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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.

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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.

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OBJECTIVE: The aim of this study was to describe the anesthetic management and perioperative outcomes in patients with Swyer-James syndrome (SJS), a rare acquired pulmonary disorder caused by postinfectious bronchiolitis obliterans resulting in airway obstruction and focal areas of emphysema or bronchiectasis. DESIGN: A retrospective computerized search of the medical records database at a large academic tertiary referral center was performed for patients with SJS from January 1, 2001 through July 31, 2012 who underwent procedures requiring anesthesia. A review of the perioperative course in the SJS patients as well as in those identified by a literature search was performed. SETTING: Academic tertiary referral center, both inpatient and outpatient settings. PARTICIPANTS: Patients with a diagnosis of SJS. INTERVENTIONS: No interventions were performed. MEASUREMENTS AND MAIN RESULTS: The authors identified 4 patients with SJS who underwent 7 uneventful anesthetics including one lung resection. Three patients were symptomatic preoperatively. The literature review identified 23 SJS patients who underwent lung resection for recurrent pulmonary symptoms. One patient developed hypoxemia during one-lung ventilation. Three patients had a prolonged hospital stay (≥10 days). All patients undergoing lung resection were young (≤42 years of age). CONCLUSION: Though SJS may be an incidental finding, these patients may have marked symptomatology, recurrent pulmonary infections, and pneumothoraces that may require lung resection. The patients tolerated anesthesia well. In severe SJS cases, pulmonary pathology and perioperative management strategies parallel that of patients with severe obstructive pulmonary disease.

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INTRODUCTION: MacLeod syndrome, also known as Swyer-James syndrome, is a rare syndrome characterized by unilateral lung hyperlucency. It is a form of constrictive bronchiolitis which is caused by repeated acute bronchiolitis and/or pulmonary infections during infancy. CASE REPORT: The patient was a 26-year-old man who had had a first left pneumothorax at the age of 21. He smokes tobacco. When he was 1-year-old he had experienced repeated episodes of acute bronchiolitis. At age 26, he suffered from a second left pneumothorax which was wrongly diagnosed as a recurrent drain-resistant pneumothorax. Misdiagnosed, he underwent a chemical pleurodesis during thoracoscopy. As a hyperlucency remained in the left upper part of the lung on chest X-ray, further investigations were undertaken and a diagnosis of MacLeod syndrome was made. CONCLUSIONS: MacLeod syndrome is rare. It can be associated with pneumothorax which can be a source of misdiagnosis and error in the management of these patients. Therefore, the diagnostic criteria and therapeutic indications are reiterated. The probable mechanism of association between pneumothorax and Macleod syndrome is also discussed.

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Swyer-James Macleod syndrome is a radiologic entity characterized by hyperlucency of one or more lobes or of the entire lung, decreased number and diameter of ipsilateral peripheral pulmonary vessels, and difficult visibility of the arterial network and unobstructed bronchial system. A 21-year-old male was admitted to our clinic on the observation of left hemithoracic hyperinflation on chest radiography. Preoperative evaluation revealed an increase of ventilation to the left lower lobe and the deletion of peripheral vascular structures. Scintigraphy revealed a perfusion defect in the left lower lobe. In this patient with congenital left upper lobe hypoplasia and Swyer-James Macleod syndrome in the lower half of the lower lobe, I present the coexistence of these two rare clinical entities and "acquired lobar emphysema."

