Consideraciones anestésicas en recién nacido con atresia y fístula traqueoesofágica / Anesthetic Considerations in Newborns with Tracheoesophageal Fistula and Atresia

Introducción: La atresia esofágica es una malformación congénita poco frecuente, que consiste en un defecto en el desarrollo del intestino anterior hacia el esófago y la tráquea que se produce aproximadamente en la cuarta semana de gestación. Objetivo: Describir los resultados de la actuación anestesiológica en un paciente con atresia esofágica y fístula traqueoesofágico, así como las consideraciones anestésicas para el tratamiento de la entidad. Presentación de caso: Paciente a término, femenina, blanca, peso al nacer 2880 g, Apgar 8-9 al min de vida, con diagnóstico de atresia esofágica ante la presencia de distrés respiratorio al nacimiento, salivación excesiva con crisis de atragantamiento a las 6 h de nacida e imposibilidad de introducir una sonda nasogástrica lo que fue confirmado en una radiografía de tórax. Fue anunciada por cirugía pediátrica y se efectuó reparación de la fístula. Se exponen las consideraciones anestésicas a seguir en el manejo de este caso, relacionadas con: los fármacos anestésicos, reposición de volumen y la estrategia de ventilación mecánica. Conclusiones: La urgente resolución quirúrgica en recién nacidos con atresia esofágica es importante para su supervivencia, para disminuir las complicaciones, los efectos adversos por la posición y el tipo de intervención que pueden presentar los pacientes. Por tanto, es imprescindible una detallada conducta anestésica. Representa un reto el manejo anestésico porque se requiere un abordaje multimodal en el pre-, intra- y posoperatorio; la seguridad de la vía aérea y una ventilación efectiva son las pautas en estas enfermedades(AU)

Introduction: Esophageal atresia is a rare congenital malformation, which consists of a defect in the development of the anterior intestine towards the esophagus and the trachea that occurs approximately in the fourth week of gestation. Objective: to describe the results of the anesthesiological intervention in a patient with EA and TEF, as well as the anesthetic considerations for the treatment of the entity. Case presentation: Term patient, female, white, birth weight 2880g, Apgar 8-9 at minute of life, diagnosed with esophageal atresia in the presence of respiratory distress at birth, excessive salivation with choking crisis at 6 hours of birth, and impossibility of inserting a nasogastric tube and confirmed by chest X-ray. She was announced for pediatric surgery and fistula repair was performed. The anesthetic considerations to be followed in the management of this case are exposed, related to: anesthetic drugs, volume replacement and the mechanical ventilation strategy. Favorable results were obtained. Conclusions: The urgent surgical resolution in newborns with esophageal atresia is important for their survival, to reduce complications, adverse effects due to the position and the type of intervention that patients may present, a detailed anesthetic conduct is essential. Anesthetic management represents a challenge because a multimodal approach is required pre, intra and postoperatively; airway safety and effective ventilation are the guidelines in these diseases(AU)

Fístula traqueoesofágica como complicación de la intubación endotraqueal prolongada / Tracheoesophageal fistula as a complication of prolonged endotracheal intubation

RESUMEN Introducción: La fístula traqueoesofágica consecutiva a intubación endotraqueal prolongada es una lesión grave con elevada morbimortalidad. El alto índice de sospecha, diagnóstico precoz, resolución de las complicaciones y un tratamiento quirúrgico definitivo son los pilares fundamentales en los cuales descansa su manejo correcto. Objetivo: Describir el comportamiento y manejo de la fístula traqueoesofágica en pacientes con intubación endotraqueal prolongada. Presentación del caso: Paciente de 34 años de edad que sufrió trauma craneoencefálico grave con necesidad de intubación endotraqueal prolongada. Su evolución fue favorable, con recuperación neurológica, pero presentaba tos incontrolable después de la deglución, aumento de las secreciones respiratorias y pérdida de 30 Kg de peso no resuelta, lo que motivó se le realizara tomografía computarizada multicorte dual sincronizada con el electrocardiograma, la que permitió de forma rápida y no invasiva, llegar al diagnóstico de la fístula traqueoesofágica. Conclusiones: La intubación endotraqueal prolongada constituye la causa principal de la aparición de la fístula traqueoesofágica. El mecanismo de producción fundamental fue la isquemia provocada por la compresión de las paredes posterior de la tráquea y anterior del esófago entre el manguito insuflado del tubo endotraqueal y la sonda nasogástrica. La tomografía computarizada multicorte dual sincronizada con el electrocardiograma permite realizar el diagnóstico de esta complicación.

ABSTRACT Introduction: Tracheoesophageal fistula following prolonged endotracheal intubation is a serious lesion with high morbidity and mortality. The high index of suspicion, early diagnosis, resolution of complications and definitive surgical treatment are the fundamental pillars on which its correct management rests. Objective: Describe the behavior and management of tracheoesophageal fistula in patients with prolonged endotracheal intubation. Case Presentation: A 34-year-old patient who suffered severe head trauma with the need of prolonged endotracheal intubation. His evolution was favorable, with neurological recovery, but he presented uncontrollable cough after swallowing, increased respiratory secretions and unsolved loss of 30 Kg of weight, which motivated to perform to him a dual multi-cut computed tomography synchronized with the electrocardiogram, which allowed quickly and non-invasively, to reach the diagnosis of tracheoesophageal fistula. Conclusions: Prolonged endotracheal intubation is the main cause of the appearance of tracheoesophageal fistula. The fundamental production mechanism was ischemia caused by compression of the posterior walls of the trachea and anterior walls of the esophagus between the inflated cuff of the endotracheal tube and the nasogastric tube. The dual multi-cut computed tomography synchronized with the electrocardiogram allows the diagnosis of this complication.

