Guía de Práctica Clínica en atresia esofágica / Clinical practice guide on esophageal atresia

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)

Resultados de la cirugía de sustitución esofágica con tubo gástrico invertido vía ascenso retroesternal en dos hospitales pediátricos / Results of esophageal substitution surgery with inverted gastric tube via retrosternal lift

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.

Comparación de vía mediastinal posterior versus vía retroesternal para el reemplazo de esófago con ascenso gástrico parcial. Motivo de un cambio de estrategia / Comparison of posterior mediastinal versus retrosternal route for esophageal replacement with partial gastric pull-up. Rationale for a change in strategy

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)

Atresia esofágica tipo III associada a ânus imperfurado: um relato de caso / Esophageal atresia type III associated to imperforate anus: a case report

Introdução: As malformações congênitas são defeitos que ocorrem durante o desenvolvimento embrio-fetal, principalmente entre a terceira e oitava semana de gestação, podendo ser suspeitado no período pré-natal. A atresia de esôfago com ou sem fístula traqueoesofágica pode estar associada a outras anomalias congênitas, como o ânus imperfurado, o qual faz parte uma síndrome denominada malformação anorretal. Objetivo: discutir o caso de um recém-nascido (RN) portador de atresia de esôfago tipo III e ânus imperfurado, bem como associar suas ocorrências simultâneas. Relato de caso: paciente do sexo masculino, advindo de uma gestação gemelar complicada por pré-eclâmpsia, apresentava ao nascimento ausência do reflexo de busca e sucção, bradicardia e cianose central. Foi diagnosticado com ânus imperfurado e atresia esofágica. Foram realizados cirurgia de Colostomia em Duas Bocas e tratamento cirúrgico da atresia de esôfago. O paciente ficou 48 dias em incubadora com intubação orotraqueal e ventilação mecânica por 26 dias. Resultado: a programação pós- alta indicada foi o acompanhamento com cirurgião pediátrico e neurocirurgião, tendo evoluído bem e aguardando anorretoplastia. Conclusão: o rápido diagnóstico é tão necessário, nesses casos, quanto as abordagens cirúrgicas. O apoio psicológico aos familiares é de extrema importância, pois visa a melhor aceitação da família frente ao caso, propiciando dessa forma, melhores prognóstico e tratamento do paciente.

Introduction: Congenital malformations are defects that occur during embryo-fetal development, especially between the third and eighth week of pregnancy, and may be suspected in the prenatal period. Esophageal atresia with or without tracheoesophageal fistula may be associated with other congenital anomalies, such as the imperforate anus, which is part of a syndrome called anorectal malformation. Objective: to discuss the case of a newborn (NB) with type III esophageal atresia and imperforate anus, as well as to associate its simultaneous occurrences. Case report: male patient, resulting from a twin pregnancy complicated by pre-eclampsia, had at birth the absence of the search and suction reflex, bradycardia and central cyanosis. He was diagnosed with imperforate anus and esophageal atresia. Double-barrel colostomy surgery and esophageal atresia surgery were performed. The patient spent 48 days in an incubator with orotracheal intubation and mechanical ventilation for 26 days. Results: the post-discharge schedule indicated was for follow-up with a pediatric surgeon and neurosurgeon. Patient evolved well and awaits anorectomy. Conclusion: rapid diagnosis is as necessary in these cases as surgical approaches. Psychological support to family members is extremely important, as it aims at better family acceptance in the case, thus providing better prognosis and treatment for the patient.

Tratamiento conservador del quilotórax posquirúrgico en paciente pediátrico / Conservative treatment of post-surgical chylothorax in pediatric patient

INTRODUCCIÓN. El quilotórax resulta de un daño al conducto torácico por ruptura, laceración, desgarro o compresión. Es una patología rara de derrame pleural en la edad pediátrica, pero frecuente como complicación posterior a cirugía cardiotorácica. La base del tratamiento conservador se ha fundamentado en: drenaje inicial, modificación de la dieta, uso de somatostatina o análogos sintéticos como octreotide, cirugía, prevención y manejo de complicaciones. Fue preciso describir la experiencia institucional clínica así como su abordaje. CASO CLÍNICO. Paciente masculino de 4 meses de edad, que ingresó a la Unidad Pediátrica Área de Emergencias del Hospital de Especialidades Carlos Andrade Marín, el 13 de septiembre de 2019 con antece-dente quirúrgico de atresia de esófago corregida en etapa neonatal. Acudió con dificultad respiratoria, radiografía de tórax que evidenció derrame pleural derecho, toracentesis diagnóstica con salida de líquido de aspecto turbio y lechoso; se colocó tubo de tórax derecho. Se prescribió ayuno inicial, nutrición parenteral durante 4 semanas hasta comprobar resolución del quilotórax. Fue dado de alta en condición estable tras 43 días de hospitalización. DISCUSIÓN. La evidencia científica registró que el tratamiento conservador del quilotórax se basó en: drenaje, reposo digestivo inicial, nutrición parenteral, modificación cualitativa de la dieta enteral y uso de octreotide; el mismo que fue aplicado al paciente de este caso clínico con evolución favorable. CONCLUSIÓN. El tratamiento conservador y multidisciplinario en el abordaje del qui-lotórax fue exitoso y no necesitó manejo quirúrgico.

