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Alpha 1-antitrypsin deficiency: the radiological features of pulmonary emphysema in subjects of Pi type Z and Pi type SZ: a survey by the British Thoracic Association.

In a multicentred survey of alpha 1-antitrypsin deficiency, radiological details have been obtained in 165 subjects homozygous for Pi type Z and in 23 subjects heterozygous for type SZ. Eight-five per cent of all type Z subjects had radiological evidence of pulmonary emphysema which was commoner in smokers than in those who had never smoked and which predominantly affected the lower zones of the lungs; 66% of the type Z subjects identified through family studies also had emphysema. Emphysema in subjects of Pi type SZ was limited to those who had smoked; this phenotype may not carry a specific risk of emphysema.

Adult↗

Surgical management of neonatal interstitial emphysema.

In the past 6 years, 50 patients with interstitial emphysema in our newborn nursery were treated with open operative procedures to remove interstitial air. Thirty-one were critically ill newborns with severe progressive pulmonary interstitial emphysema treated with pleurotomies. Five underwent pleurotomies on both sides, for a total of 36 operations in the 31 patients. All of the 31 patients underwent aggressive medical management to reduce airway pressure before resorting to operation. Ventilator manipulations, selective bronchial intubation, Forgerty catheter occlusion of the bronchus, and percutaneous lung needling were among the methods used to reduce emphysema in these patients. The operation for all 31 patients consisted of multiple linear pleurotomies to lyse blebs. The hilum was skeletonized to release perihilar blebs. A downhill course was reversed by the operation in over 50% of patients. The operation almost always permanently cured the tension emphysema on that side. Seventeen of the 31 patients lived. Eight of ten patients with unilateral disease lived. All of those patients who died had either very low birth weight, severe preoperative hypoxia, shock, or a combination of these problems.

Humans↗

Development of a canine model of pulmonary emphysema and imaging of the emphysematous lung with infrared thoracoscopy.

BACKGROUND: The surgeon must use the results of preoperative computed tomography findings and scintigraphic studies to make a subjective decision during lung volume reduction surgery with regard to the best incision line. OBJECTIVES: Our purpose was to develop an objective and real-time method of detecting areas of pulmonary emphysema by using infrared thoracoscopy. METHODS: Pulmonary emphysema was developed in various lobes of the lung in the dog by injecting porcine pancreatic elastase through the bronchi. One month after the injection, chest computed tomography and lung biopsy were performed. Infrared thoracoscopic study was then performed to reveal the presence of the emphysematous lung tissue. Simultaneously, indocyanine green was injected intravenously, and the time taken for each type of lung tissue to stain blue was measured. RESULTS: Chest computed tomography and histologic examinations revealed pulmonary emphysema-like areas in the porcine pancreatic elastase-injected lung. The computed tomography numbers of the porcine pancreatic elastase-injected lung tissue, normal lung tissue, and the tracheal lumen were -868.8 +/- 18.6, -752.2 +/- 32.5, and -1013 +/- 27.1, respectively. There were significant differences between the porcine pancreatic elastase-injected lung and the normal lung (P <.0001). The time for staining to begin was 10.7 +/- 4.8 seconds for normal lung tissue and 25.8 +/- 9.4 seconds for the emphysematous tissue; the onset of staining emphysematous lung tissue was significantly delayed (P =.003). CONCLUSIONS: We developed a successful canine model of pulmonary emphysema by injecting porcine pancreatic elastase. Infrared thoracoscopic examination revealed that the staining of emphysematous lung using indocyanine green injection was significantly greater than of normal lung.

Animals↗

The ratio of the alveolar ventilations of SF6 and He in patients with lung emphysema and in healthy subjects.

This study assess the possible impact of changes in the morphometry of acinar airways and air spaces on the efficacy of intrapulmonary gas mixing for sulphur hexafluoride (SF6) relative to that for helium (He). To that end the alveolar ventilations of He and SF6 were determined in patients with macroscopic lung emphysema and in healthy subjects. He-SF6 washout tests were performed in 17 patients (15 emphysema, 2 chronic bronchitis) and 21 healthy subjects. Using a three-compartment model, the data obtained were used to estimate the overall, effective, alveolar ventilations of SF6 and He, and their ratio VAASF6/VAHe. Mean VAASF6/VAHe (+/- S.D.) for patients (0.80 +/- 0.06) was significantly smaller (P < 0.001) than the value for the group of age-matched healthy subjects (0.90 +/- 0.05) which was non-significantly smaller than the result for the group of young, healthy subjects (0.93 +/- 0.03). In our patients, we also determined a score for emphysema using high resolution computed tomography, and this score correlated inversely with VAASF6/VAHe (r = -0.56, P = 0.018). We have interpreted our observations to mean that in patients with lung emphysema, the efficacy of intrapulmonary gas mixing for SF6 as compared with that for He reflected by VAASF6/VAHe is diminished due to increased diffusive path-lengths within the enlarged air spaces of their lungs which impair diffusive gas mixing for SF6 more than for He.

