Idiopathic giant bullous emphysema (vanishing lung syndrome): imaging findings in nine patients

OBJECTIVE: We reviewed the imaging findings in nine patients with idiopathic giant bullous emphysema. This progressive condition is characterized by large bullae, usually seen in association with several forms of emphysema, and usually occurs in young men, most of whom are smokers. MATERIALS AND METHODS: Nine patients with chest radiographic evidence of a bulla or bullae occupying at least one third of a hemithorax, who had also been examined with high-resolution CT, were included in this retrospective study. We examined the size, distribution, and locations of bullae. On high-resolution CT scans, bullae were categorized as predominantly subpleural or intraparenchymal. RESULTS: In eight of the nine cases, the chest radiographs showed variable asymmetry in the distribution of bullae. Bullous disease involved predominantly the upper lobes. High-resolution CT showed bullae from 1 to 20 cm in diameter, but most were 2-8 cm in diameter. Paraseptal emphysema and subpleural bullae were the predominant findings in all nine patients. Seven patients had separate centrilobular emphysema of various degrees and intraparenchymal bullae. None of the intraparenchymal bullae were larger than 2-3 cm. Additionally, two non-small-cell lung cancers were seen in our series. CONCLUSION: The dominant and consistent feature seen on high-resolution CT scans in both smokers and nonsmokers is extensive paraseptal emphysema merging into giant bullae. Associated centrilobular emphysema, seen in cigarette smokers, is the important variable finding for determining the extent of underlying parenchymal disease, which may help in the preoperative assessment of giant bullous lung disease.     

Experimental emphysema

This animal model of emphysema exhibits the same abnormalities in respiratory mechanics as those seen in human emphysema. The histologic and radiographic findings also closely resemble changes of panacinar disease. Moreover, the progressive hypoxemia preceding hypercarbia also parallels the clinical course seen in human disease. Drawbacks of this model include the long time period required to develop significant changes and the cost of maintaining the animals for such a time period. Large cystic areas were not noted in our animals and one would have to turn to another model to address the problem of giant bullous emphysema. There is no ideal animal model of pulmonary emphysema, and the usefulness of an experimental model should be judged on how well it answers the specific questions. Significant information has been obtained using various animal models of emphysema in lung transplantation, diaphragmatic function, pulmonary hemodynamics, and in several other areas. The dog appears to be a suitable model for thoracic surgical research on emphysema.     

Expression of the BCL6 gene in the pre- and postnatal mouse

Langerhans cell histiocytosis may be seen with goiter and histiocytic infiltration of the thyroid. We report a 2 1/2-year-old boy who had goiter and primary hypothyroidism develop, later had pulmonary disease, and died of neurologic involvement. Autopsy lesions suggested a transitional dendritic cell precursor of the epidermal Langerhans cell. Of the reported cases of Langerhans cell histiocytosis with goiter in children and adolescents, 82% were male when the relative incidence of Langerhans cell histiocytosis is two males to one female.     

Aggressive surgical management of testicular carcinoma metastatic to lungs and mediastinum

During the past six years, more than 200 patients were treated with chemotherapy for disseminated testicular cancer with a 70% complete remission rate. In 22 patients who were 17 to 46 years old, there was persistent thoracic disease, which was treated surgically. Six required a median sternotomy for bilateral pulmonary involvement or mediastinal metastasis. In 8 patients, chemotherapy had altered the histological appearance of the metastases from that of an undifferentiated primary tumor to a mature cystic teratoma. Five patients had nodules in the lungs, which were necrotic and fibrosed with no evidence of tumor. Nine showed embryonal cell carcinoma metastases in the lungs. All who had cystic teratoma are alive and free from disease. Three of the 5 with nodules and 1 of the 9 with metastases are currently free from disease. Agressive surgical intervention is important in this unique group of patients in order to determine the precise pathological category of the lesions, to remove intrathoracic malignancy, and to assess the need for additional chemotherapy. An operative mortality of zero and a low morbidity justify this approach.     