Surgical approach for iatrogenic tracheoesophageal fistula. Case report and literature review.

BACKGROUND: Post-intubation tracheoesophageal fistula is a severe complication in long-term mechanical ventilation patients with possible fatal consequences. OBJECTIVE: To describe a case of post-intubation tracheoesophageal fistula and its surgical management. In addition, a brief literature review was effectuated. CASE REPORT: 45-year-old female, suffers ischemic stroke with progressive neurological damage that requires long-term mechanical ventilation. During endoscopic gastrostomy tracheoesophageal fistula is shown. RESULTS: Undergoes surgery for esophageal repair and tracheal resection, through cervicosternotomy. Unfortunately died in the immediate postoperative period. CONCLUSIONS: This complex pathology requires structured protocols for its prevention in patients in whom long-term mechanical ventilation is expected.

ANTECEDENTES: La fístula traqueoesofágica es una complicación en pacientes con ventilación mecánica prolongada. La contaminación pulmonar permanente puede ser grave, con evolución fatal. OBJETIVO: Describir un caso de fístula traqueoesofágica posintubación y hacer una breve revisión de la literatura. CASO CLÍNICO: Mujer de 45 años que sufre un evento cerebrovascular isquémico que condiciona deterioro neurológico progresivo, ameritando ventilación mecánica prolongada. Durante la gastrostomía endoscópica se evidencia una fístula. RESULTADOS: Intervenida quirúrgicamente para reparación esofágica y resección traqueal mediante cervicoesternotomía, lamentablemente fallece en el posoperatorio inmediato. CONCLUSIONES: Esta patología es compleja. Deben implementarse protocolos enfocados en su prevención en pacientes en quienes se espera una ventilación mecánica prolongada.

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.

Asociación VACTERL con poliquistosis renal en un recién nacido: reporte de caso

Las malformaciones son causas importantes de mortalidad infantil, enfermedad crónica y discapacidad en muchos países, y ocasionan 3,2 millones de discapacidades al año. La asociación VACTERL involucra defectos vertebrales, atresia anal, fístula traqueoesofágica y displasia renal, así como anormalidades en las extremidades. Nuestro objetivo es describir las características generales de la asociación VACTERL y realizar un acercamiento a otros casos en la literatura. Nuestro caso trata de una recién nacida a las 35 semanas con grave dificultad respiratoria, que ingresa a la UCI neonatal por descompensación hemodinámica. Tiene un antecedente de poliquistosis renal bilateral a las 25 semanas dado por ecografía. En el examen físico se evidencia agenesia de órganos sexuales y ano imperforado, y en la radiografía se observa patrón atelectásico, cardiomegalia y posición dextrógira de la silueta cardiaca. La paciente fue diagnosticada con asociación VACTERL y ano imperforado. Sufrió un paro respiratorio y falleció luego de dos días.

Malformations are important causes of infant mortality, chronic disease and disability in many countries, causing 3.2 million disabilities per year. The VATERL association includes vertebral defects, anal atresia, tracheoesophageal fistula and renal dysplasia, as well as limb abnormalities. This case report aims to provide the general characteristics of the VACTERL association and discuss other cases found in the medical literature. This is the case of a female newborn delivered at 35 weeks of pregnancy with severe respiratory distress, who was admitted to the neonatal ICU due to hemodynamic decompensation. She had a history of bilateral polycystic kidney disease found by ultrasound at 25 weeks of pregnancy. A physical examination showed vaginal agenesis and imperforate anus. Atelectasis, cardiomegaly and dextroposition of the cardiac silhouette were observed in a radiography. The patient was diagnosed with VACTERL association and imperforate anus. She died 2 days after a respiratory arrest.

Primary repair: damage control surgery in esophageal trauma / El reparo primario: el control de daños en el trauma de esófago

Abstract Esophageal trauma is a rare but life-threatening event associated with high morbidity and mortality. An inadvertent esophageal perforation can rapidly contaminate the neck, mediastinum, pleural space, or abdominal cavity, resulting in sepsis or septic shock. Higher complications and mortality rates are commonly associated with adjacent organ injuries and/or delays in diagnosis or definitive management. This article aims to delineate the experience obtained by the Trauma and Emergency Surgery Group (CTE) of Cali, Colombia, on the surgical management of esophageal trauma following damage control principles. Esophageal injuries should always be suspected in thoracoabdominal or cervical trauma when the trajectory or mechanism suggests so. Hemodynamically stable patients should be radiologically evaluated before a surgical correction, ideally with computed tomography of the neck, chest, and abdomen. While hemodynamically unstable patients should be immediately transferred to the operating room for direct surgical control. A primary repair is the surgical management of choice in all esophageal injuries, along with endoscopic nasogastric tube placement and immediate postoperative care in the intensive care unit. We propose an easy-to-follow surgical management algorithm that sticks to the philosophy of "Less is Better" by avoiding esophagostomas.

