Manejo anestésico para cirurgia de atresia de esôfago em um recém‐nascido com síndrome de Goldenhar

ResumoJustificativa e objetivosA 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.Relato de casoApresentamos o caso de um recém‐nascido de 24horas de vida com síndrome de Goldenhar. Ele apresentava atresia de esôfago, com fístula traqueoesofágica distal. Decidiu‐se por uma cirurgia de emergência para reparo. Ela foi feita sob sedação, intubação com fibrobroncoscópio distal à fístula, para limitar passagem do ar para o esôfago e possível distensão abdominal. Após o reparo completo da atresia de esôfago e ligadura da fístula, o paciente foi transferido para a unidade de terapia intensiva e intubado com sedoanalgesia.ConclusõesO achado de um paciente com síndrome de Goldenhar e atresia de esôfago supõe uma situação excepcional e um desafio para os anestesiologistas, pois o manejo anestésico depende da comorbidade do paciente, do tipo de fístula traqueoesofágica, da prática hospitalar habitual e das habilidades do anestesiologista responsável, sendo que a peculiaridade principal é manter uma ventilação pulmonar adequada na presença de uma comunicação entre a via respiratória e o esôfago. A intubação com fibrobroncoscópio distal à fístula resolve o manejo da via respiratória provavelmente difícil e limita a passagem de ar para o esôfago através da fístula.AbstractBackground and objectivesGoldenhar'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 reportWe 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.ConclusionsThe 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

Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis

AbstractBackground and objectivesHypertrophic pyloric stenosis is a relatively common affection of gastrointestinal tract in childhood that results in symptoms, such as projectile vomiting and metabolic disorders that imply a high risk of aspiration during anesthetic induction. In this way, the carrying out of a technique with general anesthesia and intravenous rapid sequence induction, preoxygenation and cricoid pressure are recommended. After the correction of systemic metabolic alkalosis and pH normalization, cerebrospinal fluid can keep a state of metabolic alkalosis. This circumstance, in addition to the residual effect of neuromuscular blocking agents, inhalant anesthetics and opioids could increase the risk of postoperative apnea after a general anesthesia.Case reportWe present the successful management in 3 neonates in those a pyloromyotomy was carried out because they had presented congenital hypertrophic pyloric stenosis. This procedure was done under general anesthesia with orotracheal intubation and rapid sequence induction. Then, ultrasound-guided paravertebral block was performed as analgesic method without the need for administrating opioids within intraoperative period and keeping an appropriate analgesic level.ConclusionsLocal anesthesia has demonstrated to be safe and effective in pediatric practice. We consider the ultrasound-guided paravertebral block with one dose as a possible alternative for other local techniques described, avoiding the use of opioids and neuromuscular blocking agents during general anesthesia, and reducing the risk of central apnea within postoperative period

Manejo anestésico para la cirugía de atresia de esófago en un neonato con síndrome de Goldenhar

ResumenJustificación y objetivosEl 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.Relato del casoPresentamos un caso de un neonato con síndrome de Goldenhar de 24 h de vida. Presentaba atresia esofágica con fístula traqueoesofágica distal. Se decidió una intervención quirúrgica urgente para la reparación de la misma. Se realizó bajo sedación, intubación con fibrobroncoscopio distal a la fístula, para limitar el paso de aire a esófago y la posible distensión abdominal. Tras la completa reparación de la atresia esofágica y la ligadura de la fístula, el paciente fue trasladado a la unidad de cuidados intensivos con sedoanalgesia e intubado.Conclusionesel hallazgo de un paciente con síndrome de Goldenhar y atresia de esófago supone una situación excepcional y un reto para los anestesiólogos, por lo que el manejo anestésico depende de la comorbilidad del paciente, del tipo de fístula traqueoesofágica, de la práctica hospitalaria habitual y de las habilidades del anestesiólogo responsable, siendo la principal particularidad el mantenimiento de una adecuada ventilación pulmonar en presencia de una comunicación entre la vía aérea y el esófago. La intubación con fibrobroncoscopio distal a la fístula solventa el manejo de la vía aérea probablemente difícil y limita el paso de aire al esófago a través de la fístula.AbstractBackground and objectivesGoldenhar'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 reportWe 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.ConclusionsThe 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