Lung surface area in various morphologic forms of human emphysema

The relation between lung surface area and the extent and morphologic type of emphysema was examined at autopsy in 69 men. It was found to be conceptually preferable to express the area as internal surface area per unit lung volume (specific surface area) rather than to use the more conventional, inversely related, mean linear intercept for comparison with other morphometric data. In the whole group, fixed inflated lung volume generally increased with increasing per cent involvement of lung parenchyma by emphysema, and total internal surface area decreased, but those changes were not so regular as the decline in specific surface area with increasing per cent emphysema (r=-0.574). Lungs with no emphysema had a significantly higher specific surface area than did lungs with very little emphysema (1 to 9 per cent), suggesting noticeable effects on the physical properties of lung by minimal degrees of this disease at high levels of inflation. The way in which specific surface area decreased with increasing percentage of emphysema was not noticeably different among patients with centrilobular, panlobular, or mixed forms of emphysema, although lungs with only centrilobular emphysema were much more common and had a significantly lower mean percentage of emphysema than did those with only panlobular emphysema; the prevalence and mean percentage of emphysema of the mixed group were intermediate. These observations, coupled with the association of older mean age with increasing percentage of emphysema, were interpreted as support for the concept that many examples of panlobular emphysema are derived from confluence of extensive centrilobular emphysematous lesions.     

Environmental pollution with tobacco smoke--a threat to human health. Principles for prevention of tobacco smoking

Tobacco smoking is an important pathogenetic factor for man, but it is underestimated in our country. The great spread of this factor justifies the term "tobacco epidemic". Three epidemiological surveys made in Cracow and in the country near Lańcut indicated that 37% of the people smoked tobacco: 42% in the city of Cracow and 29% in the country. In 9.7% of adult inhabitants of Cracow, 15.6% of the males and 5.0% of the females had symptoms of chronic bronchitis. In currently smoking men chronic bronchitis was found in 20%, five times more frequent than in nonsmokers, and in 12% of the smoking women, four times more frequent than in non-smokers. In rural inhabitants chronic bronchitis existed in 6.7% of dwellers of suburban villages and in 4.4% of residents of distant villages. In currently smoking men chronic bronchitis was found in 11.7% in suburban villages and in 6.3% in distant ones. This proves the synergistic influence of industrial and transport-related air pollution and cigarette smoke. The consequences of cigarette smoking were discussed: chronic bronchitis and emphysema, heart diseases, cancer of the lung, passive smoking. In antismoking action the most important aim is to protect the young people from initiation to smoking. In adults the decision to abandon smoking is very dependent on the intellectual level of the smoker. Antismoking propaganda must be conducted with great tact, and the advantages of quitting smoking should be emphasized.     

Aspects of chronic airflow obstruction

This report questions several commonly used definitions and commonly accepted concepts. It suggest that the term, "chronic airflow obstructions," should replace the terms, "chronic obstructive pulmonary disease," "chronic obstructive lung disease," or "chronic airway obstruction," because it is flow that is obstructed. It is suggested the term, "chronic mucous hypersecretion," be used, rather than "chronic bronchitis," and that the latter be avoided. Chronic bronchitis should not be equated with narrowing of the airway and emphysema with loss of elastic recoil. Chronic bronchitis, emphysema, and lesions of the small airways probably occur together more frequently than chance will allow because of a common etiologic agent, tobacco smoke. Chronic mucous hypersecretion without other airway or parenchymal lesions seldom produces airflow obstruction and does not impair prognosis significantly. Central airways are important in chronic airflow obstruction. It is time that someone found out what is happening in subjects with abnormal results on tests of the function of small airways. The definition of "destruction" as it occurs in emphysema is deceptive, and loss of recoil and emphysema may be separate conditions. The dysfunction that occurs in emphysematous lungs is due mainly to associated airway lesions and may perhaps be due in part to the site and nature of emphysematous lesions (as opposed to loss of elastic recoil).     

