular malformations have been suggested by many authors to be part of the spectrum of the same disease. Few cases have been published on the association of SFM with lymphatic malformations. This syndrome is categorized as a mosaic RASopathy due to postzygotic mutations in the HRAS, KRAS or NRAS genes. These genes are involved in the RAF-MEK-ERK signalling pathway, which is activated by mutant cells, increasing cellular proliferation. These mutations have been found only in naevus sebaceous cells, and may be also the explanation for many of the associated pathologies. We report a case of an 18-year-old boy diagnosed with SFM syndrome associated with lymphatic malformation in the legs and agenesia of the inguinal lymph nodes. The lymphatic alterations were diagnosed by gammography of the legs. The genetic diagnosis was confirmed by the presence of a KRAS postzygotic mutation in naevus sebaceous cells of a skin specimen. Genetically confirmed cases of mosaic RASopathies should be used to more accurately characterize phenotypic presentations of this syndrome and develop a future therapeutic strategy, such as molecular targeted therapy.

[Parent controled analgesia system in postoperative pain management in patients undergoing lip and cleft palate surgery]. / Analgesia mediante bomba de infusión continua controlada por el paciente para el manejo postoperatorio del dolor en pacientes intervenidos de fisura palatina y labio fisurado.

OBJECTIVES: Craniofacial clefts surgery associates a painful postoperative pain whose management is complicated with conventional analgesia. PATIENTS AND METHODS: A parent controlled analgesia system was implanted with a continuous perfusion of tramadol, ondansetron and metamizole adjusted by weight. Parents are allowed to administer additional boluses if they observe irritability. We compared the variables of the cleft patients operated before and after the implantation of the system in our center. RESULTS: During 2016, 16 craniofacial clefts were operated (4 cheilorhinoplasties and 12 palatal clefts). No PCA (parent controlled analgesia) system was used. The average time of stay in PICU was 1.5 days. It took an average of 2.5 days to initiate tolerance. The mean of VAS (Visual Analogic Scale) was 3. 53% required major opioids (morphine, fentanyl) not being sufficient analgesia every 3 hours. During 2017, 7 palatal fissures and 4 cheilorhinoplasties were operated (11). Both of them were controlled by PCA. Patients with palatal cleft were admitted to the PICU with a total mean of 0.5 days. The beginning of tolerance was advanced to the first postoperative day. The VAS diminished to 0.5. Only one patient required opioids. 72% did not need to associate any type of analgesia. CONCLUSIONS: The PCA system is a safe and risk-free insurance for analgesia of fissured patients with benefits such as: decrease in pain, stay in PICU, the need for analgesia and initiation of early tolerance.

OBJETIVOS: La cirugía de las fisuras craneofaciales asocia un intenso dolor postoperatorio cuyo manejo resulta complicado con la analgesia convencional. MATERIAL Y METODOS: Utilizamos una bomba de analgesia controlada por los padres que contiene una perfusión continua de tramadol, ondansetrón y metamizol ajustada por peso. Se permite a los padres administrar bolos adicionales si observan irritabilidad. Comparamos variables de los pacientes fisurados intervenidos antes y después de la implantación del sistema en nuestro centro. RESULTADOS: Durante 2016 fueron intervenidos 16 fisurados (4 queilorrinoplastias y 12 fisuras palatinas). En ninguno se empleó bomba de analgesia. El tiempo medio de estancia en UCIP fue 1,5 días. Tardaron de media 2,5 días en iniciar tolerancia. La media de EVA (Escala Analógica Visual) fue de 3. El 53% precisaron opiáceos mayores (morfina, fentanilo), no siendo suficiente la analgesia c/3 horas. Durante 2017 se operaron 7 fisuras palatinas y 4 queilorrinoplastias (11). En todos empleamos bomba. Únicamente ingresaron en UCIP las fisuras palatinas (debido al manejo de la vía aérea) con una media total de 0,5 días. Se adelantó el inicio de tolerancia al primer día postoperatorio. La EVA disminuyo a 0,5. Solo un paciente precisó opiáceos. El 72% no precisó asociar ningún tipo de analgesia. CONCLUSIONES: La bomba de PCA (analgesia controlada por el paciente/por los padres) es un método seguro y exento de riesgo para la analgesia de los pacientes fisurados con beneficios como: disminución del dolor, de la estancia en UCIP, de la necesidad de analgesia e inicio de tolerancia precoz.

