[Endovascular embolization in the treatment of the pulmonary intralobar sequestration]. / Embolización endovascular en el tratamiento del secuestro pulmonar intralobar.

INTRODUCTION: Up to date the classical therapeutic approach for intralobar sequestrations (IS) has been surgical excision. However, systemic arteries embolization is presented as an alternative, and even constitutes the first line of treatment in some centers. We summarize our experience in selected cases with the aim of preserving the maximum lung parenchyma. MATERIAL AND METHODS: Retrospective study of IS who underwent endovascular embolization at our institution between 2013 and 2014. RESULTS: Three patients of 12, 14 and 21 months old were treated. Two patients had unilateral IS, one in the left lower lobe (LLL) and the other in the right lower lobe (RLL); the third one had bilateral lesions (a CAM-S complex in the RLL and a IS in the LII). The embolization of the four lesions was performed via right femoral artery. The case with bilateral lesions underwent thoracoscopic lobectomy of the CAM-S 7 months after embolization, finding adherences to the diaphragm. None of the patients had immediate complications and were discharged within 48 hours after embolization. In successive ultrasound and plain radiographs controls, with a follow-up of 6, 18 and 30 months, no complications were found. CONCLUSIONS: Systemic vessels embolization is a treatment option for the treatment of IS that avoids surgery, preserves lung parenchyma and does not preclude surgical resection in case of treatment failure or presence of parenchymal lesions. Longer follow-up is needed to determine long-term effectiveness.

INTRODUCCION: El tratamiento de elección de los secuestros intralobares (SI) es la lobectomía pulmonar. La mayoría de complicaciones se asocian a la vascularización sistémica pulmonar anómala. La embolización de las arterias sistémicas se presenta como una alternativa terapéutica, que incluso constituye la primera línea de tratamiento en algunos centros. Resumimos nuestra experiencia al aplicar esta técnica en casos seleccionados. MATERIAL Y METODOS: Estudio retrospectivo de los casos con SI tratados en nuestro centro mediante embolización entre 2013 y 2014, centrado en las indicaciones, resultados, complicaciones y seguimiento. Siendo la lobectomía la primera línea de tratamiento la embolización se reservó para casos seleccionados: bilaterales, con el objetivo de preservar parénquima pulmonar o ante el rechazo familiar a la intervención quirúrgica. RESULTADOS: Se trataron tres pacientes de 12, 14 y 21 meses. Dos presentaban un SI unilateral, uno en lóbulo inferior izquierdo (LII) y otro en el lóbulo inferior derecho (LID); el tercero lesiones bilaterales (un SI en LID y un complejo MAQ-secuestro en LII). Se embolizaron las cuatro lesiones por punción de arteria femoral derecha. El caso con lesiones bilaterales fue intervenido a los 7 meses tras la embolización, realizándose una lobectomía toracoscópica del complejo MAQ-secuestro, hallando adherencias al diafragma. Ninguno presentó complicaciones inmediatas y fueron dados de alta a las 48 horas. En los controles realizados con ecografía y radiografías simples, con seguimientos de 31, 47 y 56 meses, no se han observado complicaciones clínicas ni radiológicas. CONCLUSIONES: La embolización de los vasos sistémicos es una opción en el tratamiento del SI que permite evitar una cirugía, conservar parénquima pulmonar y no impide la exéresis quirúrgica en caso de fallo del tratamiento o lesión parenquimatosa. Es preciso un mayor seguimiento para determinar la efectividad a largo plazo.

[Current anesthesia risk of anterior mediastinal masses]. / Riesgo anestésico actual de las masas mediastínicas anteriores.

AIM: To analyze the current risk of an anesthetic event during surgical acts in pediatric patients with anterior mediastinal masses (AMM) in a tertiary oncology center, using the previously published risk factors to plan the procedure. MATERIALS AND METHODS: Retrospective study (2009-2015) of pediatric patients with AMM who underwent surgical procedures at debut. Published risk factors (symptoms, radiological findings), with special focus on the statistically significant ones, diagnosis, surgical and anesthetic procedure, special measures, and anesthetic events were recorded. Patients were classified as high or low-risk when airway or vascular compression or severe symptoms were present. MAIN RESULTS: Retrospective study (2009-2015) of pediatric patients with AMM who underwent surgical procedures at debut. Published risk factors (symptoms, radiological findings), with special focus on the statistically significant ones, diagnosis, surgical and anesthetic procedure, special measures, and anesthetic events were recorded. Patients were classified as high or low-risk when airway or vascular compression or severe symptoms were present. CONCLUSIONS: Preoperative evaluation of risk in AMM through clinical history and CT/MRI and surgical/anesthetic planning leads to excellent outcomes. The least aggressive procedures should be favored, but if needed, planned general anesthesia under experienced hands is safe even in risk patients.