Transfectable and transplantable postmitotic human neurons: a potential "platform" for gene therapy of nervous system diseases

OBJECTIVE: To evaluate F-18 fluorodeoxyglucose positron emission tomography (PET) in terms of its sensitivity and specificity in diagnosing malignant pulmonary nodules and staging bronchogenic carcinoma. METHODS: A retrospective review of any patient that presented to the VA Palo Alto Health Care System with a pulmonary nodule between 9/94 and 3/96 revealed 49 patients (four female, 45 male) age 37-85 (mean 63) with 54 pulmonary nodules who had: chest CT scan, PET scan; and tissue characterization of the nodule. Characterization of each nodule was achieved by histopathologic (N = 44) or cytopathologic (N = 10) analysis. Of the 49 patients, 18 had bronchogenic carcinoma which was adequately staged. Mediastinal PET and CT findings in these 18 patients were compared with the surgical pathology results. N2 disease was defined as mediastinal lymph node involvement by the American Thoracic Society's classification system. Mediastinal lymph nodes were interpreted as positive by CT if they were larger that 1.0 cm in the short-axis diameter. RESULTS: Sensitivity and specificity for the diagnosis of malignant pulmonary nodules using PET was 93 and 70%, respectively. All nodules (N = 3) that were falsely positive by PET scan were infectious in origin. All nodules (N = 4) that were falsely negative by PET were technically limited studies (outdated scanner, no attenuation correction, hyperglycemia) except for one case of metastatic adenocarcinoma. The sensitivity and specificity of PET in diagnosing N2 disease was 67 and 100%, compared with 56% and 100% for CT scan (not statistically significant). However, one more patient with N2 disease was correctly diagnosed by PET than by CT scan. CONCLUSION: PET is a valuable tool in the diagnosis and management of pulmonary nodules and may more accurately stage patients with bronchogenic carcinoma than CT scanning alone.     

Transscleral cyclophotocoagulation with the diode laser for neovascular glaucoma

Despite a high prevalence of canine dirofilariasis, there is no case of pulmonary dirofilariasis reported from Thailand. We herein report a case of multisystem Langerhans cell histiocytosis who had an incidental pulmonary dirofilariasis found at the time of autopsy as a solitary nodule at the periphery of the right lower lobe. This is the first reported case in Thailand. Association between pulmonary dirofilariasis and Langerhans cell histiocytosis has not been described before in the literature.     

Measurement of CT lung density in patients with chronic asthma

Evidence for the presence of emphysema in patients with asthma is controversial. We have previously shown that decreased lung density, measured by computed tomographic (CT) scanning, preoperatively, correlates with morphometric measurements of microscopic emphysema in subsequently resected lungs. The aim of this study was to compare CT lung density, in 17 patients with chronic asthma (forced expiratory volume in one second (FEV1) 1.98 (SD 0.77) L), 17 patients with chronic bronchitis and emphysema (FEV1 0.97 (0.56) L) and seven normal subjects (FEV1 3.5 (034) L). All subjects underwent CT scanning of the lungs and respiratory function testing within 2 days of each other. In five of the asthmatic patients a CT scan was performed on two occasions before and after treatment with nebulized bronchodilator. In a different group of five asthmatics these measurements were performed at the end of and 6 weeks after an exacerbation. The mean value of the lowest fifth percentile of the CT lung density in the patients with chronic obstructive pulmonary disease (COPD) was -942 (SD 36) Hounsfield units (HU), in the 17 asthmatic patients was -912 (34) HU, and in normal subjects was -880 (13). Despite a significant increase in peak expiratory flow rate from 266 (SD 102) to 406 (83) L x s(-1) (p<0.02) following nebulized beta2-agonist in five patients with chronic asthma, there was no significant change in CT lung density (p>0.05) Our study indicates that at least some patients with chronic, stable asthma develop a reduction in computed tomography lung density, similar to that in patients with emphysema.     