INTRODUCTION. Chylothorax results from damage to the thoracic duct by rupture, la-ceration, tear or compression. It is a rare pathology of pleural effusion in pediatric age, but frequent as a complication after cardiothoracic surgery. The basis of conservative treatment has been based on: initial drainage, diet modification, use of somatostatin or synthetic analogues such as octreotide, surgery, prevention and management of complications. It was necessary to describe the clinical institutional experience as well as its approach. CLINICAL CASE. A 4-month-old male patient was admitted to the Emergency Area Pediatric Unit of the Carlos Andrade Marín Specialties Hospital on september 13, 2019 with a surgical history of esophageal atresia corrected in the neonatal stage. He went with respiratory distress, chest X-ray that showed right pleural effusion, diagnostic thoracentesis with outflow of cloudy and milky fluid; a right chest tube was placed. Initial fasting was prescribed, parenteral nutrition for 4 weeks until resolution of the chylothorax was verified. He was discharged in stable condition after 43 days of hospitalization. DISCUSSION. The scientific evidence recorded that the conservative treatment of chylothorax was based on: drainage, initial digestive rest, parenteral nutrition, qualitative modification of enteral diet and use of octreotide; the same that was applied to the patient of this clinical case with favorable evolu-tion. CONCLUSION. Conservative and multidisciplinary treatment in the approach to chylothorax was successful.

Reparación toracoscópica de la atresia esofágica: implicaciones en el pronóstico del paciente / Thoracoscopic Repair of Esophageal Atresia: Implications in the prognosis of the patient

La reparación por toracoscopia de la atresia del esófago se ha constituido paulatinamente en la mejor alternativa terapéutica de los pacientes con esta entidad. Se analizaron 21 pacientes con diagnóstico de atresia esofágica operados por toracoscopia de Noviembre del 2017 a Agosto del 2020. La edad en que se efectuó la cirugía fue de 1 a 15 días de vida, promedio de 4.5. El procedimiento efectuado fue: sección y ligadura de la fistula con anastomosis termino terminal. El tiempo de cirugía fue de 90 a 240 minutos con una media de 158.5 minutos. Las complicaciones postoperatorias fueron: 02 pacientes dehiscencia parcial de la anastomosis, 05 pacientes estrechez de la anastomosis y 01 paciente divertículo traqueal. Hubo 03 pacientes que fallecieron. La reparación toracoscópica de la atresia de esófago es una opción terapéutica eficiente, con una tasa de complicaciones aceptable y una menor mortalidad. (AU)

Thoracoscopic repair of esophageal atresia has gradually become the best therapeutic alternative for patients with this entity. 21 patients with a diagnosis of esophageal atresia operated by thoracoscopy from November 2017 to August 2020 were analyzed. The age at which the surgery was performed was 1 to 15 days old, average 4.5. The procedure was: section and ligation of the fistula with end-to-end anastomosis. The surgery time was 90 to 240 minutes with a mean of 158.5 minutes. Postoperative complications were: 02 patients partial dehiscence of the anastomosis, 05 patients narrowing the anastomosis and 01 patient tracheal diverticulum. There were 03 patients who died. Thoracoscopic repair of esophageal atresia is an efficient therapeutic option, with an acceptable complication rate and lower mortality. (AU)

Effect of muscle relaxants on the prognosis of neonates with congenital esophageal atresia-tracheoesophageal fistula after surgery / 中国当代儿科杂志

OBJECTIVE@#To summarize the experience in the application of muscle relaxants in the perioperative period in neonates with congenital esophageal atresia-tracheoesophageal fistula (EA-TEF).@*METHODS@#A retrospective analysis was performed on the medical data of 58 previously untreated neonates with EA-TEF who were treated in the Neonatal Center of Beijing Children's Hospital, Capital Medical University from 2017 to 2019. The incidence rate of anastomotic leak was compared between the neonates receiving muscle relaxants for different durations after surgery (≤ 5 days and > 5 days). The correlation between the duration of postoperative use of muscle relaxants and the duration of mechanical ventilation was evaluated.@*RESULTS@#Among the 58 neonates with EA-TEF, 44 underwent surgery, among whom 35 with type III EA-TEF underwent thoracoscopic surgery. Among these 35 neonates, 30 (86%) received muscle relaxants after surgery, with a median duration of 4.75 days, and 6 (18%) experienced anastomotic leak. There was no significant difference in the incidence rate of anastomosis leak between the ≤ 5 days and > 5 days groups (@*CONCLUSIONS@#Prolonged use of muscle relaxants after surgery cannot significantly reduce the incidence of anastomotic leak, but can prolong the duration of invasive mechanical ventilation in neonates with EA-TEF. Therefore, prolonged use of muscle relaxants is not recommended after surgery.

