Tracheobronchopathia osteochondroplastica. An underrecognized entity?

Tracheobronchopathia osteochondroplastica (TPO) is an idiopathic disease of the trachea and major bronchi characterized by multiple submucosal osteocartilaginous nodules. The nodules may be either focal or diffuse, and typically spare the membranous wall of the airways. Symptoms are non-specific, and include dry cough, dyspnea, recurrent respiratory infections and occasionally hemoptysis. TPO is rarely considered as a diagnosis in part due to lack of awareness among clinicians. The diagnosis can be based on a typical bronchoscopic appearance and generally does not require biopsy of the lesions. When available, histology reveals bone formation within the submucosa with normal overlying respiratory epithelium. TPO is a benign disorder, marked by a generally favorable clinical evolution. There is currently no established treatment for the removal of airway nodules, or the prevention of further tissue growths. Interventional bronchoscopy techniques have a role in the relief of symptomatic airway obstruction, when indicated.

A case of uncomplicated pulmonary alveolar proteinosis evolving to pulmonary fibrosis.

Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterised by intra-alveolar accumulation of surfactant components and cellular debris, with minimal interstitial inflammation or fibrosis. Since surfactant accumulates abnormally, a disturbance in the normal pathway of surfactant production, metabolism, recycling or degradation has been postulated. This disease has a variable clinical course: from spontaneous resolution to respiratory failure and death due to disease progression or superimposed infections. PAP leading to pulmonary fibrosis is rarely seen, and few case reports describe this association. Here, we describe the case of a patient with a diagnosis of PAP confirmed by open lung biopsy, who developed interstitial pulmonary fibrosis years after disease onset.

Rigid bronchoscopy in the twenty-first century.

Rigid bronchoscopy, a procedure more than 100 years old, now has been performed in three centuries. The "open tube" bronchoscope provides safe access to the lower airways and has proved to be compatible with newer, more sophisticated therapies as they have been introduced into the practice of bronchology. The twenty-first century surely will provide the bronchoscopist with exciting new diagnostic and therapeutic tools for the management of lung diseases, and it is likely that the rigid bronchoscope will continue to play an important role in delivering these tools to the airways. Pulmonologists need to appreciate the use of this classic instrument and prevent rigid bronchoscopy from becoming a forgotten art.

Intraluminal brachytherapy for malignant endobronchial tumors: an update on low-dose rate versus high-dose rate radiation therapy.

Although the evolution from low-dose rate (LDR) to high-dose rate (HDR) brachytherapy for malignant endobronchial tumors was presumably based on economy, patient convenience, and radiation protection, our experience with both modalities permits assessment of the pros and cons of each technique. In November 1991, our HDR remote afterloading brachytherapy unit became operational. By that time, we had treated 110 patients (group 1) with malignant endobronchial obstruction with LDR brachytherapy. Since then, all patients have been treated with HDR brachytherapy. The outcome of our first 110 patients (group 2) treated with HDR brachytherapy is presented in this communication, using group 1 as the historic control group. In group 1, patients were treated with 1 or 2 sessions of 30-60 Gy, each calculated at a 1-cm radius. In group 2, patients received 3 or 4 weekly treatments of 7 Gy, each calculated at a 1-cm radius. The majority of patients in each group had previously received a full course of external beam irradiation, and a history of laser bronchoscopy was also similar for the 2 groups. Differences in bronchoscopic response rate (82% vs. 96%, respectively) and complications (3.6% vs. 2.7%, respectively) were statistically insignificant between the LDR group and the HDR group. We believe HDR brachytherapy is the state-of-the-art modality in intraluminal therapy for endobronchial malignancies.

Bronchoscopy training: current fellows' experiences and some concerns for the future.

