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INTRODUCTION: esophageal replacement in children is indicated when it is impossible to maintain the native esophagus, which in the pediatric population includes patients with esophageal atresia and esophageal caustic stenosis. The objective of this communication is to report the experience of a university service with two techniques of esophageal replacement. METHODS: this is a retrospective study based on the revision of hospital files. The study population consisted of patients who underwent esophageal replacement from 1995 to 2022, at the Hospital de Clínicas of the State University of Campinas. The analyzed data were age, sex, underlying disease, technical aspects, complications, and long-term results. RESULTS: during the study period, 30 patients underwent esophageal replacement. The most common underlying diseases were esophageal atresia (73.33%) and caustic stenosis (26.67%). Twenty-one patients underwent gastric transposition (70%), and nine underwent esophagocoloplasty (30%). The most frequent postoperative complication was fistula of the proximal anastomosis, which occurred in 14 patients. Most of the patients with fistulas had a spontaneous recovery. There were three deaths. Of the 27 survivors, 24 can feed exclusively by mouth. CONCLUSION: esophageal replacement in children is a procedure with high morbidity and mortality. Esophagocoloplasty and gastric transposition have similar results and complications, with the exception of proximal anastomotic fistulas, which are generally self-resolving and are more common in esophagocoloplasty. The choice of the best surgical technique must be individualized according to the patients characteristics and the surgeons experience, as both techniques offer the ability to feed orally in the short or medium term.

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OBJECTIVE: To evaluate genetic testing utilization and diagnostic yield in infants with esophageal atresia (EA)/tracheoesophageal fistula (TEF) over the past 12 years to inform future practices and individualize prognostication and management. STUDY DESIGN: A retrospective cohort study was performed for all infants with EA or EA/TEF hospitalized between January 2011 and January 2023 at a quaternary children's hospital. For each infant, demographic information, prenatal and postnatal history, and genetic testing were reviewed. RESULTS: There were 212 infants who were classified as follows: 1) complex/syndromic with EA/TEF plus an additional major anatomic anomaly (n = 114, of which 74 met VACTERL criteria); 2) isolated/nonsyndromic EA/TEF (n = 88) and 3) isolated/nonsyndromic EA (n = 10). A range of genetic tests were sent with varying diagnostic rates including karyotype analysis in 12 (all with complex/syndromic phenotypes and all positive), chromosomal microarray analysis in 189 (114 of whom were complex/syndromic with an overall diagnostic rate of 3/189), single gene testing for CHD7 in 18 (4 positive), and exome analysis in 37 complex/syndromic patients (8 positive). CONCLUSIONS: EA/TEF with and without additional anomalies is genetically heterogeneous with a broad range of associated phenotypes. While the genetic etiology of EA/TEF with or without VACTERL remains largely unknown, genome wide testing (exome or genome) including copy number analysis is recommended over chromosomal microarray testing. We anticipate that expanded genetic/genomic testing modalities such as RNA sequencing and tissue specific molecular testing are needed in this cohort to improve our understanding of the genomic contributors to EA/TEF.

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OBJECTIVE: To identify clinical and epidemiological characteristics of children evaluated by the pediatric aerodigestive program at the beginning of its activity, describe challenges in follow-up, and suggest mitigation strategies. METHODS: A case series was conducted describing the first 25 patients discussed by the aerodigestive team from a Brazilian quaternary public university hospital between April 2019 and October 2020. The median follow-up was 37 months. RESULTS: During the study period 25 children were seen by the group and the median age at first assessment was 45.7 months old. Eight children had a primary airway abnormality, five had a tracheostomy. Nine children had genetic disorders and one had esophageal atresia. Dysphagia was present in 80% of the patients, 68% had a history of chronic or recurrent lung disease, 64% had a gastroenterological diagnosis and 56% had neurological impairment. Moderate to severe dysphagia was identified in 12 children and 7 of these had an exclusive oral diet at the time. The majority of children (72%) had 3 or more comorbidities. Following team discussion, a change in feeding strategy was suggested in 56% of the children. The most frequently ordered exam was pHmetry (44%) and gastrostomy was the surgical procedure with the longest waiting list. CONCLUSIONS: Dysphagia was the most frequent issue encountered in this initial group of aerodigestive patients. Pediatricians caring for these children must be involved in aerodigestive team discussions and hospital policies must be revised to facilitate access to exams and procedures needed for this population.

