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Abstract We present a 9-year-old patient with end-stage renal disease, on peritoneal dialysis, who underwent a staged prone retroperitoneoscopic bilateral nephrectomy. Bilateral nephrectomy was indicated in preparation for renal transplant in the context of genetic predisposition malignancy when immunosuppressed. The two mirror-image surgeries enable the comparison of the anesthetic management and outcomes in a single patient. Features of interest to anesthesiologists include approach to a child with chronic kidney disease, different requirements for intraoperative antihypertensives; pain management strategies, including a comparison of erector spinae plane block with and without adjunct dexmedetomidine; anesthetic management of retroperitoneoscopic pediatric surgery and the first description of using a Foley bag attached to a peritoneal dialysis catheter to aid in diagnosis and repair of posterior peritoneal cavity entry.

Resumen Se presenta un paciente de 9 años de edad con enfermedad renal terminal, en diálisis peritoneal, quien se sometió a nefrectomía bilateral retroperitoneoscópica estadificada en posición prona. Se indicó la nefrectomía bilateral en preparación para trasplante renal en el contexto de predisposición genética hacia desarrollar una patología maligna al estar inmunosuprimido. Las dos cirugías en espejo permiten hacer una comparación del manejo anestésico y de los desenlaces en un mismo paciente. Las características de interés para los anestesiólogos incluyen el abordaje de un niño con enfermedad renal crónica, con requisitos diferentes de antihipertensivos intraoperatorios; estrategias para el manejo del dolor, incluyendo una comparación de bloqueo del plano del erector espinal con y sin dexmedetomidina adyuvante; manejo anestésico de cirugía pediátrica retroperitoneoscópica y la primera descripción del uso de una bolsa Foley conectada a un catéter de diálisis peritoneal para ayudar en el diagnóstico y la reparación de la entrada de la cavidad peritoneal posterior.

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Safe mechanical ventilation in pediatric anesthesia includes the use of protective ventilatory strategies. In anesthesia, the evidence-based literature is scarce and derives from intensive care and adult patients. New technologies, monitoring and knowledge of applied pathophysiology allow these data to be extrapolated. The technological advance in ventilators of the new anesthesia machines has allowed its use in smaller patients with greater safety, deploying more ventilatory modes for use in the operating room. The programming of the ventilator must be done looking for physiological objectives according to the stage of the child's development, step of anesthesia and surgery, in a dynamic and individualized way. This narrative review aims to summarize the available evidence about intraoperative pediatric mechanical ventilation and provide practical clinical recommendations aimed at optimizing the performance of the anesthesia machine, applying safe ventilatory strategies in pediatric patients.

Una ventilación mecánica segura en anestesia pediátrica incluye el uso de estrategias ventilatorias protectoras. En anestesia la literatura basada en la evidencia al respecto es escasa, deriva del intensivo y del paciente adulto. Las nuevas tecnologías, moni- torización y el conocimiento de la fisiopatología aplicada, permiten extrapolar estos datos. El desarrollo del avance tecnológico de los ventiladores de las nuevas máquinas de anestesia, ha permitido su uso en pacientes cada vez más pequeños y con mayor seguridad, desplegando más modos ventilatorios para uso en pabellón. La programación del ventilador debe ser buscando objetivos fisiológicos según la etapa del desarrollo del niño, la etapa del proceso anestésico y la cirugía, de manera dinámica e individualizada. La presente revisión narrativa pretende resumir la evidencia disponible sobre ventilación mecánica pediátrica intraoperatoria y entregar recomendaciones clínicas prácticas orientadas a optimizar las prestaciones de la máquina de anestesia, aplicando estrategias ventilatorias seguras en el paciente pediátrico.

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Children have the right to enjoy the highest attainable standard of health. This basic human right is anchored in the "United Nations Convention on the Rights of the Child (UNCRC)" and was adopted in 1989. Pediatric anesthesia is a high-risk specialty with anesthesia-related complications approximately 10 times more frequent when compared with adults resulting higher mor- bidity and mortality. Children are different from adults from an anatomical, physiological, pharmacological, psychological point and safe conduct of pediatric anesthesia requires profound knowledge, experience, manual skills and professional attitude of the practitioner. This also necessitates institutional support in a child appropriate clinical environment. Unfortunately, we are still far away from fulfilling this commitment in anaesthesia and continué to be hampered by the lack of human and physical resources, organization, and training. Safe Anesthesia for Every Tot (safetots.org) is an initiative of leading international pediatric anesthe- tists that advocates safe and quality anesthesia for all children. It is based on the rights of the children ('10R'), the provision of perioperative physiological homeostasis ('10N'), defines the '5W' (Who, Where, What, When and HoW), critical events '10C', provides a roadmap for education, teaching and training and identifies specific areas of critical importance for safe perioperative care. This review provides an overview of the perioperative safety in pediatric anesthesia and explores strategies to minimize risks.