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Swyer-James-Macleod syndrome (SJMS) is a rare, complex disease characterized by unilateral hyperlucent lung or lobe owing to loss of pulmonary vasculature and alveolar hyperdistention. Treatment is generally conservative, and surgical management is rare. In fact, only 4 reports on surgically treated children with SJMS are available in the literature. We describe an 8-year-old patient with a history of recurrent respiratory infections since 2 months old. The patient presented with cough and dyspnea on effort. Chest radiograph showed hyperlucency and reduction of the right lung. Computed tomography revealed reduced volume and vasculature in the right lung, whereas pulmonary scintigraphy showed that 85% of ventilation and perfusion occurred in the left lung. Pulmonary function tests showed a forced expiratory volume in 1 second of 0.85 L (56% of expected value) and a forced vital capacity of 1.20 L (70% of expected value). The child underwent right pneumonectomy because of severe compromise of pulmonary function and recurrent respiratory infections. Histologic examination revealed panacinar emphysema and chronic bronchitis/bronchiolitis. At 6-month follow-up, the child showed improved symptoms and a forced expiratory volume in 1 second of 1.15 L (77% of expected value) and a forced vital capacity of 1.4 L (83% of expected value). This report shows that although rarely indicated, surgical treatment can improve quality of life and pulmonary function in SJMS.

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Swyer-James syndrome (SJS) shows the constellation of radiographic findings of a small, hyper lucent lung, with an ipsilateral, diminished peripheral vasculature, air trapping, and a lack of peripheral fill on bronchography. We report a case of 70-year-old woman with SJS who underwent pulmonary resection of the normal side lung for lung tumor. Because of this syndrome, we could predict the hypoxia during one-lung ventilation. In fact, about ten minutes after beginning of one-lung ventilation, Sp(O2) decreased from 100% to 90%. As we could not improve the hypoxia in spite of increasing FI(O2), O2 administration to the operating side lung was started. Sp(O2) recovered after O2 administration. For anesthetic management of a patient with SJS in the normal-side-lung, it is essential to prevent the hypoxia during one-lung ventilation.

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Swyer-James-MacLeod Syndrome (SJMS) occurs as a result of childhood bronchiolitis obliterans. Typically, this disorder is diagnosed in childhood after evaluations for recurrent 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. Here, we are presenting a 22-year-old female patient who was diagnosed with SJMS who was initially misdiagnosed with a pneumothorax and treated by multiple chest tube drainages. This case highlights the significance of taking a careful history, the application of computed tomography and scintigraphy in confirming the diagnosis of SJMS and in eliminating other diseases.

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Swyer-James syndrome is an uncommon, emphysematous disease characterized by roentgenographic hyperlucency of one lung or lobe due to loss of the pulmonary vascular structure and to alveolar overdistension. We here describe a 28-year-old professional bicycle racer with Swyer-James syndrome who had dyspnea on exertion, especially during a bicycle race. Lung volume reduction surgery was performed with video-assisted thoracoscopic surgery. This treatment improved his pulmonary function, and he is active as a bicycle racer now.

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Swyer-James (MacLeod) syndrome is an acquired form of unilateral hyperlucency of the lung and is characterized by the development of severe emphysema, bronchiectasis, and/or bronchiolitis obliterans. It may develop as a complication of repeated episodes of pulmonary infection resulting in bronchiolitis obliterans and obstruction of small airways. Most patients with Swyer-James (MacLeod) syndrome can be managed clinically, and the pathologic features of the syndrome have been described in only a few reports. Placental transmogrification of the lung is a rare histopathologic finding that has been described in patients with severe emphysema associated with cigarette smoking, congenital bullous emphysema, and fibrochondromatous hamartomas of the lung and is characterized by the development of peculiar structures in the pulmonary parenchyma that resemble placental villi. To our knowledge, placental transmogrification of the lung has not been previously described in patients with Swyer-James (MacLeod) syndrome. We encountered a 32-year-old man with a history of childhood asthma who presented with progressively severe exertional dyspnea and had unilateral right lung hyperlucency. The patient underwent a right pneumonectomy. Examination of the lung revealed severe mixed centriacinar-panacinar emphysema in all lobes, bullous emphysema in the upper lobe, bronchiectases, mild interstitial pneumonia with fibrosis, and placental transmogrification of the pulmonary parenchyma of all 3 lobes. Here, we review the pathology of Swyer-James (MacLeod) syndrome and the possible pathogenesis of villous-like changes in the lung tissues.