Resumen El trauma esofágico es un evento poco frecuente pero potencialmente mortal. Una perforación esofágica inadvertida puede ocasionar la rápida contaminación del cuello, el mediastino, el espacio pleural o la cavidad abdominal, lo cual puede resultar en sepsis o choque séptico. Las complicaciones y la mortalidad aumentan con el retraso en el diagnóstico o manejo definitivo, y la presencia de lesiones asociadas. El objetivo del presente artículo es describir la experiencia adquirida por el grupo de cirugía de Trauma y Emergencias (CTE) de Cali, Colombia en el manejo del trauma de esófago de acuerdo con los principios de la cirugía de control de daños. Las lesiones esofágicas deben sospecharse en todo trauma toraco-abdominal o cervical en el que el mecanismo o la trayectoria de la lesión lo sugieran. El paciente hemodinámicamente estable se debe estudiar con imágenes diagnósticas antes de la corrección quirúrgica del defecto, idealmente por medio de tomografía computarizada del cuello, tórax y abdomen con contraste endovenoso. Mientras que en el paciente hemodinámicamente inestable se debe explorar y controlar la lesión. El reparo primario es el manejo quirúrgico de elección, con la previa colocación de una sonda nasogástrica y el seguimiento postoperatorio estricto en la unidad de cuidado intensivo. Se propone un algoritmo de manejo quirúrgico que resulta fácil de seguir y adopta la premisa "Menos es Mejor" evitando realizar derivaciones esofágicas.

Fístula traqueoesofágica en H en el período neonatal: dificultades en el diagnóstico y diferentes abordajes terapéuticos. Serie de casos / H-type tracheoesophageal fistula in neonatal period: Difficulties in diagnosis and different treatment approaches. A case series

La fístula traqueoesofágica congénita sin atresia esofágica asociada, conocida como tipo H, es una anomalía infrecuente. Se manifiesta con episodios de tos, ahogo y cianosis durante la alimentación y/o neumonía recurrente.Si bien los síntomas están usualmente presentes desde el nacimiento, el diagnóstico es difícil. La rareza de esta patología, los síntomas no específicos y las limitaciones en la demostración radiológica y endoscópica de la fístula contribuyen, a menudo, a la demora entre la presentación y la confirmación del diagnóstico.Se describen las manifestaciones clínicas, los métodos de evaluación y el tratamiento de 3 neonatos con esta patología, y se presentan recomendaciones para el diagnóstico a fin de evitar demoras innecesarias en el manejo de las fístulas en H.

Congenital tracheoesophageal fistula not associated with esophageal atresia, known as H-type fistula, is an uncommon anomaly. It presents with cough, choking, and cyanosis during feeding and/or recurrent pneumonia. Although symptoms are usually present from birth, diagnosis is difficult. The rarity of this disease, non-specific symptoms, and the limitations of radiological and endoscopic confirmation of the fistula often result in a delay between presentation and diagnosis confirmation. Here we describe the clinical manifestations, assessment methods, and management of 3 newborn infants with H-type tracheoesophageal fistula, together with diagnosis recommendations to prevent unnecessary delays in the management of this condition.

Cuidados de las fístulas traqueoesofágicas postintubación / Care of the post-intubation tracheoesophageal fistulae

RESUMEN Introducción: Las fístulas traqueoesofágicas posintubación son lesiones graves que presentan alta frecuencia de complicaciones y mortalidad. Objetivo: Actualizar el diagnóstico y tratamiento de pacientes con esta afección. Método: Se revisó la literatura en PUBMED/Medline e Infomed con las palabras clave: fístula, traqueoesofágica, fístula traqueoesofágica, postintubación y las correspondientes en inglés. Se revisaron las referencias bibliográficas de los artículos en busca de publicaciones relevantes. Se escogieron, preferentemente, trabajos prospectivos. Los informes de casos sólo se tomaron en cuenta cuando hacían referencia a alguna técnica novedosa. Desarrollo: Se encontraron 4260 artículos en Medline/Pubmed y 17 en Infomed. No se encontraron ensayos clínicos, metanálisis, ni revisiones sistemáticas. La mayoría de los artículos revisados hacen referencia a presentación de casos y algunos fueron estudios retrospectivos. Las presentaciones de casos se usaron solo si describían una técnica novedosa. Conclusiones: La intubación endotraqueal prolongada es la causa principal de FTEs. La patogenia se asocia con isquemia secundaria a compresión de las paredes traqueal posterior yesofágica anterior, entre el manguito insuflado del tubo endotraqueal y la sonda nasogástrica. El tratamiento conservador, generalmente, no cura la fístula, pero puede limitar la contaminación del árbol traqueobronquial y garantiza la nutrición. La derivación esofágica raramente está indicada, excepto cuando persiste entrada a la VR del contenido gastrointestinal. Actualmente, el tratamiento definitivo es la sutura esofágica y, en la mayoría de los casos, la resección y anastomosis traqueal(AU)