Características clínico-quirúrgicas de la atresia esofágica en el Instituto Nacional de Salud del Niño San Borja, Lima, Perú. 2015-2017 / Clinical-surgical characteristics of esophageal atresia in the National Institute of Children's Health San Borja, Lima, Peru. 2015-2017

RESUMEN Objetivo : Describir las características clínico-quirúrgicas y sociodemográficas de pacientes con atresia esofágica (AE) del Instituto Nacional de Salud del Niño San Borja (INSN-SB) 2015-2017. Material y métodos : Estudio observacional descriptivo de casos con AE 2015-2017. Los datos se procesaron y analizaron en el programa SPSS v20, las variables cualitativas fueron expresadas en frecuencias absolutas y relativas. Resultados : Se analizaron 74 casos con AE, 55,4% (n=41) fueron de sexo masculino. Según edad gestacional 45 a término, 28 pre-término y un post-término. El 59,5% tuvieron peso adecuado al nacer. 28,4% tuvieron control prenatal (CPN) completo y 68,9% no tuvieron diagnóstico prenatal de AE. La atresia con fistula traqueo-esofágica (FTE) distal fue la más frecuente con 74,3% (n=55). Se presentó sialorrea en 55 pacientes (74,3%) y disnea en 54 (72,9%). Predominaron las malformaciones cardiacas en el 27,1%, seguida por digestivas 20,3% y genitourinarias 17,6%. Solo 26 tuvieron síndromes asociados, VACTERL en 12 casos. La anastomosis termino-terminal y cierre de fistula se realizó en 55 pacientes (74,3%). 48 presentaron complicaciones tempranas, predominando sepsis (20,3%) y 46 complicaciones tardías, siendo reflujo gastroesofágico el más frecuente 29,7%. La mortalidad fue 10,8%. Conclusiones : La mayoría fue de sexo masculino, a término, con adecuado peso al nacer y sin CPN. La AE con FTE distal fue la más frecuente. Sialorrea y disnea fueron los síntomas predominantes y malformaciones cardiacas y digestivas las más asociadas. La anastomosis término-terminal y cierre de fístula fue la técnica más frecuente, como complicaciones sepsis y reflujo gastroesofágico. La mortalidad fue baja.

ABSTRACT Objective : Describe the clinical-surgical and sociodemographic characteristics of patients with esophageal atresia (EA) of the Instituto Nacional de Salud del Niño San Borja (INSN-SB) during 2015-2017. Material and methods : Descriptive observational study of cases with EA 2015-2017. The data was processed and analyzed in the SPSS v20 program, the absolute and relative frequency distributions were expressed in tables. Results : 74 cases with AE were analyzed, 55.4% (n=41) were male. According to gestational age, 45 were at term, 28 pre-term and one post-term. 59.5% had adequate weight at birth. Only 28.4% had complete prenatal care (CPN). 68.9% did not have a prenatal diagnosis of AE. Atresia with distal tracheoesophageal fistula (TEF) was the most frequent in 74.3% (n=55). Hypersalivation occurred in 55 patients (74.3%), followed by dyspnea in 54 (72.9%). Cardiac malformations predominated in 27.1%, followed by digestive 20.3% and genitourinary 17.6%. Only 26 had associated syndromes, VACTERL in 12 cases. End-to-end anastomosis and fistula closure were performed in 55 patients (74.3%). 48 presented early complications, predominating sepsis (20.3%) and 46 late complications, with gastroesophageal reflux being the most frequent 29.7%. Regarding mortality, 8 (10.8%) died. Conclusions : Most of the cases were male, at term, with adequate weight at birth and without CPN. AE with distal TEF was the most frequent. Sialorrhea and dyspnea were the predominant symptoms and cardiac and digestive malformations the most associated. End-to-end anastomosis surgery and fistula closure was the most frequent technique. Complications were sepsis and gastroesophageal reflux. Mortality was low.

