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[Clinical studies in five cases of Swyer-James syndrome].

We encountered five patients with Swyer-James syndrome. All were men, and ranged in age from 20 to 70 years (mean, 41 years). Their chest X-ray films showed left unilateral hyperlucency. Examinations revealed bilateral involvement in 3 cases and irregularity of involved regions in 2 cases. Chest CT scans revealed the distribution and severity of affected regions in greater detail than did other radiologic exams. Delayed clearance of xenon is a sign of air trapping, which is characteristics of this syndrome. Thus, 133Xe inhalation scintigrams were useful to detect air trapping, especially when mediastinal shift was not apparent on chest X-ray films. Bronchial damage is believed to cause this syndrome, and the present findings support this view. It is noteworthy that all five of these patients were men, and that in all five the affected lung was on the left. Moreover, although "respiratory tract infection is considered to be a very important" factor, many patients had no history of airway infection in childhood. Thus, some congenital factor or factors may contribute to the development of this syndrome.

Adult↗

Correlation of pulmonary function with the chest roentgenogram in chronic airway obstruction.

To evaluate the correlation of various roentgenographic indices with airway obstruction, the chest roentgenograms of 189 patients were independently examined (without knowledge of their clinical status or measured pulmonary function) by a pulmonary physician and by a chest radiologist. For most indices, there was a close correlation in the measured results between the 2 observers; the exception was assessment of areas of hyperlucency. Airflow obstruction was best correlated with the level of the right dome of the diaphragm. When this level was at or below the anterior end of the seventh rib, airflow obstruction was almost invariably present (specificity greater than 97%); however, the sensitivity of this index was low (range, 31 to 42%). There was a significant correlation with other roentgenographic indices; however, curvature of the diaphragm and retrosternal space were not as good over-all indices of airflow obstruction as was the level of the right dome of the diaphragm. When the retrosternal space was greater 4.4 cm or the transverse diameter of the heart was less than 11.5 cm, airflow obstruction was almost invariably present; but the sensitivity of these indices was low. In contrast, the presence and extent of areas of hyperlucency were poorly correlated with airflow obstruction. Parahilar bronchi were visualized in less than 50% of the chest roentgenograms by either observer; the ratio of wall thickness to diameter of these bronchi was not well correlated with the degree of airflow obstruction. The results indicate that a good assessment of the presence or absence of airflow obstruction can be made from various features of the chest roentgenogram.

Airway Resistance↗

Unilateral hyperlucency with left lower lobe mass in a patient with bronchial asthma.

Mucoepidermoid tumors are rare bronchial adenomas whose clinical presentation can mimic that of bronchial asthma. Bronchial adenoma should be included in the differential diagnosis of a patient with a persistent radiographic abnormality and clinical features of bronchial asthma. We present an adolescent female with a history suggestive of bronchial asthma and a persistent left lower lobe atelectasis, who later was found to have a low-grade mucoepidermoid tumor.

Adenoma↗

[A case of Swyer-James syndrome with bilateral lesions].

A 16-year-old female was admitted for the treatment of bronchial asthma. She had a history of measles pneumonia at four years of age. Her chest radiograph revealed right unilateral hyperlucency. She was diagnosed as having Swyer-James syndrome from the results of computed tomography, pulmonary angiography, bronchoscopy, and perfusion-ventilation scintigrams, which revealed unsuspected bilateral involvement, and irregularity of severity and distribution. In addition, she had a hypoplastic right upper bronchus, pulmonary hypertension, pneumothorax, and bronchial asthma. The findings of bilateral involvement, irregular distribution, and bronchial lesions strongly suggest that the etiology in this case was damage to the airways caused by measles infection in childhood.

Adolescent↗

[Swyer-James syndrome with pneumomediastinum and subcutaneous emphysema due to bronchial asthma].