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OBJECTIVES: This study aimed to characterize feeding/swallowing difficulties in children with esophageal atresia and/or tracheoesophageal fistula (EA/TEF) and evaluate associations among feeding difficulties, pharyngeal dysphagia (PD), and other aerodigestive evaluation findings. METHODS: This was a retrospective cohort study of feeding/swallowing characteristics of 44 patients with EA/TEF treated in the aerodigestive program of a single academic medical institution from 2010 to 2015. Demographics, comorbidities, presence and characteristics of feeding/swallowing difficulties, and results of relevant diagnostic tests [videofluoroscopic swallow studies (VFSS), clinical feeding evaluations (CFEs), chest computerized tomography (CT) scans, pulmonary bronchoscopies, and upper GI (UGI)/esophagrams] were reviewed. RESULTS: Fifty percent of the cohort had PD and 88.6% had feeding difficulties. Across 118 encounters (87 VFSS and 31 CFEs), feeding difficulties suggestive of esophageal dysphagia were most frequently seen in children over 48 months and feeding difficulties suggestive of developmental feeding problems were most frequently seen in children from 24 to 48 months. Abnormal findings were present in 59.8% of VFSS, with aspiration (34.5%) and pharyngeal residue (26.4%) the most frequently observed signs of dysphagia. Abnormal UGI/esophagram findings were not associated with significantly increased risk of feeding difficulties during visits within 3 months (risk ratio, RR = 1.33). Presence of dysphagia was associated with increased risk for some abnormal CT findings (RR= 3.0 for airspace and 3.0 for bronchiectasis). CONCLUSIONS: Feeding/swallowing difficulties are common in EA/TEF, and types of feeding difficulties vary by patient age. The presence of abnormal findings on UGI/esophagram did not increase the risk of feeding complaints; however, the presence of dysphagia increased the risk of abnormal chest CT.

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La atresia esofágica es una de las anomalías congénitas más frecuentes en la práctica quirúrgica neonatal. Se estima que tiene una incidencia de 1 por cada 3500 recién nacidos vivos a nivel mundial. La preparación de guías de actuación y protocolos asistenciales es tendencia en la práctica médica actual. Esta Guía de Práctica Clínica se elaboró respondiendo a la necesidad de protocolizar la atención médico-quirúrgica de la atresia esofágica. En el Centro Territorial de Cirugía Neonatal de Holguín, donde se regionaliza la atención a neonatos de las cinco provincias orientales del país con afecciones congénitas y quirúrgicas de alta complejidad, la atresia esofágica fue la afección quirúrgica más frecuente en los últimos diez años, con una supervivencia ascendente que alcanzó 94,4 por ciento en 2019. La guía que se presenta se aprobó en el Primer Consenso Nacional de Guías de Prácticas Clínicas en Cirugía Pediátrica, en Varadero, Matanzas en 2019. Incluye las principales pautas para el diagnóstico, tratamiento y seguimiento de los pacientes afectados y se considera una herramienta eficiente para mejorar los resultados en la asistencia médica y quirúrgica neonatal(AU)

Esophageal atresia is one of the most common congenital anomalies in neonatal surgical practice. It is estimated to have an incidence of 1 per 3500 live newborns globally. The preparation of action guides and care protocols is a trend in current medical practice. This Clinical Practice Guide was prepared in response to the need to protocolize the medical-surgical care of esophageal atresia. In the Territorial Center for Neonatal Surgery of Holguín, where the care of neonates from the five eastern provinces of the country with congenital and surgical conditions of high complexity is regionalized, esophageal atresia was the most frequent surgical condition in the last ten years, with an ascending survival that reached 94.4 percent in 2019. The guideline presented was approved in the First National Consensus of Clinical Practice Guidelines in Pediatric Surgery, in Varadero, Matanzas in 2019. It includes the main guidelines for the diagnosis, treatment and follow-up of affected patients and is considered an efficient tool to improve outcomes in neonatal medical and surgical care(AU)

