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[A case of Swyer-James syndrome with interesting chest radiographs].

A 28-year-old man was admitted for further examination after overinflation of the right lung was observed on a screening chest radiograph. The chest radiograph on admission showed increased radiolucency of the left lung and overinflation of the right lung, associated with a shift of the mediastinum toward the midline on expiration. High-resolution CT on expiration showed air trapping in the left lung and in part of the right lower lobe. Pulmonary perfusion scintigraphy showed a markedly decreased perfusion in these areas. Pulmonary angiography revealed small left pulmonary arteries with diminished peripheral vascular branches. Bronchoscopy showed no abnormality of either bronchus. Swyer-James syndrome was diagnosed on the basis of these findings. Although bronchial damage due to the lower respiratory tract infection during childhood is considered a very important factor in the pathogenesis of this syndrome, many patients have no history of airway infection, as in this case. This is a rare case of Swyer-James syndrome presenting with marked overinflation of the unaffected lung in an asymptomatic adult. Progression of this compensative overinflation probably involved asymptomatic damage caused by the weakness of the affected lung.

Adult↗

Swyer-James-MacLeod syndrome.

Swyer-James-MacLeod syndrome is a rare complication of respiratory tract infection occurring in early childhood. We report two children with chronic cough and recurrent wheezing who fulfilled the diagnostic criteria for this disorder: 1) Unilateral loss of lung volume with hyperlucency on chest x-ray. 2) Unilateral reduction in vascularity on CT scan of the chest. 3) Unilateral loss of perfusion on Technetium 99c lung scan.

Child, Preschool↗

[A case of isolated absence of the right pulmonary artery].

A 40-year-old woman was admitted to our hospital for investigation of an abnormal chest roentgenogram. She had been well with normal exercise tolerance and had no history of pneumonia. The chest roentgenogram showed decreased vascularity of the right lung, ipsilateral small hemithorax, and shift of the mediastinum toward the right. In the right lung field, hazy and confluent micronodular shadows were seen, and the left lung was emphysematous and hyperlucent. Herniation of the left lung into the contralateral thorax was seen. Although lung perfusion scintigrams showed no detectable blood flow to the right lung, a krypton ventilation study revealed diminished but homogeneous ventilation of the ipsilateral lung. A bronchogram showed normal structure of the bronchi. Cardiac catheterization revealed normal pulmonary arterial pressure. A pulmonary angiogram showed no right pulmonary artery, and absence of other cardiovascular anomalies. Therefore, the diagnosis of isolated absence of the right pulmonary artery was confirmed. An aortic angiogram demonstrated collateral vessels originating from one of the bronchial arteries, the infradiaphragmatic artery, and the right coronary artery. Chest CT disclosed cystic changes in the right lung field.

Adult↗

Differential lung function in an infant with the Swyer-James syndrome.

A previously healthy two year old boy had an adenoviral infection at the age of 13 months and developed hyperlucency of the left lung, chronic respiratory distress, and failure to thrive. Bronchodilators and steroid treatment had no effect. Radionuclide lung scans using an intravenous bolus of xenon-133 both before and after treatment showed substantially reduced function on the hyperlucent side and modestly reduced function on the other side. Fibreoptic bronchoscopy showed no structural abnormalities. Partial forced expiratory flow volume (PEFV) curves, generated from end inspiration by rapid compression of the chest wall with an inflatable jacket, were obtained from the total respiratory system and from each lung separately by inflating a Fogarty catheter in the contralateral mainstem bronchus. Expiratory flow rates and volumes during both tidal breathing and PEFV manoeuvres were considerably decreased in the hyperlucent lung. PEFV curves from the "healthy" right lung and from the total respiratory system were similar in shape and showed a moderately obstructive pattern. The right lung ventilated about four times as much as the left when measured by bronchospirometry and about three times as much when measured by the radionuclide technique. The lung scans appeared to reflect adequately the functional abnormality in this infant with the Swyer-James syndrome.

Child, Preschool↗

Post-infectious bronchiolitis obliterans: clinical, radiological and pulmonary function sequelae.

