Swyer-James Macleod Syndrome With Progressive Hyperinflation.

Swyer-James Macleod syndrome (SJMS) is a rare disorder characterized by unilateral lung or lobar hyperlucency on chest radiographs. We present a case of SJMS with progressive enlargement of the bulla. A lobectomy was performed because the bulla in the right middle lobe had enlarged and compressed the upper and lower lobes, resulting in severe dyspnea. This treatment improved severe dyspnea and pulmonary function. Surgical resection is a viable option for SJCM patients experiencing short-term progression.

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

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.

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 as a rare cause of unilateral hyperlucent lung: Three case reports.

RATIONALE: Swyer-James-Macleod syndrome (SJMS) is a rare lung disorder characterized by unilateral hyperlucent lungs which arises as a complication of bronchiolitis obliterans. It is typically diagnosed during childhood, although some patients may only be diagnosed in adulthood, often as an incidental finding, but others due to recurrent chest infections. PATIENT CONCERNS: Three patients were referred to our institution with complaints of dyspnea on exertion, chronic productive cough, and recurrent pulmonary infections. Two of them had a history of lower respiratory tract infections during childhood. DIAGNOSIS: A computed tomography scan was performed and showed unilateral reduced density of the lung and bronchiectasis in the 3 patients. Based on the clinical presentation and radiologic features, the diagnosis of SJMS was established. INTERVENTIONS: Patients started inhaled corticosteroids and long acting beta agonist, vaccines to prevent respiratory infections were administered and airway clearance techniques were taught. OUTCOMES: Two patients became asymptomatic with inhaled therapy and no recurrent chest infections were observed over a 3-year follow-up period, being discharged from our institution to the general practitioner. The 3rd patient had some improvement in the frequency of pulmonary infections and dyspnea, without improvement in respiratory function tests, maintaining vigilance at our center. LESSONS: These cases highlight the importance of being aware of this condition and its frequent association with bronchiectasis, which may adversely affect the prognosis, to manage patients appropriately and prevent recurrent pulmonary infections.

Swyer-James-Macleod Syndrome: Case Report and Brief Literature Review.

Swyer-James-Macleod syndrome is a rare radiologic entity with radiolucent lung fields which could include the entire lung, a lobe or a subsegment. The pathogenesis is unclear but is thought to be related to childhood pulmonary viral and non-viral infections with subsequent vascular and alveolar distortion. It usually presents incidentally in asymptomatic patients on imaging, but could present with recurrent pulmonary infections, shortness of breath, cough, hemoptysis or bronchiectasis. Treatment is usually observation if asymptomatic, but could potentially include inhaled steroids, bronchodilators, lobectomy segmentectomy or pneumonectomy depending on the clinical condition and severity of the symptoms. We present a case of SJMS found in a asymptomatic patient along with a brief literature review.

[Emphysema, did you say emphysema?] / Emphysème, vous avez dit emphysème ?

INTRODUCTION: Chronic obstructive pulmonary disease (COPD) is a common condition that may initially look simple but may conceal other diseases capable of accelerating its natural history or even simulating it. We describe four cases presenting as COPD with emphysema that were reclassified on the basis of certain clinical characteristics and the radiological pattern. CASE REPORTS: A 52 year old never smoking woman presenting with emphysema was eventually diagnosed as having lymphangioleiomyomatosis on the basis of an abdominal CT scan showing kidney angiomyolipomas. A 44 years old smoker presenting with rapidly evolving emphysema was eventually diagnosed as having Langerhans cell histiocytosis on the basis of a previous chest CT (four years earlier) showing cavitating nodules. An airport refueler, 73 years old, with severe emphysema despite never having smoked, was eventually diagnosed as suffering from alpha-1 antitrypsin deficiency. The last patient was a 54 year old man, a never smoker, who presented with severe airflow limitation and multilobar hyperlucency, with bronchiectasis in the same areas. He was eventually diagnosed as having a severe form of the Swyer-James-MacLeod syndrome. CONCLUSION: These four case reports underline the importance of questioning the diagnosis of COPD when certain particular phenotypic characteristics are identified.

[Referred with the working diagnosis 'pneumothorax: could it be another diagnosis?]. / Verwezen met de werkdiagnose 'pneumothorax': kan het ook iets anders zijn?