Adolescent↗

Orbital emphysema: how common, how significant?

STUDY OBJECTIVE: To describe the natural history of orbital emphysema and its management in the emergency setting. DESIGN: Retrospective medical record review. SETTING: Inner-city integrated hospital system (four divisions) including one Level I trauma center. PARTICIPANTS: All cases of orbital floor fracture and orbital emphysema from January 1, 1988, to December 31, 1993. RESULTS: The average patient was aged 33 years, and the male-to-female ratio was 7 to 1. Trauma was the underlying etiology in all patients. Seventy-four percent of patients required hospitalization, and 43% developed significant complications. Eighty-three percent were managed with antibiotics. Orbital emphysema was identified on conventional radiographs in 65% of cases. CONCLUSION: Identification of orbital emphysema in the ED should prompt a careful search for associated injury. Consultation should be sought in the presence of orbital or ocular injury. Antibiotic prophylaxis is not necessary for clean wounds.

Adolescent↗

Morphologic grading of emphysema is useful in the selection of candidates for unilateral or bilateral reduction pneumoplasty.

OBJECTIVE: Radiologic morphology of emphysema proves useful in the selection of candidates for bilateral reduction pneumoplasty. We developed a simple morphologic grading system capable of identifying subsets of patients who had maximal functional improvement after unilateral or bilateral operation. METHODS: Fifty-two patients who underwent unilateral (n=34) or bilateral (n=18) reduction pneumoplasty were evaluated. Emphysema morphology was visually scored by digital roentgenograms and high-resolution computed tomography. In each lung, severity of emphysema (ES), heterogeneity (DHT) and hyperinflation (DHF) degrees, were assessed. Asymmetric ratio of emphysema (ARE) between the lungs was expressed as: higher ES/lower ES scores. Morphometric data were correlated with absolute preoperative-postoperative FEV(1) change (DeltaFEV(1)). RESULTS: No difference was found between the unilateral and the bilateral group for ES and DHT. DHF was greater in the bilateral group (3.1 vs. 2.7, P=0.02) whereas ARE was greater in the unilateral group (1.29 vs. 1. 05, P=0.0001). Stepwise logistic regression extracted as best predictors of maximal DeltaFEV(1), ARE (odds ratio=238, Wald test P=0.04) in the unilateral group, and DHT (odds ratio=24, P=0.03) in the bilateral group. Unilateral group DeltaFEV(1) was greater in patients with ARE>/=1.3 (0.44 vs. 0.24 l, P=0.02). Bilateral group DeltaFEV(1) was greater in patients with DHT>1 (0.50 vs. 0.31 l, P=0. 03). No difference was found when comparing DeltaFEV(1) resulting from unilateral RP and ARE>/=1.3, and bilateral RP (0.44 vs. 0.41 l, not significant). CONCLUSIONS: This morphologic grading system identified subsets of patients who had maximal functional benefit from unilateral or bilateral reduction pneumoplasty and might be useful in the preoperative screening of candidates for either approach.

Adult↗

Unilateral thoracoscopic reduction pneumoplasty for asymmetric emphysema.

OBJECTIVE: We prospectively analyzed the surgical and functional results of unilateral thoracoscopic reduction pneumoplasty which we performed by choice in patients with asymmetric emphysema. METHODS: Between October 1995 and June 1997, 119 emphysematous patients were examined and 34 were operated upon. Among these, 14 selected patients with asymmetric distribution of emphysema in the lungs underwent unilateral reduction pneumoplasty (ten right, and four left). There were 13 males and one female, with a mean age of 62 years. Eligibility criteria included bullous and non-bullous end-stage emphysema with severe limitation to daily activity. RESULTS: No patient required conversion to thoracotomy. Mean operative time ranged between 70 and 240 min with a mean of 103 min. There was no postoperative mortality but five patients developed one or more complications: five prolonged air leaks (>7 days); two pulmonary infections; one empyema. No patient required postoperative mechanical ventilation. Median hospital stay was 8 days. At the 3-month follow-up the mean FEV1 increased from 0.8 l to 1.2 l (P < 0.001). Mean FVC increased from 2.6 l to 2.9 l (P < 0.001). The Medical Research Council dyspnea score decreased from a mean of 3.2 to 1.8 (P < 0.001). CONCLUSIONS: Asymmetric distribution is a frequent finding in patients with severe emphysema. Unilateral thoracoscopic reduction pneumoplasty may represent an ideal approach in this selected group of patients.