Background. There are few data on clinical, chest radiograph (CXR) or pulmonary function sequelae in children with post-infectious bronchiolitis obliterans (BO) (pulmonary crepitations, abnormalities on CXR, CT, nuclear medicine scans, or bronchography, with a history of past pulmonary infection and in the absence of other underlying pathology). Objective. To analyse the methodology of diagnosis, long-term clinical imaging and pulmonary function sequelae of post-infectious BO in children. Materials and methods. Imaging (CXRs, CT and nuclear lung scans) and clinical histories of 19 children were analysed. Results. Clinical follow-up (mean 6.8 years), revealed a high incidence of continuing problems (asthma and bronchiectasis). Fixed airway obstruction was the most common pulmonary function sequela. The sequelae on follow-up (mean 5.8 years) CXR were classified into five patterns which are illustrated: unilateral hyperlucency of an enlarged lung/part of lung; complete collapse of the affected lobe; unilateral hyperlucency of a small or normal-sized lung; bilateral hyperlucent lungs and a mixed pattern of persistent collapse, hyperlucency and peribronchial thickening. Conclusion. Long-term observations in children with post-infectious BO should be undertaken to detect bronchiectasis and obstructive airway disease. Sequelae evident on CXR, other than those previously described, can be found. Bronchography and/or lung biopsy are not usually required for the diagnosis of post-infectious BO.

Bronchiolitis Obliterans↗

[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.

Adult↗

[Chronic pulmonary thromboembolism diagnosed on the basis of characteristic mosaic patterns on lung computed tomograms].

We report on a 59-year-old woman who presented with characteristic findings on lung computed tomographic (CT) scans and was therefore suspected to have chronic pulmonary thromboembolism. She visited our hospital because of worsening exertional dyspnea over the preceding year, and because she was dissatisfied with an earlier diagnosis made by another institution. Chest roentgenograms disclosed nonhomogeneous hyperlucency in both lungs associated with linear and bundle shadows, dullness of the right costophrenic angle, and dilatation of the descending branch of the right pulmonary artery. The patient experienced moderate hypoxemia even at rest. Pulmonary function tests demonstrated a restrictive ventilatory pattern associated with reduced diffusion capacity. The lung CT scans disclosed a mosaic pattern of attenuation in the lung parenchyma, which probably reflected scattered areas of low perfusion. The unique characteristics of such CT findings drew our attention to chronic pulmonary thromboembolism as a possible diagnosis. We eventually confirmed the diagnosis on the basis of enhanced CT scans, pulmonary perfusion and ventilation scintigrams, and digital subtraction angiography. In our view, chronic pulmonary thromboembolism should be kept in mind as a possible differential diagnosis of the mosaic patterns of attenuation on lung CT scans.

Chronic Disease↗

Obliterative bronchiolitis in children.

Clinical, radiologic and histologic features of obliterative bronchiolitis (OB) in children were reviewed to define features helpful in early recognition. All autopsies (n = 2,897), lung biopsies (n = 244), and medical records of children followed at St. Christopher's Hospital for Children (SCHC) between 1960 and 1985 were screened. Nineteen cases of OB were confirmed using radiologic and histologic criteria. Twelve patients were diagnosed during life, and ten survived. Seven were diagnosed post mortem. OB should be considered when persistent respiratory signs and symptoms follow acute pneumonia, aspiration is known or suspected, areas of hyperlucency are seen on chest radiograph, or respiratory failure with overaeration is unresponsive to therapy. Diagnostic studies include ventilation-perfusion scan, bronchography and lung biopsy. Sequelae include dyspnea on exertion, obstructive lung disease, bronchiectasis, persistent atelectasis, and hyperlucent lung syndrome. Recognition and supportive treatment during the acute and chronic phases may improve the functional status of these patients.

Adolescent↗

Surgical treatment of congenital lobar emphysema in a puppy.

Congenital lobar emphysema was found in a three-month-old male springer spaniel. Clinical signs included coughing, progressive dyspnoea and abnormal lung auscultation. Radiographic examination revealed overinflation and hyperlucency of the right middle lung lobe. Following lung lobectomy, clinical signs resolved and the animal had normal exercise tolerance. Previous case reports are reviewed and discussed in relation to human congenital lobar emphysema.

