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.

Agenesia de artéria pulmonar direita: relato de caso / Agenesis of right pulmonary artery: case report

RESUMO: A agenesia de artéria pulmonar é uma malformação congênita rara, que ocorre devido ao não desenvolvimento do sexto arco aórtico. Entretanto, não é relacionada com malformações cardíacas. Além das artérias intrapulmonares, a vascularização pulmonar e a árvore brônquica geralmente não sofrem alterações. No presente relato, a paciente apresenta quadro de febre, tosse produtiva e taquipneia. Devido à clínica, optou-se por realizar radiografia simples de tórax, na qual se evidenciou a presença de opacidade interstício-alveolar peri-hilar à direita com desvio do mediastino à direita e assimetria da transparência pulmonar. Então, foi solicitada tomografia computadorizada de tórax que evidenciou pulmão direito de volume reduzido. Para melhor compreensão, realizou-se angiotomografia computadorizada do tórax, a qual detectou agenesia da artéria pulmonar direita. Importância do problema: relatar um caso de agenesia de artéria pulmonar direita. (AU)

ABSTRACT: Pulmonary artery agenesis is a rare congenital malformation, which occurs due to the non-development of the sixth aortic arch. However, it is not related to cardiac malformation, and also the intrapulmonary arteries, as well as the pulmonary vascularization and the bronchial tree usually do not change. In the present case study, the patient presents fever, productive cough, and tachypnea. A simple chest X-ray was performed because of the clinic, demonstrating right perihilar interstitial-alveolar opacity with right mediastinal deviation and asymmetry of pulmonary transparency. Due to the radiological finding, a computed tomography of the chest was requested, which showed the right lung of reduced volume. To provide a better understanding of the condition, the radiology team suggested computed angiotomography of the chest, which detected agenesis of the right pulmonary artery. The importance of the problem is to report a case of agenesis of the right pulmonary artery. (AU)

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.

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 Mounier-Kuhn: achados radiológicos e apresentação clínica / Mounier-Kuhn syndrome: radiological findings and clinical presentation

A síndrome de Mounier-Kuhn é uma doença rara, caracterizada clinicamente por infecções respiratórias de repetição. Apresentamos um relato de caso desta doença com análise da radiografia e da tomografia computadorizada de alta resolução do tórax que mostram como principais alterações aumento do calibre da traqueia, brônquios principais e bronquiectasias centrais. Estas alterações, associadas às informações clínicas, sugerem o diagnóstico.

Mounier-Kuhn syndrome is a rare disease clinically characterized by recurrent respiratory infections. The present report describes a case of this disease with analysis of chest radiography and high resolution computed tomography showing increased caliber of the trachea, main bronchi and central bronchiectasis. Such changes, in association with clinical data, suggest the diagnosis.

Collateral Ventilation to Congenital Hyperlucent Lung Lesions Assessed on Xenon-Enhanced Dynamic Dual-Energy CT: an Initial Experience

OBJECTIVE: We wanted to evaluate the resistance to collateral ventilation in congenital hyperlucent lung lesions and to correlate that with the anatomic findings on xenon-enhanced dynamic dual-energy CT. MATERIALS AND METHODS: Xenon-enhanced dynamic dual-energy CT was successfully and safely performed in eight children (median age: 5.5 years, 4 boys and 4 girls) with congenital hyperlucent lung lesions. Functional assessment of the lung lesions on the xenon map was done, including performing a time-xenon value curve analysis and assessing the amplitude of xenon enhancement (A) value, the rate of xenon enhancement (K) value and the time of arrival value. Based on the A value, the lung lesions were categorized into high or low (A value > 10 Hounsfield unit [HU]) resistance to collateral ventilation. In addition, the morphologic CT findings of the lung lesions, including cyst, mucocele and an accessory or incomplete fissure, were assessed on the weighted-average CT images. The xenon-enhanced CT radiation dose was estimated. RESULTS: Five of the eight lung lesions were categorized into the high resistance group and three lesions were categorized into the low resistance group. The A and K values in the normal lung were higher than those in the low resistance group. The time of arrival values were delayed in the low resistance group. Cysts were identified in five lesions, mucocele in four, accessory fissure in three and incomplete fissure in two. Either cyst or an accessory fissure was seen in four of the five lesions showing high resistance to collateral ventilation. The xenon-enhanced CT radiation dose was 2.3 +/- 0.6 mSv. CONCLUSION: Xenon-enhanced dynamic dual-energy CT can help visualize and quantitate various degrees of collateral ventilation to congenital hyperlucent lung lesions in addition to assessing the anatomic details of the lung.