OBJECTIVES: To determine current pulmonary fellows' perspectives about their bronchoscopy training. DESIGN: Survey of 59 pulmonary fellows selected by training program directors to represent their institutions. SETTING: "Hands-on" symposium at the CHEST 1998 annual meeting, Toronto, Canada. RESULTS: Fellows reported a mean (+/- SD) of 2.4+/- 0.7 years of training, estimated they had performed 77.7+/-34 bronchoscopies per year, and had generally high estimates of their bronchoscopy proficiency and training. Proficiency estimates correlated with number of procedures cited (r = 0.43, p = 0.001) or level of fellowship training (r = 0.40, p = 0.002). Proficiency ratings (r = 0.63, p = 0.0001) and procedure numbers (r = 0.45, p-0. 0004) correlated with program quality ratings. Approaches to bronchoscopy instruction varied, and most often consisted of one-to-one instruction by faculty (92.5%), lecture-based instruction (74.6%), and case discussions (72.9%). Use of bronchoscopy lectures (p = 0.008) or videos (p = 0.057) were associated with higher self-estimates of proficiency, whereas use of lectures (p = 0.002), a bronchoscopy text (p = 0.009), and one-on-one instruction (p = 0.05) were associated with more highly ranked programs. Major components of training varied among programs. Although most fellows had received instruction encompassed in basic bronchoscopy, fewer had experience with bronchoscopic intubation (71.2%), transbronchial needle aspiration (72.9%), quantitative bacterial culture (64.4%), stent placement (27.1%), laser photocoagulation (25.4%), or cryotherapy (6.8%). Components of bronchoscopy experiences correlated with fellows' estimates of bronchoscopy proficiency and program quality. CONCLUSIONS: Approaches to bronchoscopy instruction and the components of bronchoscopy experiences vary considerably among institutions and are associated with pulmonary fellows' perceptions of bronchoscopy proficiency and training program quality. Definition of an optimum bronchoscopy curriculum remains necessary.

Novel flexible bronchoscope and single-use disposable-sheath endoscope system. A preliminary technology evaluation.

OBJECTIVE: To measure image clarity, ease of use, and handling performance of a flexible fiberoptic bronchoscope (B-F 100; Vision Sciences; Natick, MA) and sterile single-use disposable-sheath endoscope system (BS-F21 EndoSheath; Vision Sciences). DESIGN: Prospective, multicenter diagnostic technology evaluation of a medical device newly approved by the US Food and Drug Administration. SETTING: Three tertiary care referral centers with busy bronchoscopy practices. PATIENTS: Twenty-four patients undergoing bronchoscopy with a flexible fiberoptic bronchoscope and disposable sheath endoscope system. METHODS: Flexible bronchoscopy was performed through a transnasal or transoral route after topical upper airway anesthesia using standard techniques. After each procedure, the bronchoscopist rated the performance of the medical device under study using a linear scale of 1 (poor) to 5 (excellent and equal to that of a conventional flexible fiberoptic bronchoscope). RESULTS: Mean performance ratings were > 4.0 (range, 4.17 to 4.63) for image clarity, illumination, lack of fogging, distal tip angulation, and ease of transnasal passage. All other ratings were > 3.0 (range, 3.54 to 3.87), the lowest of which was handling comfort. CONCLUSION: The flexible fiberoptic bronchoscope with sterile single-use disposable-sheath endoscope system has the potential to reduce scope downtime by eliminating the need for high-level disinfection between procedures. Illumination, image clarity, and ease of insertion are very good, justifying future prospective studies comparing this device to conventional flexible fiberoptic bronchoscopes.

Tracheobronchial amyloidosis. The Boston University experience from 1984 to 1999.

Tracheobronchial amyloidosis (TBA), an idiopathic disorder characterized by deposition of fibrillar proteins in the tracheobronchial tree, occurred in 10 patients referred to the Amyloid Program at Boston University over the past 15 years. Fewer than 100 cases of TBA have been described; only 1 series encompassed more than 3 patients. We analyzed our experience with biopsy-proven TBA to define better its natural history. Follow-up averaged approximately 8 years and was obtained in all cases, making this outcome reporting the largest and most complete to date. Three of these patients were prospectively studied for up to 24 months to examine the utility of bronchoscopy, computerized tomography (CT) imaging, and pulmonary function tests (PFTs) in monitoring disease progression. No patient with TBA developed signs or symptoms of systemic amyloidosis during the period reviewed. Conversely, tracheobronchial disease was not diagnosed in 685 patients with primary systemic (AL) amyloidosis during the 15-year study period at Boston University. Bronchoscopy proved most useful in establishing the diagnosis by biopsy. Narrowing of major airways limited its inspection of the tracheobronchial tree, however. In contrast, CT imaging provided quantitative assessment of airway narrowing and mural thickening--2 major consequences of amyloid infiltration. These CT features, in the presence of mural calcifications sparing the posterior tracheal membrane, have been reported in few disorders other than TBA. The ability of CT to map airway involvement and identify extraluminal manifestations of TBA made it the study of choice for establishing disease extent. Three patterns of disease were evident by CT imaging and bronchoscopic examination: proximal, mid, and distal airways involvement. Those with severe proximal disease had significantly decreased air flows, air trapping, and fixed upper airway obstruction on PFTs. Patients with distal disease had normal airflows. PFTs could not clearly distinguish proximal from severe mid airways disease. Thirty percent of patients died within 7-12 years after diagnosis, all having proximal or severe mid airways disease. Repeated rigid bronchoscopic debridement and laser treatments did not prevent progressive airways narrowing in patients dying from TBA. Most patients with mid airways involvement, and all distal airway cases, had either stagnant disease or slowly increasing amyloid deposits when followed for up to 14 years. In a small subset of patients followed prospectively, serial PFTs were most sensitive to disease progression. CT-derived measures of airway lumen diameter and wall thickness did not change significantly despite marked improvements in airflow after rigid bronchoscopy. Our experience suggests that serial PFTs and CT imaging together offer the best assessment of airway involvement and disease progression in patients with TBA. In the future, radiation therapy may provide more definitive treatment of TBA than debulking procedure have to date.