Manejo anestésico de épulis congénito

SummaryIntroductionepulis of the newborn is a granular cell tumor arising in the mucosa of the dental ridge. It presents as a pedunculated soft tissue mass that can be lobular or multinodular. It is more common in females than in males (8:1) perhaps due to hormonal factors. It may be accompanied by other congenital malformations. Anesthetic management is based on a potentially difficult intubation and the risk of bleeding.ObjectivesTo present the case of a newborn with congenital epulis and to review this pathology and its anesthetic management.Methods and ResultsClinical case presentation.ConclusionsSeveral types of anesthesia have been described depending, among other factors, on tumor size and on the professionals involved in excising the lesion. In our case, and given the characteristics of the tumor, we chose inhalation sedation with O2/ air / sevoflurane, lateral decubitus position and local infiltration at the base of implantation. Good collaboration between the surgeon and the anesthetist is critical for success

Estudio experimental sobre las modificaciones de la presión coloidosmótica plasmática y su relación con el desarrollo del edema pulmonar

Los mecanismos de intercambio a nivel del capilar han sido foco de atención para diversos autores en los últimos años. Sus resultados han sido dispares, quedando por aclarar la utilidad del equilibrio descrito por starling. Estas discrepancias nos han impulsado al desarrollo de este estudio. Hemos estudiado en 20 animales (perros), divididos en 4 grupos de 5, las variaciones de la presión coloidosmótica plasmática, y de los responsables de la osmolaridad del plasma, tras la agresión de la membrana del capilar pulmonar, con ácido oleico, y tras la elevación de la presión hidrostática a dicho nivel. Las determinaciones fueron efectuadas a la entrada y salida del capilar pulmonar. Los resultados han mostrado una diferencia estadísticamente significativa al comparar los niveles de presión coloidosmótica plasmática a la entrada (art. pulmonar) y salida (art. femoral) del capilar pulmonar en los animales a los que administramos ácido oleico. Estas diferencias fueron así mismo observadas en aquellos animales a los que elevamos la presión de la aurícula izquierda, si bien en este grupo se hicieron patentes en forma tardía. El resto de los parámetros estudiados, no presentaron diferencias estadísticamente significativas ni en los grupos controles ni en los grupos de estudio. A la vista de nuestros resultados estamos en condiciones de afirmar que la determinación de las diferencias de la presión coloidosmótica plasmática, medida a la entrada y salida del capilar pulmonar, puede ser utilizada como prueba diagnostica en las fases iniciales del disstres respiratorio del adulto

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

AbstractBackground and objectivesGoldenhar'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 reportWe 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.ConclusionsThe 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

EXIT procedure in twin pregnancy: a series of three cases from a single center

Background: Indications for the ex utero intrapartum therapy (EXIT) procedure have evolved and nowadays in addition to secure the airway, obtain vascular access, administer surfactant and other resuscitation medications, EXIT is used to resect cervical or thoracic masses, for extracorporeal membrane circulation (ECMO) cannulation, as well as to rescue maximum intra-thoracic space for ventilation of the remaining functional lung tissue or in cases in which resuscitation of the neonate may be compromised. EXIT procedure in twin pregnancy has been rarely reported and some doubts have been raised about its strategy and safety in such cases. Methods: We reviewed the medical records of 3 twin pregnancy cases where the EXIT procedure have been performed in our center. Results: The mean gestational age at EXIT procedure was 34 + 4 weeks. In two out the three EXIT procedures, the affected twin was delivered first. The average time on placental bypass was 9 minutes. There were no fetal or maternal complications related to the EXIT procedure. All newborns are currently doing well. Conclusion: In twin pregnancies, prenatal diagnosis combined with the EXIT procedure permits the formulation of a controlled delivery strategy to secure both newborns outcome. In those pregnancies, if intervention can be accomplished without compromise of the normal twin, EXIT can be considered. Our results support that EXIT procedure, if properly planned, safely provides a good outcome for both the fetuses as well as the mother