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Resumen Objetivo: Describir resultados de la cirugía de sustitución esofágica con tubo gástrico invertido, vía ascenso retroesternal en dos hospitales pediátricos durante el período marzo 2015 a marzo 2018. Materiales y Método: Un estudio observacional, transversal, con recolección de datos retrospectivo, donde se incluyeron todos los expedientes de pacientes que presentaban patología del esófago por causa adquirida o congénita que fueron operados de sustitución esofágica con tubo gástrico invertido en dos hospitales pediátricos durante 3 años. Resultados: Encontramos 29 niños sometidos a sustitución esofágica, de los cuales 27 cumplieron criterios de inclusión. La edad comprendida entre 2 y 17 años. El 63% corresponde al sexo femenino. La causa más frecuente de sustitución esofágica es por estenosis esofágica por ingesta caustica (92,59%). El 70% presentó algún tipo de complicación luego de la cirugía. La fístula esofagocutánea es la complicación principal con 33,33%. La permanencia en la unidad de cuidados intensivos es menor de 24 horas en un 74% de los niños. Se inicia la vía oral en casi la mitad de casos entre los 10-12 días de posquirúrgico, la estancia hospitalaria es en promedio 18,5 días. La mortalidad es 3,7%. Conclusión: La sustitución esofágica por tubo gástrico invertido vía ascenso retroesternal, es una técnica comparable en resultados a la interposición de colón. Para los autores, el estómago es un órgano ideal para realizar la reconstrucción esofágica, y sus complicaciones son manejables.

Aim: To describe the results of esophageal replacement surgery with an inverted gastric tube via retrosternal ascent in two pediatric hospitals during the period March 2015 to March 2018. Materials and Method: an observational, cross-sectional study with retrospective data collection that included all the records of patients with esophageal discontinuity due to acquired or congenital causes who underwent esophageal replacement surgery with an inverted gastric tube in two pediatric hospitals for 3 years. Results: We found 29 children undergoing esophageal replacement, of which 27 met inclusion criteria. The age between 2 to 17 years. 63% corresponds to the female sex. The most frequent cause of esophageal replacement is esophageal stricture due to caustic ingestion (92.59%). 70% presented some type of complication after surgery. The esophagocutaneous fistula is the main complication with 33.33%. The stay in the intensive care unit is less than 24 hours in 74% of children. The oral route is started in almost half of cases between 10-12 days after surgery; the hospital stay is on average 18.5 days. Mortality is 3.70%. Conclusión: The esophageal substitution by inverted gastric tube via retrosternal ascent is a technique comparable in results to the interposition of the colon. For the authors, the stomach is an ideal organ to perform esophageal reconstruction, and its complications are manageable.

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Introducción: existen dos rutas para realizar el reemplazo de esófago (RE), la retroesternal (RRE) y la mediastinal posterior (RMP). El objetivo del estudio es comparar los pacientes que recibieron un ascenso gástrico parcial empleando estas dos rutas. Material y métodos: Se revisaron las historias clínicas de 51 pacientes con ascenso gástrico parcial, en 27 años en el Hospital Garrahan. Se utilizó la vía RRE en 25 casos y la RMP en 26. Fueron comparados los datos epidemiológicos de los grupos y las variables para valorar la dificultad del acto quirúrgico, evolución inmediata y alejada. El estudio es comparativo, retro-prospectivo y longitudinal. Resultados: las características generales de los pacientes fueron similares. Los que recibieron el ascenso gástrico por vía RMP presentaron una menor incidencia de dehiscencia (p=0,017), de enfermedad por reflujo gastroesofágico (ERGE) (p=0,001) y de dumping (p=0,0001). No hubo diferencias estadísticamente significativas entre los dos grupos al comparar la duración del procedimiento, días de internación total y en Unidad de Cuidados Intensivos (UCI), días de permanencia en asistencia respiratoria mecánica (ARM), inicio de alimentación oral y estenosis de la anastomosis. Se observó una tendencia clínicamente relevante, que no alcanzó significancia estadística en las complicaciones intraquirúrgicas y número de dilataciones postoperatorias. No hubo necrosis del ascenso. Fallecieron 2 pacientes. Conclusiones: considerando la menor incidencia de dehiscencia, ERGE y dumping reemplazados por RMP, elegimos a ésta como nuestra primera opción para el reemplazo esofágico en la infancia (AU)

Introduction: The two routes for esophageal replacement (ER) are retrosternal (RRE) and posterior mediastinal (PMR). The aim of the study was to compare patients who received a partial gastric pull-up using either of these two routes. Material and methods: The clinical records of 51 patients who underwent partial gastric pull-up over 27 years at the Garrahan Hospital were reviewed. The RRE route was used in 25 and the RMP in 26 cases. The epidemiological data of the groups and the variables to evaluate the complexity of the surgical procedure, and shortand long-term outcome were compared. A comparative, retroprospective, and longitudinal study was conducted. Results: the general characteristics of the patients were similar. Those who underwent gastric pull-up via PMR had a lower incidence of dehiscence (p=0.017), gastroesophageal reflux disease (GERD) (p=0.001), and dumping (p=0.0001). No statistically significant differences were found between the two groups when comparing the duration of the procedure, days of total hospital and intensive care unit (ICU) stay, days on mechanical ventilation (MV), initiation of oral feeding and stenosis of the anastomosis. A clinically relevant trend, which did not reach statistical significance, was observed in intraoperative complications and number of postoperative dilatations. There was no necrosis of the pull-up. Two patients died. Conclusions: considering the lower incidence of dehiscence, GERD, and dumping associated with PMR, this was our first choice for esophageal replacement in infancy (AU)