Los niños tienen derecho a disfrutar del más alto estándar de salud posible. Este derecho humano básico está fundamentado en la "Convención de las Naciones Unidas sobre los Derechos del Niño (UNCRC)" y fue adoptado en 1989. La anestesia pediátrica es una especialidad de alto riesgo; con complicaciones relacionadas con la anestesia aproximadamente 10 veces más frecuentes cuando es comparado con los adultos llevando a mayor morbilidad y mortalidad. Los niños son diferentes de los adultos desde el punto de vista anatómico, fisiológico, farmacológico, psicológico y la conducción segura de la anestesia pediátrica requiere un conocimiento profundo, experiencia, habilidades manuales y actitud profesional del médico. Esto también requiere apoyo institucional en un entorno clínico apropiado para niños. Desafortunadamente, todavía estamos lejos de cumplir con este compromiso en anestesia y sigue siendo obstaculizado por la falta de recursos humanos, físicos, organizacionales y de educación y entrenamiento. Safe Anesthesia for Every Tot (safetots.org) es una iniciativa de anestesiólogos pediátricos internacionales que promueven una anestesia segura y de calidad para todos los niños. Se basa en los derechos de los niños ('10R'), el aseguramiento de la homeostasis fisiológica perioperatoria ('10N'), define las '5W' (Quién, Dónde, Qué, Cuándo y Cómo), eventos críticos '10C', proporciona una hoja de ruta para la educación, la enseñanza y la formación e identifica áreas específicas de importancia crítica para la atención perioperatoria segura. Esta revisión proporciona una descripción general de la seguridad perioperatoria en anestesia pediátrica y explora estrategias para minimizar los riesgos.

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While Non Operating Room Anesthesia (NORA) and Pediatric Ambulatory Surgery (CMA) are increasing not only in numbers but also in new different procedures, we need to know how to optimize safety so we can have the same quality standards needed in theaters. We need to get involved in patient and procedure selection as well as risk assessment. In CMA such selection seeks for healthy or low risk patients, in NORA the assessment makes the provider look for the best conditions minimizing risks. To be successful in both programs we need to work with high standards and safety while Teamwork is necessary.

Todo indica que tanto los procedimientos Cirugía Mayor Ambulatoria (CMA) como Anestesia fuera de Pabellón (NORA) seguirán aumentando, no solo en número sino también en tipo de procedimientos a realizar. Involucrarnos en los procesos previos de selección de pacientes, evaluación preanestésica y estratificación de riesgo manteniendo la misma rigurosidad con que se realiza para los niños que van a pabellón es parte de nuestra tarea. Si en CMA, la selección busca incluir pacientes de bajo riesgo, en NORA la evaluación busca la mejor adaptación para realizar el procedimiento de un paciente complejo minimizando sus riesgos. Calidad y seguridad en estos programas son fundamentales para su éxito, así como el trabajo en equipo.

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Perioperative cardiopulmonary arrest (CRP) in pediatric anesthesia is a rare but catastrophic event. Its incidence has decreased over time to advance in peri-anesthetic care for patients, as well as the availability of better equipment. It is estimated that in developed countries the incidence can reach 3-5/1,000 anesthesia when patients undergoing cardiac surgery are included. The risk factors remain similar in different studies, among them the most relevant risk factors are age less than 1 year old, ASA status III or more, urgent or emergency surgeries, type of surgery, anesthesia load and time of the day of occurrence. The respiratory and cardiovascular are most relevant immediate causes. The neurological prognosis and survival rate are superior to those of cardiopulmonary arrest in children in other settings. The key factor in the successful management of perioperative cardiopulmonary arrest in pediatric patients, is the adherence to the cardiopulmonary resuscitation protocols.