A 24-year-old woman was admitted to our hospital due to moderate asthmatic attacks. Dyspnea and hypoxemia progressed gradually despite medication. A chest roentgenogram revealed left unilateraly hyperlucency with pneumomediastiumn and subcutaneous emphysema. Swyer-James syndrome was diagnosed. Several cases of Swyer-James syndrome with bronchial asthma airway hyperresponsiveness have been reported, but we know of no reports of Swyer-James syndrome with pneumomediastinum and subcutaneous emphysema due to prolonged asthmatic attacks. Pneumomediastinum and subcutaneous emphysema may be caused by abnormally high pressures in the bronchial lumen and alveolar space during asthmatic attacks, because the emphysematous lesion may be structurally weak.

Adult↗

Respiratory dysfunction in patients with common variable hypogammaglobulinemia.

Patients with common variable hypogammaglobulinemia (CVH) are susceptible to recurrent respiratory infections. We studied the prevalence and severity of respiratory dysfunction in 32 patients with CVH by measuring routine pulmonary function tests (PFT) and interpreting chest radiographs using a quantitative score system. Pulmonary symptoms were determined by patient interview and standard questionnaire. The mean age of 29 living patients was 28 +/- 2.2 yr; there were 22 males and 10 females. The mean age at diagnosis was 17.3 +/- 2.4 yr. Sinopulmonary symptoms were present in all 32 patients and included chronic sinusitis, otitis, bronchitis, recurrent pneumonia, and bronchiectasis. Complete PFT in 25 patients revealed restrictive, obstructive, and normal patterns in 10, 6, and 9 patients, respectively. Nine patients had dyspnea on exertion. Recent chest radiographs in 32 patients showed evidence of recurrent pulmonary infection, including air trapping, linear markings, and pleural abnormalities. Panlobular emphysema, documented in one case at autopsy, was suggested in 5 other patients by the presence of lower lobe bullous lesions and marked lower lobe hyperlucency on chest radiographs. The forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) had remained stable in 19 adults (18 receiving adequate immunoglobulin replacement therapy) for 6.9 +/- 0.7 yr. The expected increase of the FEV1 and FVC with growth was normal in 6 of 8 children (all 8 receiving adequate therapy). Chest radiograph scores deteriorated only slightly from 20.3 +/- 0.6 to 18.4 +/- 0.8 (25 = normal) during the 6.9 +/- 0.7 yr in 30 patients (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Does the patient's posture affect the detection of chest radiographic findings suggestive of pulmonary embolism?

Although it is accepted that thoracic structures are enlarged in supine chest radiographs, it is not known whether the lying posture may also influence the radiographic detection of abnormalities, such as those suggestive for pulmonary embolism (PE). For this purpose, we performed planar chest radiographs both in the seated and supine positions, keeping the target-to-film distance at 2 m, in 20 patients with acute PE. Chest radiograph was then repeated 1 month later in either position with the radiological conditions unchanged to investigate the effect of posture on the detection of resolution of signs. The detection of signs due to modifications of preexisting thoracic structures (elevation of the diaphragm and enlargement of the heart, right descending pulmonary artery and azygos vein) in acute PE was different between films taken in the supine and seated positions: both hemidiaphragms were more elevated (p less than 0.001), heart and central vessels were wider (p less than 0.05 or less) when patients were supine. On the contrary, the detection of signs of new occurrences ('sausage'-like appearance of the descending pulmonary artery, linear atelectasis, densities compatible with pulmonary infarction, pleural effusion and focal hyperlucency) was not influenced by the patient's position. During recovery, both kinds of abnormalities were reduced concomitantly in seated and supine radiographs. In conclusion, in acute PE, chest radiographs should be obtained in the seated position since the supine posture may overestimate signs due to modifications of preexisting thoracic structures even though it does not influence the detection of signs of new occurrences. On the contrary, films taken in either position are useful to follow the evolution of PE, provided the film is obtained in the same position and with the same technique as in the acute phase.

Acute Disease↗

CT findings of the chest in adults with aspirated foreign bodies.