CT-image analysis of resected peripheral adenocarcinomas of the lung less than 1 cm in diameter

Conventional CT (10-mm thick) and helical thin-section CT (2-mm thick) high-resolution images were obtained to study the relationship between the appearance of small peripheral adenocarcinomas of the lung and pathological findings. Eleven cases in which adenocarcinomas less than 1.0 cm in diameter were resected were retrospectively reviewed. Conventional CT images revealed air spaces within pulmonary nodules in 82% of tumors, an ill-defined margin in 91%, and involvement of vessels in 91%. When these findings are observed in pulmonary nodules, thin-section CT should be used for further examination. Helical thin-section CT images showed inhomogeneous internal attenuation (91%), irregularly undulating margins (91%), and vascular involvement (100%).     

Pneumonitis and pulmonary abscess associated with Moraxella nonliquefaciens

A 78-year-old man with pneumonitis and pulmonary abscess associated with Moraxella nonliquefaciens is presented. This organism was found by culture of both transtracheal aspirate and sputum. No previous reports have associated M nonliquefaciens with infection of the lower respiratory tract, although sinusitis and bronchitis have been reported. Possible predisposing factors in our patient included carcinoma of the larynx, as well as alcohol ingestion and cigarette smoking.     

Pathology of the central nervous system in Chester-Erdheim disease: report of three cases

Chester-Erdheim disease is a rare form of non-Langerhans cell histiocytosis consisting of disseminated xanthogranulomatous infiltration and fibrosis that primarily involves the bones, visceral organs and systemic fatty spaces. Involvement of the central nervous system is variable, and neuropathological features have seldom been documented. We report the neuropathological findings in 3 autopsy cases. One patient had radiological and pathological bone changes characteristic of Chester-Erdheim disease. Neuropathology revealed multiple characteristic xanthogranulomas disseminated in the cerebral hemispheres, hypothalamus, cerebellum, and brainstem. The second patient presented first with cutaneous lesions characteristic of Langerhans cell histiocytosis. She subsequently developed bone abnormalities suggestive of Chester-Erdheim disease, which was confirmed by autopsy, raising the possibility of a common spectrum of histiocytosis including both diseases. Gross examination of the brain was normal, however, microscopy showed infiltration of the brain by characteristic non-Langerhans cell xanthogranulomas. The third patient presented with systemic features characteristic of Chester-Erdheim disease. Neurological signs included gait disturbance, seizures and confusion. Examination of the brain did not show any histiocytic infiltration, but did show changes suggestive of Hallervorden-Spatz syndrome. Association of Chester-Erdheim disease and Hallervorden-Spatz syndrome has not been previously reported. The relationship between both conditions is unclear.     

Cutaneous Langerhans cell histiocytosis of the genitalia in the elderly: a report of three cases

Langerhans cell histiocytosis (LCH) is a disease with a broad spectrum of clinical presentations. All of the variants have in common the proliferation of cells which are morphologically, biochemically, and immunophenotypically indistinguishable from Langerhans cells. A retrospective study of three elderly patients revealed the unique presentation of cutaneous Langerhans cell histiocytosis limited to the genitalia. These cases produced a diagnostic challenge because of their unusual clinical presentation and their morphological similarity to certain other entities, including extramammary Paget's disease and malignant melanoma, which may also show S-100-positive atypical cells. All three cases showed infiltrates of histiocytic-appearing cells with folded nuclei and moderate amounts of cytoplasm which involved the epidermis, dermis, or both. Immunoperoxidase studies using antibody to S-100, CD1a and CD68 in each case showed positive staining.     