Flexible bronchoscopy.

Fiberoptic bronchoscopy is an excellent tool for the evaluation of the airways and lung parenchyma. The history of flexible bronchoscopy, anesthesia, technique, indications, contraindications, and complications in using the bronchoscopy are reviewed.

Laser bronchoscopy.

The worldwide epidemic of cancer of the lung has stimulated the development of therapies to relieve endobronchial obstruction. Table 3 lists a number of endobronchial therapies that might be used to treat malignant central airway obstruction. With over 15 years of worldwide experience, the Nd:YAG laser has proven to be the most important of these tools. Laser bronchoscopy can be performed with rigid or flexible instruments and produces a rapid recanalization of the airway with associated relief of symptoms. The treatment is repeatable and has acceptable immediate complications and infrequent delayed complications. To be effective, laser bronchoscopy can only be used for the treatment of intraluminal obstructions. Obstruction by submucosal infiltration and external compression require other endobronchial therapies. The performance of laser bronchoscopy requires an extra commitment by the bronchoscopist. A thorough understanding of airway and mediastinal anatomy is mandatory along with an appreciation of laser physics and tissue interaction. Attendance at specialized training courses may be required to satisfy local credentialing bodies. In the past 15 years, thousands of patients have benefited from the development of laser bronchoscopy techniques. No longer a therapy of last resort, laser bronchoscopy has proven to be an excellent tool to relieve the symptoms of central airway obstruction.

Endobronchial brachytherapy.

Intraluminal endobronchial brachytherapy is a technique in which an encapsulated radioactive source is placed near a tumor for localized irradiation. It is effective, with or without other treatment modalities, in palliating problems caused by endobronchial malignancies, such as dyspnea, hemoptysis, cough, atelectasis, and postobstructive pneumonia. This article describes the different techniques and dosage schemes for brachytherapy, indications and contraindications, reported rates of efficacy and complications, and limitations of the technique.

Low dose rate versus high dose rate intraluminal brachytherapy for malignant endobronchial tumors.

Although the evolution from low dose rate to high dose rate brachytherapy for malignant endobronchial malignancies was primarily based on economy, patient convenience, and radiation protection, the difference in therapeutic index, if any, between these two modalities must be kept in mind. Our experience with both methods permits assessment of the feasibility of replacing low dose rate brachytherapy with high dose rate brachytherapy. Results with our first 110 patients (group 1) treated with low dose rate brachytherapy (133 procedures) were compared with results with our initial 59 consecutive patients (group 2) treated with high dose rate brachytherapy (161 procedures). In group 1, patients were treated with one or two sessions of 30-60 Gy each calculated at a 1 cm radius. In patients in group 2, we aimed at three weekly sessions of 7 Gy each calculated at a 1 cm radius. External beam irradiation therapy had previously been given to 88% of patients in group 1 and to 85% of patients in group 2. Laser bronchoscopy was performed in 36% of patients in group 1 and in 24% of patients in group 2 before brachytherapy. Clinical or bronchoscopic improvement was noted in 72% of patients in group 1 and in 85% of patients in group 2 (p > 0.05). Complication rates were low and comparable. Survival was similar in both groups (median < 6 months). Although both low dose rate and high dose rate brachytherapy appear equally effective in palliation for malignant endobronchial obstruction, we are now practicing the latter exclusively.

Increased resistance of hygroscopic condenser humidifiers when using a closed circuit suction system.