ABSTRACT Introduction: Post-intubation tracheoesophageal fistulae are severe lesions with high frequency of complication and high rate of mortality. Objective: To update the knowledge about the diagnosis and treatment of patients with this disease. Method: Literature review made in PUBMEDF/Medline and Infomed using the keywords: fistula, traqueoesophageal, tracheoesophageal fistula, and post-intubation in Spanish and English. References of articles were also reviewed to find out relevant publications. Prospective papers were particularly chosen. The case reports were only taken into account when they made reference to some novel technique. Results: In this search, 4260 articles were found in Medline/Pubmed and 17 in Infomed databases. No clinical assays, meta-analysis or systematic reviews were found. Most of the articles made reference to case reports and some to retrospective studies. The case reports were only used if they described a novel technique. Conclusions: Prolonged endotracheal intubation is the main cause of traqueoesophageal fistula. The pathogeny is associated to ischemia secondary to compression of posterior tracheal wall and anterior esophageal wall between the inflated cuff of the endotracheal tube and the nasogastric probe. In general, the conservative treatment does not cure the fistula but may restrict contamination of the tracheobronchial tree and guarantee nutrition. The esophageal shunt is rarely indicated, except when the gastrointestinal content continues entering into the VR. At present, the definitive treatment is the esophageal suturing and in most of cases, the resection and tracheal anastomosis(AU)

Cuidados de las fístulas traqueoesofágicas postintubación / Care of the post-intubation tracheoesophageal fistulae

Introducción: Las fístulas traqueoesofágicas posintubación son lesiones graves que presentan alta frecuencia de complicaciones y mortalidad. Objetivo: Actualizar el diagnóstico y tratamiento de pacientes con esta afección. Método: Se revisó la literatura en PUBMED/Medline e Infomed con las palabras clave: fístula, traqueoesofágica, fístula traqueoesofágica, postintubación y las correspondientes en inglés. Se revisaron las referencias bibliográficas de los artículos en busca de publicaciones relevantes. Se escogieron, preferentemente, trabajos prospectivos. Los informes de casos sólo se tomaron en cuenta cuando hacían referencia a alguna técnica novedosa. Desarrollo: Se encontraron 4260 artículos en Medline/Pubmed y 17 en Infomed. No se encontraron ensayos clínicos, metanálisis, ni revisiones sistemáticas. La mayoría de los artículos revisados hacen referencia a presentación de casos y algunos fueron estudios retrospectivos. Las presentaciones de casos se usaron solo si describían una técnica novedosa. Conclusiones: La intubación endotraqueal prolongada es la causa principal de FTEs. La patogenia se asocia con isquemia secundaria a compresión de las paredes traqueal posterior yesofágica anterior, entre el manguito insuflado del tubo endotraqueal y la sonda nasogástrica. El tratamiento conservador, generalmente, no cura la fístula, pero puede limitar la contaminación del árbol traqueobronquial y garantiza la nutrición. La derivación esofágica raramente está indicada, excepto cuando persiste entrada a la VR del contenido gastrointestinal. Actualmente, el tratamiento definitivo es la sutura esofágica y, en la mayoría de los casos, la resección y anastomosis traqueal(AU)

Introduction: Post-intubation tracheoesophageal fistulae are severe lesions with high frequency of complication and high rate of mortality. Objective: To update the knowledge about the diagnosis and treatment of patients with this disease. Method: Literature review made in PUBMEDF/Medline and Infomed using the keywords: fistula, traqueoesophageal, tracheoesophageal fistula, and post-intubation in Spanish and English. References of articles were also reviewed to find out relevant publications. Prospective papers were particularly chosen. The case reports were only taken into account when they made reference to some novel technique. Results: In this search, 4260 articles were found in Medline/Pubmed and 17 in Infomed databases. No clinical assays, meta-analysis or systematic reviews were found. Most of the articles made reference to case reports and some to retrospective studies. The case reports were only used if they described a novel technique. Conclusions: Prolonged endotracheal intubation is the main cause of traqueoesophageal fistula. The pathogeny is associated to ischemia secondary to compression of posterior tracheal wall and anterior esophageal wall between the inflated cuff of the endotracheal tube and the nasogastric probe. In general, the conservative treatment does not cure the fistula but may restrict contamination of the tracheobronchial tree and guarantee nutrition. The esophageal shunt is rarely indicated, except when the gastrointestinal content continues entering into the VR. At present, the definitive treatment is the esophageal suturing and in most of cases, the resection and tracheal anastomosis(AU)

Intubación selectiva evaluada con ecografía en neonato pretérmino llevado a corrección de atresia esofágica tipo C. Reporte de caso / Ultrasound-guided selective intubation in a preterm neonate undergoing type-C esophageal athresia correction. Case report

Abstract Introduction: Neonatal patients presenting with tracheoesophageal fistula represent a challenge to the anesthesiologist due to the multiple difficulties this pathology involves for airway management. Case discussion: Following is a description of a case of a neonate undergoing tracheoesophageal fistula repair using ultrasound-guided orotracheal intubation as an adjunct to selective intubation. Conclusion: Perioperative ultrasound is a promising tool for airway management of the pediatric patient. Further studies to assess the possibility to position the technique as a standard of care are needed.