Aged↗

Video-assisted thoracoscopic treatment of giant bullae associated with emphysema.

OBJECTIVE: Surgical treatment of bullous emphysema has received renewed attention because of recent advances in minimally invasive techniques. We describe our experience in the thoracoscopic management of patients with bullous emphysema over the last 5 years. METHODS: Twenty-five patients (24 male, one female) with a mean age of 57 years with giant bullae associated with various degree of underlying emphysema, were operated on thoracoscopically at our Institution. The severity of the emphysema was classified according to the criteria of the American Thoracic Society: five patients were in stage I (FEV 1 > 50%), eight patients were in stage II (FEV1 35 to 49%) and 12 patients were in stage III (FEV1 < 35%). Nine patients underwent operation to treat complications related to bullae, 12 presented dyspnoea and four were asymptomatic. We performed 23 unilateral and two bilateral staged thoracoscopic procedures. RESULTS: No intraoperative complications developed. Mean operative time was 107+/-25 min. No patient dead. Mean post-operative chest tube duration was 8+/-4.13 days and mean post-operative hospital stay was 11+/-5.76 days. The most frequent post-operative complication was air-leakage that in 12 patients lasted more than 7 days. Pulmonary function tests were obtained 3-6 months after the operation and statistical comparison between pre-operative and post-operative data was performed using Student's paired t-test. We observed best results in I and II stage patients, but also stage III patients experienced clinical improvement and better quality of life. CONCLUSIONS: Our experience supports the safety and effectiveness of video-assisted thoracoscopy for the treatment of giant bullae. Minimally invasive approach is fully justified especially in the group of patients with severe impairment of lung function.

Adult↗

The preoperative selection of patients for emphysema surgery.

Lung volume reduction surgery for emphysema is evolving rapidly since its re-introduction in 1993. Lung transplantation remains a viable option for others with emphysema. The major difficulty facing surgeons lies in appropriate selection of patients for either procedure. The following paper represents an attempt by review of the literature and personal experience to describe some of the important features involved in patient selection. The current literature on patient selection for lung volume reduction surgery and transplantation for emphysema was reviewed, and the results within the University of Toronto Lung Volume Reduction Program were analyzed. The review suggests that the most reliable predictors of success are heterogeneous distribution of emphysematous change as reflected by the CAT scan and the quantitative ventilation perfusion scan with new emphasis being placed on the ventilation portion of the latter. Poor prognostic indicators are hypercarbia and pulmonary hypertension. It was felt that an algorithm could be established for determination of whether lung volume reduction or transplantation should be offered to patients for emphysema surgery. The algorithm is described.

Animals↗

Results of lung volume reduction surgery for emphysema.

LVRS provides an exciting opportunity for palliation of symptoms and improvement in quality of life for patients who have severe end-stage emphysema. Because no medical therapy has been able to improve pulmonary function or reverse the inexorable decline of breathless patients who have emphysema, this opportunity to improve lung function and quality of life is one of the most innovative additions to thoracic surgery since the first successful lung transplant procedure 20 years ago. Although initial short-term, case-controlled surgeries were criticized because of incomplete and short follow-up care, substantial long-term data now exist to support the use of LVRS for select patients who have severe emphysema. Patients who have upper lobe predominant disease or low exercise capacity are more likely to have a benefit in exercise capacity and quality of life after LVRS. Selected patients who have upper lobe emphysema and poor exercise capacity are also more likely to have improved survival after LVRS. The individual contributions by the large number of investigators pioneering LVRS development, along with the collective contributions of the NETT investigators, have propelled the knowledge surrounding LVRS far beyond that of any similar new technology or procedure in its adolescence.

Humans↗

Bronchiolitis obliterans syndrome in single lung transplant recipients--patients with emphysema versus patients with idiopathic pulmonary fibrosis.