OBJETIVO: Analizar el riesgo actual de eventos anestésicos durante intervenciones quirúrgicas en pacientes con masas mediastínicas anteriores (MMA) en un centro terciario, utilizando los factores de riesgo publicados en la literatura para planificar el procedimiento. MATERIALES Y METODOS: Estudio retrospectivo (2009-2015) de los pacientes pediátricos con MMA sometidos a procedimientos quirúrgicos al debut. Se registraron: factores de riesgo publicados (clínicos, radiológicos), particularmente los estadísticamente significativos, diagnóstico, procedimiento quirúrgico y anestésico, medidas especiales y eventos anestésicos. Los pacientes se clasificaron como alto o bajo riesgo según presentasen compresión en vía aérea o vascular o síntomas de gravedad. RESULTADOS: Registramos a 38 pacientes (media de edad 11,9 años ± 4,2). El 94,7% presentaban trastornos linfoproliferativos. El 50% tenían síntomas respiratorios y el 76,3% fueron considerados como de alto riesgo, al presentar uno o más factores ES. Solo 4 casos recibieron neoadyuvancia. Se realizaron 29 biopsias (3 toracoscópicas), 11 colocaciones de catéteres centrales, 2 drenajes torácicos y 3 resecciones. La técnica anestésica consistió en sedoanalgesia (60,5%) y diversas técnicas de anestesia general (41,4% de los casos de alto riesgo). Solo registramos 2 eventos (5,3%) consistentes en dificultad para la ventilación, respondiendo ambos a medidas básicas. Todas las intervenciones se finalizaron satisfactoriamente. CONCLUSIONES: La evaluación preoperatoria del riesgo en MMA mediante historia clínica y TAC/RMN junto a la planificación anestésico-quirúrgica conduce a unos resultados excelentes. Deben favorecerse los procedimientos menos invasivos, pero si son precisos, la anestesia general planificada y en manos expertas es segura incluso en pacientes de riesgo.

[Autologous blood pleurodesis for treatment of spontaneous pneumothorax and persistent air leak in pediatric patients]. / Pleurodesis con sangre autóloga en el tratamiento del neumotórax en pacientes pediátricos.

AIM OF THE STUDY: Recurrent spontaneous pneumothorax (SP) and persistent air leak (PAL) are a therapeutic challenge in some patients. Autologous blood pleurodesis (ABP) is an alternative treatment, but its usefulness in pediatric patients has not been determined yet. MATERIAL AND METHODS: Retrospective study of pediatric patients treated with ABP at our institution between 2010 and 2014, with special assessment of its indications, description of the technique, volume of blood used, complications and outcomes. RESULTS: During this period, 29 patients were treated for SP. 5 of them (17.2%) received ABP. Indications were: 2 patients with recurrent SP after thoracoscopic bullae resection and pleurodesis and 3 patients with PAL (1 after thoracoscopic bullae resection and 2 in lung transplantation candidates who were not suitable for surgery). Median age was 14.3 years (11.9-16.6) and volume of blood used was 50 ml (26-60). The air leak stopped in a median of 2.6 days (1-7). One patient needed a second ABP for PAL and another one presented an ipsilateral recurrence of SP after ABP. Follow up time was 2.21 years (0.49-3.42). No complications were observed. CONCLUSIONS: ABP is a cheap, safe and easy to perform procedure and may be considered as a therapeutic option in some pediatric patients with SP or PAL.

OBJETTIVOS: Los neumotórax recurrentes y la fuga aérea persistente (FAP) plantean dificultades terapéuticas en determinados pacientes. La pleurodesis con sangre autóloga (PSA) constituye una alternativa en su tratamiento, aunque su utilidad en pacientes pediátricos no ha sido determinada. MATERIAL Y METODOS: Estudio retrospectivo de los pacientes pediátricos tratados con PSA en nuestro centro entre los años 2010 y 2014, centrado en las indicaciones, descripción de la técnica de administración, cantidad de sangre empleada, complicaciones y resultados. RESULTADOS: Durante este periodo, 29 pacientes presentaron neumotórax espontáneos. En 5 de ellos (17,2%) se realizó PSA como método de rescate. Las indicaciones fueron: 2 recidivas de neumotórax tras resección de bullas y pleurodesis y 3 casos de fuga aérea persistente (1 tras resección toracoscópica de bullas subpleurales y en 2 candidatos a trasplante pulmonar con bullas en los que se descartó la cirugía). La mediana de edad fue de 14,3 años (11,9-16,6) y la cantidad de sangre empleada 50 ml (26-60). La fuga aérea se resolvió en una media de 2,6 días (1-7). Un paciente requirió una nueva PSA por persistencia de fuga aérea tras la primera administración y otro presentó una recurrencia posterior del neumotórax. El tiempo de seguimiento fue de 2.21 años (0,49-3,42). No se describieron otras complicaciones. CONCLUSIONES: La PSA es un método de fácil aplicación, seguro y económico que puede ser considerado como opción terapéutica de rescate en determinados pacientes pediátricos con neumotórax o fuga aérea persistente.