Asociación VACTERL de presentación inusual: reporte de caso / VACTERL Association with Unusual Presentation: Case Report / Associação VACTERL de apresentação rara: relato de caso

Resumen: En el contexto de Asociación VACTERL, el diagnóstico prenatal de atresia esofágica concomitante con atresia duodenal es poco común. En el presente artículo se realiza el reporte de un caso con la descripción de los hallazgos ecográficos encontrados a partir semana doce de gestación, con la aparición del signo de doble burbuja intraabdominal compatible con atresia duodenal y una arteria umbilical única; y hacia la semana 31 el hallazgo de imagen anecóica y dilatación esofágica del tercio distal con comunicación con la cámara gástrica en corte longitudinal, representando una atresia esofágica, asociada además a polihidramnios. Desde el momento del nacimiento por examen físico y estudios complementarios, se evidenció además ano imperforado con fístula recto vestibular, arcos costales derechos fusionados y hemivértebras. Por los anteriores hallazgos clínicos y radiológicos, se considera que se trata de una asociación VACTERL; sin embargo, sin alteraciones cardiacas, que es una de las características más frecuentemente encontradas. Se realiza una revisión del estado del arte con respecto a la asociación VACTERL y el diagnóstico prenatal de la atresia esofágica y duodenal.

Abstract: In the context of the VACTERL Association, prenatal diagnosis of concomitant esophageal atresia with duodenal atresia is rare. In this article, a case report is described with the description of the ultrasound findings found from week twelve of gestation, with the appearance of the intra-abdominal double bubble sign compatible with duodenal atresia and a single umbilical artery; and towards week 31 the finding of anechoic image and esophageal dilation of the distal-third with communication with the gastric chamber in longitudinal section, representing esophageal atresia, also associated with polyhydramnios. Physical examination and complementary studies from birth showed an imperforate anus with a rectovestibular fistula, and hemivertebrae. Based on the above clinical and radiological findings, it is considered to be a VACTERL association; however, without cardiac abnormalities, which is one of the most frequently found characteristics. A review of the state of the art is carried out with regard to the VACTERL association and the prenatal diagnosis of esophageal and duodenal atresia.

Resumo: No contexto da Associação VACTERL, o diagnóstico pré-natal de atrésia do esôfago concomitante com atrésia de duodeno é pouco comum. Neste artigo, é realizado relato de um caso com a descrição dos achados ecográficos encontrados a partir da 12a semana de gestação, com o surgimento do sinal de dupla bolha intra-abdominal compatível com a atrésia duodenal e uma artéria umbilical única; na 3ia semana, o achado de imagem anecoica e dilatação esofágica do rádio distai com comunicação com a câmara gástrica em corte longitudinal, o que representa atrésia esofágica, associada, ainda, a polidrâmnio. Desde o momento do nascimento por exame físico e estudos complementares, é evidenciado ânus imperfurado com fístula retal vestibular, arcos costais direitos fusionados e hemivértebras. A partir dos achados clínicos e radiológicos, é considerado que se trata de uma associação VACTERL; contudo, sem alterações cardíacas, que é uma das características mais frequentemente encontradas. É realizada uma revisão do estado da arte a respeito da associação VACTERL e o diagnóstico pré-natal da atrésia esofágica e duodenal.

Comorbilidades y evolución de la función pulmonar de pacientes con atresia esofágica congénita / Comorbidities and course of lung function in patients with congenital esophageal atresia

Objetivo. Describir las características epidemiológicas, el seguimiento hospitalario y la evolución de los pacientes intervenidos por atresia esofágica y su repercusión en la función pulmonar. Población y métodos. Estudio retrospectivo, longitudinal y analítico. Se revisaron las historias clínicas de pacientes con atresia esofágica nacidos entre 1996 y 2017. Se registraron datos perinatales, tipo de atresia, malformaciones asociadas, complicaciones respiratorias y digestivas, y los datos espirométricos durante tres años. Resultados. Se incluyeron 97 pacientes. El tipo de atresia más frecuente fue el III y el síndrome más frecuente, la trisomía 21. El 13,4 % fallecieron en el período neonatal. El 23,8 % de los pacientes estuvo en seguimiento por Neumología y presentó como complicaciones respiratorias exacerbaciones (el 46,4 %), sibilancias o asma (el 36 %), neumonías (el 26,8 %). El reflujo gastroesofágico fue factor de riesgo de sibilancias (OR 5,31; p = 0,002), exacerbaciones (OR 4,00; p = 0,009) y neumonías (OR 3,24; p = 0,02). En la primera espirometría (n = 20), un 65 % presentaba patrón normal; un 30 %, restrictivo, y un 5 %, mixto. En la segunda espirometría (n = 19), un 42,1 % presentaba patrón normal; un 31,6 %, restrictivo; un 15,8 %, obstructivo, y un 10,5 %, mixto. En la tercera espirometría (n = 14), el 50 % presentaba un patrón espirométrico normal; el 21,4 %, restrictivo; el 14,3 %, obstructivo, y un 14,3 %, mixto. Conclusiones. En nuestra muestra de pacientes, una importante proporción presentó comorbilidades respiratorias y digestivas. La función pulmonar empeoró progresivamente.

