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.

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.

Chronic lung allograft dysfunction phenotype and prognosis by machine learning CT analysis.

BACKGROUND: Chronic lung allograft dysfunction (CLAD) is the principal cause of graft failure in lung transplant recipients and prognosis depends on CLAD phenotype. We used a machine learning computed tomography (CT) lung texture analysis tool at CLAD diagnosis for phenotyping and prognostication compared with radiologist scoring. METHODS: This retrospective study included all adult first double lung transplant patients (January 2010-December 2015) with CLAD (censored December 2019) and inspiratory CT near CLAD diagnosis. The machine learning tool quantified ground-glass opacity, reticulation, hyperlucent lung and pulmonary vessel volume (PVV). Two radiologists scored for ground-glass opacity, reticulation, consolidation, pleural effusion, air trapping and bronchiectasis. Receiver operating characteristic curve analysis was used to evaluate the diagnostic performance of machine learning and radiologist for CLAD phenotype. Multivariable Cox proportional hazards regression analysis for allograft survival controlled for age, sex, native lung disease, cytomegalovirus serostatus and CLAD phenotype. RESULTS: 88 patients were included (57 bronchiolitis obliterans syndrome (BOS), 20 restrictive allograft syndrome (RAS)/mixed and 11 unclassified/undefined) with CT a median 9.5 days from CLAD onset. Radiologist and machine learning parameters phenotyped RAS/mixed with PVV as the strongest indicator (area under the curve (AUC) 0.85). Machine learning hyperlucent lung phenotyped BOS using only inspiratory CT (AUC 0.76). Radiologist and machine learning parameters predicted graft failure in the multivariable analysis, best with PVV (hazard ratio 1.23, 95% CI 1.05-1.44; p=0.01). CONCLUSIONS: Machine learning discriminated between CLAD phenotypes on CT. Both radiologist and machine learning scoring were associated with graft failure, independent of CLAD phenotype. PVV, unique to machine learning, was the strongest in phenotyping and prognostication.

Pneumonectomy in an Older Patient With Swyer-James-MacLeod Syndrome With a Giant Bulla.

Swyer-James-MacLeod syndrome is a rare lung disease that appears as unilateral pulmonary hyperlucency on chest radiographs and is considered to occur as a result of childhood bronchiolitis obliterans. We report the case of a 54-year-old woman who experienced exertional dyspnea owing to Swyer-James-MacLeod syndrome associated with a giant calcified bulla that occupied the entire hemithorax. Right pneumonectomy was performed because of progressive pulmonary dysfunction caused by the compression of the bulla on the left lung. This treatment dramatically improved both her pulmonary function and symptoms. Swyer-James-MacLeod syndrome is a rare disease characterized by unilateral pulmonary hyperlucency on chest radiography. In this case, Swyer-James-MacLeod syndrome associated with a giant calcified bulla that compressed the patient's left lung was treated with right pneumonectomy.

A Case Report of Congenitally Absent Pericardium Masquerading as Recurrent Pericarditis.

A 24-year-old female with a history of Swyer-James-MacLeod syndrome presented with acute onset of pleuritic chest pain and was initially diagnosed with acute pericarditis. The 12-lead electrocardiogram demonstrated typical diffuse ST-segment elevation and PR-segment depression. Symptoms resolved rapidly with anti-inflammatory therapy consisting of ibuprofen and colchicine. After completing a 3-month course of the latter, her symptoms rapidly recurred. Workup, including labs and cardiac imaging consisting of a transthoracic echocardiogram and cardiac magnetic resonance imaging, was initially interpreted as normal. Re-review of her cardiac imaging revealed the patient had signs of a congenitally absent pericardium, including a "Snoopy Sign" on her posterior-anterior chest X-ray, which is characterized by levoposition of the cardiac silhouette, a lucent area between the pulmonary artery and aorta because of the presence of lung tissue, a lucent area between the base of the heart and the left hemidiaphragm, loss of the right heart border, a prominent pulmonary artery, and a flattened and elongated left ventricular contour. The patient had a cardiac computed tomography scan, which confirmed the diagnosis. In conclusion, a congenitally absent pericardium is a rare disorder, often undetected or misdiagnosed. There are characteristic findings on imaging such as a "Snoopy Sign" on a posterior-anterior chest X-ray, which can be easily missed because of its rarity. Our goal of this report is to educate health care providers about this rare disorder.