Bronchiolitis obliterans syndrome (BOS) after lung transplantation is a disease of small airways that is currently graded according to a decline in forced expiratory volume in 1 second (FEV(1)) even in single lung transplant recipients in whom native diseased lung may influence lung physiology. The aim of this study was to evaluate the comparative changes in lung function and survival following the onset of BOS in patients with emphysema and patients with idiopathic pulmonary fibrosis (IPF) who have undergone single lung transplantation. We analyzed data from 31 single lung transplant recipients with emphysema and 25 with IPF who were at risk of BOS. There was no difference in the incidence of BOS between the 2 groups (10 patients with emphysema and 6 patients with IPF), but after the onset of BOS the patients with emphysema had a significantly greater median survival (18 months vs 8 months) despite a poorer mean FEV(1) (1.26 liter, 45% predicted vs 2.11 liter, 67% predicted) compared with the IPF group (p < 0.05) and this difference in lung function persisted at death (0.8 liter, 30% predicted vs 1.65 liter, 51% predicted) (p < 0.05). In summary the native lung physiology appears to influence lung function and therefore survival, and this may indicate that the classification of BOS should include disease-specific characteristics.

Adult↗

Quantitative chest computed tomography as a means of predicting exercise performance in severe emphysema.

RATIONALE AND OBJECTIVES: We assessed the value of quantitative high-resolution computed tomography (CT) as a diagnostic and prognostic tool in smoking-related emphysema. METHODS: We performed an inception cohort study of 14 patients referred with emphysema. The diagnosis of emphysema was based on a compatible history, physical examination, chest radiograph, CT scan of the lung, and pulmonary physiologic evaluation. RESULTS: As a group, those who underwent exercise testing were hyperinflated (percentage predicted total lung capacity +/- standard error of the mean = 133 +/- 9%), and there was evidence of air trapping (percentage predicted respiratory volume = 318 +/- 31%) and airflow limitation (forced expiratory volume in 1 sec [FEV1] = 40 +/- 7%). The exercise performance of the group was severely limited (maximum achievable workload = 43 +/- 6%) and was characterized by prominent ventilatory, gas exchange, and pulmonary vascular abnormalities. The quantitative CT index was markedly elevated in all patients (76 +/- 9; n = 14; normal < 4). There were correlations between this quantitative CT index and measures of airflow limitation (FEV1 r2 = .34, p = 09; FEV1/forced vital capacity r2 = .46, p = .04) and between maximum workload achieved (r2 = .93, p = .0001) and maximum oxygen utilization (r2 = .83, p = .0007). CONCLUSION: Quantitative chest CT assessment of disease severity is correlated with the degree of airflow limitation and exercise impairment in pulmonary emphysema.

Aged↗

The pathobiological mechanisms of emphysema models: what do they have in common?

Emphysema results from a multi-step, complex, process of lung destruction. This review aims at organizing the available information concerning the animal models of emphysema as to which step of the pathogenesis they address. The experimental models have been classified as to whether they are based on: (a) pharmacological, (b) environmental, or (c) genetic manipulations to induce emphysema and whether they are: (a) triggers or initiators of emphysema, (b) modifiers of lung predisposition to further damage by trigger factors, or (c) mediators of lung tissue destruction.

Animals↗

Pre-vertebral surgical emphysema following functional endoscopic sinus surgery.

Functional endoscopic sinus surgery (FESS) is a widely practiced technique in the UK. This procedure has variable complication rates and can have some serious consequences. We present a case of surgical emphysema of the neck, face and the pre-vertebral space following FESS. Emphysema of the face and neck has been previously reported. However, to our knowledge, emphysema of the pre-vertebral space following FESS has not been documented. Pre-vertebral emphysema following FESS is an unusual and potentially serious complication.

Aged↗

Short- and long-term functional results after lung volume reduction surgery for severe emphysema.

Lung volume reduction surgery (LVRS) has emerged as a surgical therapeutic intervention for advanced emphysema. Designed for the relief of dyspnoea, LVRS has been demonstrated to be efficacious in a subset of carefully selected patients. Short-term improvements in dyspnoea are accompanied by improvements in forced expiratory volume in one second ranging 13-96%. Lung volumes likewise improve, with lessening of trapped gas, residual volume, and total lung capacity. Improvements in functional status and quality-of-life measures parallel the improvements in dyspnoea and lung function. One preliminary study suggests that life expectancy after 3 yrs may be improved following LVRS. Many questions regarding lung volume reduction surgery in terms of operative technique, selection of patients, and outcome remain to be answered. Data are available which begin to address some of these issues. Bilateral procedures have greater short-term improvements than unilateral procedures, but the rate of loss of function following the surgery may also be greater. Stapled resection of lung tissue has been demonstrated to be superior to laser ablation. In a majority of reports, outcome is superior in patients with heterogeneous distribution of their emphysema, and patients with alpha1-proteinase inhibitor deficiency emphysema do less well than patients with smoker's emphysema.