Objective. To describe the epidemiological characteristics, hospital follow-up, and course of patients who underwent surgery for esophageal atresia and its consequences on lung function. Population and methods. Retrospective, longitudinal, and analytical study. The medical records of patients with esophageal atresia born between 1996 and 2017 were reviewed. Perinatal data, type of atresia, associated malformations, respiratory and gastrointestinal complications, and spirometry data were recorded over 3 years. Results. A total of 97 patients were included. The most common type of atresia was III, and the most frequent syndrome, trisomy 21; 13.4 % of patients died in the neonatal period; 23.8 % were followed up by the Department of Pulmonology, and their respiratory complications included exacerbations (46.4 %), wheezing or asthma (36 %), and pneumonia (26.8 %). Gastroesophageal reflux was a risk factor for wheezing (OR: 5.31; p = 0.002), exacerbations (OR: 4.00; p = 0.009), and pneumonia (OR: 3.24; p = 0.02). In the first spirometry (n = 20), the pattern was normal in 65 %; restrictive in 30 %; and mixed in 5 %. In the second spirometry (n = 19), the pattern was normal in 42.1 %; restrictive in 31.6 %; obstructive in 15.8 %, and mixed in 10.5 %. In the third spirometry (n = 14), the pattern was normal in 50 %; restrictive in 21.4 %; obstructive in 14.3 %, and mixed in 14.3 %.Conclusions. In our sample of patients, a large proportion had respiratory and gastrointestinal comorbidities. Lung function worsened progressively.

Incidence of malnutrition, esophageal stenosis and respiratory complications among children with repaired esophageal atresia / Incidência de desnutrição, estenose esofágica e complicações respiratórias entre crianças operadas por atresia esofágica

ABSTRACT Background: Esophageal atresia is congenital anomaly with high mortality. Surgical complications and changes in nutritional status are common problems after surgical correction. Aim: To evaluate nutritional status, esophageal stenosis, and respiratory complications among children who had repaired esophageal atresia. Methods: Children aged >2 months old with repaired esophageal atresia were included in the current study. Gender, age, weight, and height were recorded for each case. Height for age and weight for age were calculated for each case. Results: According to weight for length percentile, 41.02% of the cases were underweight. Esophageal stenosis was seen in 54.76% of the obtained esophagograms. Conclusion: Underweight was present in 41.02 of the patients according to weight-for-height percentile.

RESUMO Racional: Atresia de esôfago é anomalia congênita com mortalidade alta. Complicações cirúrgicas e alterações no estado nutricional são problemas comuns após correção cirúrgica. Objetivo: Avaliar o estado nutricional, a estenose esofágica e as complicações respiratórias em crianças que tiveram a reparação de atresia de esôfago. Métodos: Crianças com idade> 2 meses de idade com atresia esofágica reparada foram incluídas no estudo atual. Sexo, idade, peso e altura foram registrados para cada caso. A altura por idade e o peso por idade foram calculados para cada caso. Resultados: De acordo com o peso para o percentil de comprimento, 41,02% dos casos estavam abaixo do peso. Estenose esofágica foi observada em 54,76% do esofagograma obtido. Conclusão: O baixo peso esteve presente em 41,02 dos pacientes, de acordo com o percentil peso/estatura.

Incidence of malnutrition, esophageal stenosis and respiratory complications among children with repaired esophageal atresia / Incidência de desnutrição, estenose esofágica e complicações respiratórias entre crianças operadas por atresia esofágica

ABSTRACT Background: Esophageal atresia is congenital anomaly with high mortality. Surgical complications and changes in nutritional status are common problems after surgical correction. Aim: T o evaluate nutritional status, esophageal stenosis, and respiratory complications among children who had repaired esophageal atresia. Methods: Children aged >2 months old with repaired esophageal atresia were included in the current study. Gender, age, weight, and height were recorded for each case. Height for age and weight for age were calculated for each case. Results: According to weight for length percentile, 41.02% of the cases were underweight. Esophageal stenosis was seen in 54.76% of the obtained esophagograms. Conclusion: Underweight was present in 41.02 of the patients according to weight-for-height percentile.

RESUMO Racional: Atresia de esôfago é anomalia congênita com mortalidade alta. Complicações cirúrgicas e alterações no estado nutricional são problemas comuns após correção cirúrgica. Objetivo: Avaliar o estado nutricional, a estenose esofágica e as complicações respiratórias em crianças que tiveram a reparação de atresia de esôfago. Métodos: Crianças com idade> 2 meses de idade com atresia esofágica reparada foram incluídas no estudo atual. Sexo, idade, peso e altura foram registrados para cada caso. A altura por idade e o peso por idade foram calculados para cada caso. Resultados: De acordo com o peso para o percentil de comprimento, 41,02% dos casos estavam abaixo do peso. Estenose esofágica foi observada em 54,76% do esofagograma obtido. Conclusão: O baixo peso esteve presente em 41,02 dos pacientes, de acordo com o percentil peso/estatura.

