Ventilación mecánica domiciliaria en niños: estudio multicéntrico español / Domiciliary mechanical ventilation in children: a spanish multicentre study

Introducción: La ventilación mecánica domiciliaria (VMD) es una técnica cada vez más frecuente en el niño. Existen pocos estudios que hayan analizado las características y necesidades de los niños sometidos a esta técnica. Material y métodos: Estudio descriptivo observacional transversal multicéntrico de pacientes entre un mes y 16 años dependientes de ventilación mecánica domiciliaria. Resultados: Se estudiaron 163 pacientes de 17 hospitales españoles con una edad media de 7,6 años. La causa más frecuente de VMD fueron los trastornos neuromusculares. El inicio de la VMD fue a una edad media de 4,6 años. Un 71,3% recibieron ventilación no invasiva. Los pacientes con ventilación invasiva tenían menor edad, menor edad de inicio de la VMD y mayor tiempo de uso diario. El 80,9% precisaban VM solo durante el sueño, y un 11,7% durante todo el día. Únicamente un 3,4% de los pacientes tiene asistencia sanitaria externa como ayuda a la familia. Un 48,2% es controlado en consultas específicas de VMD o consultas multidisciplinares. Un 72,1% de los pacientes está escolarizado (recibiendo enseñanza adaptada un 42,3%). Solo un 47,8% de los pacientes escolarizados cuentan con cuidadores específicos en su centro escolar. Conclusiones: La VMD en niños se utiliza en un grupo muy heterogéneo de pacientes iniciándose en un importante porcentaje en los primeros 3 años de vida. A pesar de que un significativo porcentaje de pacientes tiene una gran dependencia de la VMD pocas familias cuentan con ayudas específicas tanto a nivel escolar como en el domicilio, y el seguimiento sanitario es heterogéneo y poco coordinado(AU)

Introduction: Domiciliary mechanical ventilation (DMV) use is increasing in children. Few studies have analysed the characteristics of patients using this technique. Materials and methods: An observational, descriptive, transversal, multicentre study was conducted on patients between 1 month and 16 years of age dependent on domiciliary mechanical ventilation. Results: A total of 163 patients with a median age of 7.6 years from 17 Spanish hospitals were studied. The main reasons for DMV were neuromuscular disorders. The median age at beginning of DMV was 4.6 years. Almost three-quarters (71.3%) received non-invasive ventilation. Patients depending on invasive ventilation were younger, started DMV at an earlier age, and had more hours of mechanical ventilation per day. The large majority (80.9%) used DMV during sleep time only, and 11.7% during the whole day. Only 3.4% of patients had external health assistance. Just under half (48.2%) were being followed up in specific DMV or multidisciplinary clinics. Almost three-quarters (72.1%) of patients attended school (42.3% with adapted schooling). Only 47.8% of school patients had specific caregivers in their schools. Conclusions: DMV in children is used in a very heterogeneous group of patients, and in an important number of patients it is started before the third year of life. Despite there being a significant proportion of patients with a high dependency on DMV, few families receive specific support at home or at school, and health care surveillance is variable and poorly coordinated(AU)

[Domiciliary mechanical ventilation in children: a Spanish multicentre study]. / Ventilación mecánica domiciliaria en niños: estudio multicéntrico español.

INTRODUCTION: Domiciliary mechanical ventilation (DMV) use is increasing in children. Few studies have analysed the characteristics of patients using this technique. MATERIALS AND METHODS: An observational, descriptive, transversal, multicentre study was conducted on patients between 1 month and 16 years of age dependent on domiciliary mechanical ventilation. RESULTS: A total of 163 patients with a median age of 7.6 years from 17 Spanish hospitals were studied. The main reasons for DMV were neuromuscular disorders. The median age at beginning of DMV was 4.6 years. Almost three-quarters (71.3%) received non-invasive ventilation. Patients depending on invasive ventilation were younger, started DMV at an earlier age, and had more hours of mechanical ventilation per day. The large majority (80.9%) used DMV during sleep time only, and 11.7% during the whole day. Only 3.4% of patients had external health assistance. Just under half (48.2%) were being followed up in specific DMV or multidisciplinary clinics. Almost three-quarters (72.1%) of patients attended school (42.3% with adapted schooling). Only 47.8% of school patients had specific caregivers in their schools. CONCLUSIONS: DMV in children is used in a very heterogeneous group of patients, and in an important number of patients it is started before the third year of life. Despite there being a significant proportion of patients with a high dependency on DMV, few families receive specific support at home or at school, and health care surveillance is variable and poorly coordinated.

