Congenital lobar emphysema resulting from bronchial sling around a normal right main pulmonary artery

A 5-month-old female infant was transferred to our institution for evaluation of recurrent upper respiratory tract symptoms and chest x-ray films showing hyperaeration of the right upper lobe. Preoperative studies including chest fluoroscopy, lung scan, and bronchoscopy suggested congenital lobar emphysema. At thoracotomy, an anomalous right upper lobe bronchus which bifurcated around the main pulmonary artery in a sling fashion was found. This anatomic abnormality has not been described previously and is a unique cause of congenital lobar emphysema.     

Familial adenomatous polyposis

BACKGROUND: Congenital lobar emphysema is an unusual condition and its pathogenesis remains unknown. The variety of findings in pathology studies of the resected specimens led to increasing academic interest. About 50 per cent of the cases have no definitive diagnosis in pathology. The most recent theory proposes an increased number of alveoli within each acinus (polyalveolar lobe). PURPOSE: The aim of this paper is to report the morphometric measures of surgical specimens of 12 patients with congenital lobar emphysema, using the Emery and Mithal technique (radial alveolar count). METHODS: We made a case-control study, classifying the cases by age. Mann-Whitney's U test and linear regression techniques were used in data analysis: Mann-Whitney's U in comparing the cases and respective controls and linear regression to evaluate the influence of age in the measures found. RESULTS: The results revealed a significantly higher radial alveolar count than expected for the age group under 3 years; no difference was observed in the age group between 3 and 7 years and in children older than 7, the radial alveolar count was lower than expected. The normal development of the lung consists in an increasing number of alveoli increase from birth until adulthood, but this number remains constant, independent of age in congenital lobar emphysema. CONCLUSIONS: Such findings allow us to conclude that polyalveolar lobe can and must be diagnosed by a simple and practical method, such as the radial alveolar count, which decreases the incidence of the unknown etiology. The findings of an increased number of alveoli on patients younger than 3 is related to congenital lobar emphysema, since the number of alveoli does not increase in congenital lobar emphysema, just the opposite to what one would expect in the normal development of the lung.     

Bilateral Krukenberg tumors due to appendiceal mucinous carcinoid

Three cases of congenital polyalveolar lobe (pulmonary hamartoma) were diagnosed in female Thoroughbred foals. Foal 1 was born at full term but died shortly afterwards. Foal 2 was aborted at the seventh month of gestation. Parturition was induced at the tenth month of gestation in foal 3 because it developed hydrops of the amnion and ascites. In all three foals, the polyalveolar lobe occurred on the right side and affected the entire right lung. In each case, the right lung formed a tumour-like mass, and expanded into the left chest cavity. The lung masses were pink to dark red and spongy to rubbery, with marked lobular patterns on the pleural and cut surfaces. The left lung was compressed and small. The right and left lungs weighed 0.9-6.3 kg and 80 g-0.3 kg, respectively. Microscopically, the polyalveolar lobe consisted of normal alveoli, bronchioli and blood vessels, but the alveolus:artery ratio was greater than normal. In the polyalveolar lobe of two foals the numbers of alveoli per artery were 65.2 and 52.5; in contrast, the corresponding values for three control lungs were 26.9, 26.5 and 27.6. Chronic passive congestion with generalized oedema was observed in foals 2 and 3.     

Lung growth and airway function after lobectomy in infancy for congenital lobar emphysema

