Complications after Nuss bar removal procedure for pectus excavatum. Analysis and proposal of a safety protocol. / Complicaciones tras retirada de barra de Nuss en pectus excavatum. Análisis y propuesta de un protocolo de seguridad.

OBJECTIVE: The Nuss bar removal procedure may bring about different complications. Some are mild while others can be life-threatening. An adequate surgery setup and the fulfilment of some security steps may reduce their incidence. This study aims to analyze our experience with the complications that occurred during bar removal and our safety protocol for the prevention and management of these complications. MATERIALS AND METHODS: Observational cohort study from a retrospective chart review of all patients who underwent Nuss bar removal from November 2013 to March 2022 at a University hospital. Variables analyzed include patients' demographics; presence of comorbidities; time elapsed from bar placement to removal, and the occurrence of operative and postoperative complications. Study written under the 'PROCESS Guideline'. RESULTS: Fourty (40) patients were included in the study; 37 were male. One bar was removed in 17 patients and two in 22 patients. Median age at surgery: 17.5 years (Percentile 25-75%: 16.75-19.25). Time elapsed from placement to removal: 26 months (Percentile 25-75%: 23.75-30.25). Complications: 10 in 9 patients (22.5%); 6 Clavien-Dindo class I (67%); 2 class II (22%); 1 class IIIb, 1 class IV. The hemorrhagic complication motivated the development of a safety protocol to reduce incidence of complications. CONCLUSION: Nuss bar removal is a safe procedure with usually scant complications. Nonetheless, these may be serious sometimes. To prevent them, a protocol for a safe procedure is important.

OBJETIVO: La retirada de la barra de Nuss puede provocar diversas complicaciones, algunas leves y otras potencialmente mortales. Su incidencia puede verse reducida con una preparación quirúrgica adecuada y siguiendo ciertos pasos de seguridad. El presente estudio tiene por objeto analizar nuestra experiencia con las complicaciones acontecidas durante la retirada de la barra, así como nuestro protocolo de seguridad para la prevención y el manejo de dichas complicaciones. MATERIAL Y METODOS: Estudio de cohortes observacional llevado a cabo a partir del análisis retrospectivo de todos los pacientes sometidos a cirugía de retirada de barra de Nuss entre noviembre de 2013 y marzo de 2022 en un hospital universitario. Se analizaron las siguientes variables: demografía de los pacientes, presencia de comorbilidades, tiempo desde la colocación de la barra hasta su retirada, y complicaciones operatorias y postoperatorias. El estudio se realizó conforme a las directrices de la PROCESS Guideline. RESULTADOS: Se incluyó a 40 pacientes, 37 de ellos varones. En 17 pacientes se retiró una barra, y en 22, dos. La edad media en el momento de la cirugía fue de 17,5 años (percentil 25-75%: 16,75 - 19,25). El tiempo transcurrido desde la colocación hasta la retirada fue de 26 meses (percentil 25-75%: 23,75 - 30,25). Se registraron 10 complicaciones en 9 pacientes (22,5%), 6 de clase I según la clasificación de Clavien-Dindo (67%), 2 de clase II (22%), 1 de clase IIIb y 1 de clase IV. La complicación hemorrágica motivó la creación de un protocolo de seguridad para disminuir la incidencia de las complicaciones. CONCLUSION: La retirada de la barra de Nuss es un procedimiento seguro, habitualmente con escasas complicaciones, aunque en ocasiones pueden ser graves. Para evitarlas, es importante contar con un protocolo que garantice la seguridad.

Non-syndromic Hirschsprung's disease as a result of a RET gene variant. / Enfermedad de Hirschsprung no sindrómica por variante en el gen RET.

INTRODUCTION: Hirschsprung's disease (HD) is characterized by the absence of ganglion cells in the submucosal and myenteric plexuses of the colon as a result of disorders in the migration and differentiation of enteric neural crest cells during embryogenesis. It is a cross-factor condition, with more than 11 genes identified in its pathogenesis, including the RET proto-onco gene. CASE REPORTS: We present the case of two siblings with total colon HD where a potentially pathogenic variant of the RET gene was found. Their father also had this condition. DISCUSSION: Prenatal diagnosis through genetic testing allows for informed decisions and care planning for the newborn, thus reducing delayed diagnosis and treatment, and minimizing long-term complications. Mutations such as the RET gene variant highlight the importance of the genetic approach in understanding and managing HD.

INTRODUCCION: La enfermedad de Hirschsprung (EH) se caracteriza por la ausencia de células ganglionares en los plexos submucoso y mientérico del intestino grueso, resultante de deficiencias en la migración y diferenciación de las células de la cresta neural entérica durante la embriogénesis. Es una condición multifactorial, con más de 11 genes identificados en su patogénesis, incluyendo el protooncogén RET. CASO CLINICO: Se presenta el caso de dos hermanos con EH de colon total, cuyo padre también padeció la enfermedad, y en quien se encontró una variante potencialmente patogénica en el gen RET. COMENTARIOS: El diagnóstico prenatal mediante pruebas genéticas permite decisiones informadas y la planificación de cuidados para el neonato afectado, reduciendo demoras en el diagnóstico y tratamiento, y minimizando las complicaciones a largo plazo. La identificación de mutaciones como la variante en el gen RET destaca la importancia del enfoque genético en la comprensión y manejo de la EH.