Tratamiento endovascular del secuestro pulmonar con dispositivos tipo Amplatzer® / Endovascular treatment of pulmonary sequestration with Amplatzer® vascular plugs

El secuestro pulmonar es una malformación poco frecuente, que se caracteriza por tejido pulmonar sin conexión con la vía aérea y con vascularización sistémica. El tratamiento tradicional ha sido la cirugía, pero en los últimos años la embolización endovascular es una técnica cada vez más utilizada. Los materiales de embolización usados en la actualidad son los coils y los tapones vasculares tipo Amplatzer®. El tapón vascular Amplatzer® es un dispositivo de malla cilíndrico, autoexpandible, particularmente útil para la embolización de vasos grandes y de alto flujo. Presentamos a continuación 3 casos de secuestro pulmonar tratados mediante embolización con dispositivos de oclusión vascular tipo Amplatzer®. En los 3 casos se realizó el procedimiento sin incidencias, y se comprobó la oclusión total de la colateral aórtico-pulmonar. Nuestra serie aporta nuevos datos sobre la seguridad y eficacia de la embolización de los secuestros pulmonares con tapones vasculares tipo Amplatzer®, y de su preferencia sobre los coils en la embolización de vasos nutricios grandes(AU)

Pulmonary sequestration is a rare malformation characterised by non-functioning lung tissue, separated from the tracheobronchial tree and with arterial supply from the systemic circulation. The classical therapeutic approach is surgical resection. In recent years, the endovascular embolisation technique is increasingly being used for the treatment of this disease. The embolisation materials used are coils and vascular plugs. The Amplatzer® vascular plug is a self-expandable cylindrical mesh device, particularly useful for the embolisation of large vessels with high flow. Three cases of pulmonary sequestration were treated by embolisation with Amplatzer® vascular plugs. In all cases the procedure was performed without complications, and the aortopulmonary collateral vessels were completely occluded.Our study provides new data on the safety and efficacy of pulmonary embolisation with Amplatzer® vascular plugs, and its preference over coils in the embolisation of large vessels(AU)

[Endovascular treatment of pulmonary sequestration with Amplatzer® vascular plugs]. / Tratamiento endovascular del secuestro pulmonar con dispositivos tipo Amplatzer®

Pulmonary sequestration is a rare malformation characterised by non-functioning lung tissue, separated from the tracheobronchial tree and with arterial supply from the systemic circulation. The classical therapeutic approach is surgical resection. In recent years, the endovascular embolisation technique is increasingly being used for the treatment of this disease. The embolisation materials used are coils and vascular plugs. The Amplatzer® vascular plug is a self-expandable cylindrical mesh device, particularly useful for the embolisation of large vessels with high flow. Three cases of pulmonary sequestration were treated by embolisation with Amplatzer® vascular plugs. In all cases the procedure was performed without complications, and the aortopulmonary collateral vessels were completely occluded. Our study provides new data on the safety and efficacy of pulmonary embolisation with Amplatzer® vascular plugs, and its preference over coils in the embolisation of large vessels.

Enfermedad respiratoria y parálisis diafragmática en la enfermedad de Charcot-Marie-Tooth / Respiratory disease and diaphragm paralysis in Charcot-Marie-Tooth disease

La disfunción diafragmática bilateral es una entidad infrecuente. Dentro del ámbito pediátrico las causas más comunes son las asociadas a traumatismo obstétrico o cirugía cardiovascular. En el diagnóstico diferencial se incluye la enfermedad de Charcot-Marie-Tooth (CMT).Si bien en esta enfermedad es infrecuente, la afectación de la musculatura respiratoria, por su carácter distal, está descrita su asociación con neumopatía restrictiva secundaria a una disfunción del nervio frénico con paresia diafragmática bilateral o anomalías de la pared torácica. Presentamos 2 casos de CMT que ingresaron en la Unidad de Cuidados Intensivos con fallo respiratorio tipo II. En ambos casos el tratamiento con ventilación no invasiva produjo una mejoría clínica significativa. A destacar el hecho de que en uno de los pacientes la evidencia de una afectación frénica sirvió como signo guía para el diagnóstico de su enfermedad de base (AU)