Impact of Intravenous Omega-3-Enriched Lipid Emulsion on Liver Enzyme and Triglyceride Serum Levels of Children Undergoing Gastrointestinal Surgery / 대한소아소화기영양학회지

Congenital Esophageal Atresia Associated with a Tracheobronchial Remnant / 대한흉부외과학회지

A rare case of esophageal atresia/tracheo-esophageal fistula (EA-TEF) with an associated tracheobronchial remnant (TBR) is reported and discussed herein. A 13-month-old patient was seen with a complaint of vomiting of solid food 1 year after EA-TEF repair. An esophagogram showed a tapered narrowing in the lower segment of the esophagus. A re-operation was carried out and a pathologic examination of the resected stenotic segment revealed the presence of a TBR.

Esofagitis eosinofílica y atresia esofágica: ¿casualidad o causalidad? / Eosinophilic esophagitis and esophageal atresia: coincidence or causality?

La esofagitis eosinofílica es una enfermedad crónica del esófago, inmunomediada, caracterizada por síntomas de disfunción esofágica y eosinofilia tisular. Endoscópicamente, la mucosa esofágica puede ser normal o tener exudados, anillos, edema, surcos y estenosis. El tratamiento se basa en dieta de exclusión, corticoterapia tópica y/o dilatación esofágica. La atresia es la alteración congénita más frecuente del esófago, requiere corrección quirúrgica con posibles complicaciones, como reflujo gastroesofágico, estenosis y dismotilidad esofágica. Hasta el año 2015, fueron comunicados 48 casos de esofagitis eosinofílica en atresia esofágica, en los que tuvo participación la dismotilidad, el reflujo y la supresión ácida prolongada. Clínicamente, predominan la disfagia, las dificultades en la alimentación y los síntomas de reflujo, por tanto, se sugiere la endoscopía digestiva alta con biopsias para descartar esofagitis eosinofílica asociada en pacientes con atresia esofágica y síntomas persistentes antes de realizar dilatación esofágica y/o cirugía antirreflujo. Si se confirma esofagitis eosinofílica, debe tratarse con medicación apropiada y subsiguientes conductas terapéuticas dependerán de su evolución.

Eosinophilic esophagitis is an immune-mediated chronic disease of the esophagus characterized by symptoms related to esophageal dysfunction and tissue eosinophilia. In the endoscopy, the esophageal mucosa may appear normal or show exudates, rings, edema, furrows, and strictures. Its management is based on elimination diet, topical corticosteroids and/or esophageal dilation. Atresia is the most common congenital alteration of the esophagus; it requires surgical repair and poses potential complications, such as gastroesophageal reflux, strictures, and esophageal dysmotility. Up to 2015, 48 cases of eosinophilic esophagitis and esophageal atresia were reported, with dysmotility, reflux, and long-term acid suppression involvement. Prevalent clinical signs include dysphagia, difficulty eating, and reflux symptoms, so an esophagogastroduodenoscopy with biopsy is recommended to rule out associated eosinophilic esophagitis in patients with esophageal atresia and persistent symptoms before performing an esophageal dilation and/or anti-reflux surgery. If eosinophilic esophagitis is confirmed, it should be managed with the corresponding drugs, and the subsequent therapeutic approach will depend on its clinical course.

Esophageal Reconstruction with Gastric Pull-up in a Premature Infant with Type B Esophageal Atresia

Esophageal atresia (EA) with proximal tracheoesophageal fistula (TEF; gross type B) is a rare defect. Although most patients have long-gap EA, there are still no established surgical guidelines. A premature male infant with symmetric intrauterine growth retardation (birth weight, 1,616 g) was born at 35 weeks and 5 days of gestation. The initial diagnosis was pure EA (gross type A) based on failure to pass an orogastric tube and the absence of stomach gas. A “feed and grow” approach was implemented, with gastrostomy performed on postnatal day 2. A fistula was detected during bronchoscopy for recurrent pneumonia; thus, we confirmed type B EA and performed TEF excision and cervical end esophagostomy. As the infant's stomach volume was insufficient for bolus feeding after reaching a body weight of 2.5 kg, continuous tube feeding was provided through a gastrojejunal tube. On the basis of these findings, esophageal reconstruction with gastric pull-up was performed on postnatal day 141 (infant weight, 4.7 kg), and he was discharged 21 days postoperatively. At 12 months after birth, there was no catch-up growth; however, he is currently receiving a baby food diet without any complications. In patients with EA, bronchoscopy is useful for confirming TEF, whereas for those with long-gap EA with a small stomach volume, esophageal reconstruction with gastric pull-up after continuous feeding through a gastrojejunal tube is worth considering.