A case of Swyer-James Syndrome Successfully Treated with Methylprednisolone Pulse Therapy / 소아알레르기및호흡기학회지

Swyer-James syndrome (SJS) is characterized by a small- or normal-sized unilateral hyperlucent lung with decreased vascularity and air trapping on plain radiographs. SJS is considered to be a postinfectious form of bronchiolitis obliterans and has occurred following childhood pulmonary infection by adenovirus, measles, influenza virus, Bordetella pertussis, Mycobacterium tuberculosis or Mycoplasma pneumoniae. The standard treatment modality for SJS is supportive care. Despite the prominent role of inflammation in the pathogenesis of SJS, the use of corticosteroids has remained controversial. We report herein a case of SJS which was successfully treated with monthly methylprednisolone pulse therapy, resulting in complete remission as verified by high resolution computed tomography.

Pulmón hiperlucente / Lung hiperlucente

El pulmón hiperlucente unilateral también se ha denominado síndrome de Swyer-James, síndrome de Macleod o enfisema lobular o unilateral. Es una enfermedad poco frecuente que se caracteriza por un hiperclaridad pulmonar o lobar unilateral asociada a un atrapamiento aéreo en la espiración. En cuanto a la etiología, existe evidencia que se atribuye a una enfermedad adquirida que aparece tras una infección pulmonar durante la infancia, probablemente una bronquiolitis una neumonía viral. El cuadro clínico es variable: algunos pacientes se encuentran asintomáticos, otros con infecciones de vías respiratorias bajas a repetición, y los que presentan disnea de esfuerzo. El diagnóstico se suele realizar como un hallazgo incidental al hacer una radiografía de tórax a un niño por infecciones respiratorias repetidas, o en la edad adulta en una exploración radiológica de rutina, en una persona por lo demás asintomática. El diagnóstico diferencial de este síndrome se debe hacer con otras entidades torácicas que disminuyen la densidad radiológica pulmonar unilateral. A continuación se describe el caso clínico de un niño que es portador de pulmón hiperlucente.

Diagnóstico por escanografía de bronquiolitis constructiva postinfecciosa(Síndrome de Swyer-James) en un paciente con distribución atípica de edema pulmonar de las alturas / Diagnosis by CT-Scan of Swyer-James syndrome in patient with atypical distribution of high altitud pulmonary edema

Presentamos el caso de un paciente quien ingresa con un edema pulmonar de las alturas, de distribución atípica, en quien se demuestran cambios escanográficos de Síndrome de Swyer-James.

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.

Findings of chest radiograph and spiral computed tomography in Swyer-James syndrome / 中国医学科学杂志(英文版)

<p><b>OBJECTIVE</b>To evaluate the value of X-ray and spiral computed tomography (SCT) in the diagnosis of Swyer-James syndrome (SJS).</p><p><b>METHODS</b>A total of 28 patients, 12 males and 16 females, were studied retrospectively. Ages ranged from 11 to 57 years, the mean age was 32 years. All patients underwent inspiratory chest X-ray films, 5 with expiratory chest films and 1 with bronchogram. Furthermore, inspiratory and expiratory SCT scans were performed. The SCT findings were analyzed and compared with X-ray films.</p><p><b>RESULTS</b>SCT demonstrated 56 lobes with hyperlucency and diminished vascularity. The size of 51 lobes were smaller and 5 were normal. X-ray films showed that hyperlucency was only in 29 lobes, in which 19 lobes were small-sized and the other 10 lobes normal. There were 56 lobes with air-trapping on expiratory SCT scans, but only 5 lobes with air-trapping on expiratory X-ray films. Bronchogram in 1 case demonstrated bronchiectasis and bronchiolitis obliterans. SCT showed 24 patients with bronchiectasis, 9 patients with tuberculosis, 10 patients with bronchiolitis, and 2 with segmental collapse.</p><p><b>CONCLUSION</b>SCT scan is superior to chest radiography in the diagnosis and differential diagnosis of SJS.</p>