Diaphragmatic bilateral palsy is uncommon in children. The most important etiologies are thoracic surgery and obstetric trauma. Respiratory muscle impairment is a rare phenomenon in patients with Charcot-Marie-Tooth disease (CMT). However, it can be associated with restrictive pulmonary impairment, phrenic nerve dysfunction or thoracic cage abnormalities. We report two paediatric cases of CMT disease with type 2 respiratory failure due to diaphragmatic dysfunction. In both cases treatment with non-invasive mechanical ventilation resulted in satisfactory clinical improvement. Evidence of phrenic damage was the main clue in one patient in order to obtain an accurate diagnostic of her disease (AU)

[Respiratory disease and diaphragm paralysis in Charcot-Marie-Tooth disease]. / Enfermedad respiratoria y parálisis diafragmática en la enfermedad de Charcot-Marie-Tooth.

Diaphragmatic bilateral palsy is uncommon in children. The most important etiologies are thoracic surgery and obstetric trauma. Respiratory muscle impairment is a rare phenomenon in patients with Charcot-Marie-Tooth disease (CMT). However, it can be associated with restrictive pulmonary impairment, phrenic nerve dysfunction or thoracic cage abnormalities. We report two paediatric cases of CMT disease with type 2 respiratory failure due to diaphragmatic dysfunction. In both cases treatment with non-invasive mechanical ventilation resulted in satisfactory clinical improvement. Evidence of phrenic damage was the main clue in one patient in order to obtain an accurate diagnostic of her disease.

Aplicación de ventilación no invasiva en pacientes postoperados cardíacos. Estudio retrospectivo / Non-invasive ventilation after cardiac surgery. A prospective study

Objetivos: Describir según la experiencia de los autores el uso de la ventilación no invasiva (VNI) en pacientes postoperados cardíacos. Material y métodos: Estudio prospectivo de los pacientes postoperados cardíacos ingresados en la unidad de cuidados intensivos pediátricos (UCIP) entre los años 2004 y 2007 que precisaron VNI tras extubación. Resultados: Durante este período se intervinieron de cirugía cardíaca 331 pacientes, de los cuales 159 pacientes llegaron extubados a la unidad. Se aplicó VNI en 29 episodios sobre 26 pacientes. Las cardiopatías más frecuentes fueron Fallot y canal auriculoventricular. Diecinueve (65%) pacientes presentaron fallo respiratorio (FR) de tipo II y el resto presentó FR de tipo I. Los problemas respiratorios más frecuentes fueron el edema agudo de pulmón (EAP) y la atelectasia. En un 70% de los episodios la indicación fue electiva. El tipo de respirador más utilizado fue el BiPAP Vision y, en cuanto a la modalidad, la más utilizada fue el Spontaneous/Timed que supuso el 56%. La duración media de la VNI fue de 64h (rango de 41 a 88h). Los pacientes tuvieron una estancia media en la UCIP de 22 días. Nueve pacientes requirieron reintubación, ninguno de ellos de forma precoz (primeras 12h), lo que supone una eficacia del 66%. La eficacia de la VNI se correlacionó con el tipo de enfermedad y de forma inversa con las horas de uso de ésta. Se presentaron complicaciones en 12 episodios. Cuatro pacientes requirieron traqueostomía, todos ellos presentaban neumopatía asociada a hiperaflujo pulmonar previo. No falleció ningún paciente. Conclusiones: La VNI es eficaz y segura durante el postoperatorio cardíaco. Su eficacia es muy alta en caso de atelectasia o de EAP. El fracaso de la VNI se asocia de forma destacada a la afectación pulmonar previa a la cirugía, secundaria a la cardiopatía. Debe plantearse con cautela la indicación de la VNI en este subgrupo de pacientes (AU)

