Reporte de caso: hemoptisis masiva como manifestación clínica de un síndrome de Swyer-James-Mac Leod / Case report: massive hemoptysis as a clinical manifestation of Swyer-James-Mac Leod syndrome

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.

Hiperclaridad pulmonar unilateral en un niño: Síndrome de Swyer-James-Macleod / A pediatric clinical case of unilateral hyperlucency of the lung: Swyer-James-Macleod syndrome

El síndrome de Swyer-James-MacLeod es un cuadro pulmonar muy infrecuente que se caracteriza radiológicamente por hiperclaridad pulmonar lobar o multilobar, producido por la disminución de la vascularización pulmonar, por lo general, por un proceso infeccioso previo. En ocasiones, es un hallazgo casual en el estudio por infecciones respiratorias o crisis asmáticas repetidas. Se presenta el caso de un paciente de 6 años de edad que, en el contexto de probable neumonía persistente, se realizó un amplio estudio que permitió el diagnóstico del síndrome de Swyer-James-MacLeod. Se desarrolló un abordaje terapéutico multidisciplinar con el que, hasta la fecha, la evolución ha sido satisfactoria, con tratamiento médico y fisioterapia respiratoria.

Swyer-James-MacLeod syndrome is a rare pulmonary disorder that is characterized radiologically by pulmonary lobar or multilobar hyperlucency produced by decreased pulmonary vascularization usually due to a previous infectious process. It is frequently a casual finding in recurrent pulmonary infections or in the study of asthma symptoms. We present the case of a 6-year-old patient who was diagnosed with Swyer-James-MacLeod syndrome after a persistent pneumoniae. A multidisciplinary approach was conducted, with a satisfactory progress up to date, combining medical treatment with respiratory physiotherapy.

Unrecognized hyperlucent lesion on lateral film of chest X-Ray

We report an interesting case of bulla right lung, incidently found during CABG surgery.

Complication of post-infectious bronchiolitis obliterans (Swyer-James syndrome) / Complicação de bronquiolite obliterante pós-infecciosa (Síndrome de Swyer-James-Macleod)

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.

Síndrome de Swyer James-Mcload: relato de casos / Swyer James-Mc Load syndrome: case reports

A síndrome de Swyer James-Mc Load é uma entidade rara descrita pela primeira vez em 1953 sendo caracterizada por hipoplasia ou agenesia das artérias pulmonares, resultando em hiperlucência pulmonar unilateral. Embora a patogênese ainda não tenha sido completamente elucidada, acredita-se que seja uma complicação pós-infecciosa de bronquiolites ocorridas na infância. O objetivo deste estudo estudo foi analisar a melhor conduta terapêutica na síndrome de Swyer James-Mc Load e o impacto na qualidade de vida das pessoas acometidas. Relata-se dois casos desta síndrome diagnosticados em pacientes adultos, sendo que uma delas apresenta asma persistente grave e a outra é ex-tabagista e já realizou cirurgia para ressecção de um nódulo pulmonar no pulmão contralateral ao acometido. A terapêutica conservadora foi capaz de estabilizar a condição clínica dos pacientes, mantendo uma boa qualidade de vida. No entanto, pacientes que não se estabilizam com tal terapêutica provavelmente devem ser incluídos à intervenção invasiva, visto que fornece ao paciente melhora na qualidade e expectativa de vida. Uma breve revisão da literatura pertinente foi realizada para melhor compreensão do assunto...

The Swyer-James Mc Load syndrome is a rare entity first described in 1953 and is characterized by hypoplasia or agenesis of the pulmonary arteries, resulting in unilateral lung hyperlucency. Although the pathogenesis has not yet been fully elucidated, it is believed to be a post-infectious complication of bronchiolitis occurring in childhood. The objective of this article was analyzing the best treatment for SSJM and its impact on affected people's lives. We report two cases of this syndrome diagnosed in adult patients, one of whom has severe persistent asthma and the other is a former smoker and has undergone surgery for resection of a pulmonary nodule in the contralateral lung to the affected. We concluded that conservative therapy is able to stabilize the clinical condition of patients maintaining a good quality of life. However, patients who do not stabilize with such therapy are likely to be included in invasive intervention, since it improves the quality of life and life expectancy of the patient. A brief review of the literature was conducted to better understand the subject...

Síndrome de Swyer-James: Reporte de un caso / Swyer-James Syndrome: Case report

El síndrome de Swyer-James-MacLeod (SSJM) ocurre como el resultado de una bronquio-litis obliterante en la niñez. Generalmente, este desorden es diagnosticado en niños, después de evaluar infecciones respiratorias recurrentes. Una de las razones para explicar la dificultad en el diagnóstico es que cuando los pacientes desarrollan pequeñas bronquiecta-sias y pocos síntomas no es fácil reconocer sino hasta la edad adulta. Existen pocas referencias de la enfermedad en niños, por lo que consideramos de interés su descripción. Se presenta el caso de un paciente de 4 años con síntomas respiratorios de neumonía aguda y se señalan los aspectos diagnósticos de mayor relevancia clínica actual.

