RESUMO
INTRODUCCIÓN: La hipoplasia pulmonar es la anomalía congénita más frecuentemente asociada a mortalidad perinatal. MATERIAL Y MÉTODOS: Se ha realizado un estudio descriptivo retrospectivo de los casos de hipoplasia pulmonar diagnosticados entre 1995 y 2014 en un hospital universitario de tercer nivel, analizando estudios prenatales, manifestaciones clínicas, pruebas diagnósticas, tratamiento, datos de seguimiento a largo plazo y supervivencia. RESULTADOS: Se identificaron 60 casos, todos con estudio prenatal. Dieciséis recibieron intervención quirúrgica intraútero. La hernia diafragmática congénita fue la entidad más frecuentemente asociada. La manifestación clínica más habitual fue distrés respiratorio neonatal, hipoxemia grave y necesidad de soporte con ventilación mecánica. Se halló un 47% de mortalidad antes de los 60 días de vida y del 75% en las primeras 24 h de vida. Durante el seguimiento de los supervivientes se detectaron episodios de neumonías y bronquitis recidivantes, función pulmonar con patrón obstructivo y aceptable calidad de vida y tolerancia al ejercicio. CONCLUSIONES: La elevada mortalidad neonatal y la importante morbilidad a largo plazo de la hipoplasia pulmonar requieren de un diagnóstico temprano y la intervención de un equipo multidisciplinar especializado
INTRODUCTION: Pulmonary hypoplasia is the most frequent congenital anomaly associated with perinatal mortality. MATERIAL AND METHODS: A retrospective and descriptive review was conducted on cases of patients diagnosed with pulmonary hypoplasia between 1995 and 2014 in a tertiary university hospital. An analysis was made of the prenatal imaging, clinical manifestations, post-natal diagnostic tests, treatment and management, long-term follow up, and survival data. RESULTS: A total of 60 cases were identified, all of them with prenatal imaging. Sixteen patients required foetal surgery. Congenital diaphragmatic hernia was the most frequent diagnosis. Main clinical presentation was respiratory distress with severe hypoxemia and high requirements of mechanical ventilation. Mortality rate was 47% within first 60 days of life, and 75% for the first day of life. Pneumonia and recurrent bronchitis episodes were observed during follow-up. They had a lung function obstructive pattern, and their quality of life and exercise tolerance was good. CONCLUSIONS: High neonatal mortality and significant long-term morbidity associated with pulmonary hypoplasia requires an early diagnosis and a specialised multidisciplinary team management
Assuntos
Humanos , Masculino , Feminino , Criança , Hérnia Diafragmática/complicações , Hérnia Diafragmática/genética , Pulmão/anormalidades , Anormalidades Congênitas/genética , Anormalidades Congênitas/fisiopatologia , Respiração Artificial/instrumentação , Respiração Artificial/métodos , Respiração Artificial , Estudos Retrospectivos , Qualidade de Vida , Idade Gestacional , Espirometria/métodos , Espirometria , Artrogripose/complicações , Estimativa de Kaplan-MeierRESUMO
INTRODUCTION: Pulmonary hypoplasia is the most frequent congenital anomaly associated with perinatal mortality. MATERIAL AND METHODS: A retrospective and descriptive review was conducted on cases of patients diagnosed with pulmonary hypoplasia between 1995 and 2014 in a tertiary university hospital. An analysis was made of the prenatal imaging, clinical manifestations, post-natal diagnostic tests, treatment and management, long-term follow up, and survival data. RESULTS: A total of 60 cases were identified, all of them with prenatal imaging. Sixteen patients required foetal surgery. Congenital diaphragmatic hernia was the most frequent diagnosis. Main clinical presentation was respiratory distress with severe hypoxemia and high requirements of mechanical ventilation. Mortality rate was 47% within first 60 days of life, and 75% for the first day of life. Pneumonia and recurrent bronchitis episodes were observed during follow-up. They had a lung function obstructive pattern, and their quality of life and exercise tolerance was good. CONCLUSIONS: High neonatal mortality and significant long-term morbidity associated with pulmonary hypoplasia requires an early diagnosis and a specialised multidisciplinary team management.
