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.

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.

[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.

[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.

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.

[Dog bite-related injuries treated in a pediatric surgery department:analysis of 654 cases in 10 years]. / Mordeduras de perro. Análisis de 654 casos en 10 años.

BACKGROUND: Dog bites to children have increasingly come to the forefront of public attention in recent years, especially attacks by certain breeds classified as aggressive. Dog bites account for approximately 1 % of all emergency pediatric department visits and 75 % of these bites occur in children. Although these lesions are rarely serious, 20-45 % of children under the age of 15 years have been bitten. OBJECTIVE: To analyze the reality of this problem in our population in order to determine the ideal prehospital preventive measures that would reduce the incidence of dog bites. MATERIALS AND METHODS: A retrospective study of children with dog bites who received emergency treatment in the Hospital Infantil Teresa Herrera of the Complejo Hospitalario Juan Canalejo in La Coruña, Spain, between 1991 and 2000 was undertaken. A register of patients was created that included data on age and sex of the children, the race of the dog, the relationship between the child and the animal, characteristics of the injuries, circumstances of the attack, probable sequelae, need for hospital admission and complications during treatment. Nonparametric data were analyzed using Pearson's 2 test. The Ox ratio was estimated with a 95 % confidence interval (0.05). RESULTS: In the 10-year period between January 1991 and December 2000, 654 patients under the age of 14 years were treated for dog bite-related injuries. The mean age was 5.09 years. A greater number of boys had been bitten than girls (2.75/1). In 79 % of the cases the dogs were known (family, friends, neighbors). Thirty-eight percent of the attacks were made by German Shepherds and 35 % by crossed breeds. Sixty-five percent of the bites were located on the head, face and neck, especially in patients aged less than 4 years. Five percent of the lesions were severe. Thirteen patients were admitted to the pediatric intensive care unit and 98 patients required hospitalization due to the characteristics of the injuries. The mean hospital stay was 6 days. Infectious complications occurred in 32 patients. CONCLUSIONS: Although dog bite-related injuries to children in our community are rarely serious, they can produce important facial cosmetic sequelae. The high incidence of these lesions indicates the need for preventive measures and public policy strategies.

[Treatment of pyeloureteral duplication associated with ureterocele or ectopic ureter]. / Tratamiento de las duplicidades pieloureterales asociadas a ureterocele o a uréter ectópico.

INTRODUCTION: Pyeloureteral duplication is often associated to ureterocele and ectopic ureter. Therapeutic approach of ureterocele is actually a debated subject because of the differences treatments. There is controversy about the classic heminefrectomy with ureterocele aspiration and the minimal invasive endoscopic approach. MATERIAL AND METHODS: We made a retrospective study about 34 children with pyeloureteral duplication associated with ureterocele (26) or with ectopic ureter (8). The election of treatment was mainly based on renal function, showed on gammagraphy. It was performed heminephrectomy and ureterocele aspiration in patients with hypofunctioning upperpole, and surgery saving the kidney when the renal function was normal. RESULTS: Heminephrectomy was curative in 66.5% of children with ureterocele. In the others was required a correction of the vesicoureteral reflux associated with the lower pole or removal of the ureterocele with ureteral reimplantation due to failure in ureterocele collapse. 88.5% of ureterocele was collapsed. None of the heminefrectomies performed on patients with ectopic ureter needed further surgery. Pathology studies showed a high incidence of renal dysplasia (63%). CONCLUSION: We consider the heminephrectomy the treatment of choice in pyeloureteral duplication associated with ureterocele and ectopic ureter when gammagraphy studies show hypofunctioning upperpole.

[Testicular microlithiasis. Clinical significance and therapeutic implications]. / Microlitiasis testicular. Significado clínico e implicaciones terapéuticas.

Testicular microlithiasis is a rare condition usually diagnosed by scrotal ultrasound and associated with pathology of the testis. We report two pediatric cases presented with acute scrotum. Testicular sonography revealed significant bilateral testicular microlithiasis. One case was diagnosed as having torsion of the right testis and the other case as orchiepididymitis. A careful review of the literature is made and a periodic follow-up of this disease is advocated.

[Contraindications for the endoscopic treatment of vesicoureteral reflux]. / Contraindicaciones relativas para el tratamiento endoscópico del reflujo vesicoureteral.

The endoscopic treatment of vesicoureteral reflux (VUR) has acquired a great importance as a therapeutic option during the last years. We present our experience with two substances, teflon paste and macroplastic, used in 86 patients, with 147 refluxing renal units. After first injection, the successful rate was 86% for teflon pastes versus 91% for macroplastic in the middle grade VUR units. We analyze our complications rates (14.25%) and we conclude that there is a close relation with a right technic performance. In our experience we consider a major contraindication for this technical option patients less than 1 year of life. At the same time we presume that an special care must be taken in monorenal patients as well.

[Paratesticular rhabdomyosarcoma]. / Rabdomiosarcoma paratesticular.

We report a case of paratesticular rhabdomyosarcoma in a six-year-old boy. The clinical presentation was subacute. The patient underwent a radical right inguinal orchidectomy. It was classified in IRS-III stage IA (based in the Third Intergroup Rhabdomyosarcoma Study). Subsequently, the child received 3 chemotherapy courses (9 weeks) with vincristine and actinomycin D. The patient is found to be asymptomatic 1 year after the treatment.