Assessment of knowledge and skills in using an Automated External Defibrillator (AED) by university students. A quasi-experimental study. / Evaluación del conocimiento y de las habilidades para el uso de un Desfibrilador Externo Automatizado (DEA) por estudiantes universitarios. Un diseño cuasiexperimental.

AIM: To evaluate layperson (university student) ability to use an automated external defibrillator (AED). DESIGN: A repeated measures quasi-experimental study with non-probabilistic sampling and a control group was carried out. SCOPE: Teacher training degree students at the University of Santiago de Compostela (Spain). PARTICIPANTS: The sample consisted of 129 subjects (69% women and 31% men), between 19-47 years of age (mean 23.2±4.7 years). As inclusion criterion, the subjects were required to have no previous knowledge of AED. INTERVENTIONS: Times to apply defibrillation with an AED to a mannequin were recorded untrained (T0), after a theoretical and practice explanation lasting less than one minute (T1), and 6 months after the training process (T2). MAIN VARIABLES OF INTEREST: The primary endpoint was the time taken to deliver a defibrillation discharge. The "improvement effect" variable was defined by the absolute time difference between T1 and T0, while the "degree of forgetfulness effect" variable was defined as the absolute difference between T1 and T2. RESULTS: The mean times were T0=67.7s; T1=44.2s; T2=45.9s. The time to apply defibrillation was reduced after explanation training (T1

Spanish primary care in pediatric trauma (AITP) consensus: An AITP checklist. / Consenso AITP español: Una lista de verificación AITP.

INTRODUCTION: The course in Primary Care in Pediatric Trauma (ATIP in Spanish) has been taught in Spain since 1997, and there are currently 9 accredited training centers. Care of polytraumatized pediatric patients often takes place in an environment conducive to errors resulting from forgetfulness, which is why checklists - mnemonic tools widely used in industry and medicine - are particularly useful to avoid such errors. Although several checklists exist for pediatric trauma care, none have been developed within the setting of our course. MATERIALS AND METHODS: The criteria for being selected as an expert in Primary Care in Pediatric Trauma were agreed upon with the scientific polytrauma committee of the Spanish Pediatric Surgery Society. The items that make up the checklist were obtained from a review of the literature and consultation with selected experts, using the Delphi Technique. RESULTS: 10 experts representing the 9 groups or training centers in Primary Care in Pediatric Trauma were selected, and a 28-item checklist was drawn up in accordance with their design recommendations. CONCLUSIONS: With the consensus of all the groups, a checklist for the treatment of polytraumatized pediatric patients was drawn up using the Delphi Technique, an essential requirement for the dissemination of this checklist, which should be adapted and validated for use in each healthcare center.

INTRODUCCION: El curso de Asistencia Inicial al Trauma Pediátrico se imparte en España desde 1997, existiendo en la actualidad 9 centros formadores acreditados. La asistencia al paciente pediátrico politraumatizado se produce muchas veces en un ambiente proclive al error por olvido, por lo que las listas de verificación, como herramientas mnemotécnicas de amplia difusión en la industria y en medicina, serían especialmente útiles para evitarlos. Aunque existen varias listas de verificación para la asistencia al traumatismo pediátrico, ninguna se ha desarrollado en el entorno de nuestro curso. MATERIAL Y METODOS: Se acordaron los criterios para ser seleccionado como experto en Asistencia Inicial al Trauma Pediátrico con la comisión científica de politrauma de la Sociedad Española de Cirugía Pediátrica. Los ítems para formar la lista de verificación se obtuvieron a partir de una revisión bibliográfica y de la consulta a los expertos seleccionados, empleando un método Delphi. RESULTADOS: Se seleccionaron 10 expertos que representan los 9 grupos o centros formadores en Asistencia Inicial al Trauma Pediátrico y se elaboró una lista de verificación con 28 ítems, siguiendo sus recomendaciones de diseño. CONCLUSIONES: Se diseñó una lista de verificación para el manejo del paciente pediátrico politraumatizado, con el consenso de todos los grupos empleando un método Delphi, requisito fundamental para facilitar la difusión de esta lista. Sería preciso adaptar y validar dicha lista para su uso en cada centro asistencial.

Treatment with oral or topical sirolimus in complex vascular anomalies in pediatrics. Experience in a third-level hospital. / Tratamiento con sirolimus oral o tópico en anomalías vasculares complejas en pediatría. Experiencia en un hospital terciario.