Evaluation of emphysema progression as measured by computed tomography in patients with chronic obstructive pulmonary disease

It has been difficult to assess the progression of pulmonary emphysema since accurate quantification of the extent of this condition has only previously been possible on post mortem or resected lungs. Previously we have shown that measurements of CT lung density correlated with the degree of emphysema, measured morphometrically in resected lungs. We have therefore employed this technique to assess the progression of emphysema in 17 patients (12M, 5F) with wide range of chronic airflow limitation (FEV1 15-68% predicted). There was little change in the degree of airflow limitation, hyperinflation or arterial blood gas values over 30 +/- 4 months of follow up (p > 0.05). However during this period there was a significant decrease in the lowest 5th percentile of CT density, which fell from -920 +/- 32 to -940 +/- 36 Hounsfield units (p < 0.005) associated with significant fall in the diffusing capacity for carbon monoxide. We believe that these data show the ability of CT scanning to assess the progression of emphysema in patients with chronic obstructive pulmonary disease.     

Expression of macrophage markers in childhood and adult Langerhans histiocytosis (LCH)

In the diagnosis of the Langerhans cell histiocytosis several monocyte and macrophag markers have been tested in the recent years. We compared the expression of macrophage and lymphoid markers in childhood and adult type Langerhans cell histiocytosis. Ten childhood and 11 adult cases were tested using paraffin sections of biopsy samples. We have examined 6 markers: the S-100, Lysozyme, CD68 macrophag and the CD1a, CD4, HLA-DR lymphoid markers. We have found that the CD68 marker was more frequently positive than the other examined macrophag markers, and proved to be almost as reliable as the recently discovered CD1a. The most interesting result was that the expression of the markers was different in the childhood and adult type of the disease. On the basis of our experience the possibility arise that the phenotype of the childhood and adult type of the Langerhans cell histiocytosis is different.     

Chronic bronchitis. Key points in evaluation

Thorough history taking is essential in evaluation of chronic bronchitis. Patients often reveal key symptoms that help define the disorder and provide information about contributing factors, such as cigarette smoking, that can be eliminated. Useful baseline data can be collected through pulmonary function studies, electrocardiogram, chest radiographs, complete blood cell count, and measurement of electrolyte levels.     

The assessment of the severity of pulmonary edema by computed tomography. A comparison between high-resolution computed tomography, objective evaluation with density masks and functional tests

The results of high-resolution computed tomography (HRCT) were correlated with those of pulmonary function tests, chest films and CT expiratory density mask values in the evaluation of pulmonary emphysema in 33 symptomatic subjects. Emphysema was quantitated with both subjective and objective measurements. Conventional chest films were useful to diagnose severe emphysema but its actual extent was more reliably evaluated with CT scoring systems. HRCT and density mask correlated well with function tests, but the former method exhibited stronger correlation with carbon monoxide diffusion capacity. The opposite was true for hyperinflation and expiratory obstruction variables. Subjective CT estimates, which are quick and easy to perform, were seen to correspond more specifically to the pathophysiologic derangement and should therefore be used to evaluate the anatomic extent of disease. The functional severity of emphysema correlated only with the overall extent of disease and not with its regional distribution in the upper or lower lungs. Finally, in 4 cases (12.1%) with low CT scores, FEV1 was reduced but diffusion capacity values were normal. In one of these patients HRCT showed signs of bronchiolitis. In fact, small airway disease might be a more critical factor in determining functional impairment than the actual anatomical emphysema.     

A case suspected of early active pulmonary tuberculosis detected by CT with the onset of episcleritis

A 56-year-old female with the chief complaint of conjunctival injection was referred to our hospital after treatments with oral and topical corticosteroid under the diagnosis of episcleritis without any therapeutic efficacy. Possible causes of the episcleritis such as collagen vascular disease were not found, but a chest CT revealed centrilobular nodules, branching linear lesions and bronchial wall thickening which were not detected in a plain X-ray picture. We suspected pulmonary tuberculosis and tuberculous episcleritis based on these CT findings, strongly positive tuberculine skin test result and a history of contact with a smear positive tuberculosis patient. The pulmonary lesion disappeared and the episcleritis healed after the treatment with systemic antituberculous agents.