Resumen Introducción: Los pacientes neonatales con fístula traqueoesofágica representan un reto para el anestesiólogo dadas las dificultades en el manejo de la vía aérea que esta patología supone. Presentación del caso: A continuación se expone un caso de un neonato llevado a corrección de fístula traqueoesofágica con intubación orotraqueal guiada por ultrasonido como técnica adjuvante a la intubación selectiva. Conclusion: La ultrasonografía perioperatoria en el manejo de la vía aérea del paciente pediático constituye una herramienta prometedora que requiere de estudios adicionales para evaluar la posibilidad de posicionarla como un estándar de cuidado.

Manejo anestésico de atresia esofágica y fistula traqueoesofágica. Presentación de un caso / Anesthesia management of esophageal atresia and tracheoesophageal fistula in neonates. Case presentation

La atresia esofágica es una malformación congénita infrecuente incompatible con la vida y una urgencia quirúrgica neonatal. La presentación más común es la atresia esofágica con fístula traqueoesofágica distal (80-90 % de los casos). Tiene una frecuencia de presentación de uno por cada 3000 nacimientos. Por estas razones se presenta el caso de un neonato pretérmino, femenina, de 48 horas de nacimiento, 1245 gramos de peso, APGAR 8/9, con diagnóstico de atresia esofágica y fístula traqueoesofágica tipo lllb/C. Fue diagnosticada al nacimiento, ante la presencia de vómito en proyectil, distrés respiratorio e imposibilidad de introducir una sonda nasogástrica y confirmada al observarse en una radiografía de tórax. Fue anunciada para cirugía en la cual se efectuó reparación de la fistula. Se exponen las consideraciones anestésicas a seguir en el manejo de este caso, relacionadas con: los fármacos anestésicos, reposición de volumen y la estrategia de ventilación mecánica. Se obtuvieron resultados favorables.

Esophageal atresia is an infrequent congenital malformation incompatible with life and a neonatal surgical urgency. The most common presentation is esophageal atresia with distal tracheoesophageal fistula (80-90 % of cases). It presents in one of every 3000 births. For these reasons, the case of a preterm infant, 48 hours of birth, 1245 grams weight, APGAR 8/9, with diagnosis of esophageal atresia and tracheo-esophageal fistula type lllb / C is presented. She was diagnosed at birth, due to the presence of projectile vomiting, respiratory distress and the impossibility of introducing a naso-gastric tube and confirmed when seen on a chest x-ray. It was announced for surgery in which fistula repair was performed. We present the anesthetic considerations to be followed in the management of this case, related to: anesthetic drugs, volume replacement and mechanical ventilation strategy. Favorable results were obtained.

Relato de um caso de associação de VACTERL e discussão acerca de seus aspectos prognósticos / Case report of VACTERL association and discussion about its prognostic aspects

Associação de VACTERL corresponde à combinação de três ou mais das seguintes malformações: Vertebrais, atresia Anal, alterações Cardíacas, fístula Traqueo-esofágica com atresia de Esôfago, anomalias Renais e de membros (Limbs). Sua etiologia ainda é desconhecida, mas acredita-se que seja multifatorial, associada a mutações em genes como FOXF1 e ZIC3 e a fatores de risco maternos. No presente relato, descreve-se um caso de associação de VACTERL diagnosticado no Hospital Universitário Regional de Maringá (HURM). Paciente L.P.N., sexo feminino, um dia de vida, foi referenciada para UTI neonatal devido ao quadro de desconforto respiratório, cianose e salivação intensa na sala de parto, sendo diagnosticada com atresia de esôfago. No 9º dia de vida realizou-se correção cirúrgica da atresia esofá- gica e fístula traqueo-esofágica. Outras malformações foram observadas, detectando-se comunicação interventricular perimembranosa moderada e rim direito ausente, sugerindo diagnóstico de VACTERL. Com três anos e nove meses, apresenta-se eutrófica, saudável e em acompanhamento multiprofissional. Apesar de ser uma afecção rara, esta associação necessita de expertise dos profissionais de saúde, para que haja detecção precoce e tratamento de suas alterações e complicações. Além disso, se manejada da forma correta, a criança apresenta boa evolução e perspectivas de uma vida normal. O prognóstico depende da extensão e da gravidade das anomalias associadas, bem como da capacitação dos pais e equipe profissional (AU)

VACTERL Association corresponds to the combination of three or more of the following defects: Vertebral, Anal atresia, Cardiac changes, Tracheo-esophageal fistula, Esophagus atresia, Renal anomalies and Limbs. Its etiology is still unknown, but believed to be multifactorial, associated with mutations in genes as FOXF1 and ZIC3 and maternal risk factors. This report describes a case of VACTERL diagnosed in the Regional University Hospital of Maringá (HURM). L.P.N. female patient, 1 day in life, was referred to neonatal ICU due to respiratory discomfort, cyanosis and salivation in the delivery room, and diagnosed with esophageal atresia. On the 9th day of life surgical repair of esophageal atresia and tracheoesophageal fistula was performed. Other malformations were observed by detecting moderate perimembranous ventricular septal defect and absent right kidney, suggesting perimembranous ventricular septal defect and absent right kidney, suggesting VACTERL diagnostic. At the age of 3 years and 9 months, presents eutrophic, healthy, and in multiprofessional follow up. Despite being a rare disease, this association requires expertise of health professionals, in order to be early detected and to treat its alterations and complications. Also, if handled correctly, the child shows good performance and prospects of a normal life. The prognosis depends on the extent and severity of associated anomalies, as well as the training of parents and professional staff (AU)