OBJECTIVE: The use of sirolimus in vascular anomalies is a special indication not authorized in its data sheet. The objective of this study was to increase the evidence of oral or topical use of sirolimus for this indication in the pediatric population. MATERIALS AND METHODS: An observational, retrospective study of patients under 18 years of age treated with oral or topical sirolimus for vascular anomalies was carried out. Diagnosis and location of lesions, administration route and dosage of sirolimus, blood levels of sirolimus in patients who received oral treatment, treatment duration, response, and toxicity were collected. RESULTS: 18 patients - 7 with oral treatment and 11 with topical treatment - were included. With oral sirolimus, the overall response rate was 85.7%. Sirolimus was discontinued in 2 cases - as a result of full resolution and progression. 57.1% of patients had adverse effects, most of which were mild. Dyslipidemia was the most frequent adverse effect. Blood levels were monitored in all patients for dose adjustment purposes. With topical treatment, the overall response rate was 72.7%. Sirolimus was discontinued in 3 cases -due to progression in 2 cases and to stability in 1. 27.3% of patients had adverse effects, with itching standing out as the most frequent one. CONCLUSIONS: The favorable results of sirolimus treatment in our patients seem to confirm its effectiveness and safety in vascular anomalies, which make it stand as a therapeutic option in pediatric patients. However, further research is required to establish the optimal treatment regimen, treatment duration, and potential long-term adverse effects.

OBJETIVO: El uso de sirolimus en anomalías vasculares es una indicación especial no autorizada en ficha técnica. El objetivo de este estudio es incrementar la evidencia del empleo por vía oral o tópica de sirolimus en esta indicación en población pediátrica. METODO: Estudio observacional retrospectivo de pacientes menores de 18 años tratados con sirolimus oral o tópico para anomalías vasculares recogiendo: diagnóstico y ubicación de lesiones, forma de administración y dosificación de sirolimus, niveles sanguíneos de fármaco en los pacientes con tratamiento oral, duración del tratamiento, respuesta y toxicidad. RESULTADOS: Se incluyeron 18 pacientes (7 con tratamiento oral y 11 tópico). Con sirolimus oral, la tasa de respuesta global fue 85,7%. Se interrumpió sirolimus en 2 casos: por resolución completa y por progresión. El 57,1% experimentó algún efecto adverso, en su mayoría leves; siendo la dislipemia el efecto adverso más frecuente. La monitorización de niveles sanguíneos fue empleada en todos los pacientes para el ajuste de dosis. Con el tratamiento tópico, la tasa de respuesta global fue 72,7%. Se interrumpió sirolimus en 3 casos: progresión en 2 casos y estabilidad en 1. El 27,3% experimentó algún efecto adverso, siendo el prurito el más frecuente. CONCLUSIONES: Los resultados favorables del tratamiento con sirolimus en nuestros pacientes parecen confirmar la efectividad y seguridad del fármaco en anomalías vasculares y lo posicionan como una opción terapéutica en pacientes pediátricos. Aun así, parece necesaria mayor investigación que trate de aclarar, entre otros, el régimen óptimo del tratamiento, la duración del mismo y los potenciales efectos adversos a largo plazo.

[Family planning in men and women with multiple sclerosis. Analysis of the Andalusian Registry (2018-2022)]. / Planificación familiar en hombres y mujeres con esclerosis múltiple. Análisis del Registro Andaluz (2018-2022).

INTRODUCTION: The Andalusian Registry of Pregnancies in patients with multiple sclerosis is the largest Spanish registry on multiple sclerosis (MS) and family planning. For the first time, it includes information on the fertility of men with MS. The influence of the use of a disease-modifying treatment (DMT) on the health of the foetus/newborn and the impact of breastfeeding on MS are also analysed. SUBJECTS AND METHODS: This is a multicentre, prospective and observational study. Recruitment of patients took place between December 2018 and December 2020. Women were followed up for one year after delivery. Altogether 100 women and 16 men were included, with a total of 103 newborn infants. RESULTS: The annualised relapse rate of the women with MS decreased significantly during pregnancy (from 0.23 to 0.065). A total of 11.2% of patients resorted to assisted reproductive techniques in order to conceive a child. No association was found between the use of a DMT at conception and/or pregnancy and the risk of miscarriage, prematurity or low birth weight. Over half the women with MS (54.2%) chose to breastfeed (26.7% of them while on a DMT). CONCLUSIONS: MS does not affect the fertility of men. Neither does the use of a DMT at the time of conception affect their fertility or their children's health. Assisted reproductive techniques did not have a negative impact on the course of MS. Breastfeeding is a common practice among women with MS and there is no evidence of positive or negative effects on disease progression.