Muscle-sparing versus standard posterolateral thoracotomy in neonates with esophageal atresia / Toracotomia posterolateral com poupança muscular versus toracotomia padrão em neonatos com atresia esofágica

ABSTRACT Background : The muscle-sparing thoracotomy (MST) has not yet been thoroughly studied and assessed in comparison to the traditional thoracotomy method in newborns. Aim : To compare the outcomes of MST and standard posterolateral thoracotomy (PLT) in newborns. Methods : Randomized, controlled, double-blind trial on 40 neonates with esophageal atresia, comparing the time of beginning a surgery until seeing the pleura, the duration of hospitalization in the neonatal intensive care unit, the time in ventilator, the time of returning the shoulder function, the time of returning the Moro reflex, and the mortality between the two techniques. Results : The data showed no differences between the two groups in basic information (weight, height, gender, numbers of prematurity neonates and caesarean). The results on the size of the scar in the MST group was significantly lower than in the PLT group. Also, the time of returning the shoulder function in MST group was earlier than in PLT group. There were no significant differences in the duration since the beginning the surgery to see the pleura, the time of being hospitalized in intensive unit, the time that the infant required ventilator, returning time of the Moro reflex in 1st and 3rd months after the operation, and the mortality rates between MST and PLT groups. Conclusion : It seems that the advantages of using MST over PLT procedure in neonates include the earlier shoulder function recovery and also superior cosmetic results.

RESUMO Racional : A técnica de toracotomia poupadora de músculo (MST) ainda não foi estudada e avaliada em relação ao método tradicional de toracotomia em recém-nascidos. Objetivo : Comparar os resultados da MST e toracotomia posterolateral padrão (PLT) em recém-nascidos. Métodos : Ensaio randomizado, controlado, duplamente cego em 40 neonatos com atresia esofágica, comparando o tempo de início da incisão até ver a pleura, a duração da hospitalização na unidade de terapia intensiva neonatal, o tempo em ventilador, o tempo da volta da função do ombro, tempo de retorno do reflexo Moro e mortalidade entre as duas técnicas. Resultados : Os dados não mostraram diferenças entre os dois grupos em informações básicas (peso, altura, gênero, número de neonatos de prematuridade e cesariana). Os resultados sobre o tamanho da cicatriz no grupo MST foram significativamente menores do que no grupo PLT. Além disso, o tempo de retorno da função do ombro no grupo MST foi mais precoce do que no grupo PLT. Não houve diferenças significativas na duração desde o início da operação até a pleura ser vista, o tempo de hospitalização em unidade intensiva, o tempo que a criança necessitou de ventilador, retorno do reflexo Moro nos 1º e 3º meses após a operação, e as taxas de mortalidade entre os grupos. Conclusão : As vantagens de usar o procedimento MST sobre PLT em neonatos incluem a recuperação da função do ombro e também resultados cosméticos superiores.

Resultados de la cirugía de reconstrucción esofágica en pacientes pediátricos con patología esofágica compleja en dos hospitales de alto nivel de Medellín, Colombia, 2006-2016 / Esophageal reconstruction results in surgery of pediatric patients with complex esophageal pathology in two high level hospitals of Medellín, Colombia, 2006-2016 / Resultados da cirurgia de reconstrução esofágica em pacientes pediátricos com patologia esofágica complexa em dois hospitais de alto nível de Medellín, Colômbia, 2006-2016

RESUMEN Objetivos: el esófago difícil (complejo) representa la vía final común de un conjunto de enfermedades que requieren múltiples procedimientos o cirugía de reemplazo esofágico; ya sea porque desde el inicio es la única alternativa de manejo o porque han fallado previamente otros tipos de intervenciones menos radicales. El objetivo de este estudio es describir los resultados de la cirugía de reconstrucción esofágica en pacientes con patología esofágica compleja en dos hospitales de alto nivel de Medellín (Colombia) entre el 1° enero de 2006 y el 31 de junio de 2016. Métodos: estudio descriptivo retrospectivo en el que se revisaron las historias clínicas del Hospital Pablo Tobón Uribe (HPTU) y el Hospital Universitario San Vicente Fundación (HSVF), recopilando 40 pacientes que cumplieron con los criterios de inclusión. Resultados: el diagnóstico más frecuente de esófago difícil corresponde a atresia de esófago, seguido de quemadura esofágica. Las causas directas de la condición fueron estenosis esofágicas persistentes, fístulas traqueoesofágicas recurrentes, fístulas esofagopleurales y brecha larga. Las intervenciones quirúrgicas fueron en orden de frecuencia: ascenso gástrico, corrección de la fístula, interposición de colon, esofagostomía más gastrostomía, resección, anastomosis y esofagoplastias. Las principales complicaciones posoperatorias fueron la estenosis, las fístulas, la infección del sitio operatorio y los trastornos funcionales. Conclusiones: los niños con patología esofágica compleja representan un reto para los cirujanos pediátricos, tanto por la dificultad de los procedimientos a los que son sometidos como por la morbilidad de las enfermedades que conllevan y de las cirugías que requieren para dar continuidad al tracto digestivo.