[Nissen fundoplication in children under 1 year of age: is age important?] / Funduplicatura de Nissen en niños menores de 1 año: ¿la edad importa?

AIM: Nissen fundoplication (NF) is a procedure with technical difficulties and variable functional prognosis the lower the patient's age is. Our objective is to analyze the peculiarities of this procedure when performed in children under 1 year. MATERIALS AND METHODS: Retrospective study of the NF in our center from 1999 to 2014. We review the differences between children under 1 year of age and the leftover of the series: history, indications, surgical approach and postoperative outcomes. RESULTS: A total of 233 patients (57.1% male) were operated at a median age of 2.3years (1 month-17.31years), of which 82 (35.2%) were younger than 1 year. It Open surgery was performed in 118 patients (86.6% of children under 1 year and 31.1% over 1 year, p <0.05) and laparoscopic in 115. The median follow-up was 3.92 ± 3.24 years. Patients under 1 year had a higher number of comorbidities (91.5% vs 81.5%), respiratory symptoms (76.8% vs 49.7%) and postoperative complications (20.7% vs 9.9% OR = 2.4), with statistically significant differences (p <0.05). There were not differences in the Nissen's failure rate (15.9% vs 8.6%) or the need of reoperation (15.9% vs 7.9%). CONCLUSIONS: Patients under 1 year operated by NF form a group with particular indications and comorbidities. Although the outcomes among these patients are favourable, surgical complications are more frequent than in older children.

INTRODUCCION Y OBJETIVOS: La funduplicatura de Nissen (FN) es un procedimiento que plantea dificultades técnicas y un pronóstico funcional variable cuanto menor es la edad del paciente. Nuestro objetivo es analizar las peculiaridades de esta intervención cuando se realiza a niños menores de 1 año. MATERIAL Y METODOS: Estudio retrospectivo de las FN en nuestro centro de 1999 a 2014. Se evaluaron las diferencias entre menores de 1 año de vida y el resto de la serie en cuanto a antecedentes, indicaciones, abordaje quirúrgico y evolución postoperatoria. RESULTADOS: Un total de 233 pacientes (57,1% varones) fueron intervenidos a una mediana de edad de 2,3 a (1 mes-17,31 a), de los que 82 (35,2%) eran menores de 1 año. La cirugía fue abierta en 118 pacientes (86,6% de los menores de 1 año y 31,1% de los mayores de 1 año, p <0,05) y laparoscópica en 115. La mediana de seguimiento fue de 3,92 años. Los pacientes menores de 1 año presentaron mayor número de comorbilidades (91,5% vs 81,5%), sintomatología respiratoria (76,8% vs 49,7%) y complicaciones postoperatorias (20,7% vs 9,9%, OR=2,4), siendo estas diferencias estadísticamente significativas (p <0,05). No hubo diferencias en cuanto al fallo del Nissen (15,9% vs 8,6%) ni a la necesidad de reintervención (15,9% vs 7,9%). CONCLUSIONES: Los pacientes menores de 1 año operados mediante FN constituyen un grupo con indicaciones y comorbilidades particulares. Aunque los resultados son favorables, las complicaciones quirúrgicas son más frecuentes.

[Laryngotracheal traumatic injuries (LTI) in children: when to operate?] / Lesiones laringotraqueales traumáticas en niños ¿cuándo operar?