To characterize the outcome of lobectomy in infancy and the low expiratory flows which persist after lobectomy for congenital lobar emphysema, 15 subjects with this history were studied at age 8-30 yr. Total lung capacity was normal in all, but higher values (P < 0.05) were observed in nine subjects with upper lobectomy than in five subjects with right middle lobectomy. Ratio of residual volume to total lung capacity was correlated (P < 0.05) with the amount of lung missing as estimated from normal relative weights of the respective lobes. Xe(133) radiospirometry in eight subjects showed that the operated and unoperated sides had nearly equal volumes at total lung capacity, but that the operated side was larger than the unoperated side at residual volume. Perfusion was equally distributed between the two sides. Similar findings were detected radiographically in four other subjects. Forced expiratory volume in 1 s and maximal midexpiratory flow rate averaged 72 and 45% of predicted, respectively. Low values of specific airway conductance and normal density dependence of maximal flows in 12 subjects suggested that obstruction was not limited to peripheral airways. Pathologic observations at the time of surgery and morphometry of the resected lobes were not correlated with any test of pulmonary function. These data show that lung volume can be completely recovered after lobectomy for congenital lobar emphysema in infancy. The volume increase occurs on the operated side, and probably represents tissue growth rather than simple distension. The response to resection is influenced by the particular lobe resected and may be associated with decreased lung recoil near residual volume. Low expiratory flows in these subjects could be explained by several mechanisms, among which a disproportion between airway and parenchymal growth in infancy (dysanaptic growth) is most compatible with our data.     

Usefulness of computed tomography and scintigraphy in diagnosis of emphysematous bullae in the lung

The x-ray examinations usually do not reveal morbid changes after lung expansion in the treatment of spontaneous pneumothorax. In our observation computed tomography (CT) and scintigraphy enable not only the exact determination of the extent of changes but also they disclose bullae invisible in conventional chest radiographs. 15 patients with cured spontaneous pneumothorax and 10 patients with radiographic evidence of a bulla or bullae were examined. CT scans showed bullae from 3 to 18 cm in diameter involving predominantly the upper lobes. 4 patients had additionally subpleural or intraparenchymal bullae of various degrees. In all patients with cured spontaneous pneumothorax, CT scans revealed intraparenchymal bullae, and in 6 cases bilateral intraparenchymal bullae were revealed. Only in sites of large bullae, no isotopic marker or its low elimination was shown in perfusion and inhalation scintigraphy. 10 patients with giant bullous emphysema were operated; in 6 patients enucleation of bullae, in 3 lobectomy and in one patient bullectomy were performed. CONCLUSION: CT is a method of choice in the diagnosis of lung emphysematous bullae and it enables the detection of the changes undetectable in chest radiographs. Perfusion and inhalation scintigraphy is helpful in the diagnosis of large emphysematous bullae and postoperative follow-up examination.     

Flap of the musculus latissimus dorsi to prevent alveolar air leakage from sectional plane of the lung after resection of metastatic pulmonary and chest wall tumor

Alveolar air leakage after pulmonary resection usually heals with adequate pleural drainage, but must be more actively treated if leakage may be severe. If left untreated, the postresection space can lead to empyema. We used a muscle flap to prevent alveolar air leakage from a large sectional plane of the lung resected because of metastases in the lung and chest wall. A 48-year-old man complained of pain and a mass on the right side of his back. He had undergone resection and chemotherapy for clear cell sarcoma that originated on the back of the left hand when 43 years of age, wedge resection of the right lower lobe of the lung for a metastatic pulmonary tumor at 46 years, and lobectomy of the same lobe for a recurrence of the metastatic pulmonary tumor at 47 years. The diagnosis was of a metastatic tumor to the right chest wall with peripheral pulmonary tumors of the right upper and middle lobes. Resection of the chest wall and the lung including the tumors was done. Much air leakage from the extensive sectional plane of the right upper and middle lobes was seen intraoperatively, and this plane was therefore covered with a flap of the musculus latissimus dorsi. Chest tubes were removed on day 7 postoperatively when air leakage was no longer seen. Subcutaneous emphysema, which appeared on day 14 postoperatively, required redrainage of the pleural air space, but pleurodesis was effective. Use of a muscle flap was simple and effective for covering of a sectional plane of the lung, and should be considered when alveolar air leakage may be extensive.     

Lung volume reduction surgery as an emergency and life-saving procedure

Lung volume reduction surgery (LVRS) is emerging as a promising and unique therapeutic option for rigorously selected patients with severe debilitating emphysema. A 51 yr old man with generalized emphysema developed bilateral pneumothoraces during his first holiday abroad. Due to respiratory insufficiency, intubation and mechanical ventilation were necessary. In total, six chest tubes were inserted but massive air leak persisted and his respiratory condition deteriorated due to bronchopneumonia and sepsis. The patient was transferred to Belgium. As a last resort, bilateral LVRS was performed through a median sternotomy. The most diseased areas of the upper lobes containing the air leak were resected bilaterally and a pleurectomy was associated. Three months after operation, there was a remarkable improvement in spirometric values with an increase in forced expiratory volume in one second of almost 100%. The results were sustained after a follow-up of 18 months. In this dramatic case, lung volume reduction surgery proved to be effective, and was even a life saving procedure.     