TITLE: Planificación familiar en hombres y mujeres con esclerosis múltiple. Análisis del Registro Andaluz (2018-2022).Introducción. El Registro Andaluz de Embarazos en pacientes con esclerosis múltiple (EM) es el mayor registro español sobre EM y planificación familiar. Por primera vez se incluye información sobre la fertilidad de hombres con EM. También se analizan la influencia del uso de un tratamiento modificador de la enfermedad (TME) en la salud del feto o recién nacido y el impacto de la lactancia materna en la EM. Sujetos y métodos. Es un estudio observacional, prospectivo y multicéntrico. El reclutamiento de pacientes se hizo entre diciembre de 2018 y diciembre de 2020. El seguimiento de las mujeres tras el parto fue de un año. Se incluyó a 100 mujeres y 16 hombres, con un total de 103 recién nacidos. Resultados. La tasa anualizada de brotes de las mujeres con EM descendió durante el embarazo de forma significativa (de 0,23 a 0,065). Un 11,2% de los pacientes recurrieron a técnicas de reproducción asistida para conseguir la gestación. No se encontró relación entre el uso de un TME en la concepción y/o embarazo y el riesgo de aborto, prematuridad o bajo peso al nacer. El 54,2% de las mujeres con EM optaron por dar lactancia (el 26,7% de ellas usando un TME). Conclusiones. La EM no afecta a la fertilidad de los hombres. Tampoco influye en ésta, ni en la salud de sus hijos, el uso de un TME en el momento de la concepción. Las técnicas de reproducción asistida no impactaron negativamente en la evolución de la EM. La lactancia se impone como una práctica habitual entre las mujeres con EM y no se evidencian efectos positivos o negativos sobre la evolución de la enfermedad.

Vascular malformations in childhood.

Vascular malformations are inborn errors of vascular embryogenesis present at birth that should be diagnosed in childhood and, when necessary, treated to prevent later complications. The current trend is to classify these lesions according to flow characteristics and the predominant type of vascular channel affected. Given the complexity, and in many cases, the rarity, of vascular malformations, they should be managed by multidisciplinary teams at vascular anomalies centers. Furthermore, because the association between vascular malformations and certain syndromes is becoming increasingly recognized, a better understanding of these lesions will help to improve overall patient management in this setting.

[Is experimental surgery necessary or essential in the training program of a pediatric surgeon?]. / ¿Es necesaria o imprescindible la cirugía experimental en el programa formativo de un cirujano pediátrico?

Many surgical procedures performed in pediatric surgery have a slow learning curve, the volume of patients and the existence of complex diseases that require extensive training and surgical skill, have taken our service to create a global training program of experimental surgery. This program based on the simulation and training invasive procedures in real anatomical models, aims to educate our residents in a global and efficiently way in order to obtain an improvement of technical training, and increased patient safety result of experience and expertise wined in the experimental animal. This paper presents the main features, objectives and results obtained with this training program and seeks to promote the incorporation of simulation programs in live animal as an essential part of the training of pediatric surgery resident.

[Neonatal pyloroduodenal duplication cyst. A case report].

Intestinal duplications located in the duodenum are rare conditions that generally affect the first or second duodenal portion. It is extremely unusual for this condition to be located in the pyloroduodenal area, accounting for less than ten documented cases. This research presents a case of a female newborn who showed on the second day of life signs of upper intestinal obstruction which required exploratory laparotomy. The surgery revealed a pyloroduodenal cyst that was successfully excised. This is an extremely rare congenital anomaly that encompasses challenging symptoms and signs, and the purpose of the surgery should be the complete resection of the lesion. If the complete excision endangers the surrounding organs, partial resection through mucosectomy is a valid alternative to prevent the complications of more invasive surgery.

[Quality of life in children operated on for anal atresia]. / Calidad de vida en los pacientes intervenidos de atresia de ano.