Association between subglottic stenosis and endotracheal intubation in tracheostomized pediatric patients. / Asociación entre estenosis subglótica e intubación endotraqueal en pacientes pediátricos traqueostomizados.

OBJECTIVE: Considering that intubation time is the primary cause of subglottic stenosis, tracheostomy is suggested in adult patients following 10-15 days. The objective of this study was to analyze the association between intubation time and stenosis in pediatric patients, as well as to establish whether there is an adequate timing for tracheostomy in order to reduce the incidence of stenosis. MATERIALS AND METHODS: A retrospective study (2014-2019) of tracheostomized newborns and children after an intubation period was carried out. Endoscopic findings at tracheostomy were analyzed. RESULTS: Tracheostomy was conducted in 189 patients, 72 of whom met inclusion criteria. Mean age was 40 months (1 month - 16 years). The incidence of stenosis was 21%, with a mean age of 23 months and a mean intubation time of 30 days vs. 19 days in the non-stenosis group (p= 0.02). The incidence of stenosis increased by 7% five days following intubation, reaching 20% after one month. Patients under 6 months old had greater tolerance to intubation periods without stenosis (incidence < 6% after 40 days, and median time to stenosis of 56 days vs. 24 days in patients over 6 months old). CONCLUSIONS: In patients with long intubation periods, preventive measures should be taken in order to avoid laryngotracheal injuries, and early tracheostomy should be considered.

OBJETIVOS: Considerando el tiempo de intubación la principal causa de estenosis subglótica, en adultos se sugiere realizar una traqueostomía a los 10-15 días. Se buscó determinar la asociación entre tiempo de intubación y estenosis en pediatría y establecer si existe, un momento en el que realizar una traqueostomía reduciría la incidencia de estenosis. MATERIAL Y METODO: Estudio retrospectivo (2014-2019) de neonatos y niños traqueostomizados luego de un período de intubación. Se analizaron los hallazgos endoscópicos al realizar la traqueostomía. RESULTADOS: Se traqueostomizaron 189 pacientes y 72 cumplieron criterios de inclusión. La edad media fue de 40 meses (1 mes a 16 años). La incidencia de estenosis fue de 21%, con edad media de 23 meses e intubación media de 30 días versus en el grupo sin estenosis fue de 19 días (p= 0 ,02). La incidencia de estenosis aumentó un 7% a los cinco días de intubación alcanzando el 20% al mes. Los menores de 6 meses presentaron mayor tolerancia a períodos de intubación sin estenosis (incidencia < 6% luego de 40 días y mediana de tiempo hasta la estenosis de 56 días, versus 24 días en mayores de 6 meses). 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.

Beyond image defined risk factors (IDRFs): a delphi survey highlighting definition of the surgical complexity index (SCI) in neuroblastoma.

PURPOSE: Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs do not all carry the same weighting in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize a surgical complexity (Surgical Complexity Index, SCI) in NB resection. METHODS: A panel of 15 surgeons was involved in an electronic Delphi consensus survey to identify and score a set of shared items predictive and/or indicative of surgical complexity, including the number of preoperative IDRFs. A shared agreement included the achievement of at least 75% consensus focused on a single or two close risk categories. RESULTS: After 3 Delphi rounds, agreement was established on 25/27 items (92.6%). A severity score was established for each item ranging from 0 to 3 with an overall SCI range varying from a minimum score of zero to a maximum score of 29 points for any given patient. CONCLUSIONS: A consensus on a SCI to stratify the risks related to neuroblastoma tumor resection was established by the panel experts. This index will now be deployed to critically assign a better severity score to IDRFs involved in NB surgery.

Tumores de parótida en población pediátrica: 10 años de experiencia / PAROTID TUMORS IN PEDIATRIC POPULATION: 10 YEARS OF EXPERIENCE

Introducción: Los tumores de las glándulas salivales son infrecuentes en la población en general y comprenden menos del 3% de los tumores de cabeza y cuello1 . En pediatría el 90% corresponden a tumores de la glándula parótida,2 siendo en su mayoría tumores benignos. Objetivo: Describir la incidencia de tumores parotídeos en un centro de referencia, haciendo hincapié en la variabilidad etiológica y la presentación por grupos etarios. Materiales y métodos: Se trata de un estudio de cohorte retrospectivo de pacientes menores de 18 años con tumores de parótida en un periodo de 10 años: de 2011 a 2021, seguidos en el Servicio de Cirugía General Pediátrica del Hospital Italiano de Buenos Aires. Se incluyeron todos los pacientes pediátricos que presentaron tumor en la región parotídea seguidos o tratados en este centro, se excluyeron aquellos que no se pudieron recabar los datos de forma completa. Las variables analizadas fueron edad, sexo, forma de presentación, estudios complementarios, tipo de tratamiento, histología, complicaciones. Resultados: Se analizaron un total de 33 pacientes, de estos un 64% (N = 21) fueron mujeres, con una relación mujer/hombre de 1,7. La media de edad al diagnóstico fue de 8.5 años, siendo 2 de diagnóstico prenatal y hasta los 18 años. La localización fue en un 57% (N = 19) izquierda, el 43% restante derecha. La forma de presentación más frecuente fue la masa palpable en región parotídea 75% (N=25), en dos pacientes fue un hallazgo en estudios por imágenes: resonancia prenatal y otro por resonancia de cerebro. Conclusiones: en pediatría, a diferencia de la población adulta, se presentan una gran variedad de diagnósticos, que incluye desde lesiones vasculares hasta tumores malignos. Debido a que las lesiones parotídeas malignas son clínicamente indistinguibles de las benignas, es importante establecer un diagnóstico preciso. Esta serie representa esta diversidad etiológica en pediatría, así como la distribución etaria comparada con la descrita por la literatura