Aim: To report our experience with non-invasive ventilation (NIV) after cardiac surgery. Materials and methods: Prospective study of cardiac surgery patients admitted to our PICU between 2004 and 2007 who required NIV after extubation. Results: A total of 331 patients were admitted to the unit after cardiac surgery during this period. Of these, 159 were extubated in the operatin groom. NIV was introduced in 29 episodes on 26 patients. Fallot’s tetralogy and AVD were the most common heart diseases, and 65% had type II respiratory failure. The respiratory problems usually involved were acute pulmonary oedema and atelectasis. Indication was elective in 70% of episodes. BiPAP Vision was the common est ventilator used and in the S/T mode(56%). Average length of use was 64h. Average length of stay in PICU was 22 days. Nine patients required reintubation, none of them at an early stage (first12h) which represents an efficiency of 66%. The effectiveness of NIV was related to the type of disease and inversely with the time NIV was needed. There were complications in 12 episodes. Four patients required tracheostomy, all of which were associated with previous lung disease. Survival was 100%. Conclusions: NIV is effective and safe after cardiac surgery. It has very good results in respiratory failure due to atelectasis or pulmonary oedema. NIV failure in these patients is strongly associated with preoperative pulmonary sequelae secondary to heart disease. NIV indication in these patients has to be carefully evaluated (AU)

[Non-invasive ventilation after cardiac surgery. A prospective study]. / Aplicación de ventilación no invasiva en pacientes postoperados cardíacos. Estudio retrospectivo.

AIM: To report our experience with non-invasive ventilation (NIV) after cardiac surgery. MATERIALS AND METHODS: Prospective study of cardiac surgery patients admitted to our PICU between 2004 and 2007 who required NIV after extubation. RESULTS: A total of 331 patients were admitted to the unit after cardiac surgery during this period. Of these, 159 were extubated in the operating room. NIV was introduced in 29 episodes on 26 patients. Fallot's tetralogy and AVD were the most common heart diseases, and 65% had type II respiratory failure. The respiratory problems usually involved were acute pulmonary oedema and atelectasis. Indication was elective in 70% of episodes. BiPAP Vision was the commonest ventilator used and in the S/T mode (56%).Average length of use was 64h. Average length of stay in PICU was 22 days. Nine patients required reintubation, none of them at an early stage (first 12h) which represents an efficiency of 66%. The effectiveness of NIV was related to the type of disease and inversely with the time NIV was needed. There were complications in 12 episodes. Four patients required tracheostomy, all of which were associated with previous lung disease. Survival was 100%. CONCLUSIONS: NIV is effective and safe after cardiac surgery. It has very good results in respiratory failure due to atelectasis or pulmonary oedema. NIV failure in these patients is strongly associated with preoperative pulmonary sequelae secondary to heart disease. NIV indication in these patients has to be carefully evaluated.

Protocolo de ventilación no invasiva neonatal: cuidado con recomendar presiones demasiado bajas / No disponible

No disponible

Rendimiento de los cultivos practicados a pacientes hemato-oncológicos con enfermedades infecciosas graves / Yield of the cultures performed in hemato-oncology patients with serious infectious diseases

Introducción: Los avances en el campo de la oncología pediátrica han aumentado la supervivencia pero las complicaciones infecciosas son frecuentes y la necesidad de los Cuidados Intensivos (UCI-P) se incrementa. El objetivo del trabajo es valorar la rentabilidad de los cultivos practicados a pacientes oncológicos que precisaron ingreso en la UCI.P por una patología infecciosa. Material y Métodos. Estudio descriptivo, retrospectivo, de los pacientes oncológicos ingresados en una UCI-P por patología infecciosa en un hospital de tercer nivel. Resultados: Se incluyeron 22 pacientes que tuvieron 25 episodios de infección. Destacó la presentación en forma de insuficiencia respiratoria aguda en 14 pacientes (56%). Los síntomas infecciosos al diagnóstico previos al ingreso en la UCI-P fueron: sobreinfección respiratoria en 12 caos (48%); sospecha clínico-analítica de sepsis en 8 (16%). En un 43% de los pacientes ingresados en planta, se aisló algún germen en los cultivos realizados previos al ingreso en intensivos. Se aisló el microorganismo patógeno en 17 de los pacientes ingresados en UCI (68%), en un 82% de los casos gracias al hemocultivo. El diagnóstico final más frecuente en la UCI-P fue sepsis en 13, seguida de neumonía en 10 pacientes. Fueron exitus one pacientes (48%). Discusión: El elevado número de cultivos negativos probablemente se deba a que quedaran decapitados por la política antibiótica de amplio espectro. El conocimiento estricto de la epidemiología infecciosa de cada hospital y el estudio etiológico precoz y agresivo permitirían avanzar el tratamiento antibiótico correcto de forma empírica e incrementar la efectividad de la antibioterapia (AU)