INTRODUCTION: Most children with anorectal malformations have some type of intestinal dysfunction. A correct follow up in this aspect after surgery affects their quality of life. MATERIAL AND METHODS: We gathered a sample of 20 children that were lost in their follow up after posterior sagittal anorectoplasty (PSARP). We got contact with them and they were interviewed and examined in our department. RESULTS: We collected 12 girls and 8 boys. Age range was between 3 and 14 years. 70% had good prognosis for continence (low fistula) and 30% poor prognosis (high fistula). 35% suffered from postoperative complications being the most frequent prolapse especially in high atresias. 85% had good rectal sensitivity, 15% had poor sensitivity that was directly related to incontinence. Anal tone was decreased in our exploration in 35% of patients which was not directly related to their continence. 65% suffered constipation with or without fecaloma. 5% of cases had intestinal hipermotility. The total incidence of fecal loose was 40%. More than a half (62,5%) kept on loosing stool despite treating their constipation or hipermotility, so we consider them true incontinents (no voluntary bowel movements). 67% of children with high fistula were true incontinents, just 7% of those with low fistula. Subjective quality of life in patients with soling was 6.4. In clean patients it was 9.3. Objective quality of life (Score/13) in dirty patients was 6.6. In Clean patients: 11.9. After our bowel management protocol we got 100% of patients clean during school time, thereby improving their quality of life. CONCLUSIONS: Incontinence determines the long-term quality of life in our patients in addition to the psycho-social consequences. They are clearly more frequent in patients with high fistula. Much assume incontinence as an unavoidable part of their disease so do not always demand treatment if they are not followed by a surgeon.

[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.

[Correction of lop ear using minimally invasive approach]. / Corrección de las orejas procidentes mediante un abordaje mínimamente invasivo.

INTRODUCTION: Helix valgus or procident ears is a common problem that affects about 5% of the population. The folds of the antehelix and the overdevelopment of the concha are the most commonly found anatomic alterations of the ear pavilion. In children this pathology usually causes anxiety and an emotional trauma that may interfere in their normal development. MATERIALS AND METHODS: There are a few tipes of techniques to correct helix valgus. We present the application of the technique in our service. We conduct the otoplastia with an outer puntiform technique which allows us to cut the cartilage partially from the outside. Next we fold from the rear the antehelix and hide the concha. RESULTS: We analysed 7 years of the application of this technique and we now present 87 otoplastias conducted to 44 children. The 97% of them were bilateral. No precocious complications have been observed after the surgery. All cases except for one of them have been bilateral. All the patients were satisfied with the aesthetic results. None of them showed relapse. In one case there was a hypertrophic scar that required cutting and in 2 of the cases there was a slight hypercorrection. CONCLUSIONS: Procident ears may occasion a psychological trauma in children. We believe that this technique, which is minimally invasive, provides very satisfactory aesthetic results, the puntiform scar being hardly noticed fifteen days before surgery. The patients need to stay in hospital for a short period, 24-48 hours, and complications are very rare, recidiva has not been described. We strongly recommend this technique for the correction of procident ears.

[Non-palpable testes: laparoscopic or inguinal approach?]. / Testes no palpables: abordaje laparoscópico o inguinal?

INTRODUCTION: Twenty per cent of the operated patients suffering from cryptorchidisim show no palpable testis in the physical check-up. The use of a non-palpable testis in the initial stages is considered to be controversial when deciding between a laparascopic or an inguinal approach. Our aim is to compare the results obtained with these two approaches and evaluate which one of them would be the most relevant as an initial option. MATERIALS AND METHODS: We examined the patients who have been subjected to surgical intervention for non-palpable testis in the last three years. We had a sample of 53 patients. Bilateral non-palpable testes were disregarded. In the case of 35 patients the initial approach was through the groins while in 18 of them the approach was laparascopic. Relevant data were recorded, such as the age of the patient, right or left side, surgical findings, need for a laparascopic or groin approach and associated hernia. RESULTS: The average age of the patients at the time of the surgical treatment was 3.7 years R (1-13 years). 42% of the testes were on the right side and 58% on the left. In an initial stage the inguinal approach was used with 35 patients, 2 of these requiring laparascopic exploration due to a non-concluding check-up; it was concluded that they were 2 cases of anorchia. An initial laparoscopic approach was used with 18 patients. Internal vessels in the canal were found which required an inguinal approach. In 12 patients normal or atrophic testes were observed. Testicular descent was achieved through the groin in 10 of them while in the remaining 2 a combined approach was adopted. CONCLUSIONS: 80% of the patients subjected to a first laparoscopic approach needed a groin approach later on. 11% of the patients subjected to a first inguinal approach required laparascopic examination. In view of the results obtained, we conclude that inguinal exploration diminishes the need for second procedures.