The aim of this study was to assess the imaging findings in adult patients with tracheobronchial foreign body aspiration. Nineteen patients (11 men and 8 women; age range 26-89 years) with foreign-body aspiration were retrospectively reviewed. Nine patients were outpatients with non-specific symptoms and ten were hospitalized with nonresolving pneumonia (n = 6), after detection of a dental fragment on a chest radiograph following intubation (n = 3), and there was one mentally retarded patient with empyema. An aspirated dental fragment was seen on a chest radiograph in 3 patients and an endobronchial foreign body on CT in 16, appearing as a dense structure within the bronchial lumen. The foreign body was right sided in 14 cases and left sided in 5. Three cases were missed at first interpretation. Associated findings on CT were volume loss, hyperlucency with air trapping and bronchiectasis in the affected lobe. Thirteen patients were managed with bronchoscopy, whereas 2 needed thoracotomy. In 1 patient bronchoscopy failed to detect a foreign body, indicating a false-positive CT diagnosis. One patient expelled an aspirated tablet and two refused invasive procedure. The foreign bodies found mainly were bones and dental fragments. A high clinical suspicion is necessary to diagnose a foreign body. Since CT is often used to evaluate various respiratory problems in adults, it may be the first imaging modality to discover an unsuspected aspirated foreign body in the bronchial tree.

Adult↗

Thoracic CT in pediatric patients with difficult-to-treat asthma.

OBJECTIVE: . The aim of this study was to establish objective, simple criteria for bronchial wall thickening in children with difficult-to-treat asthma. SUBJECTS AND METHODS: . Bronchial sections were counted at five levels in both lungs and at three levels in the right lung on high-resolution CT and plotted against lung function. Findings from 27 children with persistent symptoms of asthma (mean age, 11.4 years; SD, +/-3.1 years) that were severe (group A, n = 15) or moderate (group B, n = 12) were compared with findings from 21 control subjects (mean age, 10.8 years; SD, +/-3.0 years) using the Student's t test, analysis of variance, and Dunn-Bonferroni test. RESULTS: . A bronchial wall thickening score based on the number of visible bronchi at three levels (three-level score) proved to be as valuable as and simpler to obtain than a score based on the number of bronchi at five levels (five-level score). The three-level scores for groups A and B were similar (mean +/- SD, 16.8 +/- 4.2 vs 18.4 +/- 3.4, respectively; p = not significant), but these scores were significantly higher than those for the control subjects (mean +/- SD, 8.2 +/- 3.4, respectively; p < 0.001). There was no correlation between the three-level score and forced expiratory volume in 1 sec or forced expiratory flow between 25% and 75% of forced vital capacity. In contrast with adults with severe asthma, our pediatric patients with difficult-to-treat asthma did not have CT evidence of mucoid impaction, emphysema, areas of hyperlucency, bronchiectasis, or sequellar line shadows. CONCLUSION: . Bronchial wall thickening visible on high-resolution CT may constitute an additional criterion of asthma severity in children. CT evidence of bronchial wall thickening might help to identify patients with a higher risk of airway remodeling.

Adolescent↗

Congenital lobar emphysema: evaluation and long-term follow-up of thirty cases at a single center.