Introduction: Salivary gland tumors are rare in the general population and comprise less than 3% of head and neck tumors1. In pediatrics, 90% correspond to tumors of the parotid gland,2 being mostly benign tumors. Objective: To describe the incidence of parotid tumors in a reference center, emphasizing the etiological variability and the presentation by age groups. Materials and methods: Tis is a retrospective cohort study of patients under 18 years of age with parotid tumors over a period of 10 years: from 2011 to 2021, followed up at the Pediatric General Surgery Service of the Italian Hospital of Buenos Aires. All pediatric patients who presented tumor in the parotid region followed up or treated in this center were included, those who could not collect the data completely were excluded. Te variables analyzed were age, sex, form of presentation, complementary studies, type of treatment, histology, and complications. Results: A total of 33 patients were analyzed, of which 64% (N = 21) were women, with a female/male ratio of 1.7. Te mean age at diagnosis was 8.5 years, with 2 prenatal diagnoses and up to 18 years. Te location was 57% (N = 19) left, the remaining 43% right. Te most frequent form of presentation was a palpable mass in the parotid region 75% (N=25), in two patients it was a finding in imaging studies: prenatal MRI and another by brain MRI. Conclusions: in pediatrics, unlike the adult population, a wide variety of diagnoses are presented, ranging from vascular lesions to malignant tumors. Because malignant parotid lesions are clinically indistinguishable from benign ones, it is important to establish an accurate diagnosis. Tis series represents this etiological diversity in pediatrics, as well as the age distribution compared to that described in the literature

Minimally invasive surgery for neuroblastic tumours: A SIOPEN multicentre study: Proposal for guidelines.

INTRODUCTION: Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. MATERIALS & METHODS: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. RESULTS: A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. CONCLUSIONS: MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours.

Prostatic Rhabdomyoma in a Toddler: A Case Report With Molecular Characterization.

We present a 29-month-old male patient in follow-up due to pyelocaliceal dilation with a prostatic nodule incidentally found during ultrasound evaluation. Cysto video endoscopy was performed and a prostate biopsy, obtained. Microscopic evaluation showed a haphazardly distributed population of muscular cells with cross striations without evidence of mitosis or necrosis. Immunohistochemistry was positive for myogenin and desmin and negative for smooth muscle actin. Next generation sequencing was performed without finding any pathogenic variant or fusion in the tumor RNA. The patient received no further treatment, remained asymptomatic and continues in follow up, 3 years after initial diagnosis. We report a case of prostate rhabdomyoma in a toddler, an exceptional location that raises concern about differential diagnosis with its malignant counterpart, rhabdomyosarcoma, especially at this age.

Usefulness of combined ultrasonography and scintigraphy in the preoperative assessment of secondary or tertiary hyperparathyroidism. / Utilidad de la combinación de ecografía y gammagrafía en evaluación preoperatoria de hiperparatiroidismo secundario o terciario.

OBJECTIVE: To determine whether combined ultrasonography and parathyroid scintigraphy improves hyperplastic parathyroid gland detection in the pediatric population for parathyroidectomy planning in patients with secondary or tertiary hyperparathyroidism. MATERIAL AND METHODS: An observational and analytical retrospective cohort study was carried out. Patients diagnosed with secondary or tertiary hyperparathyroidism from 2011 to 2018 undergoing total or subtotal parathyroidectomy were included - provided there was information available on pathological examination and surgical protocol. RESULTS: N = 15 patients. A total of 53 parathyroid glands diagnosed with hyperplasia using either of the imaging methods were analyzed. For each method (ultrasonography and scintigraphy) and the combination of both, sensitivity and area under the curve were calculated, using pathological examination result as a reference. Ultrasonography and scintigraphy diagnostic match was 66%. DISCUSSION AND CONCLUSIONS: The intraoperative difficulty of parathyroid gland identification as well as the anatomical variation that these present is well-known. Ultrasonography detected more glands than scintigraphy when diagnosing parathyroid hyperplasia. The combination of both methods allows patients with a first negative study to be detected.