Introduction: Recent advances in pediatric oncology have improved survival but infectious complications are frequent and the necessity of pediatric intensive care (PICU) is growing. The objective of this study is to determine the profitability of cultives in oncologic pediatric patients who required PICU admittance because of a infectious pathology. Materials and methods: Retrospective review of oncologic patients medical records, admitted to a reference pediatric intensive care unit (PICU), due to an infectious pathology. Results_ 22 patients who suffered 25 infectious processes were recruited. Acute respiratory insufficiency was the most frequent form of presentation (56%). Infectious symptoms before PICU admittance were: respiratory infection 12 cases (48%); clinical sepsis suspicion or biochemical markers compatible with sepsis in 8 patients (16%). 43% of the cultures recollected before PICU´s admittance were positive. Pathogenic microorganisms were isolated in 17 PICU´s patients (68%); in 82% of the cases, it was isolated in a blood sample. The final diagnoses were; sepsis in 13 cases, pneumonia in 10 cases. 11 patients died (48%). Discussion: The high number of negative cultures could be the result of the empiric broad-spectrum antibiotic therapy that is often used in this group of patients. The know-ledge of every hospital epidemiology and the precocious and aggressive etiologic search could improve the empiric antibiotic treatment and improve antibiotic effectiveness (AU)

[Seat belt syndrome. Acute spinal cord injury due to incorrect use of two-point seat belts]. / Síndrome del cinturón de seguridad: lesión medular aguda por uso incorrecto del cinturón de seguridad de dos puntos.

Traumatic spinal cord injury (SCI) is a rare entity in the pediatric age group. These injuries are mainly caused by road traffic accidents (RTA), especially in children not wearing a seat belt. The use of child safety devices such as seat belt restraints has decreased morbidity and mortality in RTA but their incorrect use can also produce serious injuries that are grouped under the term "seat-belt syndrome". This syndrome associates vertebral and spinal cord injuries, intra-abdominal, cutaneous, and muscle-skeletal lesions. We present three patients with complete spinal cord and intra-abdominal injuries, requiring urgent surgery in two of them. On examination, all three patients had seat belt marks on the lower abdominal region. The three patients can be included in this syndrome and its main cause was the use of a two-point seat belt.

Síndrome del cinturón de seguridad: lesión medular aguda por uso incorrecto del cinturón de seguridad de dos puntos / Seat belt syndrome. Acute spinal cord injury due to incorrect use of two-point seat belts

Las lesiones medulares traumáticas son entidades poco frecuentes en la edad pediátrica. La principal causa son los accidentes de tráfico y se dan generalmente en pacientes no portadores del cinturón de seguridad. El uso de los dispositivos de seguridad, como el cinturón, ha disminuido la morbimortalidad en los accidentes, pero el uso incorrecto puede también producir lesiones graves que se describen dentro del denominado síndrome del cinturón de seguridad (seat-belt syndrome). Este síndrome asocia la presencia de lesiones vertebrales, medulares, de vísceras abdominales sólidas y/o huecas y lesiones musculoesqueléticas y cutáneas. Presentamos 3 casos clínicos de pacientes con lesiones medulares completas y lesiones intraabdominales, requiriendo cirugía urgente en dos de ellos. Destaca la presencia en todos ellos de la marca del cinturón de seguridad a nivel abdominal. Los 3 casos pueden ser incluidos en este síndrome y su principal causa es la utilización del cinturón con banda abdominal de 2 puntos