Síndrome de Swyer-James-Macleod / Swyer-James-Macleod syndrome

A Síndrome de Swyer-James é uma afecção relativamente rara, sendo que a maioria dos casos relatados na literatura foi descrita em pacientes adultos. Com o aprimoramento dos métodos diagnósticos, sobretudo dos de imagem, o diagnóstico na infância vem-se tornando mais freqüente. Como a abordagem dessa síndrome em livros-texto especializados é feita de maneira sucinta e os artigos publicados em periódicos são escassos, sobretudo na faixa etária pediátrica, muitas vezes o pediatra e o pneumologista infantil encontram dificuldades no tratamento desses pacientes. Este artigo tem como objetivo fazer uma revisão dessa síndrome, com ênfase especial nos métodos diagnósticos e aspectos terapêuticos.

Swyer-James syndrome, in spite of being a quite rare disease during childhood, is now being more easily diagnosed with the help the new image methods like computerized tomography and scintilography. A revision of this syndrome is made, with emphasis on diagnosis and treatment besides a brief historical review since its first description in 1952.

Clinical spectrum of Swyer-James-Macleod syndrome in adults

The aim of this study is to describe the clinical and imaging features of Swyer-James-Macleod syndrome [SJMS] in 9 adults. We reviewed the charts of 9 patients diagnosed with SJMS at the King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia over a 10 year period. The patients mean age was 38.1 years; males were more affected than females [7:2]. Seven of the patients had symptoms referable to the chest and a similar number had compatible abnormalities on physical examination. The left lung was involved in all cases. Bronchiectasis was present in 7 [77.8%]. Eight patients who underwent pulmonary function tests had combined defects. Two patients demonstrated significant reversibility. All patients had a stable course over at least before a 3 year follow-up period. Swyer-James-Macleod syndrome has a diverse manifestations in adults and can mimic other pulmonary disorders, which may lead to incorrect diagnosis and inappropriate therapy. The course is generally a stable one

Small Airway Disease after Mycoplasma Pneumonia in Children: HRCT Findings and Correlation with Radiographic Findings

PURPOSE: To assess the high-resolution CT (HRCT) findings of small airway abnormalities after mycoplasma pneumonia and correlate them with the findings of chest radiography performed during the acute and follow-up phases of the condition. MATERIALS AND METHODS: We retrospectively evaluated HRCT and chest radiographic findings of 18 patients with clinical diagnosis of small airway disease after mycoplasma pneumonia (M:F=8:10, mean age: 8.3 years, mean time interval after the initial infection; 26 months). We evaluated the lung parenchymal and bronchial abnormalities on HRCT (n=18). In addition, presence of air-trapping was assessed on expiratory scans (n=13). The findings of HRCT were correlated with those of chest radiography performed during the acute phase of initial infection (n=15) and at the time of CT examination (n=18), respectively. RESULTS: HRCT revealed lung parenchymal abnormalities in 13 patients (72%). A mosaic pattern of lung attenuation was noted in ten patients (10/18, 56%), and air-trapping on expiratory scans was observed in nine (9/13, 69%). In nine of 14 (64%) with negative findings at follow-up chest radiography, one or both of the above parenchymal abnormalities was observed at HRCT. In four patients (27%), parenchymal abnormalities were seen at HRCT in areas considered normal at acute-phase chest radiography. Bronchiectasis or ateclectasis was observed in eight (44%) and four (22%) patients, respectively, at HRCT. The CT features of Swyer-James syndrome such as a unilateral hyperlucent lung with reduced lung volume and attenuated vessels were noted in two patients (11%). CONCLUSION: HRCT can clearly demonstrate lung parenchymal and bronchial abnormalities of small airway disease after mycoplasma pneumonia in children.

Bronchogenic Squamous Cell Carcinoma in Patient with Swyer-James Syndrome: One Case Report / 대한흉부외과학회지

Swyer-James syndrome is a rare disease with patients presenting with unilateral hyperlucent lungs and hypoperfusion due to hypoplasia of the pulmonary artery and bronchiolitis obliterans. A unilateral hyperlucent lung generally develops after a lower respiratory tract infection during early childhood. In extremely rare cases, an association of bronchogenic carcinoma with Swyer-James syndrome has been reported. We report a case of bronchogenic squamous cell carcinoma associated with Swyer-James syndrome that performed right upper lobectomy and lymph node dissection with a relevant literature review.