OBJETIVO: Determinar si la combinación de la ecografía y la gammagrafia paratiroidea mejora la capacidad de detección de glándulas paratiroideas hiperplásicas en población pediátrica para la planificación de paratiroidectomía en pacientes con hiperparatiroidismo secundario o terciario. MATERIAL Y METODOS: Estudio observacional y analítico de una cohorte retrospectiva. Se incluyeron pacientes con hiperparatiroidismo secundario o terciario, entre 2011 y 2018, que fueron operados de paratiroidectomía total o subtotal, en los que haya podido recabarse información de la anatomía patológica y protocolo quirúrgico. RESULTADOS: N = 15 pacientes. Se analizaron un total de 53 glándulas paratiroides con diagnóstico de hiperplasia en alguno de los métodos por imágenes evaluados. Para cada método (ecografía y gammagrafía) y para la combinación de ambos, se obtuvieron la sensibilidad y área bajo la curva, tomando como referencia el resultado obtenido por anatomía patológica. La concordancia en el diagnóstico de la ecografía y de la gammagrafía fue del 66%. DISCUSION Y CONCLUSIONES: Es bien conocida la dificultad intraquirúrgica que se plantea en cuanto a la localización de las glándulas paratiroides así como la variación anatómica que estas presentan. La ecografía detectó más glándulas que la gammagrafía en el diagnóstico de hiperplasia paratiroidea. La combinación de ambos métodos permite detectar a aquellos pacientes en los cuales un primer estudio resultó negativo.

[Thyroid nodules in children: risk of malignancy for each Bethesda category]. / Nódulo tiroideo en pediatría: riesgo de malignidad para cada categoría Bethesda.

PURPOUSE: Thyroid nodules are unusual findings in children. Some authors highlight the higher rate of malignancy in this group. The Bethesda system, created in adults to stratify thyroid nodules according to the risk of malignancy, constitutes a reference system for the management of this pathology. The American Thyroid Association promotes its use in the pediatric population, although there is no available data showing an equivalent risk. The aim of this study was to assess the risk of malignancy represented by each Bethesda stage in a pediatric study population. METHODS: A retrospective cohort study was performed in pediatric patients with thyroid nodules biopsied by fine needle aspiration, during the period 2005-2017. During the follow-up, the outcome was assessed by comparing the Bethesda stage (cytology) with the surgical specimen histology. For patients not surgically treated, Bethesda Class was compared with the clinical and imaging follow up. RESULTS: 105 patients with fine needle aspiration of a thyroid nodule were analyzed and classified by the Bethesda system. 47 patients were excluded for incomplete follow-up. All Bethesda I nodules were benign, 6.6% of Bethesda II were malignant and all Bethesda IV, V and VI nodules were histologically malignant. CONCLUSION: The rate of malignancy among patients with Bethesda II, IV, V and VI was higher than published in Bethesda classification for adults. The risk of malignancy in pediatric patients might be greater than expected. These results may have a significant impact on follow-up strategies and also in therapeutic algorithms.

OBJETIVO: El nódulo tiroideo es un hallazgo infrecuente en pediatría. Algunos autores destacan la mayor tasa de malignidad en este grupo. La clasificación Bethesda, creada en pacientes adultos para estratificar los nódulos tiroideos según el riesgo de malignidad, constituye un sistema de referencia en el algoritmo terapéutico de esta patología. La American Thyroid Association propone homologar esta clasificación a la población pediátrica, si bien no existen datos que demuestren que el riesgo sea equivalente. El objetivo fue evaluar el riesgo de malignidad de cada categoría Bethesda en la población pediátrica. MATERIALES Y METODO: Se realizó un estudio de cohorte retrospectivo en pacientes pediátricos con nódulos tiroideos punzados con aguja fina durante el periodo 2005-2017. Luego se analizó su evolución ulterior comparando la categoría Bethesda asignada (citología) con la histología de la pieza quirúrgica. Para los pacientes sin indicación quirúrgica, se comparó con la evolución clínica e imagenológica. RESULTADOS: 105 pacientes cumplieron los criterios de inclusión. Se excluyeron 47 por seguimiento inadecuado. El 100% de los Bethesda I fueron nódulos benignos. El 6,5% de los Bethesda II fueron malignos. Todos los nódulos Bethesda IV, V y VI fueron malignos por histología. CONCLUSION: El porcentaje de malignidad entre los nódulos Bethesda II, IV, V y VI fue mayor al publicado. El riesgo de malignidad entre los pacientes pediátricos podría ser mayor al descrito en adultos para cada categoría de Bethesda. Estos resultados podrían ser significativos a la hora de establecer las estrategias tanto terapéuticas como de seguimiento.

Serotype b of Aggregatibacter actinomycetemcomitans triggers pro-inflammatory responses and amyloid beta secretion in hippocampal cells: a novel link between periodontitis and Alzheimer´s disease?

Introduction: Previous reports have proposed that Periodontal disease (PDis) predisposes to Alzheimer's disease (AD), both highly prevalent pathologies among the elderly. The bacteria Aggregatibacter actinomycetemcomitans (Aa), associated with the most aggressive forms of PDis, are classified in different serotypes with distinct virulence according to the antigenicity of their lipopolysaccharide (LPS). Methods: Here, we determined the effects of purified LPS, from serotypes a, b or c of Aa, on primary cultures of microglia or mixed hippocampal cells. Results: We found that both culture types exhibited higher levels of inflammatory cytokines (IL-1ß, IL-6 and TNFα) when treated with serotype b-LPS, compared with controls, as quantified by qPCR and/or ELISA. Also, cultures treated with serotype a-LPS displayed increased mRNA levels of the modulatory cytokines IL-4 and IL-10. Mixed hippocampal cultures treated with serotype b-LPS exhibited severe neuronal morphological changes and displayed increased levels of secreted Aß1-42 peptide. These results indicate that LPS from different Aa serotypes triggers discriminatory immune responses, which differentially affect primary hippocampal cells. Conclusion: Altogether, our results show that treatment with serotype b-LPS triggers the secretion of proinflammatory cytokines by microglia, induces neurite shrinking, and increases the extracellular Aß1-42 levels, all features strongly associated with the etiology of AD.