Respiratory function and surgical reduction of lung volume

Emphysema is characterised by an enlargement of the terminal air spaces. Destructions of alveolar walls lead to a loss of the lung elastic recoil. The driving pressure for expiration is decreased and the outward forces acting on the bronchioles are lost, leading to bronchiolar collapse and airflow limitation. Hyperinflation of the lungs and overdistension of the chest wall cause the respiratory muscles to operate in unfavourable conditions. Patients with advanced emphysema have decreased quality of live: they are dyspneic at rest and are unable to perform exercise. Surgical excision of parts of diffusely emphysematous lungs (Lung Volume Reduction Surgery, LVRS) has been proposed since many years. Expansion of the remaining lung should increase lung elastic recoil and restore the outward forces on the bronchioles. It has been demonstrated that LVRS reduces dyspnea symptoms, improves exercise tolerance and enhances the quality of live. LVRS increases lung elastic recoil, airway conductance and maximal expiratory flow, reduces dynamic hyperinflation and improves the efficiency of the respiratory muscles. These improvements are maintained for at least 12 to 18 months. Preoperative evaluation, surgical-induced modifications of pulmonary functions and postoperative exercise training are exposed.     

Aftermath of failed diagnosis of late-presenting congenital diaphragmatic hernias

A congenital posterolateral diaphragmatic (Bochdalek) hernia generally presents with symptoms within a day after birth. This article reports on 3 children whose hernias produced symptoms for the first time beyond a week of age. One 3-year-old child died acutely with symptoms resembling that of a tension pneumothorax. In 2 children symptoms only developed at 12 and 23 days of age. They were initially thought to have lower lobe pneumonias and delayed diagnosis led to the death of one. Although the late presentation of a congenital posterolateral diaphragmatic hernia is rare, it is important to recognise it because with appropriate therapy all such children should have a normal life expectancy, whereas unsuspected hernias in older children carry a mortality that is even worse than that of neonatal cases. The first requisite for the diagnosis of a congenital diaphragmatic hernia (CDH) is a high index of suspicion. Cystic lesions or masses in the lower lung fields should suggest the possibility of a CDH with herniated abdominal content at any age. Congenital diaphragmatic hernias should be included in the differential diagnosis of apparent lower lobe pneumonias in all children below a month of age.     

Tonoko pneumoconiosis with deposition of titanium

A 56-year-old man had worked as a furniture painter and had been exposed to tonoko polishing powder for 30 years. He had complained of dyspnea on exertion for 7 years, and was admitted to our hospital in 1991. A chest X-ray film revealed large opacities and emphysematous changes in both lung fields. Tonoko pneumoconiosis was diagnosed after transbronchial lung biopsy. Home oxygen therapy was given because of progressive hypoxemia, but the patient died of respiratory failure. At autopsy, examination of the lungs showed severe emphysematous changes, and collapse of alveoli. Many gray masses resembling coating cement were seen especially in the upper lobes. Microscopical examination showed that the large opacities were composed of hyaline nodules, similar to silicotic nodules, with anthracosis. Scanning electron microscopy with X-ray microanalysis revealed a high concentration of titanium in the gray mass. A low dose of titanium would not be expected to induce fibrotic changes in the lung, but a high dose and long-term exposure might have that effect. Titanium contained in paint might have exacerbated tonoko pneumoconiosis in this patient.     

Diagnostic accuracy of technologies used in LBP assessment

Postoperative improvement of respiratory function has been reported with lung volume reduction surgery (LVRS) in patients with severe emphysema. Since smoking is an established risk factor for lung cancer, vascular diseases and emphysema, it is not infrequent to find these diseases associated in the same patient. Combined treatment of lung cancer and emphysema has already been reported. Surgical treatment of vascular diseases in emphysematous patients could also benefit from the application of LVRS techniques. We report resection of an aortic aneurysm combined with LVRS in a patient with concomitant thoracic aortic aneurysm and severe emphysema. Respiratory function improved in the postoperative period.     