Animals↗

Neonatal pulmonary infarction. A cause of 'cystlike' lucencies on the chest roentgenogram.

Perinatal pulmonary infarction is a difficult and infrequently made diagnosis. A male newborn had infarction of most of the right lung secondary to perinatal pulmonary thromboembolism (PTE). Serial chest roentgenograms initially showed opacity of most of the right lung, followed by the appearance of well-defined radiolucencies resembling pneumatoceles associated with mass effect. By the 25th day of life the localized "cystlike" lucencies were no longer evident and the right lung appeared predominantly hyperlucent. Although the presence of underlying disease makes the diagnosis of PTE in the infant and young child difficult, the clinical features and methods of diagnosis of PTE in this age group are similar to those for the adult. The diagnosis of PTE should be considered in the infant with respiratory distress and unusual findings on chest roentgenography.

Cysts↗

Air trapping in children: evaluation with dynamic lung densitometry with spiral CT.

PURPOSE: To evaluate the feasibility of the use of a simple method of dynamic lung densitometry with spiral computed tomography (CT) to differentiate air trapping from compensatory hyperinflation in children. MATERIALS AND METHODS: Eight children (mean age, 4 years 2 months) who had focal areas of radiolucency on chest radiographs underwent spiral CT (5-second acquisition time, fixed table position) during quiet breathing. Data were reconstructed with 0.7-second temporally overlapping scans. Lung attenuation in selected regions of interest was plotted against time. RESULTS: In children with air trapping (n = 5), mean lung attenuation (+/- 1 standard deviation) in hyperlucent regions was -815 HU +/- 52, and mean respiratory excursion was 28 HU +/- 7. In patients with compensatory hyperinflation (n = 4), mean lung attenuation was -664 HU +/- 31, and respiratory excursion was 84 HU +/- 15. The differences were statistically significant (P = .001 for mean attenuation; P < .0005 for respiratory excursion). CONCLUSION: Dynamic spiral CT lung densitometry is a quick, simple method for quantitative confirmation of the presence of air trapping and differentiation from compensatory hyperinflation.

Absorptiometry, Photon↗

Scintigraphic images of Swyer-James syndrome.

The Swyer-James Syndrome, first described in 1953, is a rare entity which consists of severe oligemia of one or more pulmonary lobes accompanied with bronchial deformation. Recurrent bronchial infections in childhood are believed to be the cause, leading to obliteration of small airways, alveolar overdistension and, ultimately, the destructive changes characteristic of emphysema. Secondary vascular alterations occur resulting in oligemia of the afflicted lobe. The clinical presentation is variable ranging from the totally asymptomatic patient to dyspnea on exertion or recurrent lower respiratory tract infections. The physical examination may reveal hyperresonance over the affected area, restricted chest expansion on one side, or occasionally rales on auscultation. Radiological findings are characteristic and include a pronounced one sided hyperlucency of the lung (due to oligemia), a mediastinal shift toward the affected side on inspiration, a small ipsilateral hilus, and bronchial deformities as disclosed by bronchography. Another radiologic feature, described as a "sine quanon" condition for diagnosis by Fraser and Paré is the development of air trapping during expiration. Various diagnostic modalities including roentgenography, bronchoscopy, bronchography, angiography and radionuclide imaging are necessary to obtain the diagnosis.

Adult↗

Bronchial atresia with transient spontaneous disappearance of a mucocele.

We report the transient spontaneous disappearance of a mucocele due to bronchial atresia. Two years before presentation, a chest radiograph showed a hyperlucent right upper lung and a mucocele near the right hilum. A chest radiograph taken 1 year later showed that the mucocele had disappeared leaving an ovoid outline of a dilated bronchus. A chest radiograph obtained 3 months before presentation showed that the mucocele was present again. Atresia of the B3b bronchus of the right upper lobe was noted on thoracotomy. The "disappearance" of the mucocele probably was due to the clearance of mucoid material through collateral airways.

Adolescent↗