INTRODUCTION: Paediatric LTI is associated with significant morbid-mortality. Although historically first line treatment was surgical, conservative management is making headway. The purpose of this study was to analyze the management and surgical indications of LTI treated at our institution. MATERIAL AND METHODS: Retrospective study of patients with LTI treated between 2007 and 2014. RESULTS: Six out of seven patients, with a median age of 4 years (2-15), had blunt traumas and one had an open trauma. Respiratory distress, pain and subcutaneous emphysema were presented in 71.4% of the patients. Five children were intubated (3 pre-hospital care and 2 after reaching the hospital), all of them underwent fibrobronchoscopy, determining the location, the extention, and assessing the possibility of extubation. Esophagoscopy was made in 2 cases. The remaining 2 cases were bounded to observation as they did not have respiratory symptoms or progressive emphysema, precluding bronchoscopy. Conservative management was followed in 71.4% of the patients, with a 100% success rate. Conservative management criteria were no progression of emphysema or respiratory distress, regardless the degree and location of the lesions. Two patients needed surgery: one tracheoesophageal fistula caused by a button battery, and the other was a tracheal open section associated with vascular injury. Both of them recovered uneventfully. CONCLUSIONS: If the respiratory symptoms or the emphysema do not progress, patients with LTI can be managed conservatively, regardless of size or location of the injury. Associated esophageal lesions and open injuries require surgical management. An early diagnosis is mandatory in order to minimize morbidity and mortality.

INTRODUCCION: Las lesiones laringotraqueales traumáticas pediátricas están asociadas a una importante morb-imortalidad. Aunque históricamente el tratamiento ha sido quirúrgico, en la actualidad el enfoque conservador ha adquirido una mayor importancia. Valoramos el tratamiento y las indicaciones quirúrgicas de los traumatismos laringotraqueales tratados en nuestro centro. MATERIAL Y METODOS: Análisis retrospectivo de pacientes con traumatismos laringotraqueales del 2007 al 2014. RESULTADOS: Siete pacientes, mediana de edad 4 años (r: 2-15), 6 traumatismos cerrados y 1 abierto. El 71,4% presentaban dificultad respiratoria, dolor y enfisema subcutáneo. Cinco fueron intubados (3 en prehospitalaria y 2 en hospital). En todos ellos se realizó fibrobroncoscopia determinando la localización, la extensión de la lesión y la posibilidad de extubación. En dos de ellos se asoció esofagoscopia. Dos casos no presentaban clínica respiratoria y el enfisema no progresó por lo que se tomó una actitud expectante y no precisaron broncoscopia. El 71,4% se trataron conservadoramente, siendo el criterio de tratamiento conservador la no progresión del enfisema ni empeoramiento del estado respiratorio, independientemente del grado y localización de la lesión, con una tasa de éxito del 100%. Dos pacientes se intervinieron: una fístula tráqueo-esofágica producida por una quemadura (pila de botón) que requirió resección traqueal; y una sección traqueal abierta asociada a lesión vascular, ambas con correcta evolución posterior. CONCLUSIONES: En caso de no progresión de la clínica respiratoria o del enfisema, e independientemente del grado y localización, las lesiones laringotraqueales pueden tratarse de manera conservadora con o sin intubación. Lesiones esofágicas asociadas y lesiones abiertas obligan a un abordaje quirúrgico. Un diagnóstico precoz minimiza la morbimortalidad.

[Spontaneous pneumothorax in children: factors associated with their recurrence]. / Neumotórax espontáneo en la edad pediátrica: factores asociados a su recidiva.

INTRODUCTION: Recurrence of spontaneous pneumothorax (SP) is more frequent in pediatric patients. The purpose of our study was to identify factors associated with this recurrence. MATERIAL AND METHODS: Retrospective review of the chart of patients with SP treated at our institution between 2004 and 2014. Statistic methods (χ2 and logistic regression) were used analyzing age, gender, side and size of pneumothorax and therapeutic approach as possible predictive factors of SP recurrence. RESULTS: A total of 81 SP cases in 43 patients (29 men, 14 women) were reviewed: 50 primary (PSP) and 31 secondary (SSP). Median age at presentation was 15,1 years (0.5-18.59). Follow up was 3.52 years (0.21-18.59). Twenty-three cases (28.4%) underwent surgery (22 thoracoscopies, 1 thoracotomy), consisting of bleb resection with abrasive and chemical pleurodesis. Ipsilateral recurrence after surgery was 21.7% (22.2% in PSP, 20% in SSP, p = 0.096) and 41.4% after non-surgical treatment. Age was a risk factor for recurrence (p= 0.049; OR = 1.16), especially between 10 and 15 years (44.6% in front of 19%, p = 0.013). Male gender and size >20% were associated with higher recurrence risk in SSP that received non-surgical treatment (p<0.05). CONCLUSIONS: Recurrences of SP in children are frequent even after surgical treatment. Age (preadolescence and early adolescence), male gender and initial size of pneumothorax may be factors associated with higher recurrence risk.