County funding of mental health services in a rural state

A case of lobar torsion after lung surgery is reported here. She developed a right middle lobar torsion diagnosed by postoperative bronchial fiberscopy, suggesting incomplete obstruction of the intermedial bronchus. Re-operation was performed successfully either by fixing the right lower lobe to both the middle lobe and the thoracic wall. Careful postoperative observation with chest radiography, bronchial fiberscopy, are important for precise diagnosis of and therapy for lobar torsion following pulmonary lobectomy.     

Shear bond strength of amalgam adhesives to dentin

Recent studies have suggested that failure of pentamidine prophylaxis against Pneumocystis carinii pneumonia (PCP) may be due to reduced deposition of pentamidine in the upper lobes. In this study, we performed bronchoalveolar lavage from the apical segment of the upper lobe and the middle lobe in 51 HIV-positive patients, all of whom were receiving prophylaxis with aerosolized pentamidine, who had presented with acute respiratory symptoms. Lavage fluid from each lobe was assayed for pentamidine using high-performance liquid chromatography (HPLC). The number of clusters of P carinii were counted after staining with a Wright-Giemsa stain. The patients were subclassified as PCP-positive (32 patients) and PCP-negative (19 patients) on the basis of the presence/absence of P carinii clusters in their BAL fluid. The concentration of pentamidine in the upper lobe compared with the middle lobe was no different (using paired Student's t tests) for either PCP-positive patients or PCP-negative patients. In comparing the positive with the negative subjects, using unpaired Student's t test, there was no difference in the concentration of pentamidine in the upper lobe or the middle lobe. For PCP-positive patients, the numbers of P carinii clusters were on average higher in the upper lobes (mean +/- SD: upper = 14.9 +/- 16.6, middle 7.5 +/- 10.8, p = 0.013, paired Student's t test), but there was no correlation between lobar P carinii cluster counts and pentamidine levels. We conclude that the absence of a relationship between cluster count and pentamidine level, the similarity in regional pentamidine levels between upper and middle lobes, as well as the similarity in pentamidine levels between the PCP-positive and PCP-negative groups indicate that the regional dose of pentamidine is not the determining factor as to whether aerosolized pentamidine prophylaxis will succeed or fail.     

Transient giant negative T wave in acute anterior myocardial infarction predicts R wave recovery and preservation of left ventricular function

Resection of large bullae to decompress adjacent lung tissue with the goal of improving pulmonary function has been an accepted surgical approach for many years. However, the indication for lung volume reduction is not bullous disease but diffuse emphysema and the surgical approach is based on an entirely different concept. The resection of the most affected parts of the emphysematous parenchyma aims at a reduction of the over expansion of the chest with the goal of improving respiratory mechanics. This concept was introduced by Brantigan in 1959, but has failed to gain widespread acceptance until recently. Based on the extensive experience in lung transplantation for patients with end stage emphysema J. D. Cooper reevaluated the idea successfully. He reported remarkable improvements in FEV1 and a reduction in hyperinflation after performing bilateral lung volume reduction through a median sternotomy. During the last 2 years we performed bilateral lung volume reduction in more than 30 patients with diffuse emphysema using video assisted thoracoscopy (VAT) and studied the results prospectively. In the first 20 patients preoperative mean forced expiratory volume in 1 second (FEV1) was 765 ml/sec and improved by a mean of 42% (0-100%) three months postoperatively. This gain in FEV1 was already observed at the end of hospitalisation approximately two weeks after surgery. The 12 minute walking distance improved over 40%. In our highly selected study population we had no perioperative mortality. Lung volume reduction is a palliative treatment of severe pulmonary emphysema. Currently no data is available on the duration of the improvement. In this selected group of patients dyspnea is reduced and pulmonary mechanics are improved, with a resulting increase in quality of life.     