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.

Complication of post-infectious bronchiolitis obliterans (Swyer-James syndrome).

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.

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.

VATS bullectomy and apical pleurectomy for spontaneous pneumothorax in a young patient with Swyer-James-Mc Leod syndrome: case report presentation and literature review focusing on surgically treated cases.

BACKGROUND: Swyer-James-McLeod Syndrome (SJMS) is an uncommon, emphysematous disease characterized by radiologic hyperlucency of pulmonary parenchyma due to loss of the pulmonary vascular structure and to alveolar overdistension. CASE REPORT: We herein describe a 15-year-old Caucasian patient with well-established SJMS since childhood who presented with spontaneous pneumothorax. Video-assisted thoracoscopic bullectomy with apical pleurectomy was performed. Since SJMS is considered an on-going inflammatory process, the patient one year after surgery exhibits excellent quality of life with no pneumothorax recurrence.

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.

Bronquiolitis obliterante postinfecciosa con presentación radiológica como pulmón hiperclaro unilateral / Postinfective obliterative bronchiolitis presenting as a hyperlucent lung

El síndrome de Swyer-James McLeod, o pulmón hiperclaro unilateral idiopático, es una entidad fundamentalmente radiológica caracterizada por una hiperclaridad de uno o más lóbulos, o de un pulmón entero. El tamaño pulmonar puede ser normal o reducido. Existe una desviación mediastínica hacia el lado afectado durante la inspiración, con disminución del movimiento diafragmático homolateral; se observa una escasa vascularización del pulmón, con un patrón broncográfico peculiar, que puede acompañarse, como en este caso, de bronquiectasias. Se presenta el caso de un varón diagnosticado a los 2 meses de vida de bronquiolitis, que evolucionaría hacia el desarrollo de este síndrome(AU)

Swyer-James McLeod syndrome or unilateral hyperlucent lung is basically a radiologic entity, defined by one or more hyperlucent lung lobus, or a complete hyperlucent lung. Lung size may be normal or reduced. There is a mediastinal deviation towards the affected hemithorax during inhalation and a limited diaphragmatic movement. Poor lung vascularization is also appreciated and a characteristic bronchographyc pattern. It may be associated to bronchiectasias as it happened in our patient. We introduce a case of a two months old male infant diagnosed of serious RSV + bronchiolitis, needing intensive care(AU)

Swyer-James-McLeod's syndrome and pneumothorax on same side: delay in chest drain removal despite full expansion.

Swyer-James-McLeod's syndrome (SJMS) occurs as a result of childhood recurrent respiratory infections. As this condition presents with few symptoms, if any, it may pass unnoticed until adulthood. The authors are presenting a case of a 17-year-old male admitted with spontaneous pneumothorax. Even though his lung expanded, his chest x-rays still mimicked a pneumothorax. Chest CT pulmonary angiogram was needed to confirm the diagnosis of unilateral SJMS. Diagnostic difficulty arose because both conditions were on the same side.