Congenital lobar emphysema (CLE) is characterized by overdistension and air-trapping in the affected lobe, and is one of the causes of infantile respiratory distress. In this report, we review our 27 years of experience with 30 CLE patients. Patients' medical records were evaluated with regard to age, clinical presentation, diagnostic methods, associated diseases, treatment, histopathologic findings, and final clinical and laboratory findings at the end of a long-term period. The mean age of 30 patients (18 male) at diagnosis was 4.9 +/- 6.7 months (range, 2 days-2.5 years). Tachypnea, dyspnea, cough, cyanosis, wheezing, hoarseness, and decreased breath sounds on the affected side were the main symptoms and clinical findings. On chest X-rays, emphysema was seen in all patients; shift/herniation to the opposite lung, atelectasis, and pneumothorax were observed in 16, 5, and 2 cases, respectively. Computerized tomography of the thorax was performed in 16 cases and revealed emphysema at affected lobe/lobes in all, a shift/herniation to the opposite side in 12 cases, and atelectasis of neighbor lobe/lobes in 7 cases. All 8 patients who had perfusion scintigraphy showed reduced perfusion in the affected lobe. Narrowed and flaccid bronchi were detected in one patient by using flexible bronchoscopy. Blood gas analysis was performed in 11 patients, and hypoxia and hypercarbia were revealed in 9 and 7 of these patients, respectively. The most common affected lobe was the left upper lobe (57%), followed by the right upper lobe (30%) and right middle lobe (27%). Two lobes were involved in 4 patients. Associated abnormalities were observed in 5 patients. Twenty-one patients underwent lobectomy; 9 were followed conservatively. Ages at diagnosis were significantly younger in surgically treated patients. Emphysema was detected in all pathological specimens, with an additional bronchial cartilage deficiency in 2 patients. In the surgically treated group, 2 patients died and 2 patients were lost to follow-up. In the conservatively treated group, one patient was lost to follow-up. Mean follow-up duration of all patients was 63.2 +/- 56.2 months (range, 1-209 months). At follow-up visits, all patients were doing well. In surgically treated patients, chest X-rays were normal (9 cases), or showed hyperlucency on the operated side (6 cases) or chronic changes in the operation area (2 cases). Hyperexpansion in the affected lobe was found to be reduced in all cases in the conservatively treated group.

Adolescent↗

Bronchopulmonary dysplasia: value of CT in identifying pulmonary sequelae.

OBJECTIVE: Pulmonary dysfunction is common in children who survive bronchopulmonary dysplasia. Chest radiographs are not satisfactory for the identification of the sequelae of bronchopulmonary dysplasia because, although they often show abnormalities, the abnormalities are usually minor and sometimes absent. We therefore assessed the value of CT for identifying the sequelae of bronchopulmonary dysplasia. MATERIALS AND METHODS: Twenty-three children (mean age, 4 years) who had survived neonatal bronchopulmonary dysplasia and had signs of chronic pulmonary dysfunction (recurrent episodes of coughing, wheezing, dyspnea, pneumonia, respiratory insufficiency) were examined with chest radiographs and high-resolution CT scans of the chest. Two reviewers qualitatively analyzed the chest radiographic and CT findings by describing the most consistently found lesions and their frequencies. RESULTS: The chest radiographs showed hyperexpansion in 17, hyperlucent areas in 11, and linear opacities in 10 of the 23 children. Pleural thickening was not observed, and four children had normal findings on chest radiographs. All 23 CT scans showed abnormalities, including multifocal areas of hyperaeration, well-defined linear opacities, and triangular subpleural opacities with an external base and an internal apex. In 20 of 23 children, all three abnormalities were present. For the three other children, two of these three abnormalities were found. No bronchiectasis was observed in any of the cases. CONCLUSION: Lesions in survivors of bronchopulmonary dysplasia with chronic pulmonary dysfunction are visualized better on CT scans than on chest radiographs. Importantly, CT findings of multifocal areas of hyperaeration, numerous linear opacities facing triangular subpleural opacities visible on several consecutive sections, and no bronchiectasis should suggest the presence of sequelae of bronchopulmonary dysplasia.

Bronchopulmonary Dysplasia↗

[Chronic hypersensitivity pneumonitis or idiopathic pulmonary fibrosis? Diagnostic role of high resolution Computed Tomography (HRCT)].