BACKGROUND: Patients who are referred to an emergency department (ED) often have a working diagnosis. Hospital physicians may be inclined to accept this diagnosis and incorrectly link the findings of investigations to it. This may lead to an incorrect definitive diagnosis and therapy. CASE DESCRIPTION: We present the case of a 36-year-old female who was referred to the ED with a pneumothorax seen on chest x-ray. Findings from history-taking, physical examination and a second chest x-ray taken on the ED were diagnosed as being consistent with pneumothorax and a chest-drain was inserted. However, further investigation showed that she had Swyer-James-Mcleod syndrome which could have been treated conservatively. CONCLUSION: This patient was given a chest drain after the working diagnosis of pneumothorax was made. Due to the definite diagnosis of Swyer-James-Mcleod the indication for chest drainage may have been incorrect. If a diagnosis of pneumothorax is suspected, but diagnostic findings suggest otherwise, further investigations should be performed before a chest drain is inserted.

Síndrome de Swyer-James-MacLeod. Presentación de 2 casos y consideraciones clínicas / Swyer-James-MacLeod syndrome. Two case reports and a clinical review

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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...

Recurrent pneumothorax related to Swyer-James syndrome.

A 21-year-old man with a history of bronchial asthma during childhood presented with left recurrent pneumothorax. Chest x-ray showed collapse of the left lung. Computed tomography showed hyperlucency and some bullae in the left upper lobe. Thoracoscopic bullectomy and pleurodesis were performed. Pneumothorax recurred twice, for which thoracoscopic bullectomy and pleurodesis were performed. During the third operation, pleurodesis of almost the entire left upper lobe was performed. Since this third operation, the patient has been free from recurrences of pneumothorax for three years. The lung perfusion scintigram performed during the absence of pneumothorax showed a lack of accumulation in the left upper lobe. The lung ventilation scintigram performed during the absence of pneumothorax showed low accumulation and delay of washout in the left upper lobe. These findings are compatible with Swyer-James syndrome. To prevent the recurrence of pneumothorax related to Swyer-James syndrome, some kind of procedure, such as extensive pleurodesis, is necessary.

Adult diagnosis of Swyer-James-Macleod syndrome: retrospective analysis of four cases.

Swyer-James-Macleod syndrome (SJMS) is a rare constrictive bronchiolitis with air-flow obstruction and a decreased number and diameter of ipsilateral peripheral pulmonary vessels. This syndrome is characterized by unilateral hyperlucency on chest radiography. Computed tomography provides useful additional information. The diagnosis is usually made in childhood but sometimes occurs in adulthood. The disease often presents with dyspnea, decreased exercise tolerance, cough, hemoptysis, and recurrent pulmonary infections. SJMS may be confused with asthma or pulmonary embolism due to similar symptoms and may result in inappropriate therapy. This case series examined the clinical and imaging spectrum of four patients who were diagnosed with SJMS in adulthood.

The vanishing lung: an important cause of hyperlucency on chest radiograph.

Giant bullous emphysema is an uncommon condition characterised by large asymmetric bullae with upper lobe predominance. This condition is most frequent in young male smokers. Patients usually present with progressive breathlessness which is secondary to enlargement of the bullae leading to compression of the lung parenchyma. Large asymmetrical bullae may appear as a unilateral hyperlucency on a plain chest radiograph, and may mimic the appearances of pneumothorax. A computed tomography scan is needed to delineate the lung pathology. We describe two cases with this condition that presented acutely and discuss the management of bullous emphysema.

[Management of a child with Swyer-James syndrome who underwent flexible bronchoscopy under general anesthesia].

Swyer-James syndrome (SJS) is a rare syndrome characterized by hyperlucency of one or more lobes of the unilateral lung and decreased pulmonary vascularity which is considered to be secondary to the childhood obliterative bronchiolitis. We report anesthetic management of a girl with SJS who underwent flexible bronchoscopy. A 15-year-old girl weighing 47 kg was referred to our hospital with suspected SJS. She had a history of bronchiolitis in early childhood. On admission, chest radiograph showed a hyperlucent lung with diminished pulmonary vasculature on the left lung. Pulmonary function test revealed slightly restrictive pattern (%VC 69.3%, %FEVa1.0 77.9%). Ventilation and perfusion scanning demonstrated the markedly decreased ventilation (left : right, 33 : 67) and perfusion (left : right, 10 : 90) of the left lung. To rule out the foreign body and bronchial stenosis, flexible bronchoscopy under general anesthesia was planned. Anesthesia was induced with propofol and maintained with propofol and remifentanil. Spontaneous respiration was maintained by using laryngeal mask airway to prevent the pressure alveolar damage by positive pressure ventilation. Flexible bronchoscopy was performed safely by using topical laryngeal lidocaine administration and additional bolus of propofol. Her postoperative course was uncomplicated and she was discharged on the following day.