SUMMARY Introduction: complex esophagus represents a final condition that occurs secondary to diseases that require multiple procedures or esophageal replacement surgery, either because it is the only treatment or because others interventions have failed. Our objective is to describe the results of esophageal reconstruction surgery in patients with complex esophageal pathology attended in two high-level hospitals in Medellin, Colombia, between January 1- 2006 and June 31- 2016. Methods: descriptive and retrospective study with review of clinical records at Hospital Pablo Tobón Uribe (HPTU) and Hospital Universitario San Vicente Fundación (HSVF), collecting 40 patients. Results: the most frequent diagnosis was esophageal atresia, followed by esophageal burn. The main causes that led these patients to be classified as complex esophagus were persistent esophageal strictures, recurrent tracheoesophageal fistulas, esophageal fistulas and the long gap. The surgical interventions were in order of frequency: gastric replacement, closure of the fistula, colon replacement, esophagostomy and gastrostomy, resection and anastomosis and esophagoplasty. The main postoperative complications of patients undergoing esophageal reinterventions or esophageal replacement were stenosis, fistulas, surgical site infection and functional disorders. Conclusions: children with complex esophageal pathology represent a challenge for pediatric surgeons, because of the difficulty of the procedures they undergo as well as the morbidity of their diseases and surgeries they require to give continuity to their digestive tract.

RESUMO As crianças com patologia esofágica complexa representam um desafio para os cirurgiões pediátricos, tanto pela dificuldade dos procedimentos aos que são submetidos, como pela morbidez das doenças que implicam e das cirurgias que requerem para dar continuidade ao trato digestivo. O objetivo do estudo é descrever os resultados da cirurgia de reconstrução esofágica em pacientes com patologia esofágica complexa, definida como a falha no manejo endoscópico ou na cirurgia de reparação inicial em dois hospitais de alto nível de Medellín, Colômbia entre o dia 1° janeiro de 2006 e o dia 31 de junho de 2016. Se revisaram as histórias do Hospital Pablo Tobón Uribe (HPTU) e Hospital San Vicente Fundación (HSVF), recopilando 40 pacientes. O diagnóstico mais frequente de esôfago difícil corresponde a atresia de esôfago, seguido de queimadura esofágica. As principais causas que levaram a estes pacientes a catalogarse como esôfago complexo foram: estenose esofágicas persistentes e fístulas traqueoesofágicas recorrentes, fístulas esôfago-pleurais e brecha longa. As intervenções cirúrgicas foram em ordem de frequência: Ascenso gástrico, correção da fístula, interposição de colón, esofagostomia mais gastrostomia, ressecção, anastomose e esofagoplastias. Apresentam-se as principais complicações pós-operatórias e o tempo de estância hospitalar, nutrição parenteral e em reiniciar a via oral, ademais, mostramos o seguimento pós-cirúrgico.

Rare cause of dysphagia in children

Dysphagia in children is generally caused by strictures secondary to gastroesophageal reflux disease or post-repair of trachea-esophageal atresia/fistula

A four-year-old female presented with a history of dysphagia for solid food and vomiting of undigested food. Barium swallow showed esophageal duplication cyst, which was confirmed by esophagoscopy

Surgical resection was curative

Causes other than stricture or dysmotility should be considered in children with unrelenting dysphagia.

Clinical Study of Congenital Esophageal Stenosis: Comparison according to Association of Esophageal Atresia and Tracheoesophageal Fistula / 대한소아소화기영양학회지

PURPOSE: Congenital esophageal atresia (CES) is a rare congenital disease. The severity of symptoms is variable; thus, diagnosis is difficult and tends to be delayed. CES is frequently accompanied by esophageal atresia (EA) with/without tracheoesophageal fistula (TEF). We investigated the characteristics of CES by reviewing our experience with CES patients and researched the differences between CES with EA-TEF and isolated CES. METHODS: A total of 31 patients underwent operations for CES were reviewed retrospectively. The patients were divided into two groups according to the association with EA-TEF, and compared the differences. RESULTS: Sixteen boys and 15 girls were included. The mean age at symptom onset was 8 months old, and the mean age at diagnosis was 21 months old. Nine patients with EA-TEF were included group A, whereas the other 22 patients were assigned to group B. There were no differences in sex, gestational age, associated anomalies and pathologic results between the groups. In group A, the age at diagnosis and age at surgery were younger than in group B despite the age at symptom occurrence being similar. Postoperative complications occurred only in group A. CONCLUSION: In this study, symptoms occurred during the weaning period, and vomiting was the most frequent symptom. CES patients with EA-TEF tended to be diagnosed and treated earlier despite the age at symptom occurrence being similar. CES patients with EA-TEF had more postoperative complications; therefore, greater attention should be paid during the postoperative period.