Using ARROWSMITH: a computer-assisted approach to formulating and assessing scientific hypotheses

The usefulness of Xe-133 and Tc-99m-MAA single photon emission computed tomography (SPECT) in identifying areas to be resected during video-assisted thoracoscopic lung reduction surgery for emphysema was examined. Twenty-nine patients with advanced emphysema were examined using Xe-133 and Tc-99m-MAA SPECT prior to and following surgery. For the Xe-133 dynamic SPECT, patients inhaled Xe-133 gas for 6 minutes. Equilibrium and subsequent washout SPECT images were acquired every 30 seconds for 6 to 7 minutes during spontaneous breathing. Ventilation was quantified by Xe-133 clearance time (T1/2) in addition to visual assessment. The patients underwent unilateral thoracoscopic volume reduction in the regions with abnormal Xe-133 retention and Tc-99m-MAA defect. All patients demonstrated marked, heterogeneous Xe-133 retention and Tc-99m-MAA defects preoperatively. The worst functioning areas were identified as nonventilated and noflow areas, or areas with air trapping and low perfusion. These changes were found even in patients with diffuse and symmetrical impairments on chest CT. After surgery, most of these "target areas" disappeared and pulmonary function tests demonstrated significant improvement. T1/2 correlated closely with the percent predicted FEV1 (%FEV) and 6-minute walk distance before and after surgery (p<0.0001). Xe-133 and Tc-99m-MAA SPECT imaging was useful in identifying "target areas" in the emphysematous lung. Directed unilateral thoracoscopic volume reduction based on these SPECT images is an effective treatment for emphysema.     

Pleural pressure with lobar obstruction in dogs

The effects of acute lobar obstruction on pleural surface pressure in supine dogs were examined. The right lower lobes (RLL) were obstructed at FRC in some dogs while in others the left lung and RLL were both obstructed at FRC. At the end of the subsequent inspiration, costal surface pressure was less over the obstructed lobe than over the unobstructed right upper lobe. Alveolar pressure within the obstructed RLL decreased relative to tracheal pressure at end inspiration resulting in an inflating pressure being applied to the obstructed lobe. In most dogs elastic recoil increased at the lateral costal margin of the obstructed RLL implying the application of a deforming pressure to the obstructed lobes. The tendency to inflate and deform the obstructed RLL was greater during spontaneous breathing than during artificial ventilation.     

Cystic changes in the lungs in childhood (author's transl)

Two boys with presumably congenital cystic changes in the lungs are described. In one child multiple cystic deformations led to acute symptoms of severe respiratory distress necessitating urgent resection of the right middle and upper lobes. The other boy had a large thick-walled cyst in the left upper lobe causing no distress whatsoever. This patient was observed for more than 6 years before operation. Multiple vascular anomalies and anastomoses were revealed during surgery. Both cases are presented in detail and the completely different clinical course is outlined. Only when careful supervision of intrapulmonary cystic changes is guaranteed can operation be postponed for longer than 6 months. Pulmonary cysts may lead to emergency procedures. They are always a potential source of serious complications such as rupture or infection. When no tendency for regression can be discovered surgical removal is indicated. Conservative treatment will not influence the course. Differential diagnosis is discussed briefly.     

Tracheobronchial injuries in blunt thoracic trauma

Tracheobronchial lesions after blunt chest injury are seldom (0,5-0,7%). Diagnostic and therapeutic strategies in 16 own cases and a review of the literature are presented. Own experiences: Locations of the lesions were main bronchus (10), bronchus intermedius (2), and trachea (4). Rupture was total in five cases, and partial in seven. In four patients the mucosa only was ruptured. Initial symptoms: Subcutaneous emphysema (13), pneumothorax (9), respiratory insufficiency (5), lung lesion (5), but tracheal bleeding in five cases only. Diagnosis mainly by bronchoscopy (8 early, 4 late), but in 4 cases after thoracotomy. Treatment: In cases of total rupture, there were three anastomoses of the bronchus and one of the trachea, and one pneumonectomy. In all partial ruptures, there was suturing of the lesion. Mucosa lesions were treated conservatively. RESULTS: 1 empyema, 2 ex. leth. (bilateral pneumonia 7.d., multiple organ failure 20. d). FOLLOW UP: 9 patients free of symptoms, 5 patients with respiratory problems. The symptoms "mediastinal emphysema and continuous air leakage through the chest tube or persistent atelectasis of the lung" are indications for urgent bronchoscopy and early surgery. Long-term results are good in 70%-90% of the cases. Not diagnosed lesions can result in tracheobronchial stenosis and infections of the lung later on, to be treated by lung resection only. Total bronchial ruptures can result in strictures and non-infected atelectasis, resection of the stricture and reinflation of the lung being successful in 60%-70% of these late diagnosed cases.     

