Vía aérea difícil en un paciente pediátrico con mucopolisacaridosis tipo i (síndrome de Hurler) / Difficult airway in a pediatric patient with mucopolysaccharidosis type i (Hurler syndrome)

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Corrección intrauterina de mielomeningocele: experiencia del programa de medicina y terapia fetal del Hospital Universitario Virgen del Rocío / Intrauterine myelomeningocele repair: Experience of the fetal medicine and therapy program of the Virgen de Rocío University Hospital

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 (AU)

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 (AU)

[Intrauterine myelomeningocele repair: experience of the fetal medicine and therapy program of the Virgen de Rocío University Hospital]. / Corrección intrauterina de mielomeningocele: experiencia del programa de medicina y terapia fetal del Hospital Universitario Virgen del Rocío.

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.

[Comparative analysis of the criteria for surveillance and monitoring in anesthesia, resuscitation and pain therapy. Recommendation of the Sociedad Española de Anestesia y Reanimación]. / Análisis comparativo de los criterios de vigilancia y monitorización en anestesia, reanimación y terapia del dolor. Recomendaciones de la SEDAR.

INTRODUCTION: One part of morbidity and mortality associated with anesthesia is due to accidents. It is thought that an additional monitoring can prevent and avoid most of anesthetic accidents. OBJECTIVES: In order to improve patient's safety and quality of anesthesia, Harvard University hospital approved in 1985 the rules for intraoperative monitoring. These were adopted by the American Society of Anesthesiologists (ASA) in 1986. In line with this procedure, professional associations of several countries pronounced their own rules. SEDAR did it in 1989. The purpose of this study was to compare spanish rules with those of America (Harvard and ASA), Australia, England and France. RESULTS: Comparative analysis revealed that the spanish norms are more extensive since they include not only the intraoperative anesthetic activities, but also those related to recovery, pain, and obstetric anesthesia. However, it has some deficiencies such as the lack of a periodical revision, and of an adaptative period and assistance to the anesthesiologist provided by auxiliary personnel. Successful points were the recognition that pulse oximetry is essential, the preoperative verification of all material, and, more importantly, is the only one that considers essential capnography in the assessment of ventilation and pulse oximetry during regional anesthesia and postoperative phase. CONCLUSION: Spanish norm is comparable to that of the other countries considered in this study. It shows important successful points and at the same time some significant deficiencies.