[Therapeutic approach of enuresis based on the differentiation of the enuretic syndrome and monosymptomatic enuresis]. / Enfoque terapéutico de la enuresis basándose en la diferenciación del síndrome enurético y la enuresis monosintomática.

INTRODUCTION: Enuresis affects 15% of the children under 5. Possible etiopatogeny explaining the mechanism of production of nocturnal enuresis has been described, resulting in different terapeutical approaches; however, we cannot speak up to now of general guidelines for its treatment. MATERIALS AND METHODS: On the basis of 544 children who were treated in our hospital in the last 12 years, we analysed a sample of 124 patients corresponding to the last two years. We now present the therapeutical protocol used and analyse the results. In the initial therapeutical approach a distinction is made between monosymptomatic enuresis and eneuretic syndrome. RESULTS: We studied a total of 120 patients (89 boys and 31 girls). 63% of them showed monosymptomatic enuresis while 37% suffered from enuretic syndrome. In the case of 15% of them, this was associated with heavy sleep and difficulties to wake up. 92% was the general percentage of recovery. In the patients suffering from nonosymptomatic eneuresis, the problem was solved with desmopressin in 87% of them (demospressin on its own in 65% or in combination with oxibutine in the remaining 35%). In the group of enereutic symdrome, the problem was solved with oxibutine in 71% (on its own in 40% or in combination with desmopressin in 54%. CONCLUSIONS: The treatment and solution of enuresis improve the child's self-esteem and the anxiety caused in the family. A good medical history with a clear distinction between enuretic syndrome and monosyntomatic enuresis leads us to a suitable therapeutical approach for every patient, allowing us to find earlier the right treatment for every individual.

[25 years experience in cerebrospinal shunt. Are new systems better?]. / 25 años de experiencia en válvulas de derivación ventrículo-peritoneal. Son mejores los nuevos sistemas?

INTRODUCTION: Since the introduction of the first ventricular shunt in 1949, the cerebrospinal shunts are the most used choice for the management of hydrocephalus in children. With the technological advance, systems have been developing increasingly sophisticated. Our aim is to study the incidence of complications after the implantation of these shunts and the variables associated to it. MATERIAL AND METHODS: We perform a retrospective study between 1981 and 2006, gathering the patients between 0 and 15 years treated at our Hospital for the placement of a cerebrospinal shunt or as consequence of a problem of the device. We analyze the tipe of shunt (Hakim, Pudentz, Delta, Orbis-Sigma, Strata, Codman), etiology of hydrocephalus, age, immediate and late complications, permanence of the system, changes of the system and cerebrospinal fluid. The qualitative data were analyze by c2 test. We also estimate survival of shunts with Kaplan-Meier stimator and make a multivariant Cox regression analysis. RESULTS: A total of 75 patients. The main etiology for hydrocephalous was myelomeningocele, followed by congenital and posthemorrhagic. Most of the patients presented complications of the system in the follow-up. They were adjusted for age, sex, etiology, shunt and surgeon. The variable that had an independent effect for predicting the presence of a complication was the type of shunt, being the most ancient systems those with major survival. The most frequent complication in the immediate period (< 3 month) was the obstruction and infection of the proximal catheter or shunt. Distal catheter disconnection or break prevailed in the late one. We got few functional complications, these were not related with the type of shunt. CONCLUSIONS: In our experience, the shunts with the less short-term probability of complication (< 3 months) have been old simple systems like Hakim and Pudentz. The functional complications, that are those which presumably should be reduced by the new programmable systems, don,t seem do it, neither mean longer survival of the system.

Effect of 3basic life support training programs in future primary school teachers. A quasi-experimental design. / Efecto de 3métodos de enseñanza en soporte vital básico en futuros maestros de Educación Primaria. Un diseño cuasiexperimental.