INTRODUCCION: Las recurrencias de los neumotórax espontáneos (NE) son más frecuentes en niños que en adultos. Nuestro objetivo es analizar qué factores pueden estar asociados a su recidiva. MATERIAL Y METODOS: Análisis retrospectivo de los casos de NE tratados en nuestro centro entre 2004 y 2014. Se utilizaron métodos estadísticos (χ2 y regresión logística) para identificar factores asociados a su recurrencia analizando edad, sexo, lateralidad, tamaño del neumotórax y tratamiento recibido. RESULTADOS: Se recogieron 81 episodios en 43 pacientes (29 hombres, 14 mujeres): 50 primarios y 31 secundarios (14 pacientes: 10 procesos bronquíticos, 1 displasia broncopulmonar, 1 fibrosis quística y 2 fibrosis pulmonares) con una mediana de edad de 15,1 años (0,5-18,59). El seguimiento fue de 3,52 años (0,21-9,93). Veintitrés casos (28,4%) fueron intervenidos (22 toracoscopias, 1 toracotomía), realizándose resección de bullas asociada a pleurodesis abrasiva. La tasa de recidiva ipsilateral tras la cirugía fue del 21,7% (22,2% en los primarios y 20% en los secundarios, p = 0,92) y del 41,4% en pacientes no intervenidos (p = 0,096). La edad se comportó como factor de riesgo para la recidiva (p = 0,049; OR = 1,16), principalmente entre los 10 y los 15 años (44,6% frente al 19%, p = 0,013). El sexo masculino y el tamaño >20% se asociaron a la recidiva de los NE secundarios no intervenidos (p<0,05). CONCLUSIONES: Las recurrencias de los NE en niños son frecuentes incluso tras la cirugía. La preadolescencia y el inicio de la adolescencia, el sexo masculino y el tamaño inicial del neumotórax pueden ser factores asociados a un mayor índice de recidiva.

[Cervico-sternotomy for thoracic inlet conditions in children]. / La cérvico-esternotomía como abordaje de patología tumoral u ortopédica tóraco-cervical.

BACKGROUND/AIM: Neither cervicotomy nor postero-lateral thoracotomy allow safe surgical access to the lower cervical spine and high posterior mediastinum with full control of the vascular and neural structures involved. We report our favorable experience with cervico-sternotomy for accessing this region. PATIENTS AND METHODS: Six patients were operated upon between 1998 and 2011 for either removal of huge cervico-thoracic neural ganglioneuromas (n = 2) or anterior arthrodesis for congenital (n = 2), neuropathic (n = 1) or osteolytic scoliosis (n = 1). In all cases, cervicotomy was followed by sternotomy, thymectomy, division of the innominate vein and dissection of jugular veins, carotid arteries and vagus nerves. RESULTS: The tumors measured 10.9 x 3.9 x 8.7 cm and 8 x 6 x 5 cm, and involved the paravertebral chain from the aortic arch to the base of the skull and from the left lung hilus to the thyroid region respectively. In the scoliosis patients, anterior vertebral fixation between C5 and T5 was readily feasible. Blood transfusion was avoided. Horner's syndrome and transient lymphedema were the only complications. Median operative time was 210 minutes (range 180-240 minutes) and median estimated blood loss was 2.7 cc/kg (0-13.8 cc/kg). Median hospital stay was 7 days (range 5-18 days). CONCLUSIONS: Cervico-sternotomy is an optimal approach for this anatomical region in children. It offers better exposure of the anterior cervico-thoracic spine and the thoracic inlet than cervicotomy or thoracotomy. Control of the nervous and vascular structures was safely achieved in all cases and postoperative discomfort was surprisingly limited.

[Experimental model of amniotic band in rats: model description and initial morphological study]. / Modelo experimental de brida amniótica en ratas: descripción del modelo y estudio morfológico inicial.

PURPOSE: Our objective is to describe a new experimental model of amniotic band in rats and to show the initial morphological results. METHODS: The model was created by ligation of a hind limb with silk on day 17 of gestation; in selected fetuses ligation was withdrawn on day 19 of gestation. Fetuses were removed by cesarean section on day 21 of gestation, analyzing their weights and morphological characteristics of their hind legs. We used 10 pregnant SD rats, weighing 263 g (231-279). One hundred and thirteen fetuses were analyzed divided into 6 groups: fetuses from control rats (n=28), uterine opening and closing (n=7), band creation (n=28), band creation and subsequent removal (n=14) and not intervened fetuses of the litters in which the band was created (n=19) and that the band was created and removed (n=17). Comparisons between groups were made using parametric tests considering p<0.05 as a threshold for significance. RESULTS: Operated fetuses (band creation and band creation and removal) showed statistically significant differences in weight (mean=4.56 g and 4.4 g) and length of their hind limbs (mean=3.97 mm and 5.34 mm) compared to the rest of the groups. The rate of abortions in operated fetuses was 16.6% and the rate of amputations was 8.3%. DISCUSSION: A viable and reproducible experimental model of amniotic band is described, with potential applications in both the study of the disease and the role of fetal surgery thereon.