Chest illness in infancy and chronic respiratory disease in later life: an analysis by month of birth

BACKGROUND: Cohorts born at different times of year differ in their risk of exposure to seasonal respiratory infections in early life, but are likely to have similar socioeconomic status and lifestyle thereafter. METHODS: We investigated the long-term consequences of acute chest illness in infancy for later development of chronic respiratory disease by analysing variations by month of birth in hospital admissions for respiratory illness (total n = 49,866), chronic respiratory symptoms and ventilatory function among British school children (n = 11,482) and middle-aged adults (total n = 55,829). RESULTS: Admission for bronchiolitis in the first year of life was three times more common for infants born September to November (autumn) than those born March to May, yet people born in the autumn experienced fewer respiratory symptoms and had better ventilatory function. In two surveys of middle-aged men, forced expiratory volume in one second/forced ventilatory capacity (FEV1/FVC) was significantly (P = 0.025) higher among autumn births. Hospital admissions for chronic bronchitis/emphysema and pneumonia varied little with season of birth. Admissions for asthma were significantly (P < 0.05) more common among children and young adults born in the autumn. CONCLUSIONS: These findings do not support the hypothesis of a causal link between chest illness in infancy and the later development of chronic bronchitis and emphysema. The variation in asthma admissions with month of birth deserves further investigation.     

The surgical physiopathology of essential pulmonary emphysema and volume-reduction intervention

The breaking of the interalveolar septa represents, in the pathogenetic mechanism of emphysema, a final event, common to the different etiologic agents. This elementary injury causes a series of consequences, essentially of mechanic-structural type (intrapulmonary aerial spaces-confining parenchyma collapse, bronchial obstruction, dead space augmentation) on the thin and articulate bronchoalveolar architecture, whose final rearrangement determines, at least in part, the clinical picture. In short, the break of alveolar septa involves the formation of intraparenchymal aerial spaces with collapse of the confining lung; the compensatory mechanism to this situation, involves the hyperexpansion of the thoracic cage and flattening of the diaphragm, with the aim of allowing ventilation of the healthy residual parenchyma. Because of the finite capability of expansion of the thoracic cage and of the diaphragm in respect to the theoretical capability of the lung of large intraparenchymal aerial spaces formation, it is easy to imagine that emphysema can cause a serious functional respiratory deficit even before a significant quantity of pulmonary parenchyma is destroyed by the pathogenic process. It may then be hypothesized that a simple reduction of the volume of the lung, even sacrificing a part of "working" parenchyma, might allow the residual lung to come back to a normal ventilation, wholly ameliorating the respiratory exchanges. The clinically more remarkable consequence of lung volume reduction is the amelioration of ventilation mechanics with a decreased respiratory work due to the shift of the tidal volume toward values less proximal to the maximal expandability of the thoracic wall and of the diaphragm. On the other end, it is possible to anticipate an equally significant effect on bronchial obstruction, due to the more favorable matching of the compliance of the thoracic wall and that of the lung. LVRS has significant effect on the TV sharing ratio between emphysematous spaces and residual healthy parenchyma; the hyperexpansion of the residual lung in fact causes the distension of the emphysematous spaces, continuing in the natural compensatory mechanism of the emphysema. The decreased ventilation and thus re-breathing of the residual emphysematous spaces, together with the improved ventilation may ameliorate hypercapnia. Obviously no direct effects can be expected from LVRS on the conditions of the alveolar membrane and thus on gas diffusion capacity through it. The time duration of the amelioration achieved with the lung volume reduction is still to be demonstrated.