Swyer-James-MacLeod Syndrome (SJMS) occurs as a result of childhood bronchiolitis obliterans. Typically, this disorder is diagnosed in childhood after evaluations for recu-rrent respiratory infections. One of the reasons to explain the difficulty in diagnosis is that when patients develop little bronchiectasis, and therefore, few symptoms, then this syndrome may not be recognized until adulthood. There are few references of the disease in children sowe think your description is interes-ting, we present the case of a 4 year old patient with acute respiratory symptoms of pneumonia, high lighting the most important aspects of clinical diagnosis today.

Aplicação terapêutica da ventilação colateral com drenagem pulmonar no tratamento do enfisema pulmonar difuso: relato dos três primeiros casos / Therapeutic application of collateral ventilation with pulmonary drainage in the treatment of diffuse emphysema: report of the first three cases

OBJETIVO: Relatar os resultados obtidos no pré- e pós-operatório de três pacientes portadores de enfisema pulmonar difuso grave, empregando uma nova técnica: ventilação colateral com drenagem do parênquima pulmonar. MÉTODOS: Para a avaliação da drenagem pulmonar, foram selecionados pacientes que já haviam sido submetidos à terapêutica clínica máxima, incluindo a reabilitação pulmonar, e que ainda assim sofriam de falência respiratória com dispnéia incapacitante. Os pacientes foram submetidos, no pré- e no pós operatório, à pletismografia e ao teste da caminhada de seis minutos, assim como responderam aos seguintes questionários de qualidade de vida: Medical Outcomes Study 36-item Short-Form Health Survey, Saint George's Respiratory Questionnaire, Eastern Cooperative Oncology Group Performance Status e Medical Research Council Scale. Todos os pacientes tiveram seguimento de no mínimo 300 dias de pós-operatório. Os testes foram realizados no pré-operatório, entre 30 e 40 dias de pós-operatório e após 300 dias de pós-operatório. Os dados foram analisados pelo método de gráficos de perfis de médias. RESULTADOS: Quando comparados os resultados do pré-operatório com os do pós-operatório nos dois momentos, verificou-se que houve melhora em todos os parâmetros estudados. CONCLUSÕES: Os resultados sugerem que a técnica operatória proposta para o tratamento de doentes portadores de enfisema pulmonar difuso grave foi capaz de diminuir os sintomas debilitantes destes pacientes, tornando sua qualidade de vida muito melhor.

OBJECTIVE: To report the results obtained in three patients with diffuse pulmonary emphysema during the pre- and post-operative periods following a new surgical technique: collateral ventilation with lung parenchyma drainage. METHODS: Patients suffering from pulmonary failure and disabling dyspnea, despite having received the gold standard treatment, including pulmonary rehabilitation, were selected for the evaluation of pulmonary drainage. During the pre- and post-operative periods, patients were submitted to plethysmography and six-minute walk tests, as well as completing the following quality of life questionnaires: Medical Outcomes Study 36-item Short-Form Health Survey, Saint George's Respiratory Questionnaire, Eastern Cooperative Oncology Group Performance Status and Medical Research Council Scale. In all three cases, the postoperative follow-up period was at least 300 days. The tests were performed at the following time points: during the pre-operative period; between post-operative days 30 and 40; and on post-operative day 300. Data were analyzed using profile plots of the means. RESULTS: When the results obtained in the pre-operative period were compared to those obtained at the two post-operative time points evaluated, improvements were observed in all parameters studied. CONCLUSIONS: The results suggest that the surgical technique proposed for the treatment of patients suffering from severe diffuse emphysema successfully reduces the debilitating symptoms of these patients, improving their quality of life considerably.

Aplicação terapêutica da ventilação colateral no enfisema pulmonar difuso: apresentação de um protocolo / Therapeutic application of collateral ventilation in diffuse pulmonary emphysema: study protocol presentation

Apresentação de um protocolo, para testar uma nova opção de tratamento operatório nos doentes portadores de enfisema pulmonar difuso, nos quais a terapêutica clínica máxima, incluindo a reabilitação pulmonar, foi realizada e ainda assim, existe falência respiratória com dispnéia incapacitante. Serão avaliados dez doentes portadores de hiperinsuflação pulmonar grave. O método propõe promover passagens expiratórias alternativas à via aérea principal para o ar aprisionado no pulmão enfisematoso, por meio de uma drenagem do parênquima pulmonar, comunicando os alvéolos ao meio exterior. Serão selecionados dez doentes, com os consentimentos informados assinados, e com a aprovação do Comitê de Ética em Pesquisa da Faculdade de Ciências Médicas da Santa Casa de São Paulo. Os doentes selecionados deverão obedecer os critérios de inclusão para participar deste estudo. O protocolo de avaliação do procedimento proposto é viável e ao final será capaz de mostrar, se de fato há ou não benefício para um doente debilitado e sofrido, quando hoje as únicas soluções são o transplante de pulmão ou a cirurgia redutora de volume pulmonar.

