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PubMed2005

Swyer-James (MacLeod) syndrome with placental transmogrification of the lung: a case report and review of the literature.

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.

Adult↗
PubMed2002

Video-assisted thoracoscopic bullectomy for spontaneous pneumothorax in a Swyer-James syndrome patient.

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.

Adolescent↗
PubMed1998

[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.

Adult↗
PubMed1994

[A case of Swyer-James syndrome--a morphological study with inflated fixed lung].

Morphological features of Swyer-James syndrome were studied in a specimen obtained during left pneumonectomy. A 67-year-old woman was admitted because her left lung was hyperlucent and contained a solitary nodule. Primary lung adenocarcinoma complicated with Swyer-James syndrome was diagnosed, and left pneumonectomy done. The left upper lobe was inflated and fixed for morphological study. The volume of the specimen was obviously lower than normal but the bronchial branching was normal. Macroscopically, the specimen had irregular bronchial walls, bronchiectatic changes, and dilation of distal air spaces, similar to panlobular emphysema. Microscopically, there was scarring in periarteriolar regions that suggested bronchiolitis obliterans, enlargement of distal airspaces, and an abnormally low number of capillaries. These findings suggest hypoplasia of the lung due to abnormal lung growth after birth.

Aged↗
PubMed2006

Ventricular septal defect and Swyer-James (Macleod's) syndrome together: a case report.

Swyer-James (MacLeod's) syndrome is a rare entity that is characterized by the hyperlucent appearance of one or more lobes of one lung, diminished ipsilateral hilar shadow and hardly visible arterial structure on chest radiography. Ventricular septal defect is the most common childhood congenital heart defect and, if left untreated, this will result to irreversible pulmonary hypertension, reversing of the shunt and Eisenmenger's syndrome. In this case report, we describe a 19-year-old female patient who had diagnosis of VSD and Swyer-James syndrome at the same time.

Adult↗
PubMed1976

Swyer-James (Macleod's) syndrome. Variations in pulmonary-bronchial arterial blood flow.

The Swyer-James (Macleod's) syndrome (or unilateral hyperlucency of the lung) frequently presents a diagnostic problem. Two cases of this entity are reported that demonstrate its similarity to and differentiation from pulmonary embolism and other intrapulmonic disorders. Detailed roentgenologic and physiologic studies were performed to define the diagnostic criteria of this syndrome. The reciprocal relationship of the bronchial arterial circulation in the hyperlucent lung was described.

Adult↗
PubMed2001

Black, white, and shades of gray: common abnormalities in chest radiographs.

Chest radiographs remain one of the oldest, noninvasive methods for identifying chest diseases. This tool is relatively inexpensive, low risk, and important for the advanced practice nurse to use in making an initial differential diagnosis. Timely intervention depends on the advanced practice nurse's ability to recognize the radiographic abnormalities commonly associated with acute pulmonary disease. When linked with the history, presenting signs and symptoms, and a thorough physical assessment, the chest radiograph is key to appropriate diagnosis and management. This article reviews the basic radiographic lung densities, describes a systematic approach to ensure thorough chest radiographic interpretation, and presents characteristic chest radiographs found in the acute care setting. Selected examples of diffuse lung diseases, localized alveolar opacities, pleural effusions, and hyperlucency of the lungs will also be presented.

Heart Failure↗
PubMed1998

Imaging of asthma and allergic bronchopulmonary mycosis.

The chest radiograph of the patient with asthma is characterized by bronchial wall thickening and hyperinflation. On CT scanning of patients with asthma one may see airway wall thickening, thickened centrilobular structures, and focal or diffuse hyperlucency. Apparent bronchial dilation may be seen, but the diagnosis of bronchiectasis should be made with caution. Quantification of changes in the airway wall and lung parenchyma may be valuable in understanding the mechanisms of asthma and in evaluating the effects of treatment. Central bronchiectasis occurs in most, but not all, cases of ABPA. Patchy airspace opacity may be the sole radiologic manifestation of ABPA in some cases. Other fungi can rarely cause a similar syndrome. The challenge for the radiologist evaluating the images of a patient with asthma is to find complications, such as ABPA, or alternative diagnoses.

Aspergillosis, Allergic Bronchopulmonary↗
PubMed1991

[Chest radiograph of foreign bodies in the tracheobronchial tree].

Chest radiographs in 14 children with foreign bodies in the tracheobronchial trees were evaluated retrospectively. The most common causative materials were nuts, and both main bronchi were most commonly involved. The initial chest radiographs that were used for analysis were obtained one hour to 50 days after aspiration or onset of symptoms. Of the nine cases in which chest radiographs were taken within 7 hours after aspiration, six showed hyperlucency with (three cases) or without overinflation (three cases) in the affected lungs, and the other three showed normal chest radiographs. Two patients had indeterminate diagnoses on chest radiographs at inspiration: one patient underwent chest radiographs at expiration and the other underwent fluoroscopy. Air-trapping was demonstrated in both patients. Of another five cases in which chest radiographs were taken 18 hours after aspiration of a foreign body, three cases showed atelectasis or consolidation and the other two showed hyperlucent lung. From these observations, hyperlucent lung indicates an early stage of the disorder while atelectasis or consolidation indicates a fairly advanced stage. In patients with clinically suspected foreign bodies, we advocate that additional examinations be performed to establish a final diagnosis, even when chest radiographs are normal or indeterminate.

Bronchi↗
PubMed1993

Bronchial atresia: report of a case and review of the literature.

We report herein an unusual presentation of bronchial atresia in a 28-year-old woman, in whom hyperlucency of the ventral segment, distal to a right extrahilar mass found on a routine chest X-ray, was not recognized. Atresia of the medial branch of the ventral segmental bronchus (B3b) with mucoid impaction in the dilated bronchus was finally disclosed by a right upper lobectomy. The patient had been asymptomatic, and physical examination demonstrated no abnormal findings such as decreased breathing sounds over the affected lung. Localized hyperlucency and a mass are the characteristic radiographic features of bronchial atresia. In the present case, however, hyperlucency distal to the mass, which was retrospectively evident on a computed tomogram of the chest, was not recognized. The preoperative diagnosis was also made difficult by the fact that the atresia was located on a subsequential branch (B3b) of the ventral segmental bronchus of the right upper lobe. Since computed tomography and magnetic resonance imaging are able to make an accurate diagnosis of bronchial atresia possible, surgery is often not indicated for asymptomatic patients. Moreover, although surgical intervention is required for patients with complications such as encroachment of normal pulmonary tissue or infection, resection should be as limited as possible to preserve normal lung tissue.

Adult↗
PubMed2005

Lung volume reduction surgery for a professional athlete with Swyer-James syndrome.

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.

Adult↗
PubMed2006

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.

Adolescent↗
PubMed2006

Developments in neuroacanthocytosis: expanding the spectrum of choreatic syndromes.

As with other neurodegenerative disorders, research into the group of diseases known under the umbrella term of "neuroacanthocytosis" has greatly benefited from the identification of causative genes. The distinct and unifying aspect of these disorders is the presence of thorny deformations of circulating erythrocytes. This may be due to abnormal properties of red cell membranes, which could lead to insights into mechanisms of neurodegeneration. Research approaches in this field, in addition to examining functions and protein interactions of the affected proteins with particular respect to neurons, have also drawn upon the expertise of hematologists and red cell membrane biologists. In this article, recent developments in the field are presented.

Animals↗