[Total parathyroidectomy, autoimplant and cryopreservation for the treatment of hyperparathyroidism of renal origin in children and young adults]. / Paratiroidectomía total, autoimplante y criopreservación para el tratamiento del hiperparatiroidismo de origen renal en pediatría y adultos jóvenes.

OBJECTIVES: To describe our initial experience in the treatment of hyperparathyroidism (HPP) of renal cause using total or subtotal parathyroidectomy, autoimplant and cryopreservation in pediatric patients. Secondary HPP is the increased function of the parathyroid hormone (PTH) due to an abnormal phosphocalcic metabolism in patients with chronic renal failure (CRF). This situation produces increased bone resorption resulting in osteodystrophy and endovascular calcifications. Surgical treatment is aimed to diminish the level of PTH in CRF patients, to avoid HPP complications. METHODS AND MATERIALS: Descriptive, monocentric and retrospective study of a case series of patients with secondary and tertiary hyperparathyroidism, who went through total or subtotal parathyroidectomy, autoimplant and cryopreservation between 2009 and 2016. We analyzed the following variables: age, calcemia, PTH, phosphatemia, alkaline phosphatase (ALP), follow-up and complications. The continuous variables are expressed in median and interquartile range or in mean and SD, according to their distribution. The categorical variables were expressed in percentages and frequencies (repeated sentence). RESULTS: Number of patients included: 13. Mean age of the patients was 16.7 years old. Preoperative median calcium dosage was 9.1 mg/dl (IQR: 8.9-9.5). Median PTH was 2,600 pg/ml (IQR: 1,400 pg/ml to 2,785 pg/ml). Intraoperatory dosage of PTH reported a median drop of 86.6% in the first 15 minutes (IQR: 80.5-95.9). After the first 48 hours, median calcemia was 9 mg/dl (IQR: 7.7-9.4) and median PTH was 40 pg/ml (IQR: 20-113). We did not identify intraoperatory complications. In the immediate post operatory stage, mean IV calcium therapy was 4 days (SD: 2.39). Median time of follow-up was 18 months (IQR 9-36). Two patients had hungry bone syndrome and one patient had a recurrence of the pathology as remote post operatory complications. After a year, median calcemia, was 9 mg/dl (IQR: 7.6-9.3) and median PTH was 50 pg/ml (IQR: 28.5-108). The decrease in PTH and ALP were statistically significant with p value < 0.05. CONCLUSION: In our study, total parathyroidectomy with auto implant is a safe and effective option for the treatment of secondary and tertiary hyperparathyroidism in pediatric patients. This could also prevent bone complications.

OBJETIVOS: Describir la experiencia en el tratamiento del hiperparatiroidismo (HPP) de origen renal con la técnica de la paratiroidectomía total o subtotal, autoimplante y criopreservación en pacientes pediátricos y adultos jóvenes. El HPP secundario es el aumento de la función de las hormonas paratiroideas debido a una alteración en el metabolismo fosfocálcico a partir de la insuficiencia renal crónica (IRC). Esto produce una mayor resorción ósea, provocando alteraciones en los huesos y calcificaciones en el endotelio vascular. El tratamiento quirúrgico se indica para disminuir el nivel de parathormona (PTH), y así prevenir las complicaciones del hiperparatiroidismo en pacientes con IRC terminal. MATERIAL Y METODOS: Estudio descriptivo, retrospectivo, monocéntrico de una serie de pacientes con diagnóstico de HPP secundario y terciario sometidos a paratiroidectomía total, autoimplante y criopreservación entre 2009 y 2016. Se analizaron las siguientes variables: Edad, Calcemia, PTH, Fosfatemia, Fosfatasa alcalina (FAL), tiempo de seguimiento y complicaciones. Las variables continuas se expresan en mediana y rango intercuartil o en media y DS, según su distribución. Las variables categóricas se expresaron en porcentajes y frecuencias. RESULTADOS: Se incluyeron 13 pacientes. La edad media fue 16,7 años (R= 11:24). La mediana de calcemia preoperatoria fue 9,1 mg/dl (RIQ: 8,9-9,5). La mediana preoperatoria de PTH fue 2.600 pg/ml (RIQ: 1.400-2.785). La medición del descenso porcentual de PTHi ultrarrápida informó una mediana de 86,6% en los primeros 15 minutos (RIQ: 80,5-95,9). No se identificaron complicaciones intraoperatorias. A las 48 horas posquirúrgicas, la mediana de calcemia fue de 9 mg/dl (RIQ: 7,7-9,4) y la mediana de PTH de 40 pg/ml (RIQ: 20-113). La media de aporte de calcio endovenoso posquirúrgico fue de 4 días (DS 2,39). La mediana de seguimiento fue de 18 meses (RIQ 9-36). Como complicaciones posoperatorias tardías se identificaron dos pacientes con síndrome de hueso hambriento y un paciente con recidiva de la enfermedad. Al año postoperatorio la mediana de calcemia, fue de 9 mg/dl (RIQ: 7,6-9,3). La mediana de PTH fue de 50 pg/ml (RIQ: 28,5-108)y la de FAL fue de 116 UI/L (RIQ 102:273). El descenso de PTH y de FAL fueron estadísticamente significativos con p valor < 0,05. CONCLUSIONES: En nuestro estudio, la paratiroidectomía total con autoimplante es una alternativa segura y efectiva para el tratamiento del hiperparatiroidismo secundario y terciario en pediatría, permitiendo prevenir así complicaciones óseas y cardiovasculares.