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OBJECTIVE: The purpose of this study was to evaluate the CT appearance, management, and temporal course of persistent pulmonary interstitial emphysema in neonates. MATERIALS AND METHODS: Criteria for inclusion in the study group included neonates with a history of prematurity who required ventilation for lung disease, development of hyperexpanded radiolucent lung lesions after typical radiographic findings of pulmonary interstitial emphysema, and CT documentation of lung abnormalities. Radiographs and CT scans were reviewed for the anatomic distribution, appearance, and presence of classic lines or dots within a radiolucent mass. We compared the management (surgical vs nonsurgical) and the temporal course in nonsurgical cases for patients in the United States and patients outside the United States. RESULTS: From seven institutions, we identified 17 patients who had persistent pulmonary interstitial emphysema with CT documentation. On CT, all lesions consisted of hyperexpanded cystic radiolucencies. Distribution was single-lobe in nine patients (left upper lobe, n = 5; left lower lobe, n = 3; right middle lobe, n = 1), multilobar in eight patients, and bilateral in six patients. Fourteen patients (82%) showed the characteristic line-and-dot pattern. All patients were initially treated conservatively; nine eventually underwent surgical resection with confirmation at pathology. Of 10 patients who underwent nonsurgical treatment for at least 1 year, lesions resolved in four patients, decreased in size in three asymptomatic patients, and enlarged in three patients, with eventual resection in two. Surgical resection was performed in 89% (8/9) of patients treated in the United States and in 13% (1/8) of patients treated outside the United States. CONCLUSION: Our study found that 82% of patients with persistent pulmonary interstitial emphysema had characteristic CT findings (central lines and dots surrounded by radiolucency). Although most patients in this series eventually underwent surgical resection, initial treatment can be conservative. The decision to perform surgery may have reflected the local medical culture.

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We treated a 15-year-old patient with spontaneous pneumothorax associated with Swyer-James syndrome using video-assisted thoracoscopic surgery (VATS). Thoracic computed tomography showed hyperlucent areas in the bilateral lungs. Due to major air leakage continuing for a week, we conducted VATS bullectomy. Because the opposite lung suffered hypoplasia, intermittent bilateral pulmonary ventilation was required to sustain an adequate PaO2 in arterial blood gas analysis during surgery. Because of recurrent pneumothorax, we performed reoperation 10 months later, finding a few newly generated bullae. To the best of our knowledge, this is the first report of VATS used to treat a Swyer-James syndrome patient with pneumothorax.

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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.

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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.

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Because patients with Swyer-James syndrome have almost always been treated conservatively, few reports exist of pathological findings of the lung in this syndrome. We report a case of this rare disease treated surgically and discuss pathological findings. A 36-year-old woman repeatedly contracted bronchitis and pneumothorax since adolescence, until April 26, 1997, when she reported chest pain and dyspnea. Chest X-ray on admission showed left pulmonary collapse with a slight deviation of the mediastinum toward the right. Chest computed tomography showed an apical bulla and emphysematous change in the left upper lobe. Pulmonary arteriography at age 17 showed hypoplasia of left pulmonary artery branches in the left upper lobe. Based on a diagnosis of Swyer-James syndrome, we conducted left upper lobectomy on May 2, 1997. Pathological examination of the resected left upper lobe showed marked emphysematous change, including an emphysematous bulla with destruction of alveolar structure and peribronchiolar fibrosis. No vascular abnormality was recognized in histology. Emphysematous change secondary to repeated bronchiolitis is believed to have led to her repeated pneumothorax.

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Os autores relataram dois casos de hipertransparência pulmonar unilateral,ou síndorme de Swyer-James-MacLeod,envolvendo o hemitórax direito e caracterizados por diminuição do murmúrio vesicular no lado afetado,hipoplasia da artéria pulmonar e akterações da árvore brônquica. Feita uma discussão das prováveis causas dessa entidade clínica e identificadas as outras doenças que podem simular a síndrome.O tratamento proposto nesses casos é conservador,reservando-se a cirurgia aos casos complicados por infecções intercurrentes ou hemorragia

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