Megalencephaly-capillary malformation syndrome and associated hydrocephalus: treatment options and revision of the literature.

PURPOSE: We describe our series of 4 patients with megalencephaly-capillary malformation syndrome (MCAP) and review the literature in order to assess the optimal treatment for the associated hydrocephalus. METHODS: We review our institutional series of hydrocephalus associated with MCAP and review the literature, analyzing the causes that could originate the hydrocephalus and the different types of treatments proposed for them. RESULTS: Of our patients treated with ventriculoperitoneal (VP) shunt, one suffered a surgical revision of the shunt and died due to a cranial trauma unrelated to her syndrome or the previous shunt surgery, and the other did not undergo surgical revisions until the end of her follow-up. Our patients treated with endoscopic third ventriculostomy (ETV) have improved their symptomatology and have not suffered of any complications related to the hydrocephalus after the ETV surgery. CONCLUSIONS: We update the treatment of MCAP-associated hydrocephalus and propose ETV as a valid treatment, as it seems a safe procedure with a low rate of complications.

Instituto Pediátrico de Enfermedades Raras del Hospital Sant Joan de Déu / Pediatric Institute of Rare Diseases of the Hospital Sant Joan de Déu

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Meningoencefalitis de tronco como presentación de Behçet / Meningoencephalitis as a presentation form of Behçet

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Papel de las exploraciones complementarias y de la monitorización domiciliaria en los pacientes de riesgo de episodio aparentemente letal, apneas y síndrome de muerte súbita del lactante / The role of complementary examinations and home monitoring in patients at risk from apparent life threatening event, apneas and sudden infant death syndrome

INTRODUCCIÓN: Los monitores de apneas detectan anomalías en la frecuencia cardíaca y respiratoria, sin utilidad demostrada para el diagnóstico de alteraciones respiratorias relacionadas con el sueño en los niños como se pensaba en su origen. OBJETIVO: Describir el tipo de pacientes que se monitorizan, durante cuánto tiempo y la evolución de los mismos. MATERIALES Y MÉTODOS: Estudio descriptivo retrospectivo de los pacientes con monitorización cardiorrespiratoria domiciliaria controlados, desde octubre del 2008 hasta septiembre del 2012, en consultas externas de un hospital materno-infantil de tercer nivel. RESULTADOS: Durante el periodo de estudio fueron incluidos 88 pacientes, el 58% de ellos de sexo masculino, con una mediana de edad de 15,5 días y durante un periodo de 4,7 meses. El motivo de monitorización fue en un 20,5% por antecedente de muerte súbita, sin encontrar patología subyacente; un 25% por apnea de la prematuridad; un 20,5% por episodio aparentemente letal, y un 14.8% por atragantamiento. Otras causas suponen el 19,3% (apnea/hipopnea, desaturaciones y respiración periódica). De estos 3 últimos grupos, en el 50% se registraron eventos patológicos: reflujo patológico (9), apneas de la prematuridad (2), causa neurológica (3) y apneas de causa desconocida (10). CONCLUSIONES: La sospecha de apnea del lactante es un motivo de consulta que crea gran preocupación tanto a la familia como al pediatra. La monitorización domiciliaria es útil en la detección de alteraciones en la frecuencia cardíaca y respiratoria pero es necesario limitar sus indicaciones y realizar un buen seguimiento de estos pacientes, evitando el abuso de otras pruebas complementarias o tratamientos

INTRODUCTION: Home apnea monitors detect abnormalities in cardiac and respiratory frequency, but their use in the diagnosis of respiratory -related sleep disturbances in children has not been demonstrated, as was originally thought. OBJECTIVE: To describe the type of patients being monitored, for how long and their outcome. MATERIAL AND METHODS: A retrospective descriptive study was conducted on patients with controlled home cardiorespiratory monitoring from October 2008 to September 2012 in the Outpatient department of a Maternity tertiary hospital. RESULTS: During the study period 88 patients were included, 58% of them were male, with a median age of 15.5 days, and followed up for a period of 4.7 months. The reason for monitoring was due to a history of sudden death without finding underlying pathology in 20.5%, 25% due to apnea of prematurity, 20.5% due to apparent life-threatening event, and 14.8% due to choking. Other causes accounted for 19.3% (apnea/hypopnea, desaturation and periodic breathing). Of these last three groups, pathological events were observed in 50% of them: reflux disease (9), apnea of prematurity (2), neurological causes (3), and apnea of unknown cause (10). CONCLUSIONS: Suspected infant apnea is a cause for consultation that creates a great deal of concern to the family and the pediatrician. Home monitoring is useful in detecting changes in cardiac and respiratory frequency, but is necessary to limit its indications and ensure proper monitoring of these patients, avoiding the abuse of other tests or treatments

Tóxicos vegetales: un problema aún vigente / Poisonous plants: An ongoing problema