Neonatal Serial Transverse Enteroplasty (STEP): Case Report.

CASE REPORT: Gastroschisis is the most frequent congenital abdominal wall defect. When associated with intestinal atresia (complex gastroschisis), short bowel syndrome may occur. Complicated gastroschisis is the most frequent cause of short bowel syndrome in our series. The serial transverse enteroplasty procedure has been used to lengthen the bowel and achieve intestinal rehabilitation in patients with dilated gut. The use of this technique in the newborn period, for tailoring the bowel while preserving absorptive mucosa, has been recently described. We present a video showing the surgical treatment of an intestinal obstruction produced by a complex intestinal atresia in a newborn baby in whom a primary closure of a gastroschisis had been done at birth. During laparotomy at the 21st day of life, a type IVa intestinal atresia was found, associated with a colonic stenosis. Proximal dilated jejunum was tailored with a serial transverse enteroplasty procedure, as shown in the video. End-to-end jejunal-ileal anastomosis was performed. Postoperative entero-cutaneous fistula occurred and was treated with vacuum-assisted therapy. Enteral feedings were initiated at 15 days after surgery. Parenteral nutrition was withdrawn at 30 days. After 16 months follow-up, actual weight was 8.7 kg (percentile 10% to 25%). The patient remained on full enteral feedings. CONCLUSIONS: In cases of intestinal atresia, short bowel syndrome, and proximal dilated bowel, we propose an aggressive surgical approach to preserve bowel mucosal surface while tailoring the jejunal loop to improve motility. The serial transverse enteroplasty procedure is an acceptable alternative to tailoring methods that resect a segment of the bowel wall.

[Diagnosis and treatment of the tumors of the parotid region in Pediatrics: cohort]. / Diagnóstico y tratamiento de los tumores de la región parotídea en Pediatría: cohorte.

INTRODUCTION: Parotid tumors are rare in children and usually have multiple differential diagnoses that require different diagnostic and treatment methods. MATERIAL AND METHODS: Retrospective cohort study of all consecutive pediatric patients with parotid tumors that were treated in Pediatric Surgery Service of Hospital Italiano de Buenos Aires. RESULTS: Twenty-two patients with parotid tumors were included and 72% (n=16) were female patients. The median age was 10 years (range: prenatal to 19 years). Imaging studies: ultrasound, CT and MRI. Diagnostic methods: needle or open biopsy (14), biopsy of lymph nodes (3), imaging (4), blood test (1). Treatment: surgical resection (12), medical treatment (7), sclerotherapy (2). Diverse histological diagnosis were obtained: pleomorphic adenoma (4), infantile hemangioma (4), PNET (2), low flow vascular malformation (2), Castleman's disease (1), schwannoma (1), neuroblastoma (1), alveolar rhabdomyosarcoma (1), mucoepidermoid carcinoma (1) RICH (Rapidly Involuting Congenital Hemangioma) (1), granulomatous disease (1), reactive adenitis (1), parotid cyst (1), ALL (Acute Lymphoblastic Leukemia) (1). Follow-up (42,5 months): 18 patients are free of disease, 3 are currently under medical treatment and 1 died. CONCLUSIONS: Our series shows the diversity that parotid tumor can present in pediatric population and is consistent with the literature there the incidence of benign lesions are predominant. However, it is difficult to predict risk of malignancy. An exhaustive diagnosis is important, using complementary methods and biopsy whenever that is necessary.

INTRODUCCION: Los tumores de la región parotídea son raros en Pediatría, y suelen presentar múltiples diagnósticos diferenciales que requieren diferentes métodos diagnósticos y de tratamiento. MATERIAL Y METODOS: Estudio de cohorte retrospectivo de todos los pacientes pediátricos consecutivos con tumores de la región parotídea en el periodo 2003-2016, que fueron atendidos en el Servicio de Cirugía Pediátrica del Hospital Italiano de Buenos Aires. RESULTADOS: Se incluyeron 22 pacientes con tumores de la región parotídea. El 72% (n=16) fueron pacientes del sexo femenino. La mediana de edad fue de 10 años (rango: prenatal a 19 años). Estudios complementarios: Ecografía, TC y RMN. Confirmación diagnóstica: biopsia por punción o quirúrgica (14), biopsia de adenopatías (3), diagnóstico por imágenes (4), laboratorio (1). Tratamiento: resección quirúrgica (12), tratamiento médico (7), escleroterapia (2). Diagnósticos diferenciales: adenoma pleomorfo (4), hemangioma infantil (4), malformación vascular de bajo flujo (2), PNET (2), enfermedad de Castleman (1), schwannoma (1), neuroblastoma (1), rabdomiosarcoma alveolar (1), carcinoma mucoepidermoide (1), RICH (hemangioma congénito rápidamente involutivo) (1), enfermedad granulomatosa (1), adenitis reactiva (1), quiste parotídeo (1), LLA (leucemina linfoblástica aguda) (1). Seguimiento medio de 42,5 meses: libres de enfermedad (18), realizando tratamiento médico (3), óbito (1). CONCLUSIONES: Nuestra serie demuestra la gran variedad diagnóstica que pueden presentar los tumores de la región parotídea en la población pediátrica y concuerda con la literatura donde se observa mayor incidencia de lesiones benignas. Sin embargo, es difícil predecir el riesgo de malignidad. Es importante realizar un diagnóstico exhaustivo, utilizando métodos complementarios de imágenes y biopsia ante la duda diagnóstica.