Analysis of perceived risk and satisfaction with telematic follow-up in patients and families of congenital diaphragmatic hernia patients during SARS-CoV-2 pandemia.

BACKGROUND: The impact of COVID-19 in families and patients with congenital diaphragmatic hernia (CDH) is unknown, this situation has generated uncertainty not only in family members but also in the optimal outpatient follow-up. Telehealth has become a fundamental tool for the follow-up during the pandemic. The objective of this survey is to evaluated the impact of SARS-CoV-2 in families and patients with CDH and the satisfaction with telematic follow-up. METHODS: Telephone survey of patient's caregivers with CHD, aged 1-16 years, followed in neonatal surgery outpatients, from January 31, 2020 to November 15, 2020. The ethical clearance for this study was taken from the Clinical Research Ethics Committee of our Research Institute vide letter number VHIR/239283/01.01.2021. RESULTS: 81 surveys of 100 patients with active follow-up were carried out. There were no refusals in any contacted parents. There were 30 contacts (37%), 44.8% at school and 27.6% from cohabiting family members. Four infections (4.9%) were diagnosed, half symptomatic. In 40 patients (49.4%) the follow-up was telematic, with a mean score of 3.1±1.3 out of 5. For future controls, 65% prefer presential follow-up, 25% alternate and 10% telematics. 50.6% reported greater anxiety and 34.6% (28/81) extreme measures of isolation, being more accentuated in the group of 3-6 years (p<0.05). CONCLUSION: The impact of COVID19 in patients with CHD is not greater than in the general pediatric population. Although the incorporation of the telehealth was well valued, most of the caregivers prefer the face-to-face outpatient follow-up.

[Technical aspects of experimental intestinal transplant]. / Aspectos técnicos del trasplante intestinal experimental.

PURPOSE: Our objective is to analyze the variables that influence the outcome of Small Bowel Transplantation (SBT) in rats in an experimental microsurgery program. The surgical technique and perioperative care are described in detail. METHODS: Retrospective study of the SBT in rats conducted in our experimental surgery laboratory from 2002 to 2010. The animals were divided into group A (those who survived more than 48 hours) and group B (those who died earlier without justificable cause). We compared in both groups: number of transplants performed by the surgeon, warm ischemia time, cold ischemia time and duration of the procedure. RESULTS: Five surgeons with different degrees of microsurgical training participated in the study. A total of 521 SBT were performed with an overall survival of 48%. The first successful transplant was performed after a median of 46 (25-68) transplants. Total procedure time (3.5 vs 2.9 hours) and warm ischemia time (51 vs 35 minutes) were higher in group B (p < 0.05). DISCUSSION: The number of transplants required for learning the technique is high. However, survival is acceptable when the time needed for vascular anastomosis is reduced. The SBT in rats is a valuable model for surgical training and research of the phenomena related to SBT.

Minimally invasive surgery for pediatric dumbbell neuroblastoma: systematic literature review and report of a single-stage neurosurgical and thoracoscopic approach.

INTRODUCTION: Traditional surgical strategies for dumbbell neuroblastoma entail, among others, high risk of spinal deformity. Less invasive procedures might reduce these sequelae, however, there is small evidence comparing different strategies. Indications of minimally invasive surgery in neuroblastoma are still developing. Our aim is to identify and analyze different surgical approaches described in the recent literature and to suggest a minimally invasive option. METHODS: A systematic review of the literature was conducted in PubMed (Jan 2000-Dec 2021) to identify reports describing surgical resection of dumbbell neuroblastoma in children, according to the PRISMA guidelines. Only full-text articles were included. RESULTS: 7 articles met the inclusion criteria which, added to the present case, represent a total of 43 patients. All were retrospective studies, most of them small series. Tumor location was mostly thoracic. Most of combined approaches were performed in two stages. Spinal deformity after surgery was reported in 3 patients. Minimally invasive approach was described in only one paper, with no reported cases of its use in a single-stage combined surgery. We also report, to our knowledge, the first single-stage posterior neurosurgical approach combined with thoracoscopy for resection of a dumbbell neuroblastoma in an infant. CONCLUSION: Surgical resection of dumbbell neuroblastomas is challenging. There is no consensus on best surgical approach. Dumbbell tumors should not be considered a contraindication for minimally invasive surgery. A single stage and minimally invasive strategy is proposed.