El paro cardiorrespiratorio (PCR) perioperatorio en anestesia pediátrica es un evento raro, pero catastrófico. Su incidencia ha ido disminuyendo con el tiempo gracias a los avances en los cuidados perianestesicos de los pacientes, así como a la disponibilidad de mejor equipamiento. Se estima que en países desarrollados la incidencia puede alcanzar a 3-5/1000 anestesias cuando se incluyen los pacientes sometidos a cirugía cardiaca. Los factores de riesgo se mantienen similares en los distintos estudios, entre ellos los más relevantes son la edad menor de un año, el ASA elevado (III o más), cirugía de urgencia o emergencia, el tipo de cirugía, la carga de anestesias y el horario de ocurrencia. Las causas inmediatas más importantes son las respiratorias y las cardiovasculares. El pronóstico neurológico y la tasa de sobrevida son mejores que los de un paro cardiaco en niños en otros escenarios. La adherencia a protocolos de resucitación cardiopulmonar son claves en el manejo exitoso de paro cardiorrespiratorio perioperatorio en pediatría.

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Fluid therapy is the most widely used medical treatment, with indications and risks that are important to know. Its indication in the pediatric perioperative period has continuously changed with the upward understanding of the physiology of newborns, infants and children, as well as in the knowledge of the composition of the different fluids available for use in this patient's population. This is evident in the various existing guidelines on this topic. It is important to consider the preoperative fasting of our patient, which must be decreased to the maximum. We must differentiate the two objectives of fluid therapy: maintenance and replacement fluid therapy. The latest recommendations on maintenance fluid therapy indicate preferring the use of balanced solutions, and if not having these, preferring lactate ringer serum by adding glucose for a concentration of 1-2.5%, especially in patients with a higher risk of hypoglycemia. On the other hand, in replacement fluid therapy, it should always be started with crystalloids preferring isotonic balanced solutions of electrolytes. The use of colloids is controversial given the scarce scientific evidence in pediatric patients. Finally, when indicating blood products, the risks associated with transfusion should always be considered, however, we should also consider the threshold of hematocrit and platelets to transfuse if necessary, and calculate the maximum volume of blood loss allowed to be transfused.

La fluidoterapia es el tratamiento médico más ampliamente utilizado, con indicaciones y riesgos que es importante conocer. Su indicación en el perioperatorio pediátrico ha cambiado continuamente a medida que se avanza en la comprensión de la fisiología de los recién nacidos, lactantes y niños, como también en el conocimiento de la composición de los diferentes fluidos disponibles para su uso en esta población de pacientes. Esto queda en evidencia en las diversas guías existentes respecto al tema. Es importante considerar el ayuno preoperatorio de nuestro paciente, que debe ser disminuido al máximo. Debemos diferenciar los dos objetivos de la fluidoterapia: fluidoterapia de mantención y de reposición. Las últimas recomendaciones sobre fluidoterapia de mantención indican preferir el uso de soluciones balanceadas, y de no disponer de éstas, preferir suero ringer lactato añadiendo glucosa para una concentración de 1%-2,5%, especialmente en pacientes con mayor riesgo de hipoglicemia. Por otro lado, en la fluidoterapia de reposición, se debe iniciar siempre con cristaloides prefiriendo soluciones balanceadas isotónicas de electrolitos. El uso de coloides es controversial dado la escasa evidencia científica en pacientes pediátricos. Por último, al indicar hemoderivados, siempre se deben considerar los riesgos asociados a transfusión, sin embargo, también debemos considerar el umbral de hematocrito y plaquetas para transfundir en caso necesario, y calcular el volumen de pérdida sanguínea máxima permitida para transfundir.

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Abstract Perioperative morbidity and mortality are high among patients in the extremes of life undergoing anesthesia. Complications in children occur mainly as a result of airway management-related events such as difficult approach, laryngospasm, bronchospasm and severe hypoxemia, which may result in cardiac arrest, neurological deficit or death. Reports and new considerations that have changed clinical practice in pediatric airway management have emerged in recent years. This narrative literature review seeks to summarize and detail the findings on the primary cause of morbidity and mortality in pediatric anesthesia and to highlight those things that anesthetists need to be aware of, according to the scientific reports that have been changing practice in pediatric anesthesia. This review focuses on the identification of "new" and specific practices that have emerged over the past 10 years and have helped reduce complications associated with pediatric airway management. At least 9 practices grouped into 4 groups are described: assessment, approach techniques, devices, and algorithms. The same devices used in adults are essentially all available for the management of the pediatric airway, and anesthesia-related morbidity and mortality can be reduced through improved quality of care in pediatrics.