Diagnosis and management of a large oropharyngeal teratoma (epignathus) in a twin pregnancy

2 pages, 2 figures.-- PMID: 19036373 [PubMed].-- Available online Nov 25, 2008.Epignathus refers to a teratoma of the oropharyngeal region composed of cells from the ectodermal, mesodermal, and endodermal layers. When it arises from the palate or pharynx and protrudes from the mouth it may result in life-threatening airway obstruction after birth [K. Izadi, M. Smith, M. Askari, D. Hackam, A.A. Hameed and J.P. Bradley, A patient with an epignathus: management of a large oropharyngeal teratoma in a newborn, J Craniofac Surg 14 (4) (2003), pp. 468–472]. The ex utero intrapartum treatment (EXIT) procedure maintains fetoplacental circulation for sufficient oxygenation in a fetus at risk for airway obstruction.Peer reviewe

Intrauterine myelomeningocele repair: Experience of the fetal medicine and therapy program of the Virgen de Rocío University Hospital

[ES] La forma más frecuente de espina bífida es el mielomeningocele, para el que no existe un tratamiento postnatal óptimo. Además del trastorno motor o sensitivo dependiente del nivel de la lesión, los niños suelen tener asociada la malformación de Arnold Chiari ii. El mielomeningocele presenta una alta mortalidad y puede acompañarse, hasta en el 80-90%, de hidrocefalia que es responsable de la gran afectación neurocognitiva, precisando de derivación para su supervivencia. La reparación intrauterina de malformaciones fetales mediante acceso abierto a través de histerotomía se ha convertido en una opción terapéutica gracias a la mejora de las técnicas quirúrgicas y anestésicas, y de la correspondiente instrumentación, que han convertido este tipo de intervenciones en una práctica relativamente frecuente. El tratamiento anestésico debe orientarse tanto a la madre como al feto, siendo importante mantener controlados los factores hemodinámicos que regulan el flujo placentario, la dinámica uterina, las pérdidas sanguíneas y el bienestar fetal. Dentro de nuestro Programa de Medicina y Terapia Fetal se han realizado 21 procedimientos de cirugía fetal abierta, 17 procedimientos EXIT y 4 procedimientos para la corrección intrauterina de mielomeningocele fetal. Describimos nuestra experiencia en la corrección intrauterina de mielomeningocele fetal mediante cirugía fetal abierta.[EN] The most frequent form of spina bifida is myelomeningocele. There is no optimal postnatal treatment for this defect. In addition to the motor or sensory deficits, which depend on the location of the lesion, the defect is usually associated with Chiari ii malformation in affected children. Myelomeningocele has high mortality and, in up to 80% to 90% of patients, can be accompanied by hydrocephalus, which causes severe neurocognitive impairment and requires the patient to be shunted for survival. Intrauterine repair of fetal malformations employing open access through hysterotomy has become a therapeutic option due to improved anesthetic and surgical techniques and instrumentation, which have allowed this type of intervention to become relatively frequent. Anesthetic treatment should focus on both the mother and fetus and the hemodynamic factors regulating placental flow, uterine dynamics, blood loss and fetal well-being must remain well-controlled. Within our Program for Fetal Medicine and Therapy, 21 open fetal interventions have been performed: 17 EXIT procedures and 4 procedures for the intrauterine correction of fetal myelomeningocele. We describe our experience of the intrauterine repair of fetal myelomeningocele through open fetal surgery.Peer Reviewe