Duodenal atresia with apple peel associated with congenital diaphragmatic hernia: an exceptional case and a literature review. / Atresia duodenal con apple peel asociada a hernia diafragmática congénita: un caso excepcional y revisión de la literatura.

INTRODUCTION: Duodenal atresia associated with apple peel is extremely rare. Duodenal atresia occurs as a result of absence of recanalization at an early stage, whereas intestinal atresia is seemingly due to vascular causes at later stages. The presence of abnormalities associated with diaphragmatic hernia is frequent, but association with duodenal atresia has been little explored. CASE REPORT: This is the case of a female neonate born at gestational week 31, with duodenal atresia and apple peel, associated with left diaphragmatic hernia and major heart disease. An abdominal muscle flap was performed for diaphragmatic defect closure purposes, and duodenojejunal anastomosis was carried out following resection of part of the non-viable apple peel. DISCUSSION: To our knowledge, this is the first case described with this rare association. The combination of duodenal atresia and apple peel had been previously described 11 times. However, the association of both with congenital diaphragmatic hernia had not been reported yet.

INTRODUCCION: La atresia duodenal asociada con appel peel es extremadamente infrecuente. La primera se produce por un defecto en la recanalización en etapas tempranas, mientras que la atresia intestinal parece deberse a causas vasculares en etapas más tardías. La presencia de anomalías asociadas a la hernia diafragmática es común, pero la asociación con la atresia duodenal apenas está descrita. CASO CLINICO: Presentamos un recién nacido de 31 semanas de gestación y sexo femenino, con atresia duodenal y apple peel, asociada a hernia diafragmática izquierda y cardiopatía mayor. Se realizó un flap muscular abdominal para el cierre del defecto diafragmático y anastomosis duodenoyeyunal tras la resección de parte del apple peel inviable. COMENTARIOS: A nuestro entender, es el primer caso descrito con esta asociación singular. La combinación de atresia duodenal con apple peel se ha descrito previamente en 11 ocasiones; la asociación de ambas con hernia diafragmática congénita no había sido descrita.

[Pilot experience with indocyanine green navigation in pediatric surgery]. / Experiencia piloto con navegación mediante fluorescencia con verde de indocianina en cirugía pediátrica.

INTRODUCTION: Indocyanine Green (ICG) fluorescence is a new tool for navigated minimal invasive and open surgery, with multiple possible uses, that can increase safety and improve surgical results, facilitating intraoperative decision making. We hereby present our pilot series using ICG navigation in different procedures of pediatric surgery. MATERIAL AND METHODS: As a proof of concept, between May 2017 and March 2019, we have used this technique as a help for decision making in these scenarios: visualization of the biliary tract, vascular and lymphatic structures, neoplastic tissue and other anatomic landmarks. The route of administration, timing and dosage changed depending on the indication. A 30º optic, with a conventional and near-infrared light emitter, connected to a high definition system specially equipped was used. RESULTS: We considered that the technique might be useful in 20 patients (22 procedures): 6 involving the biliary tract (5 cholecystectomies, 1 choledochal stenosis), 9 oncologic procedures (5 laparoscopic and 4 open), 7 miscellanea (pulmonary nodule resections, long-gap esophageal atresia, anastomotic leak, etc). There were no complications regarding ICG administration. We considered that the system provided relevant information or affected intraoperative decision making in 90% of the cases. CONCLUSIONS: ICG navigation was easy to perform and complication free. Our preliminary results suggest that ICG navigation, in open and endoscopic procedures, might provide a qualitative leap regarding safety and facilitate the performance of certain pediatric surgical procedures, particularly in oncology, liver surgery and neonatal surgery.