Resumen Los pacientes en extremos de la vida sometidos a anestesia tienen la más alta morbimortalidad perioperatoria. Los niños se complican principalmente por eventos derivados del manejo de la vía aérea pediátrica (VAP), como dificultad en su abordaje, laringoespasmo, broncoespasmo e hipoxemia severa, que pueden terminar en paro cardiaco, déficit neurológico o muerte. En los últimos años se han informado y retomado aspectos que cambian la práctica clínica sobre la VAP Esta revisión narrativa de la literatura busca concretar y resumir estos hallazgos sobre la primera causa de morbimortalidad en anestesia pediátrica y enfatizar en lo que los anestesiólogos deben conocer, con base en los informes científicos que vienen cambiando la práctica anestésica pediátrica. Esta revisión busca identificar las conductas "nuevas" y concretas que han surgido en los últimos 10 años, y que ayudan a disminuir las complicaciones derivadas del manejo de la VAP Se señalan y describen al menos nueve conductas agrupadas en 4 bloques: Evaluación, técnicas de abordaje, dispositivos y algoritmos. Actualmente se cuenta con prácticamente todos los dispositivos de adultos para el manejo de la VAP y con consideraciones específicas se puede mejorar la calidad de la atención y reducir la morbimortalidad anestésica en pediatría.

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Crisponi syndrome is a rare and severe heritable disorder characterised by muscle contractions, trismus, apnea, feeding troubles, and unexplained high fever spikes with multiple organ failure. Here we report perioperative care for endoscopic gastrostomy of a 17 month-old female child with Crisponi syndrome. Temperature in the surgery room was strictly monitored and maintained at 19°C. The patient was exposed to both inhaled and intravenous anesthetic agents. Surgical and perioperative periods were uneventful. Episodes of fever in Crisponi syndrome arise from CRLF1 mutation, which differs from the physiological pathway underlying malignant hyperthermia.

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Abstract Crisponi syndrome is a rare and severe heritable disorder characterised by muscle contractions, trismus, apnea, feeding troubles, and unexplained high fever spikes with multiple organ failure. Here we report perioperative care for endoscopic gastrostomy of a 17 month-old female child with Crisponi syndrome. Temperature in the surgery room was strictly monitored and maintained at 19ºC. The patient was exposed to both inhaled and intravenous anesthetic agents. Surgical and perioperative periods were uneventful. Episodes of fever in Crisponi syndrome arise from CRLF1 mutation, which differs from the physiological pathway underlying malignant hyperthermia.

Resumo A Síndrome de Crisponi é uma condição clínica hereditária grave e rara caracterizada por contrações musculares, trismo, apneia, distúrbios na alimentação, picos de febre alta e inexplicável, e falência de múltiplos órgãos. Descrevemos o cuidado perioperatório de paciente pediátrica com 17 meses de idade, portadora da Síndrome de Crisponi, submetida a gastrostomia endoscópica. A temperatura da sala de cirurgia foi cuidadosamente monitorizada e mantida a 19ºC. A paciente foi submetida a agentes anestésicos inalatórios e venosos. O cuidado cirúrgico e perioperatório desenvolveram-se sem incidentes. As crises de febre na Síndrome de Crisponi originam-se de mutação no gene CRLF1, o que as diferenciam do mecanismo fisiopatológico da hipertermia maligna.

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RESUMEN Introducción: las máscaras laríngeas son dispositivos supraglóticos utilizadas ampliamente en anestesia para el abordaje y mantenimiento de la vía respiratoria. Una de ellas, la Ambu® Aura 40TM, tiene un diseño que le permite ajustarse al entorno de la hipofaringe con su lumen dirigido a la apertura laríngea. Ha demostrado ser adecuada para procedimientos quirúrgicos donde no es necesaria la intubación endotraqueal. Objetivo: determinar la efectividad dicha máscara laríngea para anestesia general en cirugía ortopédica pediátrica. Materiales y métodos: se realizó un estudio descriptivo, prospectivo, transversal, en 135 pacientes intervenidos quirúrgicamente de forma electiva en el Hospital Pediátrico Eliseo Noel Caamaño, de Matanzas, en el periodo enero de 2015- junio 2017. Se tuvo en cuenta el número de intentos y el tiempo para insertar la máscara laríngea, la necesidad de reemplazarla por un tubo endotraqueal y las complicaciones relacionadas con su uso. Resultados: la ML Ambu® Aura 40TM fue efectiva en la mayoría de los pacientes ya que permitió realizar la cirugía sin necesidad de sustituirla por el tubo endotraqueal y la aparición de complicaciones relacionada con su uso fue baja. Conclusiones: la ML Ambu® Aura 40TM fue efectiva ya que en la mayoría de los pacientes fue insertada correctamente en el primer intento y un tiempo inferior a los 20 segundos. Solo una minoría necesitó que fuera reemplazada por el tubo endotraqueal, y la aparición de complicaciones relacionada con su uso fue baja (AU).