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OBJECTIVE: To know the cardiac malformations frequency associated with esophageal atresia and its type in patients of the Children's Specialties Hospital of Chihuahua as well as related sociodemographic characteristics. METHOD: The epidemiology, clinic and evolution of patients with esophageal atresia diagnosis who were admitted to this hospital for a period of two years were studied. Variables such as sex, gestational age, birth weight, Apgar score, atresia type, associated congenital malformations, hospital complications and parental related aspects were analyzed. RESULTS: Twelve patients were studied, 50% of them were male, most of them were products of term pregnancies with adequate birth weight. There were mestizo ethnicity prevalence, young mothers children with a medium socio-economic level, without geographical predominance. 82% of the cases corresponded to type III esophageal atresia, the most frequent congenital malformations associated were cardiac in 83% of which 90% corresponded to atrial septum defects. CONCLUSIONS: Esophageal atresia is a relatively common congenital malformation of multifactorial etiology. A complete approach to patients with this pathology is necessary to identify a concomitant illness and provide adequate treatment.

OBJETIVO: Conocer la frecuencia de malformaciones cardiacas asociadas en pacientes con atresia de esófago y su tipo en el Hospital Infantil de Especialidades de Chihuahua, así como las características sociodemográficas relacionadas. MÉTODO: Se estudiaron la epidemiología, la clínica y la evolución de los pacientes con diagnóstico de atresia esofágica que ingresaron a dicho nosocomio durante un periodo de 2 años. Se analizaron variables como sexo, edad gestacional, peso al nacer, Apgar, tipo de atresia, malformaciones congénitas asociadas, complicaciones durante la estancia hospitalaria y aspectos relacionados con los padres. RESULTADOS: Se estudiaron 12 pacientes, de los cuales el 50% eran de sexo masculino, y la mayoría de ellos fueron producto a término con peso adecuado al nacimiento. Predominio de etnia mestiza, hijos de madres jóvenes con nivel socioeconómico medio, sin predominio geográfico. El 82% de los casos correspondían a atresia esofágica tipo III, y las malformaciones congénitas más frecuentes asociadas fueron las cardiacas en el 83% de los casos, de las cuales el 90% correspondían a defectos del tabique auricular. CONCLUSIONES: La atresia esofágica es una malformación congénita relativamente común y de etiología multifactorial. Es necesario realizar un abordaje completo de los pacientes con esta patología para poder identificar otra afección y brindar el tratamiento adecuado.

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OBJECTIVES: To describe growth and feeding outcomes in patients with type C esophageal atresia who underwent early primary repair and to identify predictors for poor growth. STUDY DESIGN: This single-center, retrospective, cohort study included all patients with type C esophageal atresia who underwent early primary repair from 2013 to 2019. Weight-for-age z score (WAZ) was calculated at birth, and every 6 months until 3 years postoperatively. Longitudinal median regression was used to evaluate WAZ over time. A multivariable logistic regression model explored predictors of growth outcomes. RESULTS: Of 46 infants who met the inclusion criteria, 72% were term. The median age at repair was 1.5 days of life (IQR, 1-2 days of life) and the hospital length of stay was 20 days (IQR-14, 30 days). Two patients had esophageal leak (4.3%). The median WAZ at birth was below average (-0.72; IQR, -1.37 to -0.40), but improved to reach average by 3 years (-0.025; IQR, -0.85 to 0.97, P < .001). At discharge, 72% of patients were receiving full oral nutrition, which improved to 95% by 3 years. The only independent predictor of poor growth at 1 year (WAZ < -1 [33%]) was WAZ at discharge (P = .02). CONCLUSIONS: Infants with esophageal atresia who undergo early primary repair are capable of achieving standard growth curves by 3 years of age. However, poor discharge WAZ score was predictive of poor WAZ score at 1 year. Efforts to identify at-risk patients and institute targeted inpatient and outpatient nutrition interventions are needed to improve their growth trajectory.

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