INTRODUCCION: La fluorescencia con verde de indocianina (ICG) es una nueva herramienta de navegación por imagen con múltiples posibles aplicaciones, orientada a aumentar la seguridad y mejorar los resultados quirúrgicos, facilitando la toma de decisiones. Presentamos nuestra serie piloto con el uso del ICG en distintos procedimientos de cirugía pediátrica. MATERIAL Y METODOS: A modo de prueba piloto, entre mayo 2017 y marzo de 2019, hemos usado dicha técnica como ayuda en la toma de decisiones en los siguientes supuestos: visualización de vía biliar, estructuras vasculares y linfáticas, tejido tumoral y otras estructuras. El momento y dosis de ICG varió en función de la indicación. Se utilizó una óptica de 30º, con un emisor de luz convencional y de luz cercana al infrarrojo, conectada a un sistema de alta definición especialmente equipado. RESULTADOS: Se consideró que la técnica podría ser de utilidad en 20 pacientes (22 procedimientos): 6 sobre la vía biliar (5 colecistectomías, 1 estenosis de la vía biliar), 9 procedimientos oncológicos (5 laparoscópicos y 4 abiertos), 7 procedimientos miscelánea (resección nódulos pulmonares, atresia esófago, fallo sutura, etc.). No hubo complicaciones relacionadas con la inyección del ICG. Se consideró que aportó información relevante o influenció en la toma de decisiones en un 90% de los casos. CONCLUSIONES: El uso del ICG resultó sencillo y exento de complicaciones. Nuestros resultados sugieren que la navegación mediante ICG, en cirugía laparoscópica y abierta, puede suponer un salto cualitativo en seguridad y facilitar la realización de determinados procedimientos, especialmente en oncología, cirugía hepática y cirugía neonatal.

Brain perfusion SPECT in patients with PHACES syndrome under propranolol treatment.

INTRODUCTION: Children with PHACES syndrome (PS) and visual impairment or stridor show a dramatic and immediate response to propranolol. However, this beta-blocking drug could be responsible for an eventually increased risk of ischemic stroke due to the underlying cerebral vascular disease. To more accurately understand the effects of propranolol on brain vascularization, we examined PS patients treated with this drug for airway or visual complications using brain perfusion SPECT (Single Photon Emission Computed Tomography). In the past, this examination has been shown to be useful in the management of patients with different neurovascular disorders. METHODS: Clinical records and imaging studies were reviewed in 7 patients with a diagnosis of PS. All patients underwent magnetic resonance angiography (MRA), echocardiography, chest X-ray and ophthalmologic, neurological, and cardiologic assessments. They received 2-3 mg/kg/day propranolol in an attempt to treat stridor or avoid ophthalmologic occlusion. We performed SPECT after 3-6 months of treatment. RESULTS: SPECT showed a normal uptake in the frontal and temporal regions despite vascular abnormalities found with MRA imaging. Significant improvements of symptoms and in the volume of the hemangioma were noted in all cases without signs of a reduction of brain blood perfusion. CONCLUSIONS: Propranolol treatment was safe in our patients who did not show signs of perfusion changes. The high sensitivity for detecting functional impairment makes brain perfusion SPECT useful in the diagnosis and follow-up of patients with PS considered at risk of neurovascular impairment. Accurate knowledge of its pathophysiological basis, together with the appropriate technique and careful interpretation of reporting, will enhance the clinical use of brain SPECT in those patients.

Liver transplantation in children with cystic fibrosis: experience in our centre and preliminary results with a combined en bloc liver-pancreas graft.

AIM OF THE STUDY: Cystic fibrosis (CF) is a multisystemic disease, with some patients developing end-stage liver disease (ESLD), requiring liver transplantation (LT). These children usually present with severe mutations of the CFTR gene. Almost 100% of patients with severe mutations develop exocrine pancreatic insufficiency, leading later to endocrine insufficiency. Immunosuppression accelerates the development of insulin-dependent diabetes (IDD) in transplanted children with CF. Our aims were: (1) to analyze our experience with CF-related ESLD children who received LT, and the relationship to the development of IDD; (2) to report our preliminary results with en bloc liver-pancreas transplantation (CLPT). METHODS: 9 children (6M/3F) with CF and ESLD underwent LT between 1993 and 2010; median age and weight were 12.3 years (range: 5.4-17.0) and 36.7 kg (range: 14.2-58.5), respectively. 4 patients received a whole graft, 4 had reduced grafts (1 split) and 1 underwent CLPT. Immunosuppression followed the protocols at the time of transplantation. RESULTS: Liver function was restored in all patients and none of them needed re-transplantation. Median follow-up was 105 months (range: 4-206). 1 child died of respiratory failure at 23 months after transplantation while awaiting pulmonary transplantation. Survival (Kaplan-Meier) at 105 months was 87.5%. 4 children already had IDD before transplantation and 3 developed diabetes immediately after transplantation. 2 had not developed IDD at the end of the study: the youngest at the time of LT (5.4 years, follow-up 7.1 years) and the girl who had had CLPT and who recovered normal exocrine and endocrine pancreatic function after transplantation. CONCLUSIONS: LT is a realistic option to treat CF-related ESLD children. IDD is common in these patients. En bloc liver-pancreas transplantation is an appealing option, since it simultaneously restores exocrine function and prevents IDD. This procedure has clear technical advantages over simultaneous isolated liver and pancreas transplantation.