ABSTRACT Introduction: laryngeal mask are supra glottal devices widely used in anesthetics for approaching and maintaining the airway. One of them, the Ambu® Aura 40TM, has a design allowing to adjust to the surroundings of hypo-pharynx with its lumen directed to the pharyngeal opening. It has demonstrated to be adequate for surgical procedures when there is no need of endotracheal intubation. Objective: to determine the effectiveness of that laryngeal mask for general anesthetic in pediatric orthopedic surgery. Materials and methods: a cross-sectional, prospective, descriptive study was carried out in 135 patients who underwent elective surgical intervention in the Pediatric Hospital ?Eliseo Noel Caamaño?, of Matanzas, in the period January 2015- June 2017. The number of attempts and the time needed to insert laryngeal mask, the necessity of changing it for an endotracheal tube, and complications related to its usage were taking into account. Results: Ambu® Aura 40TM laryngeal mask was effective in most patients, due to it allowed to perform the surgery without need of changing it for an endotracheal tube, and the low appearance of complications related to its use. Conclusions: Ambu® Aura 40TM laryngeal mask was effective because it was inserted correctly at the first attempt, in a time less than 20 seconds. Only a minority needed to replace it by endotracheal tube, and the appearance of complications related to its use were low (AU).

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Abstract Background and objectives: Conjoined twins are monozygotic twins physically joined at some part of the body. This is a rare phenomenon, estimated between 1:50,000 and 1:200,000 births. The objective of this report is to present the anesthetic management and the perioperative challenges for a separation surgery. Case report: Thoraco-omphalopagus twins were diagnosed by ultrasound and were followed by the fetal medicine team of the service. After 11 h of cesarean surgery, the pediatric surgical team chose to separate the twins. They were monitored with cardioscopy, oximetry, capnography, nasopharyngeal thermometer, urinary output, and non-invasive blood pressure. We chose inhaled induction with oxygen and 4% Sevoflurane. T1 patient was intubated with a 3.5 uncuffed endotracheal tube, and, after three unsuccessful intubation attempts of patient T2, a number 1 laryngeal mask was used. After securing the twins' airway, the induction was supplemented with fentanyl, propofol, and rocuronium. Mechanical ventilation in controlled pressure mode (6 mL.kg−1) and lumbar epidural (L1-L2) with 0.2% ropivacaine (2.5 mg.kg−1) were used. The pediatric surgical team initiated the separation of the twins via sternotomy, ligation of hepatic vessels. After 2 hours of procedure, the separation was completed, continuing the surgical treatment of T1 and the support of T2 until his death. Conclusions: Conjoined twin separation surgery is a challenge, which requires planning and coordination of a multidisciplinary team during all stages.

Resumo Justificativa e objetivos: Gêmeos conjugados são gêmeos monozigóticos conectados por alguma parte do corpo. Esse é um fenômeno raro, estimado entre 1:50.000 a 1:200.000 nascimentos. O objetivo deste relato é apresentar o manejo anestésico e os desafios perioperatórios para cirurgia de separação. Relato de caso: Gêmeos toraco-onfalópagos foram diagnosticados por ultrassonografia e acompanhados pela equipe de medicina fetal do serviço. Após 11 horas da cesárea, a equipe cirúrgica pediátrica optou pela separação dos gêmeos. Foram monitorados com cardioscopia, oximetria, capnografia, termômetro nasofaríngeo, débito urinário e pressão arterial não invasiva. Optou-se por indução inalatória com oxigênio e sevoflurano a 4%. O G1 foi intubado com tubo orotraqueal 3,5 sem cuff e após três tentativas de intubação do G2 sem sucesso usou-se máscara laríngea número 1. Após obtenção da via aérea nos gêmeos, complementou-se indução com fentanil, propofol e rocurônio. Ventilação mecânica no modo pressão controlada 6 ml.kg-1 e peridural lombar L1-L2 com ropivacaína 0,2% (2,5 mg.kg-1). A equipe cirúrgica pediátrica iniciou a separação dos gêmeos através de esternotomia, ligadura de vasos hepáticos. Após duas horas de procedimento, a separação foi concluída, prosseguiram-se o tratamento cirúrgico de G1 e os cuidados de G2 até o óbito. Conclusões: A cirurgia de separação de gêmeos conjugados é um desafio, requer planejamento e coordenação de uma equipe multidisciplinar durante todos os estágios.