PURPOSE: In the diagnosis of extrinsic allergic alveolitis, high-resolution computed tomography (HRCT) is more sensitive and more specific than radiography; however, the accuracy of HRCT is not absolute. The role of HRCT in this field has scarcely been investigated in the literature. The purpose of this paper is to explore the possibilities of HRCT in this field and to analyse the correlations between HRCT and functional parameters. MATERIALS AND METHODS: We performed a retrospective blind evaluation the HRCT scans of 24 patients (19 males and 5 females). Twelve were affected by idiopathic pulmonary fibrosis (IPF); 12 had chronic extrinsic allergic alveolitis (EAA) or hypersensitivity pneumonitis. The HRCT findings were studied by two radiologists, who were not aware of the diagnoses. The possible differences in evaluation were subsequently resolved by consensus. In the presence of interstitial fibrosis, we recorded the prevailing site and the anatomical extension (with the method of the visual percentage score, described in the literature). In all the cases, we recorded the blood gas values (PaO2, PaCO2, and pH), total lung capacity (TLC), forced expiratory volume in 1 second (FEV1), Vital Capacity (VC), Tiffeneau index (FEV1/VC) and monoxide carbon diffusion (DLCO). The statistical significance of the correlations was investigated using the Mann-Whitney and Wilcoxon tests. Student "t"-test and Pearson's chi squared were used to compare the variables of the different groups. A value of p< or =0.05 was considered significant. RESULTS: Overall, the diagnosis was formulated with a high degree of confidence in 13 of 24 cases (54.1%); in these patients, the result was correct in 84.6% of cases (11 of 13). In the diagnosis of EAA, HRCT sensitivity was 50%, with 91.6% specificity, 70.8% accuracy. In the diagnosis of IPF, HRCT sensitivity was 75%, with 83.3% specificity, 79.1% accuracy. The HRCT signs of interstitial fibrosis were visible in 11 cases of IPF (91.6%) and in 4 cases of EAA (33.3%), with significant statistical difference (p<0.005). In the analysis of the whole series (24 patients) we found a significant inverse correlation between the HRCT score of fibrosis and the extension of the areas with air-trapping (p<0.003). There was no significant difference between IPF and EAA considering age, FEV1, PaO2, PaCO2 and pH. We found inverse correlation (p<0.001) between HRCT extension of the fibrosis and DLCO. There was a significant difference between pulmonary fibrosis and extrinsic allergic alveolitis considering TLC (in average, respectively: 65% and 84.3%; p<0.02), and DLCO (respectively 40.4% and 74.7%; p<0.03). DISCUSSION AND CONCLUSIONS: HRCT is a reliable and strong method to be used in particularly complex fields. We found signs of interstitial fibrosis more frequently in IPF than in EAA (91.6% versus 33.3%). In IPF cases, the extension of the fibrosis--evaluated with the visual score method--was higher then in EAA (34.7% versus 6%). The presence of fibrosis with basal and peripheral distribution was characteristic of idiopathic pulmonary fibrosis, with good sensitivity and specificity (75%), whereas in chronic extrinsic allergic alveolitis the areas of fibrosis often presented an irregular and heterogeneous distribution, in 91.6% of cases. However, 25% of extrinsic allergic alveolitis cases had a distribution mimicking idiopathic pulmonary fibrosis. The presence of areas with increased ground-glass opacity is more common in EAA than in IPF (66.6% versus 33.3%). Areas of mosaic attenuation, visible in inspiratory CT scans, are not rare in IPF (41.6% of cases), and generally have a basal distribution. The presence of hyperlucent lobules inside the fibrotic areas does not exclude the diagnosis of IPF. The finding of the expiratory air-trapping is more common in chronic EAA than in IPF, and the extension of this areas is greater in EAA (17.3% versus 6.5%). We found an inverse correlation between the extension of the fibrosis and the expiratory trapping. Signs of emphysema were observed in 25% of cases, wiema were observed in 25% of cases, with equal prevalence and the same extension in IPF and in EAA. All patients were smokers or ex-smokers. Smoking has not been demonstrated to have a protective effect against EAA. The areas of mosaic perfusion during inspiration do not represent a sound criterion for the differential diagnosis between IPF and chronic EAA. In fact, they can also be frequently found in IPF. HRCT can be employed in clinical practice for the differential diagnosis between IPF and chronic EAA with good (but not absolute) accuracy.

Alveolitis, Extrinsic Allergic↗