ABSTRACT
El síndrome de Swyer-James-Mac Leod es una entidad poco frecuente adquirida en la infancia, generalmente tras una infección pulmonar moderada a grave de tipo bronquiolitis o neumonía, generalmente virales. Ocasionalmente se tiene el antecedente de infecciones repetidas de este tipo. Consiste en el desarrollo de enfisema hipoplásico pulmonar unilateral, que puede a veces relacionarse con bronquiectasias ipsilaterales o bilaterales, obstrucción fija al flujo aéreo y puede también asociarse a reducción del flujo sanguíneo del pulmón hipoplásico, de manera focal o difusa, con o sin tortuosidad de la vascularización proximal y a veces con una compensación del pulmón contralateral, en forma de sobredistensión e hiperflujo vascular relativo. Presentamos el caso de un varón de 79 años de edad con antecedentes de infecciones tipo bronquiolitis virales repetidas en la infancia, obstrucción fija grave al flujo aéreo y hemoptisis masiva secundaria a una infección por Pseudomonas aeruginosa sensible a la terapia antibiótica habitual.
Swyer-James-Mac Leod syndrome is a rare condition acquired in childhood, usually after a moderate to severe lung infection such as bronchiolitis or pneumonia, usually viral. Occasionally there is a history of repeated infections of this type. It consists of the development of unilateral pulmonary hypoplastic emphysema, which can sometimes be related to ipsilateral or bilateral bronchiectasis, fixed airflow obstruction, and may also be associated with reduced blood flow in the hypoplastic lung, with or without tortuosity of the proximal vascular supply and sometimes with compensation from the contralateral lung, in the form of overdistension and relative vascular hyperflow. We present the case of a 79-year-old man with a history of recurrent viral bronchiolitis-type infections in childhood, severe fixed airflow obstruction, and massive hemoptysis secondary to a Pseudomonas aeruginosa infection sensitive to usual antibiotic therapy.
Subject(s)
Humans , Male , Aged , Pulmonary Emphysema/complications , Lung, Hyperlucent/complications , Hemoptysis/etiology , Pulmonary Emphysema/therapy , Pulmonary Emphysema/diagnostic imaging , Radiography, Thoracic , Lung, Hyperlucent/therapy , Lung, Hyperlucent/diagnostic imaging , Computed Tomography AngiographyABSTRACT
Summary Swyer-James syndrome is a complication of post-infectious bronchiolitis obliterans that causes inflammation and fibrosis of the bronchial walls. There are two types: asymptomatic, with most cases diagnosed in adults during routine radiological examinations; and symptomatic, most commonly found in children. Here, we report the case of a 6-year-old child with recurrent dyspnea since the age of 3, who showed signs and symptoms of bronchiolitis obliterans and radiological signs of bronchial wall thickening and air trapping. The clinical and radiological findings led to the diagnosis of Swyer-James syndrome. Treatment of this syndrome is intended to reduce the pulmonary lesions and improve the patient's quality of life.
Resumo A síndrome de Swyer-James-Macleod é uma complicação da bronquiolite pós-infecciosa, ocasionando inflamação e fibrose das paredes dos bronquíolos. Pode se manifestar de duas formas: assintomática, sendo a maioria diagnosticada na fase adulta, quando o paciente se submete a exames radiológicos de rotina, e a forma sintomática, que é mais encontrada em crianças. Relatamos um caso de uma criança de 6 anos de idade com crises de dispneia de repetição desde os 3 anos, apresentando sinais e sintomas de bronquiolite obliterante e sinais radiológicos de espessamento brônquico e aprisionamento aéreo. Por meio da clínica e achados radiológicos, foi feito o diagnóstico de síndrome de Swyer-James-Macleod. O tratamento dessa síndrome visa a reduzir as lesões pulmonares e a melhorar a qualidade de vida do paciente.