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RESUMEN Introducción: la agitación durante la emergencia de la anestesia general es una complicación frecuente en pediatría que puede causar daños físicos, retrasar el alta y aumentar los costos. Objetivo: caracterizar los episodios anestesia general en el paciente pediátrico. Materiales y métodos: se realizó un estudio descriptivo, prospectivo, longitudinal de 246 pacientes que presentaron anestesia general en el Hospital "Eliseo Noel Caamaño" entre septiembre de 2015 y diciembre de 2018. Se estudiaron las variables: edad, género, ASA, tiempo quirúrgico y tiempo anestésico, tipo de cirugía, método de anestesia, agentes usados para la inducción y el mantenimiento, severidad de los episodios y necesidad de tratamiento. Resultados: la mayoría de los pacientes que presentaron anestesia general tenían entre 2 y 6 años (63,4%), eran masculinos (67,9%), ASA I (78,1%) y fueron operados de excéresis de lesiones de partes blandas (27,6%). El tiempo quirúrgico fue de 31,2 ± 10,4 minutos y el anestésico 43,5±8,8 minutos. El método anestésico más indicado fue la anestesia balanceada (84,2%), como inductor el propofol (86,2%) y para el mantenimiento isoflurano (34,1%) y sevoflurano (26,4%). Predominaron los episodios severos (51,2%) y el 56,9% necesitó intervención farmacológica. Conclusiones: esta anestesia fue más frecuente en los menores de seis años, masculinos, sanos, a los cuales se les realizaron procederes cortos, con anestesia balanceada, se usó propofol para la inducción e isoflurano y sevoflurano para el mantenimiento de la anestesia. Prevalecieron los episodios severos y la mayoría requirió tratamiento farmacológico.

ABSTRACT Introduction: agitation during the emergence from general anesthesia is a frequent complication in Pediatrics that can cause physical damages, delay discharge and increase costs. Objective: to characterize the episodes of general anesthesia in the pediatric patient. Materials and methods: a descriptive, prospective, longitudinal study was carried out with 246 patients who presented general anesthesia in the Hospital "Eliseo Noel Caamaño" from September 2015 to December 2018. The studied variables were age, gender, ASA, surgical time, anesthetic time, kind of surgery, anesthetic method, agents used for the induction and maintenance, episodes severity and treatment necessity. Results: most of patients treated with general anesthesia were aged 2-6 years (63.4 %), male (67.9 %), ASA I (78.1 %), and underwent the removal of soft parts lesions (27.6 %). The average surgical time was 31,2 ± 10,4 minutes and the anesthetics one was 43,5±8,8 minutes. The most used anesthetics method was balanced anesthesia (84.2 %), the most used inductor was propofol (86.2 %) and for the maintenance isoflurane (34,1%) and sevoflurane (26,4%). Severe episodes (51,2%) predominated, and 56.9 % needed pharmacologic intervention. Conclusions: this kind of anesthesia is more frequently used in children aged less than 6 years, male, healthy, who underwent short procedures with balanced anesthesia and the use of propofol for the induction and isoflurane and sevoflurane for maintaining it. Severe episodes predominated, and most of them required pharmacologic treatment.