[Retrocaval ureter in children: surgical approach based on the obstructive pattern in the diuretic renogram with 99mTc DTPA]. / Uréter retrocavo en edad pediátrica: actitud quirúrgica en base al patrón obstructivo en el renograma diuretico 99mTc DTPA.

Retrocaval ureter is a rare disease in childhood which is due to a abnormal development of the inferior cava vein. We report a new case of retrocaval ureter in a 9 year-old girl who had consulted for macrohematuria and right flank pain. Preoperative intravenous urography and DTPA diuretic renogram suggested this entity. CT scan was no necessary for the diagnosis. We consider the diuretic renogram the best diagnostic method in the surgical decision of this disease.

[Use of Tc 99m DTPA in the follow-up of 2 pediatric patients diagnosed with megacalycosis or Puigvert's disease]. / Utilización del Tc 99 m DTPA en el seguimiento de 2 pacientes pediátricos diagnosticados de megacaliosis o enfermedad de Puigvert.

OBJECTIVE: Megacalycosis or Puigvert's disease is a congenital anomaly of renal development characterized by caliceal dilatation, an increased number of calyces, associated with hypoplasia of the pyramids of Malpighi, and a normal renal pelvis. Renal function is always normal and there is no evidence of obstruction to urinary flow. The foregoing are important in distinguishing megacalycosis from congenital hydronephrosis. We have studied two pediatric patients with this renal anomaly by means of non invasive techniques, in order to demonstrate they had no urinary flow obstruction despite the caliceal dilatation. METHODS: Two cases of megacalycosis that had been diagnosed at our hospital from 1991 to 1995 are described and the literature is reviewed. Diagnosis was basically by intravenous urography. A diuretic renogram with technetium 99m diethylenetriaminepentaacetic acid (Tc 99m DTPA) showed no urinary flow obstruction in the anomalous kidney. This test was repeated several times, together with renal function studies like the technetium 99m dimercaptosuccinic acid scan (DMSA Scan). RESULTS: Both patients are asymptomatic and have a normally functioning kidney with no scars demonstrable on the DMSA scan. Renal urinary flow studies have remained within the normal ranges (elimination of more than 40% of the radionuclide 20 min after the administration of furosemide). US control evaluations have shown adequate renal growth and persistent caliceal dilatation in both cases. CONCLUSIONS: This congenital malformation must be considered when investigating renal dilatation, since megacalycosis does not require surgical treatment. Intravenous urography is useful in the diagnosis of this condition and Tc 99m DPTA is the best test for subsequent control evaluations.

[Ureteral hydronephrosis secondary to appendicular abscess]. / Ureterohidronefrosis secundaria a absceso apendicular.

OBJECTIVE: To report a case of ureterohydronephrosis secondary to an undiagnosed appendiceal abscess. METHODS/RESULTS: Herein we describe a case of a 4-year-old girl with right ureterohydronephrosis arising from extrinsic compression of the right ureter due to an undiagnosed appendiceal abscess. The patient was treated with intravenous antibiotics and the abscess was drained. Regular isotope and US assessments showed both the residual retroperitoneal fibrosis and renoureteral dilation had decreased. CONCLUSIONS: Acute appendicitis is still the most common cause of emergency abdominal operations in children. Although the symptoms are easily recognizable and generally lead to the correct diagnosis in most cases, the peculiarities of childhood can lead to errors in the diagnosis resulting in the complications reported herein. We emphasize the usefulness of ultrasound in the diagnosis and conservative treatment is advocated.

[Benign gingival granular cell tumor in the newborn: congenital epulis]. / Tumor gingival benigno de células granulares en el recién nacido: epulis congénito.

Gingival granular cell tumor, or congenital epulis, was first described by Neumman in 1871 and subsequently 201 cases have been published in 173 patients. It is an uncommon benign tumor that is present like a pedunculated, smooth surfaced, isolated lesion on the alveolar mucosa of the maxillar of the newborn child. Its firm consistency and variable size can occasionally cause problems in the child's breathing or feeding. This tumor is easily diagnosed clinically and, although spontaneous regression of the tumor mass has occasionally been reported, the current treatment is surgical removal. Two new cases of congenital epulis are reported and a literature review is included.

[Blunt abdominal trauma. Nonoperative management]. / Traumatismos abdominales cerrados: un enfoque conservador.

During the 7 year period, 1987 to 1994, 48 patients with blunt abdominal trauma were treated in our Hospital. There were splenic injury in 50% cases, liver trauma in 25% and renal injury in 40%. Seven patients suffered injuries to multiple sites. The clinical findings were confirm with ultrasound (47 children) and Computed Tomography scanning (CT) (19 children). Intravenous pyelography were performed in nine cases with previously suspect of renal affection. The CT scan provided diagnosis in four patients with negative findings in the sonography. In the present serine, 42 cases were managed by nonoperative means with 100% survivals. One patient surgically treated died from associated severe head injury. The postoperative complications appeared in ten nonoperatively cases (recurrence of bleeding in four children). 14 of these non surgical patients required blood transfusion, and the mean volume of transfusion was 30 ml/kg. A follow-up abdominal sonography was obtained in all survivors, showing resolution of the injury in all of them. We conclude that non surgical managements must be the first choice in all blunt abdominal trauma in pediatrics. Laparotomy is mandatory in masive bleeding or hemodynamically inestable patients. Ultrasonography is a simple method without risks and fast available, well indicated for diagnosis and follow-up of this children.