Síndrome de Swyer-James-McLeod; descripción de un caso y breve revisión de la literatura / Swyer-James-McLeod Syndrome: a case report and brief review of the literature

Resumen. El síndrome de Swyer-James-McLeod se caracteriza por ser una patología adquirida, generalmente de causa infecciosa, en las primeras etapas de la vida. Ello provoca una hipoplasia del pulmón afectado, con características anatomopatológicas que se describen como una bronquiolitis obliterante adquirida. La clínica que presentan los pacientes es muy variada, desde formas paucisintomáticas hasta cuadros graves con infecciones respiratorias severas de repetición (AU)

Abstract. The Swyer-James-McLeod syndrome is characterized by being an acquired condition, generally due to an infectious cause, in the early stages of life. It causes hypoplasia of the affected lung, with anatomic-pathological characteristics that are described as acquired bronchiolitis obliterans. The symptoms presented by the patients vary greatly, going from paucisymptomatic forms to severe pictures with recurrent severe respiratory infections (AU)

Swyer James syndrome: a case report.

Swyer James syndrome is a rare condition clinically characterized by bronchiolitis and pneumonitis early in life. A 2 year old girl referred to us with complaints of wheezing, chronic cough and fever since birth time. Based to her clinical symptoms and radiograph and CT scan findings, diagnosis of Swyer James syndrome was confirmed. She is under supportive treatment and her respiratory symptoms have been improved.

Regression of giant bullous emphysema.

Spontaneous resolution of bulla associated with infection or tumor is occasionally observed. However, resolution of progressively enlarging giant bullous emphysema (GBE) after medical therapy has not been reported. A 51-year-old man smoker with GBE on the right lung was referred to Samsung Medical Center for the consideration of bullectomy. A review of his medical records revealed that right-side bullous emphysema had been detected 4 years previously and it had progressively enlarged with a concomitant deterioration of lung function. Although he had a history of chronic asthma, he had never been treated on a regular basis. After combination therapy, including regular tiopropium, a salmeterol/flucatisone inhaler, a salbutamol inhaler as needed, and oral theophylline therapy, right side bulla showed marked regression. This regression of bulla was associated with an improved forced expiratory volume in one second and normalization of residual volume. This case serves as a reminder to clinicians that medical treatment for underlying diseases should precede surgical consideration in patients with GBE.

A child with respiratory distress having unilateral obstructive emphysema and contralateral opaque hemithorax on chest radiograph: role of multi-detector computerized tomography with multi-planar reconstruction and virtual bronchoscopy.

We describe a case of mucus plug in the right main bronchus with ipsilateral obstructive emphysema and contralateral opaque hemithorax in an 18-month-old girl who presented with respiratory distress. The role of multi-detector CT scan with multi-planar reconstructions and virtual bronchoscopy is highlighted.

Hemoptisis como manifestación clínica en el síndrome de Swyer- James / Macleod / Hemoptysis as clinical manifestation in Swyer-James (Macleod) Syndrome

El síndrome de Swyer-James o de MacLeod es una enfermedad infrecuente que asocia hipoperfusión pulmonar y atrapamiento aéreo con enfisema. En la edad adulta suele pasar desaparecido por su escasa sintomatología y puede diagnosticarse tras el hallazgo en una radiografía simple de tórax una imagen de hiperclaridad pulmonar unilateral. Presentamos un caso que destaca por su inhabitual forma de presentación clínica con hemoptisis masiva, no referida en la literaturamédica previamente según nuestra revisión, y que fue controlada con éxito mediante angiografía y embolización de los vasos anómalos. Después de cuatro años de seguimiento en la consulta externa no volvió a ocurrir de nuevo ningún episodio de hemoptisis y la paciente realiza su vida normal

Swyer-James or MacLeod syndrome is an infrequent disease associated with pulmonary hypoperfussion, air trapmentand emphysema. At adult age this disease can pass unnoticed because of its scarce symptoms but can be diagnosed in a plain chest radiograph showing a unilateral pulmonary hyperlucency. We present a case with an unusual clinic debut: massive hemoptysis. Such way of presentation was not refered previously in the medical literature according to our review.The patient was successful managed through angiography and embolization of the anomalous vessels. After four years of follow-up at the outpatient clinic no hemoptysis occurred again, and she can keep a normal activity life1