14 years of experience with the artificial urinary sphincter in children and adolescents without spina bifida.

PURPOSE: The efficacy of the artificial urinary sphincter to treat sphincteric incontinence in pediatric patients with spina bifida has been clearly reported. The possibility of maintaining spontaneous voiding has usually been the main reason for prosthetic device surgery. We reviewed our experience with the artificial urinary sphincter in patients without spina bifida who had had previous surgery of the bladder neck or proximal urethra. MATERIALS AND METHODS: From 1990 to 2004, 112 children and adolescents underwent implantation of an AMS 800 artificial urinary sphincter. Of the patients 19 males and 4 females (20.5%) between ages 4 and 17 years (mean 8.1) had no spina bifida. Instead there were bladder exstrophy in 12 patients, anorectal malformation with a rectourethral or vesical fistula in 7 and epispadias in 4. A bladder neck cuff between 5.5 and 7.5 cm, and a 61-70 balloon were used in all patients. RESULTS: Only 1 patient was lost to followup. In 22 patients (95.6%) mean followup was 80 months (range 4 to 155). Three sphincters in patients with exstrophy were removed because of erosion and/or infection 5, 49 and 60 months after initial surgery, respectively. A total of 19 sphincters remained in place (86.3% survival rate) with 5 revisions (26.3%) because of the pump (2), the cuff (2) or balloon fluid leakage. In this group 13 patients (68.4%) voided spontaneously and 6 (31.6%) performed clean intermittent catheterization, although 3 also voided spontaneously. Overall continence was good in 87% of patients because 2 were still incontinent at night. CONCLUSIONS: The artificial urinary sphincter is a good long-term solution to urinary incontinence secondary to sphincter incompetence despite multiple previous surgeries of the bladder neck or proximal urethra. Patients with bladder exstrophy and many previous bladder procedures are more exposed to complications such as erosion compared with patients with epispadias or anorectal malformation. The high percent of patients maintaining spontaneous voiding and the good rate of continence are the most important benefits of this type of surgical option for sphincter incompetence.

Asistencia endoscópica en la cirugía de la fístula traqueoesofágica / Assistance endoscopic in the surgery of the tracheoesophageal fistula

La fístula traqueoesofágica congénita, sin atresia de esófago, conocida como fístula en H, es una malformación rara de la vida aérea, que ocurre en sólo en 4 por ciento de las malformaciones traqueoesofágica y su incidencia es de 1:50.000-80.000 nacimientos. El tratamiento de elección es el cierre quirúrgico de la fístula. Para este procedimiento es siempre preferido el abordaje cervical sobre el torácico ya presenta una mejor complejidad y morbilidad. La asistencia endoscópica por broncoscópia esofagoscopía rígidas permite la cateterización del trayecto fistuloso, la individualización de la fístula y la movilización de la misma dentro del cuello, para ofrecer un acceso más fácil por cervicotomía lateral. Se presenta al Servicio de Otorrinolaringología del Hospital Italiano de Buenos Aires una paciente de 2 meses de edad con falta de progreso de peso, tos emetizante y fiebre sin foco. Como antecedente habia padecido una neumonía tratada en los quince días previos.Frente a la sospecha de episodios aspirativos, se realizó Videodeglución donde se detectó Fístula Traqueoesofágica en H. Se realizó también Rinofibrolaringoscópia para evaluación dinámica de laringe. Se decide cierre quirúrgico de la fístula a través de abordaje crevical, para lo que se planeó asistencia endoscópica para cateterización e identificación de la misma.

Cirugía Conservadora en el tratamiento de los tumores benignos / Conservative surgery in the treatment of benign testicular tumors in childhood

El tratamiento clásico de los tumores testiculares ha sido la orquidectomía radical.En los últimos años esta conducta se ha modificado mediante un tratamiento más conservador:la tumorectomía con conservación de parénquima sano.Esta terapeútica está basada en datos preoperatorios,como la ecografía de alta definición,el dosaje negativo de marcadores serológicos y fundamentalmente,en la anatomía patológica intraoperatoria(congelación)dato esencial para decidir una conducta conservadora.En esta revisión presentamos la experiencia de nuestro servicio en 4 niños con tumores testiculares benignos,que fueron tratados con conservación de la gónada

Sigmoid cystoplasty with argon beam without mucosa.