Síndrome de Klippel-Feil con fistula traqueoesofagica, pulgar bifido y angiolipoma cerebral / Klippel-Feil syndrome with tracheoesophageal fistula, bifid thumb and cerebral angiolipoma

El síndrome de Klippel-Feil es una malformación congénita de la charnela cráneo-cervical compleja que involucra vértebras y visceras, caracterizada por la tríada clásica de cuello corto, limitación de movimientos de la cabeza por la fusión de vértebras cervicales e implantación baja del cabello en la región occipital. Se presenta por falla de segmentación en el esqueleto axial del embrión. Su incidencia se estima en 1/40 000-42 000nacimientos y predomina en el sexo femenino. El objetivo del presente trabajo es describir el cuadro clínico de un paciente con síndrome de Klippel-Feil y múltiples malformaciones asociadas, entre ellas, fístula traqueoesofágica, pulgar bífido y lipomas/angiolipomas intracraneales, las cuales, hasta ahora, no han sido descritas en el síndrome, por lo que se considera un hallazgo excepcional.

The Klippel-Feil syndrome is a congenital malformation of the skull flap involving complex cervical vertebrae and organs, characterized by a classic triad: short neck, limitation of movement of the head due to cervical spine fusion and low hairline in occipital region. It results from an error in the axial skeleton segmentation of the embryo; its incidence is estimated at 1/40,000-42,000 births and predominates in females. The aim of this paper is to describe the clinical picture of a patient with Klippel-Feil syndrome and multiple malformations, including tracheoesophageal fistula, bifid thumb and intracranial lipomas/angiolipomas,that have not been previously described in the syndrome, so it is considered an exceptional finding.

Anesthetic management for surgery of esophagus atresia in a newborn with Goldenhar's syndrome / Manejo anestésico para cirurgia de atresia de esôfago em um recém-nascido com síndrome de Goldenhar / Manejo anestésico para la cirugía de atresia de esófago en un neonato con síndrome de Goldenhar

Goldenhar's syndrome is a polymalformative condition consisting of a craniofacial dysostosis that determines difficult airway in up to 40% of cases. We described a case of a newborn with Goldenhar's syndrome with esophageal atresia and tracheoesophageal fistula who underwent repair surgery.

A síndrome de Goldenhar é um quadro de polimalformação que consiste em uma disostose craniofacial que determina uma via respiratória difícil em até 40% dos casos. Nós descrevemos um caso de um recém-nascido com síndrome de Goldenhar com atresia de esôfago e fístula traqueoesofágica para a qual foi feita cirurgia de reparo.

El síndrome de Goldenhar es un cuadro polimalformativo consistente en una disostosis craneofacial que condiciona una vía aérea difícil hasta en el 40% de los casos. Describimos un caso de un neonato con síndrome de Goldenhar con atresia de esófago y fístula traqueoesofágica al que se practicó cirugía de reparación de la misma.

Anesthetic management for surgery of esophagus atresia in a newborn with Goldenhar's syndrome.

BACKGROUND AND OBJECTIVES: Goldenhar's syndrome is a polymalformative condition consisting of a craniofacial dysostosis that determines difficult airway in up to 40% of cases. We described a case of a newborn with Goldenhar's syndrome with esophageal atresia and tracheoesophageal fistula who underwent repair surgery. CASE REPORT: We report the case of a 24-h-old newborn with Goldenhar's syndrome. He had esophageal atresia with distal tracheoesophageal fistula. It was decided that an emergency surgery would be performed for repairing it. It was carried out under sedation, intubation with fibrobronchoscope distal to the fistula, to limit the air flow into the esophagus, and possible abdominal distension. Following complete repair of the esophageal atresia and fistula ligation, the patient was transferred to the intensive care unit and intubated under sedation and analgesia. CONCLUSIONS: The finding of a patient with Goldenhar's syndrome and esophageal atresia assumes an exceptional situation and a challenge for anesthesiologists, since the anesthetic management depends on the patient comorbidity, the type of tracheoesophageal fistula, the usual hospital practice and the skills of the anesthesiologist in charge, with the main peculiarity being maintenance of adequate pulmonary ventilation in the presence of a communication between the airway and the esophagus. Intubation with fibrobronchoscope distal to the fistula deals with the management of a probably difficult airway and limits the passage of air to the esophagus through the fistula.

[Anesthetic management for surgery of esophagus atresia in a newborn with Goldenhar's syndrome]. / Manejo anestésico para cirurgia de atresia de esôfago em um recém-nascido com síndrome de Goldenhar.