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BACKGROUND AND OBJECTIVES: Conjoined twins are monozygotic twins physically joined at some part of the body. This is a rare phenomenon, estimated between 1:50,000 to 1:200,000 births. The objective of this report is to present the anesthetic management and the perioperative challenges for a separation surgery. CASE REPORT: Thoraco-omphalopagus twins were diagnosed by ultrasound and were followed by the fetal medicine team of the service. After 11hours of cesarean surgery, the pediatric surgical team chose to separate the twins. They were monitored with cardioscopy, oximetry, capnography, nasopharyngeal thermometer, urinary output, and noninvasive blood pressure. We chose inhaled induction with oxygen and 4% Sevoflurane. T1 patient was intubated with a 3.5 uncuffed endotracheal tube, and, after three unsuccessful intubation attempts of patient T2, a number 1 laryngeal mask was used. After securing the twins' airway, the induction was supplemented with fentanyl, propofol, and rocuronium. Mechanical ventilation in controlled pressure mode (6mL.kg-1) and lumbar epidural (L1-L2) with 0.2% ropivacaine (2.5mg.kg-1) were used. The pediatric surgical team initiated the separation of the twins via sternotomy, ligation of hepatic vessels. After 2hours of procedure, the separation was completed, continuing the surgical treatment of T1 and the support of T2 until his death. CONCLUSIONS: Conjoined twin separation surgery is a challenge, which requires planning and coordination of a multidisciplinary team during all stages.

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Thoracotomy is associated with intense pain. In the pediatric population, the pain affects the ventilatory mechanics, which is also strongly influenced by the characteristics of the respiratory tract and chest according to the age. Therefore, regional techniques are strongly recommended. In 2016, ESP is described for the first time, which generates extensive sensory block in the chest wall, without approaching the pleura and the neuroaxial space from the technical point of view. At present, there is increasing experience in different surgical settings, but it is still scarce in pediatric patients. We present the successful application of the technique in 2 pediatric cases of thoracic surgery and various outcomes are described.

La toracotomía está asociada con intenso dolor. En la población pediátrica, el dolor afecta la mecánica ventilatoria, que además se ve fuertemente influida por las características propias de las vías respiratorias y del tórax según la edad. Por lo anterior, las técnicas regionales están fuertemente recomendadas. En 2016 se describe por primera vez el ESP, que genera bloqueo sensitivo extenso en la pared torácica, sin aproximarse desde el punto de vista técnico a la pleura y al espacio neuroaxial. En la actualidad, existe experiencia en aumento en diversos settings quirúrgicos, pero es aún escasa en pacientes pediátricos. Presentamos la aplicación exitosa de la técnica en 2 casos pediátricos de cirugía torácica, así como la descripción de diferentes resultados posoperatorios.

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Abstract Patients with Patau's syndrome (Trisomy 13) have multiple craniofacial, cardiac, neurological and renal anomalies with very less life expectancy. Among craniofacial anomalies cleft lip and palate are common. These craniofacial and cardiac anomalies present difficulties with anesthesia. We therefore describe the anesthetic management in the case of a Trisomy 13 child for operated for cleft lip at 10 months of age.

Resumo Os pacientes com síndrome de Patau (trissomia 13) apresentam várias anomalias craniofaciais, cardíacas, neurológicas e renais, com expectativa de vida bem menor. Entre as anomalias craniofaciais, o lábio leporino e a fenda palatina são comuns. Essas anomalias craniofaciais e cardíacas apresentam dificuldades na anestesia. Portanto, descrevemos o manejo anestésico em uma criança de 10 meses com trissomia 13 submetida à cirurgia de lábio leporino.

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Patients with Patau's syndrome (Trisomy 13) have multiple craniofacial, cardiac, neurological and renal anomalies with very less life expectancy. Among craniofacial anomalies cleft lip and palate are common. These craniofacial and cardiac anomalies present difficulties with anesthesia. We therefore describe the anesthetic management in the case of a Trisomy 13 child for operated for cleft lip at 10 months of age.

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Abstract Neuroblastoma is the most common, non-central nervous system tumor of childhood. It has the potential to synthesize catecholamines. However, the presences of hypertension are uncommon. We report the perioperative management of a 15-month-old infant with giant abdominal neuroblastoma who presented severe hypertension. The pathophysiological alterations of neuroblastoma are reviewed and perioperative management presented.

Resumo Neuroblastoma é o tumor mais comum do sistema nervoso não central na infância. Esse tumor tem o potencial de sintetizar catecolaminas; entretanto, a presença de hipertensão é rara. Relatamos o manejo perioperatório de uma criança de cinco meses com neuroblastoma abdominal gigante que apresentou hipertensão grave. As alterações fisiopatológicas do neuroblastoma foram revistas e o manejo perioperatório é apresentado.