PURPOSE: Intestinal bladder augmentations have well recognized complications, including mucus production, metabolic abnormalities and perforation. These complications may be avoided if the intestinal mucosa is not incorporated in the urinary tract. We report our experience with sigmoid cystoplasty without mucosa using argon beam, and describe the clinical, urodynamic, ultrasound and pathological results. MATERIALS AND METHODS: We performed sigmoid cystoplasty without mucosa in 26 patients and with argon beam over the mucosa before it was removed in 6 boys and 4 girls with a mean age of 8 years (range 3 to 14). All patients had neurogenic bladder as the initial disease. Indications for augmentation were poor bladder compliance, low bladder capacity, hydronephrosis and urinary incontinence. Mean followup was 18 months (range 8 to 40) and included ultrasound, urodynamic evaluation, renal function and clinical assessment. In all patients intraoperative biopsies were done. In 8 of the 10 patients endoscopic biopsies of the augmented segment were obtained between 6 months and 2 years postoperatively. The operation consisted of the Goodwin technique without mucosa, which was treated with argon beam before it was removed. RESULTS: The 10 patients are dry on intermittent clean catheterization with intervals of greater than 4 hours. There have been no clinical urinary tract infections. Two patients presented with peristaltic contractions and no symptoms. Bladder capacity increased from 80 (range 45 to 200) to 300 (220 to 400) ml., and mean postoperative compliance was 15 ml./cm. H2O (range 9 to 38). There were no significant changes in the urodynamic data between patients treated with sigmoid cystoplasty without mucosa only and with argon beam. Intraoperative biopsies after treatment with argon beam showed damaged mucosa and muscularis mucosa, and intact serosa, muscularis and submucosa layers. On the 8 argon beam postoperative biopsies the sigmoid submucosa was covered with a pseudostratified metaplasia of connective tissue with collagen fibers without scars (trichromic technique). CONCLUSIONS: Sigmoid cystoplasty without mucosa with argon beam is easy to perform. The clinical and urodynamic results have been satisfactory, and use of argon beam prevents postoperative bleeding and residual glands.

Transferrina carbohidrato deficiente, gammaglutamil transferasa y volumen corpuscular medio en la evaluación de la ingesta alcohólica reciente de bebedores excesivos / Carbohydrate deficient transferrin for the assessment of recent alcohol intake in excessive drinkers

Background: There are no reliable markers to detect heavy drinking or as a tool to control abstinence compliance in alcoholic treatments. The Mean Corpuscular Volume (MCV), and the gammaglutamyl transpeptidase (GGT), are widely used although their predictive value is somewhat limited due to their low specificity. On the other hand, the Carbohydrate-deficient transferrin (CDT) described in the eighties is highly specific and would be of value in early detection of problem drinking. Aim: To compare the sensitivity and specificity of CDT, GGT, and MCV in order to evaluate their single and combined use as markers for detection of heavy drinking behaviour. Patients and Methods : CDT, GGT, and MCV values were determined in blood samples from (a) alcoholics (drinking more than 100 9 alcohol/day; n=47) and (b) healthy volunteers, teetotalers from the Church of Saints of Later Days (n=34). At the time of sampling alcoholics were presently drinking or had been abstinents for no more than six weeks. ROC curves were used to determine the best cut-off point for each marker. Results: Sensitivity was found to be similar for all three markers. Specificity was found higher for GGT (90.9 percent) and CDT (91.0 percent). The combined use of MCV, GGT and CDT, that is, when at least one of the markers is altered, was shown to detect 83 percent of the patients. No correlation was observed between the markers and the level of alcohol intake. Conclusions: CDT could be of value as a marker to detect heavy drinking when used with GGT and MCV values combined. CDT is particularly higher in drinking alcoholics and remains significantly high for at least six weeks after they stop drinking

Pólipo fibroide inflamatorio: causa de invaginación intestinal / Inflammatory fibroid polyp: cause of bowel intussusception

Se presentan dos casos de invaginación intestinal cuya causa fue un pólipo fibroide inflamatorio. Ellos corresponden a un niño y un paciente adulto. En ambos pacientes la invaginación fue precedida por una historia de dolor abdominal recurrente. Los estudios de imagen previo a la intervención quirúrgica fueron ultrasonografía abdominal y tomografía axial computarizada (TAC) en ambos casos. Se destacan las características del tumor y su presentación en el estudio de imágenes

Obstrucción intestinal en el prematuro de extremado bajo peso / Small bowel obstruction in very low birth weight preterm newborns

Seis prematuros con extremado bajo peso al nacer(PEBP,< 600 g)presentaron un cuadro de obstrucción intestinal seguido de perforación,ninguno evolucionó como una enterocolitis necrotizante (ECN).Los seis fueron operados,cuatro por neumoperotoneo,uno por una masaabdominal palbable y el restante por obstrucción intestinal.Los hallazgos quirúrgicos fueron,en todos los casos,impactación meconial en íleon distal con perforación inmediatamente proximal.Se realizó una resección segmentaria del íleon y una ileostomía.Tres pacientes fallecieron antes de los 45 días postoperatorios y el resto evolucionó favorablemente.La perforación intestinal no relacionada con ECN ocurre con cierta freceuncia en los PEBP.Prevenir la perforación y encontar la causa que la produce,constituyen el mayor desafío en el tratamiento de esta patología