AIM: To evaluate the learning of basic life support (BLS) measures on the part of laypersons after 3different teaching programs. DESIGN: A quasi-experimental before-after study involving a non-probabilistic sample without a control group was carried out. SCOPE: Primary school teacher students from the University of Santiago (Spain). PARTICIPANTS: A total of 124 students (68.8% women and 31.2% men) aged 20-39 years (M=22.23; SD=3.79), with no previous knowledge of BLS, were studied. INTERVENTIONS: Three teaching programs were used: a traditional course, an audio-visual approach and feedback devices. MAIN VARIABLES OF INTEREST: Chest compressions as sole cardiopulmonary resuscitation skill evaluation: average compression depth, compression rate, chest recoil percentage and percentage of correct compressions. Automated external defibrillator: time needed to apply a shock before and after the course. RESULTS: There were significant differences in the results obtained after 2minutes of chest compressions, depending on the training program received, with feedback devices having a clear advantage referred to average compression depth (p<0.001), compression rate (p<0.001), chest recoil percentage (p<0.001) and percentage of correct compressions (p<0.001). Regarding automated external defibrillator, statistically significant differences were found in Tafter (p=0.025). CONCLUSIONS: The teaching course using feedback devices obtained the best results in terms of the quality of chest compressions, followed by the traditional course and audio-visual approach. These favorable results were present in both men and women. All 3teaching methods reached the goal of reducing defibrillation time.

[Platelet-rich plasma (platelet gel) in secondary alveoloplasty in cleft patients]. / Plasma enriquecido en plaquetas en la alveoloplastia de pacientes fisurados.

INTRODUCTION: The use of Platelet-rich plasma (platelet gel--PRP--) was introduced in the oral and maxilofacial surgery 10 years ago. Its good results are due to the quickly generation of new bone and the acceleration of the period of surgical scar formation. Its employment in the alveolar reconstruction of the cleft patient is not still consistent and the works published in the literature are infrequent. The objective of this preliminary study is presenting our experience with the use of PRP in the reconstruction of the alveolar congenital defects of cleft patients. PATIENTS AND METHODS: Between July 2002 and January 2004 were operated 14 patients with congenital alveolar cleft carrying out an standard secondary alveoloplasty. In 12 cases was employed cancellous bone of iliac crest and in two cases the donor area was tibial. The laboratory of Hematology of our Hospital prepared the plasma gel rich in platelets by means of a double centrifuge of autologous serum of the patient. After adding calcium a rich gel in platelets in approximate quantities of 1-2 ml was obtained. In this preliminary report we have studied the aspect of the surgical injury the 3er postoperative day, and the first and second weeks after intervention. These results were compared carried out previously with the Standard alveoloplasty without use of the PRP. Likewise we evaluated the bony density by means of intraoral Rx at 3 and 6 months postoperative. RESULTS: The injury of the alveoloplasty healed more quickly in the patients in which plasma enriched gel was employed. The patients referred less pain and edema in the first days of the postoperative period. The alveolar bony regeneration was faster to the 3 months, though the result was similar in the intraoral Rx to the 6 months. The necessary quantities of cancellous bone were smaller in all cases in which the plasma enriched gel was used (30% less). CONCLUSIONS: The use of Platelet-rich plasma (PRP) is a valid protocol for the reconstruction in patients with congenital alveolar clefts. Its low price and morbidity do it recommendable for its employment in cleft patients.

[Combination of pulsed dye laser and propranolol in the treatment of ulcerated infantile haemangioma]. / Combinación de propranolol oral y láser de colorante pulsado en el tratamiento de los hemangiomas infantiles ulcerados.

INTRODUCTION: Ulceration is the most common complication of infantile haemangioma, with 15.8% of them usually appearing in the proliferative phase. They can be managed in several ways. We present our experience in the treatment of ulcerated haemangioma with the combination of pulsed dye laser and propranolol. MATERIAL AND METHODS: A retrospective observational study was conducted on patients with ulcerated infantile haemangioma treated with pulsed dye laser in association with propranolol. The study included 7 patients, 3 cases in labial area and 4 cases in the nappy area. A review was also performed on a historical cohort of 5 children with ulcerated haemangiomas with the same features, but treated only with propranolol, topical agents and occlusive dressings. RESULTS: The median size of the ulcer was 1.0 cm, and there was a mean time of onset pre-treatment of 2 weeks. Pain and bleeding was present in all patients. After 2 weeks of combined propranolol and laser treatment, all lesions were healed. The pain disappeared after the first laser session. Patients with ulcerative haemangioma in the labial area obtained a better response than patients with haemangioma in the nappy area. The cohort of patients treated with propranolol required a mean healing time of 5.2 weeks, with the addition of an occlusive dressing with ointment. CONCLUSIONS: We believe that our results suggest that combined treatment, laser and propranolol, has synergistic effects that accelerate the healing of ulcerated haemangioma, as observed in our patients. Further studies with larger numbers of patients are needed to confirm this fact.

[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.