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RESUMO

Neuroblastoma is the most common, non-central nervous system tumor of childhood. It has the potential to synthesize catecholamines. However, the presences of hypertension are uncommon. We report the perioperative management of a 15-month-old infant with giant abdominal neuroblastoma who presented severe hypertension. The pathophysiological alterations of neuroblastoma are reviewed and perioperative management presented.

Assuntos

RESUMO

Neuroblastoma is the most common, non-central nervous system tumor of childhood. It has the potential to synthesize catecholamines. However, the presences of hypertension are uncommon. We report the perioperative management of a 15-month-old infant with giant abdominal neuroblastoma who presented severe hypertension. The pathophysiological alterations of neuroblastoma are reviewed and perioperative management presented.

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RESUMO

ABSTRACT BACKGROUND AND OBJECTIVES: Laparoscopic surgery has become a popular surgical tool when compared to traditional open surgery. There are limited data on pediatric patients regarding whether pneumoperitoneum affects cerebral oxygenation although end-tidal CO2 concentration remains normal. Therefore, this study was designed to evaluate the changes of cerebral oxygen saturation using near-infrared spectroscope during laparoscopic surgery in children. METHODS: The study comprised forty children who were scheduled for laparoscopic (Group L, n = 20) or open (Group O, n = 20) appendectomy. Hemodynamic variables, right and left regional cerebral oxygen saturation (RrSO2 and LrSO2), fraction of inspired oxygen, end-tidal carbon dioxide pressure (PETCO2), peak inspiratory pressure (Ppeak), respiratory minute volume, inspiratory and end-tidal concentrations of sevoflurane and body temperature were recorded. All parameters were recorded after anesthesia induction and before start of surgery (T0, baseline), 15 min after start of surgery (T1), 30 min after start of surgery (T2), 45 min after start of surgery (T3), 60 min after start of surgery (T4) and end of the surgery (T5). RESULTS: There were progressive decreases in both RrSO2 and LrSO2 levels in both groups, which were not statistically significant at T1, T2, T3, T4. The RrSO2 levels of Group L at T5 were significantly lower than that of Group O. One patient in Group L had an rSO2 value <80% of the baseline value. CONCLUSIONS: Carbon dioxide insufflation during pneumoperitoneum in pediatric patients may not affect cerebral oxygenation under laparoscopic surgery.

RESUMO JUSTIFICATIVA E OBJETIVOS: A cirurgia laparoscópica se tornou uma ferramenta cirúrgica popular em comparação com a cirurgia aberta tradicional. Há poucos dados sobre pacientes pediátricos no que se refere ao pneumoperitônio afetar a oxigenação cerebral enquanto a concentração de CO2 no fim da expiração continua normal. Portanto, este estudo teve como objetivo avaliar as alterações da saturação de oxigênio cerebral com espectroscopia de infravermelho próximo durante cirurgia laparoscópica em crianças. MÉTODOS: O estudo recrutou 40 crianças programadas para apendicectomia laparoscópica (Grupo L, n = 20) ou aberta (Grupo A, n = 20). Variáveis hemodinâmicas, saturação de oxigênio cerebral regional direita e esquerda (RrSO2 e LrSO2), fração inspirada de oxigênio, pressão expiratória final de dióxido de carbono (PETCO2), pico de pressão inspiratória (Ppico), volume minuto respiratório, concentrações de sevoflurano inspirado e expirado e temperatura corporal foram registrados. Todos os parâmetros foram registrados após a indução da anestesia e antes do início da cirurgia (T0, basal), 15 minutos após o início da cirurgia (T1), 30 minutos após o início da cirurgia (T2), 45 minutos após o início da cirurgia (T3), 60 minutos após o início da cirurgia (T4) e no fim da cirurgia (T5). RESULTADOS: Houve diminuição progressiva em ambos os níveis de RrSO2 e LrSO2 nos dois grupos, mas não foi estatisticamente significativa em T1, T2, T3, T4. Os níveis de RrSO2 do Grupo L em T5 foram significativamente menores do que os do Grupo A. Um paciente do Grupo L apresentou um valor rSO2 < 80% do valor basal. CONCLUSÕES: A insuflação de dióxido de carbono durante o pneumoperitônio em pacientes pediátricos pode não afetar a oxigenação cerebral em cirurgia laparoscópica.

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