We present a protocol to test a new surgical procedure for the treatment of patients with diffuse lung emphysema who, after having received the golden standard treatment (pulmonary rehabilitation), continue to present respiratory failure with disabling dyspnea. Ten patients with severe lung hyperinflation will be evaluated. The method proposed is designed to create alternative expiratory passages for air trapped in the emphysematous lung by draining the lung parenchyma, thereby establishing communication between the alveoli and the external environment. The ten patients selected will be required to meet the inclusion criteria and to give written informed consent. Those ten patients will be included in the study pending the approval of the Ethics in Research Committee of the São Paulo Santa Casa School of Medicine, São Paulo, Brazil. The protocol we will employ in order to evaluate the proposed procedure is feasible and will show whether debilitated patients suffering from diffuse pulmonary emphysema can benefit from this procedure, which could represent an alternative to lung transplant or lung volume reduction surgery, the only options currently available.

Clinical Features of Swyer-James Syndrome / 소아알레르기및호흡기학회지

PURPOSE: Swyer-James syndrome is an uncommon abnormality characterized radiographically by a hyperluncent lobe or lung and functionally by normal or reduced volume during inspiration and air trapping during expiration. The condition typically follows certain respiratory infections in infancy or early childhood. Thus, it is a postinfectious form of bronchiolitis obliterans. Most patients have chronic cough, sputum, and abnormal breathing sounds, and present with repeated pulmonary infections. The syndrome affects pulmonary functions and may cause anatomical changes such as bronchiectasis and emphysema. We reviewed cases of Swyer-James syndrome, in order to better understand the clinical manifestations and outcomes of the disease. METHODS: The diagnostic criteria were small or normal-sized unilateral hyperlucent lung with air-trapping during expiration based on chest X-ray or CT and history of severe lung infection. A total of 12 patients were found. Medical records, radiological studies, and other clinical test results were reviewed. RESULTS: Most of the patients had chronic respiratory symptoms and signs. All patients had pneumonia or bronchiolitis as initial insult. Microbial agents were determined in six patients, of whom three were adenovirus and the others, Mycoplasma pneumoniae. They had restrictive and obstructive patterns of pulmonary function with bronchial hyperresponsiveness for methacholine. Two patients underwent lobectomy for emphysema and bullous emphysema, respectively. CONCLUSION: In this study, Swyer-James syndrome is a severe chronic pulmonary disease, presenting with respiratory symptoms, and functional and anatomical changes including bronchiectasis and emphysema. Further rejearch is needed to investigate initial etiologic agents and pathogenesis; further research is also needed for the care of chronic respiratory problems.

A Case of Swyer-James Syndrome / 소아알레르기및호흡기학회지

The Swyer-James Syndrome is a acquired pulmonary disease following bronchiolitis obliterans in young aged children, in which characteristic unilateral hyperlucence radiologically. Since Swyer and James first reported a case in 1953, it also has been named unilateral hyperlucent lung syndrome or MacLeod syndrome. Frequently there are repeated pulmonary infections which cause bacterial pneumonia and brochiectasis. In cases of pulmonary symptoms, it is diagnosed at early childhood, but there will be delayed detection until later ages in asymptomatic ones unless occasional chest radiography. Altogether the syndrome begins at childhood and manifested decreased exercise tolerance and failure to thrive, which means important disorder in pediatrics. Presently we experienced Swyer-James syndrome of 4 years old boy who had productive cough for 1 month diagnosed with a plain chest X-ray, lung computerized tomography and isotope lung scanning.

A Case of Unilateral Hyperlucent Lung by Main Bronchus Obstruction / 결핵

A 32-year-old woman was admitted to the hospital complaining of cough, sputum, and chest discomfort for the past ten days. The radiologic findings were transradiant left lung with reduced number and size of vessels, mediastinal shifting to the right at expiration, matched entilation-perfusion defect on ventilation-perfusion scan, and diffuse hypoplasia of the left pulmonary artery and its branches on pulmonary angiography. We describe a case of unilateral hyperlucent lung by main bronchus obstruction in a patient who presents with a clinical picture suggestive of Swyer-James syndrome.

A case of pulmonary disease with unilateral hyperlucent lung / 결핵

The Swyer-James syndrome is a relatively uncommon disease entity presented with unilateral hyperlucent lung due to hypoplasia of a pulmonary artery and bronchiectasis of the affected lung. The main finding is a hyperlucent lung with small hilar shadows on the chest X-ray. Pulmonary angiography is the standard method for diagnosis. We report a case of the Swyer-James syndrome with a brief review of literature.