BACKGROUND AND OBJECTIVES: Goldenhar's syndrome is a polymalformative condition consisting of a craniofacial dysostosis that determines difficult airway in up to 40% of cases. We described a case of a newborn with Goldenhar's syndrome with esophageal atresia and tracheoesophageal fistula who underwent repair surgery. CASE REPORT: We report the case of a 24-hour-old newborn with Goldenhar's syndrome. He had esophageal atresia with distal tracheoesophageal fistula. It was decided that an emergency surgery would be performed for repairing it. It was carried out under sedation, intubation with fibrobronchoscope distal to the fistula, to limit the air flow into the esophagus, and possible abdominal distension. Following complete repair of the esophageal atresia and fistula ligation, the patient was transferred to the intensive care unit and intubated under sedation and analgesia. CONCLUSIONS: The finding of a patient with Goldenhar's syndrome and esophageal atresia assumes an exceptional situation and a challenge for anesthesiologists, since the anesthetic management depends on the patient comorbidity, the type of tracheoesophageal fistula, the usual hospital practice and the skills of the anesthesiologist in charge, with the main peculiarity being maintenance of adequate pulmonary ventilation in the presence of a communication between the airway and the esophagus. Intubation with fibrobronchoscope distal to the fistula deals with the management of a probably difficult airway and limits the passage of air to the esophagus through the fistula.

Útero doble asociado a atresia esofágica con fístula traqueoesofágica / Double uterus associated with esophageal atresia with tracheoesophageal fistula

Se reporta una asociación de defectos congénitos no descritos con anterioridad en la literatura, dados por útero doble y atresia esofágica con fístula traqueoesofágica e historia obstétrica de dos embarazos, un aborto espontáneo y gestación en curso. El estudio cromosómico en sangre periférica materna y en líquido amniótico informa cariotipos normales. A las 22 semanas aparece ventriculomegalia fetal severa, que se confirma en biopsia fetal. La información disponible no es suficiente para determinar las bases genéticas de la asociación: útero doble atresia esofágica fístula traqueoesofágica (AU)

It is reported an association of congenital defects not previously described in the literature, given by double uterus and esophageal atresia with tracheoesophageal fistula and obstetric history of two pregnancies, spontaneous abortion and ongoing pregnancy. The chromosomal study in maternal peripheral blood and amniotic fluid reports normal karyotypes. At 22 weeks appears severe fetal ventriculomegaly, which is confirmed in fetal biopsy. The information available is not sufficient to determine the genetic basis of the association: double uterus - esophageal atresia - tracheoesophageal fistula (AU)

Agenesia Traqueal. Anomalía congénita rara con alta mortalidad / Tracheal Agenesis. a Rare Congenital Anomaly with High-Mortality

Introducción: La agenesia traqueal es una malformación congénita muy rara, incompatible con la vida, el defecto consiste en una ausencia parcial o total de la tráquea debajo de la laringe permitiendo que el tracto respiratorio inferior se conecte con el tracto gastrointestinal por medio de una fístula traqueó-esofágica distal. Caso Clínico: Se presenta el caso clínico de un recién nacido, con antecedente materno de polihidramnios, nace con dificultad respiratoria, ausencia de llanto y dificultad para la intubación orotraqueal, Apgar 4/8, pasa a Unidad de Cuidados Intensivo neonatales (UCIN) y es conectado a Asistencia Respiratoria Mecánica (ARM) a las 6hs de vida se extuba en forma accidental, con imposibilidad para la reintubación, se realizan Tomografía de cuello y tórax donde se constata agenesia traqueal con fistula traqueoesofágica distal, sin otras malformaciones congénitas asociadas, se realizan varias cirugías paliativas. A pesar de todos los esfuerzos médicos, el paciente fallece a los 6 días de vida, ante colapso de vías aéreas. Discusión: Se presenta este caso por ser la agenesia traqueal una malformación extremadamente rara, y con muy pocos casos descriptos en la literatura. Es el primer caso en nuestro hospital, se presenta como un caso clínico complejo, de manejo multidisciplinario, sometido a varias cirugías paliativas, el paciente fallece, la evolución es generalmente fatal y de mal pronóstico debido a que aún no se han desarrollado técnicas adecuadas de reemplazo traqueal .

Introduction: Tracheal a genesis is a very rare congenital malformation that is incompatible with life.The anomaly consists of the partial or total absence of the trache a below the larynx that allows connection of the lower respiratory...

Reparación toracoscópica de atresia esofágica con y sin fistula traqueoesofágica / Thoracoscopic repair of esophageal atresia with and without tracheoesophageal fistula

Introduction: Esophageal atresia (EA) is the interruption of the continuity of the esophagus, with or without persistent communication with the trachea. Recent advances in surgical techniques have made possible correction with minimally invasive surgery (MIS). Objective: To evaluate the management of thoracoscopic technique in the treatment of EA. Patients and Method: Retrospective analysis of medical records in two centers was carried out between 2007 and 2012. Variables such as gestational age, gender, weight, type of esophageal atresia, malformations, surgery and postoperative complications were recorded. Results: Twenty patients, 15 of them with type III EA, 4 with type I and one unclassifiable, were part of the study. 13 patients underwent ligation, cut of tracheoesophageal fistula and end to end anastomosis. Two underwent laparoscopic gastrostomy and fistula ligation. One patient required conversion and underwent esophagostomy plus gastrostomy. 4 patients without fistula required laparoscopic gastrostomy and aspiration of the proximal esophagus to be able to perform esophageal anastomosis. In the postoperative period, pleural drainage and a transanastomotic feeding tube were installed. Seven patients required esophageal dilations and 4 patients developed stenosis due to reflux. Two had recurrent fistula, one with spontaneous resolution and another with endoscopic resolution. Three children died: two of them due to pathologies not related to surgery and one as a result of limitation of therapeutic effort after fistula patency. Discussion: Esophageal atresia repair with MIS is a safe and effective option with excellent exposure and visualization of anatomic landmarks and little associated morbidity.