INTRODUCCIÓN: La consulta por ingesta de plantas es poco habitual en los Servicios de Urgencias pediátricas pero puede conllevar una elevada toxicidad. La formación sobre toxicología botánica del personal sanitario suele ser escasa y puede resultar difícil llegar al diagnóstico o establecer el tratamiento adecuado. OBJETIVO: Estudiar las características epidemiológicas y clínicas de las intoxicaciones por ingesta de sustancias vegetales con el fin de aumentar su conocimiento en el personal sanitario. MATERIALES Y MÉTODOS: Estudio descriptivo retrospectivo de los pacientes atendidos por ingesta de sustancia vegetal, entre enero del 2008 y diciembre del 2012, en el Servicio de Urgencias de un hospital materno-infantil de tercer nivel. RESULTADOS: Durante el periodo de estudio fueron atendidos 18 pacientes con ingesta de un posible tóxico vegetal. En 14 casos la ingesta se consideró potencialmente tóxica: retama, adelfa, muérdago, acebillo, judía vulgar (2), tomate de Jerusalén, ricino (2), estramonio, potus, marihuana y setas con toxicidad digestiva (2). De estos, en 10 la ingesta fue no intencionada, 2 casos pueden enmarcarse en el maltrato infantil, se produjo un caso con fin recreativo y otro con fin suicida. Destacaron por su toxicidad las ingestas de adelfa, ricino y estramonio. CONCLUSIONES: La potencial gravedad de la ingesta de sustancias vegetales y la variedad de los mecanismos de exposición al tóxico obligan al pediatra a tener presente esta posibilidad y estar preparado para su diagnóstico y manejo. Es necesario diseñar medidas preventivas específicas, como la información a las familias o la regulación de la presencia de plantas tóxicas en zonas de recreo

INTRODUCTION: A medical visit for plant ingestion is rare in the pediatric emergency services but may involve a high toxicity. The botanical toxicology training of health staff is often very limited, and it can be difficult to make a diagnosis or decide on the appropriate treatment. OBJECTIVE: To study the epidemiological and clinical characteristics of poisoning due to plant ingestion in order to increase the knowledge of the health professional. MATERIAL AND METHODS: A descriptive retrospective study was conducted on patients seen in a pediatric emergency department after the ingestion of plant substances from January 2008 to December 2012. RESULTS: During the period of study, 18 patients had ingested possible toxic plants. In 14 cases, it was considered to be potentially toxic: broom, oleander, mistletoe, butcher's-broom, and vulgar bean (2), Jerusalem tomato, castor (2), Jimson weed, potus, marijuana, and mushrooms with digestive toxicity (2). Among the potentially toxic cases, the ingestion was accidental in 10 patients, 2 cases were classed as infantile mistreatment, 1 case had recreational intention, and another one suicidal intentions. The ingestion of oleander, castor and Jimson weed had major toxicity. CONCLUSIONS: The potential gravity of the ingestion of plant substances and the variety of the exposure mechanism requires the pediatrician to bear in mind this possibility, and to be prepared for its diagnosis and management. Specific preventive information measures need to be designed for the families and for the regulation of toxic plants in playgrounds

[The role of complementary examinations and home monitoring in patient at risk from apparent life threatening event, apneas and sudden infant death syndrome]. / Papel de las exploraciones complementarias y de la monitorización domiciliaria en los pacientes de riesgo de episodio aparentemente letal, apneas y síndrome de muerte súbita del lactante.

INTRODUCTION: Home apnea monitors detect abnormalities in cardiac and respiratory frequency, but their use in the diagnosis of respiratory -related sleep disturbances in children has not been demonstrated, as was originally thought. OBJECTIVE: To describe the type of patients being monitored, for how long and their outcome. MATERIAL AND METHODS: A retrospective descriptive study was conducted on patients with controlled home cardiorespiratory monitoring from October 2008 to September 2012 in the Outpatient department of a Maternity tertiary hospital. RESULTS: During the study period 88 patients were included, 58% of them were male, with a median age of 15.5 days, and followed up for a period of 4.7 months. The reason for monitoring was in a 20.5% due to a history of sudden death without finding underlying pathology in 20.5%, 25% due to apnea of prematurity, 20.5% due to apparent life-threatening event, and 14.8% due to choking. Other causes accounted for 19.3% (apnea/hypopnea, desaturation and periodic breathing). Of these last three groups, pathological events were observed in 50% of them: reflux disease (9), apnea of prematurity (2), neurological causes (3), and apnea of unknown cause (10). CONCLUSIONS: Suspected infant apnea is a cause for consultation that creates a great deal of concern to the family and the pediatrician. Home monitoring is useful in detecting changes in cardiac and respiratory frequency, but is necessary to limit its indications and ensure proper monitoring of these patients, avoiding the abuse of other tests or treatments.

[Poisonous plants: An ongoing problem]. / Tóxicos vegetales: un problema aún vigente.

INTRODUCTION: A medical visit for plant ingestion is rare in the pediatric emergency services but may involve a high toxicity. The botanical toxicology training of health staff is often very limited, and it can be difficult to make a diagnosis or decide on the appropriate treatment. OBJECTIVE: To study the epidemiological and clinical characteristics of poisoning due to plant ingestion in order to increase the knowledge of the health professional. MATERIAL AND METHODS: A descriptive retrospective study was conducted on patients seen in a pediatric emergency department after the ingestion of plant substances from January 2008 to December 2012. RESULTS: During the period of study, 18 patients had ingested possible toxic plants. In 14 cases, it was considered to be potentially toxic: broom, oleander, mistletoe, butcher's-broom, and vulgar bean (2), Jerusalem tomato, castor (2), Jimson weed, potus, marijuana, and mushrooms with digestive toxicity (2). Among the potentially toxic cases, the ingestion was accidental in 10 patients, 2 cases were classed as infantile mistreatment, 1 case had recreational intention, and another one suicidal intentions. The ingestion of oleander, castor and Jimson weed had major toxicity. CONCLUSIONS: The potential gravity of the ingestion of plant substances and the variety of the exposure mechanism requires the pediatrician to bear in mind this possibility, and to be prepared for its diagnosis and management. Specific preventive information measures need to be designed for the families and for the regulation of toxic plants in playgrounds.