Traumatic spinal cord injury (SCI) is a rare entity in the pediatric age group. These injuries are mainly caused by road traffic accidents (RTA), especially in children not wearing a seat belt. The use of child safety devices such as seat belt restraints has decreased morbidity and mortality in RTA but their incorrect use can also produce serious injuries that are grouped under the term 'seat-belt syndrome'. This syndrome associates vertebral and spinal cord injuries, intra-abdominal, cutaneous, and muscle-skeletal lesions. We present three patients with complete spinal cord and intra-abdominal injuries, requiring urgent surgery in two of them. On examination, all three patients had seat belt marks on the lower abdominal region. The three patients can be included in this syndrome and its main cause was the use of a two-point seat belt

Mielomalacia: una causa de insuficiencia respiratoria tardía. A propósito de 2 casos / Myelomalacia: a cause of delayed respiratory failure. Apropos of two cases

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[Ondine syndrome: diagnosis and management]. / Síndrome de Ondine: diagnóstico y seguimiento.

Ondine's syndrome is a congenital central hypoventilation syndrome due to a disorder in the autonomic control of breathing in the absence of any primary disease that would explain it. Although the incidence of this entity is low, it may be underestimated due to the variable clinical expression of this syndrome, depending on its severity. Early diagnosis is of great importance to provide appropriate management to prevent the acute and chronic asphyxia that determines the long-term prognosis of this disease. This review aims to present practical management guidelines to provide doctors unfamiliar with this syndrome with basic knowledge of the diagnosis, treatment and follow-up of these patients.

[Non-invasive ventilation in neonates with Ondine syndrome: a real indication?]. / Ventilación no invasiva en lactantes afectados de síndrome de Ondine: 'una indicación real?

Ondine's syndrome is a congenital central hypoventilation syndrome due to a disorder in the autonomic control of breathing in the absence of any primary disease that would explain it. Noninvasive ventilation (NIV) has been reported to be effective in the management of these patients, thus avoiding the need for tracheotomy for prolonged mechanical ventilation. We describe our experience of NIV in infants with Ondine's syndrome. Two infants with Ondine's syndrome were transferred to our center for management and adjustment of therapy. On admission NIV (BiPAP VISION) was started with nasal interphase in S/T (spontaneous/timed) mode, which failed to provide suitable ventilation (PCO2 > 70 mmHg). Finally, tracheotomy for continuous mechanical ventilation was performed. Although NIV has been reported to be successful in some patients with Ondine's syndrome, its application in patients younger than 5 years does not seem to be the general norm of treatment.

Síndrome de Ondine: diagnóstico y seguimiento / Ondine´s syndrome: diagnosis and treatment

El síndrome de Ondine consiste en un síndrome de hipoventilación central congénita secundario un trastorno del sistema nervioso central en el cual el control autonómico de la respiración está ausente o se encuentra deteriorado en ausencia de una enfermedad primaria que lo justifique. Aunque la incidencia de esta entidad es baja, es probable, dada la variable expresividad clínica según el grado de intensidad, que se trate de una enfermedad infradiagnosticada. En estos casos es de gran importancia un diagnóstico precoz que permita un buen manejo a fin de evitar episodios de hipoxia e hipercapnia que marcarán el pronóstico de esta enfermedad a largo plazo. El objetivo de esta revisión es presentar una guía de manejo práctica que facilite al facultativo poco adiestrado en esta enfermedad un conocimiento básico del diagnóstico, tratamiento y control de estos pacientes

Ondine's syndrome is a congenital central hypoventilation syndrome due to a disorder in the autonomic control of breathing in the absence of any primary disease that would explain it. Although the incidence of this entity is low, it may be underestimated due to the variable clinical expression of this syndrome, depending on its severity. Early diagnosis is of great importance to provide appropriate management to prevent the acute and chronic asphyxia that determines the long-term prognosis of this disease. This review aims to present practical management guidelines to provide doctors unfamiliar with this syndrome with basic knowledge of the diagnosis, treatment and follow-up of these patients