Introducción: Atresia esofágica (AE) es la interrupción de la continuidad del esófago, con o sin comunicación con la tráquea. Los avances en cirugía han permitido su corrección con cirugía mínimamente invasiva (CMI). Objetivo: Evaluar el manejo toracoscópico en AE. Pacientes y Método: Revisión retrospectiva de fichas de 2 centros entre 2007 y 2012. Se registraron variables como edad gestacional, sexo, peso, tipo de atresia esofágica, malformaciones, cirugías y complicaciones postquirúrgicas. Resultados: Veinte pacientes, 15 tipo III, 4 tipo I y uno no clasificable. En 13 pacientes se realizó ligadura, sección de fístula tráqueo-esofágica y anastomosis término-terminal. Dos fueron sometidos a gastrostomía laparoscópica más ligadura de fístula. Un paciente requirió conversión y se realizó esofagostomía más gastrostomía. En 4 pacientes sin fístula, se realizó gastrostomía laparoscópica y aspiración del esófago proximal hasta lograr realizar la anastomosis esofágica. En el post-operatorio se instaló drenaje pleural y sonda trans-anastomótica. Siete pacientes requirieron dilataciones esofágicas y 4 desarrollaron estenosis por reflujo. Dos presentaron fístula recidivada, uno con resolución espontánea y el segundo de resolución endoscópica. Tres niños fallecieron: dos por patologías no relacionadas con la cirugía y uno con limitación de esfuerzo terapéutico con repermeabilización de fístula. Discusión: La atresia esofágica reparada con CMI es una opción segura y efectiva en esta serie, permitiendo excelente exposición y visualización de reparos anatómicos, con poca morbilidad asociada.

A new proposal for evaluation of the pharyngoesophageal segment and its relation with the narrow-band spectrogram in tracheoesophageal speakers / Uma nova proposta de avaliacao do segmento faringoesofagico e sua relacao com a espectrografia acustica na voz traqueoesofagica

PURPOSE: The primary purpose of this study was to assess the relationship between pharyngoesophageal segment (PES) configuration and narrow-band spectrogram of tracheoesophageal voices. METHODS: This study included 30 total laryngectomees tracheoesophageal speakers. Patients were assessed by videofluoroscopy (VF), during deglutition and voicing, and the vowel /a/ was recorded for spectrographic analysis. The evaluation of VF recording consisted of visual perceptual rating of degree of contact between the prominence of PES and its anterior wall, defined as absent/mild (hypo contact), moderate (normo contact) and intense (hyper contact); and quantitative measures of PES: anteroposterior distance (APD) and length of the PES (lenPES); PES surface area in swallowing (areaSw), and phonation (areaPh), and the area of the air reservoir (areaAir). Visual inspection of a narrow-band spectrogram was made and four different acoustic signal typing were defined as Type I, II, III or IV. RESULTS: Type I-II is correlated with moderate contact; Type III, with intense and Type IV, with absent/mild contact. Type I-II has bigger APD and PES with lower length than Type IV. There is a correlation between bigger APD and shorter PES. CONCLUSION: The group with I-II signal typing has PES with normo contact; Type III with hyper contact and Type IV has PES with hypo contact. The best tracheoesophageal voices are achieved by PES with moderate contact of the prominence and with shorter and larger anteroposterior PES distances. What differentiates the PES with hyper contact from PES with normal one is only the degree of contact between the prominence of the PES and its anterior wall. .

OBJETIVO: Avaliar a relação entre a configuração do segmento faringoesofágico (SFE) e a espectrografia acústica nas vozes traqueoesofágicas (TE). Métodos: Participaram 30 sujeitos laringectomizados totais com fonação TE, que se submeteram à gravação da vogal /a/, para análise espectrográfica acústica, e ao exame videofluoroscópico (VF), durante a deglutição e a fonação. A análise espectrográfica foi feita pela avaliação visual do tipo do traçado e classificado como Tipo I, II, III ou IV. No exame, foi avaliado o grau de contato da proeminência do SFE com sua parede anterior, definido como ausente/leve (hipocontato), moderado (contato normal) e intenso (hipercontato); e mensurados: distância anteroposterior (DAP), comprimento do SFE (cSFE); área do reservatório de ar (área AR); área do SFE na deglutição (área DE) e na fonação (área FO). RESULTADOS: O tipo de sinal espectrográfico I-II caracteriza-se por contato normal; Tipo III, hipercontato; e Tipo IV, hipocontato. O grupo com sinal Tipo I-II apresenta DAP maior e cSFE menor do que o Tipo IV. Há correlação entre DAP maior e cSFE menor. CONCLUSÃO: Os sinais espectrográficos Tipo I e II caracterizam o SFE com contato normal; Tipo III, com hipercontato e Tipo IV, com hipocontato. SFE com contato normal, curtos e com DAP maior caracterizam as melhores vozes TE. Somente o grau de contato da proeminência do SFE com sua parede anterior diferencia SFE com contato normal de segmentos com hipercontato. .