[Anesthesia in a patient with Ellis-van Creveld syndrome]. / Tratamiento anestésico de una paciente con síndrome de Ellis-Van Creveld.

Ellis-van Creveld syndrome is a rare type of developmental chondroectodermal dysplasia. We report the case of a 32-year-old woman with this syndrome who was scheduled for cesarean section. She had no related heart defect. A spinal block was attempted but after confirming that no sensory blockade had been achieved, general anesthesia was administered. Both the operation and the anesthetic procedure were without complications. The clinical manifestations of Ellis-van Creveld syndrome are short-limbed dwarfism, postaxial polydactyly, fingernail dysplasia, cleft palate and lips, and heart defects. Diagnosis is based on clinical manifestations and radiography. Treatment involves correction of heart defects and orthopedic problems. Perioperative airway management problems may develop. A preoperative echocardiogram should be done to assess heart function and ascertain anatomical abnormalities. Thoracic deformities may make mechanical ventilation difficult and there is risk of barotrauma. Intraoperative management requires rapid control of the airway and prevention of bronchial aspiration. Vigilance in preventing hemodynamic instability and myocardial depression is essential. Postoperative analgesia must be managed carefully and adverse cardiorespiratory events avoided.

Tratamiento anestésico de una paciente con síndrome de Ellis-Van Creveld / Anesthesia in a patient with Ellis-van Creveld syndrome

INTRODUCCIÓN: El síndrome de Ellis-Van Creveld es una enfermedad rara del desarrollo, perteneciente al grupo de las displasias condroectodérmicas. CASO CLÍNICO: Mujer de 32 años diagnosticada de síndrome de Ellis van Creveld, programada para cesárea. Carecía de patología cardiaca. Se realizó un bloqueo subaracnoideo pero tras la comprobación de que no había bloqueo sensitivo, se decidió anestesia general. Tanto la cesárea como el procedimiento anestésico trascurrieron sin incidencias. DISCUSIÓN: Las manifestaciones clínicas del síndrome de Ellis van Creveld son enanismo con extremidades muy cortas, polidactilia postaxial, tórax pequeño, displasia ungueal, alteraciones de la boca y labios y anomalías cardiacas. El diagnóstico es clínico-radiológico. El tratamiento viene dado por la corrección de las alteraciones cardiacas y el tratamiento ortopédico. La morbilidad perioperatoria puede venir dada por las dificultades en el manejo de la vía aérea. Es necesaria la realización de una ecocardiografía preoperatoria para valorar la función cardiaca y concretar las anormalidades anatómicas. Las anormalidades torácicas también pueden dificultar la ventilación mecánica, incluido el riesgo de barotrauma. El manejo intraoperatorio debe basarse en un rápido control de la vía aérea, con prevención de la broncoaspiración. Debe evitarse la inestabilidad hemodinámica y la depresión miocárdica. En el postoperatorio, es primordial el correcto manejo de la analgesia y la prevención de episodios cardiorrespiratorios desfavorables(AU)

Ellis-van Creveld syndrome is a rare type of developmental chondroectodermal dysplasia. We report the case of a 32-year-old woman with this syndrome who was scheduled for cesarean section. She had no related heart defect. A spinal block was attempted but after confirming that no sensory blockade had been achieved, general anesthesia was administered. Both the operation and the anesthetic procedure were without complications. The clinical manifestations of Ellis-van Creveld syndrome are short-limbed dwarfism, postaxial polydactyly, fingernail dysplasia, cleft palate and lips, and heart defects. Diagnosis is based on clinical manifestations and radiography. Treatment involves correction of heart defects and orthopedic problems. Perioperative airway management problems may develop. A preoperative echocardiogram should be done to assess heart function and ascertain anatomical abnormalities. Thoracic deformities may make mechanical ventilation difficult and there is risk of barotrauma. Intraoperative management requires rapid control of the airway and prevention of bronchial aspiration. Vigilance in preventing hemodynamic instability and myocardial depression is essential. Postoperative analgesia must be managed carefully and adverse cardiorespiratory events avoided(AU)

Visitas domiciliarias en un centro de salud de Zaragoza (2005) / Home visits in a health care center of Zaragoza (2005)

INTRODUCCIÓN. Determinar las características de los pacientes que solicitan las visitas domiciliarias, así como la distribución temporal y horaria de los mismos. MÉTODOS. Análisis descriptivo retrospectivo de las visitas domiciliarias realizadas por los médicos de un equipo de Atención Primaria durante el año 2005. Se obtuvieron datos cuantitativos de las visitas domiciliarias solicitadas como espontáneas y recogidas en un registro informatizado, su distribución temporal, horaria y datos relativos a los pacientes que solicitaron las visitas. RESULTADOS. Entre los resultados cabe destacar que se solicitaron 1.668 visitas, las cuales supusieron el 2,5% del total de consultas realizadas y sólo el 0,5% se registraron como urgentes; a las 14 horas el 88% de éstas ya se habían recogido, siendo los lunes y los meses de diciembre y enero en los que mayor número de visitas se asumieron. El 5,85% de la población solicitó, al menos, una visita domiciliaria; el 72% de los pacientes solicitó una única visita, y las visitas múltiples fueron solicitadas por los pacientes de mayor edad. CONCLUSIONES. El volumen de visitas se sitúa en un nivel intermedio en relación con otros estudios. Las visitas solicitadas como urgentes, que son las que más preocupan a los facultativos por la gran distorsión que provocan en el quehacer diario, dada la inmediatez que exigen, sólo suponen el 0,5%. Con el aumento de la edad crece el número de visitas, también lo hace con los meses de invierno. La atención domiciliaria adquiere cada vez más importancia en una asistencia sanitaria integral y continuada, siempre a expensas del estilo del propio médico

INTRODUCTION. To determine the characteristics of the patients who ask for a home visit, as well as their temporal and timetable distribution. METHODS. Retrospective descriptive study of the home visits make by the doctors of a Primary health care team during 2005. Quantitative data were obtained from spontaneous home visits made by the doctors and were stored in a computerized register where the temporal distribution (months), timetable distribution and all the personal information of each patient visited were analyzed. RESULTS. From the results obtained, it stands out that 1,668 spontaneous home visits were required in 2005. This accounts for 2.5% of the all consultations made in this period. Only 0.5% of the home visits required were recorded as urgent and 88% of the total home visits required were recorded before 2 p.m. Mondays as weekday and December and January as months were the periods when the most visits were attended. A total of 5.85% of the population asked for a home visit, and 72% of those asked only once. Those patients who required more than one home visit during 2005 were in the elderly age group. CONCLUSIONS. The volume of spontaneous home visits analyzed in this study is similar to other studies. Urgent home visits are the most difficult ones to manage by the doctors, due to the reaction time they require and the distortion of the programmed timetable, however these types of visits only account for 0.5%. The older a person is, the higher the number of visits, and wintertime is the highest period. Home visits are more important than ever in an integral and continuous health care, but always bearing in mind the style of each doctor