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.

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.

Swyer-James-MacLeod Syndrome in a Patient with Eisenmenger Syndrome.

Swyer-James-MacLeod syndrome is characterized radiologically by hyperlucency in a single lung lobe, accompanied by reduced vascularity, alveolar hyperdistention, and air trapping, without bronchial airway obstruction. The most common congenital heart defect in childhood, ventricular septal defect, leads to irreversible pulmonary hypertension and Eisenmenger syndrome if not treated promptly. This case report presents a 25-year-old patient with Swyer-James-MacLeod syndrome and Eisenmenger syndrome. It is crucial to include Swyer-James-MacLeod syndrome in the differential diagnosis of patients with atypically distributed pulmonary emphysema and unilateral hyperlucency for early diagnosis and timely intervention.

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.

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.

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.

Findings of chest radiograph and spiral computed tomography in Swyer-James syndrome.

OBJECTIVE: To evaluate the value of X-ray and spiral computed tomography (SCT) in the diagnosis of Swyer-James syndrome (SJS). METHODS: 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. RESULTS: 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. CONCLUSION: SCT scan is superior to chest radiography in the diagnosis and differential diagnosis of SJS.

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.

Clinical, physiologic, and roentgenographic changes after pneumonectomy in a boy with Macleod/Swyer-James syndrome and bronchiectasis.

Macleod/Swyer-James syndrome is an uncommon and complex disease characterized by roentgenographic hyperlucency of one lung or lobe due to loss of the pulmonary vascular structure and to alveolar overdistension. This syndrome seems to be an acquired disease that follows viral bronchiolitis and pneumonitis in childhood. It must be differentiated from many other causes of unilateral lung "transradiancy" on the chest roentgenogram, such as those related to congenital bronchial and/or vascular abnormalities. We here describe an 11-year-old patient with Macleod/Swyer-James syndrome and bronchiectasis resulting in severe recurrent bronchopulmonary infections. Despite the severe impairment of pulmonary function, the patient underwent resection of the right lung with progressive improvement of clinical and physiologic parameters.

Swyer-James syndrome with myocardial bridge: a case report.

Swyer-James syndrome is an uncommon disease with unknown aetiology. It is generally discovered on a chest radiograph as increased translucency involving one hemithorax because of diminished vascular markings. Pulmonary vascularization on the affected side is greatly reduced leading to radiological translucency. Myocardial bridging describes an angiographic entity, which is any degree of systolic narrowing of a coronary artery observed in at least one angiographic projection. It is a congenital anomaly of the coronary artery and may occur in 5-12% of the human population. We report a patient suffering from chest tightness and with diagnosed Swyer-James syndrome also having myocardial bridge.

Basilar hyperlucency in a patient with emphysema due to hypocomplementemic urticarial vasculitis syndrome.

Disproportionate emphysematous involvement of the lung bases, compared with the apices, sometimes called basilar hyperlucency, is an unusual radiographic pattern that has been reported primarily in patients with alpha-1 antitrypsin deficiency, but also in individuals with emphysema caused by intravenous injection of methylphenidate. We present a patient with emphysema associated with hypocomplementemic urticarial vasculitis syndrome and whose chest radiograph demonstrated basilar hyperlucency. To the extent that basilar hyperlucency has not been well recognized as a feature of hypocomplementemic urticarial vasculitis syndrome, this report extends the spectrum of causes of this unusual radiographic pattern of emphysema.

Swyer-James syndrome complicated by lung abscess.

Swyer-James syndrome, a rare disease with unilateral hyperlucent lung due to bronchiolitis obliterans and pulmonary artery hypoplasia, generally develops after lower respiratory tract infection during early childhood. Invasive procedures, including bronchoscopy and angiography, are often necessary for a definitive diagnosis. We report a 17-year-old man admitted because of cystic bronchiectasis complicated by lung abscess. Chest roentgenography showed the typical findings of Swyer-James syndrome. Noninvasive magnetic resonance angiography was used to confirm hypoplasia of the right pulmonary artery. The patient received antibiotic therapy, underwent a right lower lobectomy for the lung abscess, and recovered.

A case of bronchogenic carcinoma and concomitant Swyer-James syndrome.

A 46-yr-old male patient with the rare concomitance of Swyer-James syndrome in the left lung and small cell lung carcinoma in the right is presented in the light of pertinent literature.

[Diagnostic image (23). Congenital lobar emphysema]. / Diagnose in beeld (23). Congenitaal lobair emfyseem.

A 6-months-old-boy failed to thrive. Congenital emphysema of the left upper lobe of the lung was diagnosed, and lobectomy was performed, with good results.

[Two cases of Swyer-James syndrome].

Two cases of Swyer-James syndrome are reported. Case 1; A 25-year-old man was admitted to our hospital to be treated for Mycobacterium avium infection. Chest X-ray film revealed hyperlucency in the right lower lung. High-resolution CT showed a low attenuation area and bronchiectasis in the right lower lobe. He had had two episodes of pneumonia in his childhood. 3D CT scan showed a narrowing of right lower pulmonary arteries. Case 2; A 65-year-old woman was admitted to our hospital with dyspnea on effort. Chest X-ray film revealed hyperlucency in the right lung. Chest CT scan on inspiration and expiration detected air-trapping, which is characteristic of this syndrome. In both cases, the volume of the hyper lucent lung was normal. From these clinical findings, we diagnosed these two cases as Swyer-James syndrome and in this paper described the clinical features and treatment.

[Swyer-James syndrome with pneumothorax].

We report a case of Swyer-James syndrome with pneumothorax. A 20 year-old man was admitted to our hospital with dyspnea. Right pneumothorax was diagnosed by chest X-ray. He had a history of current pneumonia in childhood. After improvement of the pneumothorax, chest X-ray revealed hyperlucency of the right upper lung. Chest-CT revealed a low attenuation area and a cystic space in the right upper lobe. Xenon-133 clearance was delayed in ventilation scintigraphy. Bronchography showed cylindrical bronchiectasis in the proximal bronchi and poor filling of the peripheral bronchi in the right upper lobe. Pulmonary angiography showed a small right upper pulmonary artery. Swyer-James syndrome was diagnosed. The pneumothorax was probably caused by weakness of the cystic spaces.

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.