Humans↗

Functional and morphological heterogeneity of emphysema and its implication for selection of patients for lung volume reduction surgery.

Lung volume reduction surgery (LVRS) in patients with advanced pulmonary emphysema aims to alleviate symptoms and enhance quality of life by improving respiratory mechanics. The theoretical concepts of the operation predict the greatest functional benefit in patients with marked hyperinflation, and with airflow obstruction due to loss of elastic recoil. Consistent observations in several centres, have confirmed these expectations. To achieve maximal reduction in lung volume at the least cost of functional tissue, resection is targeted to the lung zones with the most severe destruction by emphysema, leaving zones with relatively well-preserved tissue intact. Heterogeneity in emphysema distribution as assessed by visual scoring of the chest computed tomography scan according to a simple grading system has been shown to correlate with LVRS outcome variables. Therefore, evaluation of lung volume reduction surgery candidates has to include the functional and morphological characteristics of the emphysema as well as a general assessment of perioperative risk. However, the knowledge of potential predictive factors of lung volume reduction surgery outcome is so far based on retrospective analysis of highly selected patients. Therefore, many questions in respect of the selection of ideal candidates for this procedure remain unanswered at the present time.

Elasticity↗

Retinoic acid treatment abrogates elastase-induced pulmonary emphysema in rats.

Pulmonary emphysema is a common disease in which destruction of the lung's gas-exchange structures (alveoli) leads to inadequate oxygenation, disability and frequently death; lung transplantation provides its only remediation. Because treatment of normal rats with all-trans-retinoic acid increases the number of alveoli, we tested whether a similar effect would occur in rats with emphysema. Elastase was instilled into rat lungs, producing changes characteristic of human and experimental emphysema: increased lung volume reflecting a loss of lung elastic recoil, larger but fewer alveoli and diminished volume-corrected alveolar surface area due to destruction of alveolar walls. Treatment with all-trans-retinoic acid reversed these changes providing nonsurgical remediation of emphysema and suggesting the possibility of a similar effect in humans.

Animals↗

The presence of emphysema does not affect the systemic bioactivity of inhaled fluticasone in severe chronic obstructive pulmonary disease.

AIMS: To assess the systemic bioactivity of fluticasone proprionate (FP) 2000 micro g daily on sensitive adrenal and bone markers in severe chronic obstructive pulmonary disease (COPD) patients with or without significant emphysema. METHODS: Ten patients without emphysema (COPD group: age 55 years, FEV(1) 51% predicted and DL(CO) 83% predicted) and 10 patients with emphysema (COPDE group: age 59 years, FEV(1) 43% predicted and DL(CO) 49% predicted) received FP 2000 micro g daily via a spacer for 2 weeks. There was a 1-week washout period prior to FP treatment where patients were given salmeterol and oxitropium, after stopping their usual inhaled corticosteroids for the duration of the study. Measurements including overnight 10 h urinary cortisol excretion corrected for creatinine (OUCC) and serum osteocalcin concentrations were performed at baseline following washout and after 2 weeks of FP. RESULTS: Values for OUCC and serum osteocalcin concentrations pre- and post-FP were not significantly different between the COPD and COPDE groups. There was significant suppression of OUCC (nmol mmol(-1)) by FP treatment within the COPD group (P = 0.03): 7.86 vs 4.64 (95% CI on the difference 0.47, 5.98), and within the COPDE group (P = 0.006): 7.13 vs 4.27 (95% CI on the difference: 1.03, 4.69). Likewise, there was significant suppression of osteocalcin concentration (nmol l(-1)) by FP treatment within the COPD group (P = 0.04): 7.24 vs 6.34 (95% CI on the difference: 0.01, 1.78), and within the COPDE group (P = 0.03): 6.92 vs 5.72 (95% CI on the difference: 0.12, 2.29). CONCLUSIONS: Severe COPD patients who are receiving high dose FP are susceptible to the development of systemic adverse effects, irrespective of the presence of emphysema.

Administration, Inhalation↗