OBJECTIVES: To examine a hygroscopic condenser after clinical use and to describe the interaction of a hygroscopic condenser and a closed circuit suction system used simultaneously. DESIGN: Prospective evaluation of hygroscopic condensers used clinically, and laboratory investigation of a hygroscopic condenser used with a closed circuit suction system. SETTING: Tertiary referral centers. MEASUREMENTS AND MAIN RESULTS: The hygroscopic condenser used during mechanical ventilation was removed and peak inflation pressure was measured by delivering a standard tidal volume and inspiratory flow across the isolated hygroscopic condenser while recording the peak inflation pressure. In the laboratory, four 10-mL aliquots of saline were instilled via closed circuit suction system into a test lung with fresh hygroscopic condensers (n = 15) inline. At baseline and after each instillation, the hygroscopic condenser was weighed and the peak inflation pressure was measured while in five condensers, peak expiratory flow rate was also measured. In these five devices, hygroscopic condenser resistance was measured with 100 L/min of constant gas flow while measuring the pressure drop across the hygroscopic condenser. In 11 hygroscopic condensers used for 27.5 +/- 11.9 hrs with no closed circuit suction system, the peak inflation pressure was 3.74 +/- 0.58 cm H2O. In the laboratory, instillation of saline via closed circuit suction system was associated with an increase in hygroscopic condenser weight. Peak inflation pressure increased in a quadratic fashion with the increase in hygroscopic condenser weight, while peak expiratory flow rate decreased in a linear fashion. After four saline instillations, hygroscopic condenser resistance increased from 5.66 +/- 0.31 to 13.9 +/- 2.42 cm H2O/L/sec. CONCLUSIONS: Clinical use of a hygroscopic condenser alone is not associated with a significant increase in peak inflation pressure. We caution the use of a hygroscopic condenser and a closed circuit suction system simultaneously, as an increase in hygroscopic condenser resistance may develop and may be poorly tolerated in patients with marginal ventilatory reserve.

Graded comprehensive cardiopulmonary exercise testing in the evaluation of dyspnea unexplained by routine evaluation.

The evaluation of dyspnea is problematic when a cause is inapparent after initial diagnostic studies. We examined the results and role of cardiopulmonary exercise testing (CPET) in 50 patients with a mean 23 months of dyspnea and normal FEV1 and FVC. The CPET studies were interpreted by a panel and a consensus reached. Subsequent tests ordered by the primary physician were reviewed, and a final diagnosis was agreed on by the panel. Seven of 50 patients had cardiac limitation, 17 of 50 had pulmonary limitation, 14 of 50 had obesity and/or deconditioning, 1 of 50 had gastroesophageal reflux, and 16 of 50 had either psychogenic dyspnea or no identifiable disease. Five patients had more than one clinical diagnosis accounting for 55 diagnoses in the 50 patients. Those with a normal CPET had a higher VO2max and O2 pulse than those with cardiac disease, deconditioning, or hyperactive airways disease (HAD) (p < 0.05). Electrocardiographic changes identified cardiac disease while studies demonstrating ventilatory limitation identified a pulmonary process. In 24, deconditioning could not be distinguished from cardiac limitation. Of these, 14 responded to exercise training and/or weight loss, whereas 3 had cardiac disease, 7 had HAD, and 4 had psychogenic dyspnea (4 had more than one clinical diagnosis). In the 13 patients with normal CPET results, one had gastroesophageal reflux, two had HAD, four had psychogenic dyspnea, and six had no identifiable disease. We conclude that a diagnosis can be made in most patients with chronic dyspnea; however, further studies including bronchoprovocation are often required. Cardiopulmonary exercise testing is useful in identifying a cardiac or pulmonary process, but it is insensitive in distinguishing cardiac disease from deconditioning.

Efficacy of short term versus long term tube thoracostomy drainage before tetracycline pleurodesis in the treatment of malignant pleural effusions.

BACKGROUND: A study was undertaken to compare the efficacy of short term tube thoracostomy drainage with standard tube thoracostomy drainage before instillation of tetracycline for sclerotherapy of malignant pleural effusions. METHODS: The study consisted of a randomised clinical trial in a sequential sample of 25 patients with malignant pleural effusions documented cytopathologically. Fifteen patients were randomly assigned to group 1 (standard protocol) and 10 to group 2 (short term protocol). Patients in group 1 had tube thoracostomy suction drainage until radiological evidence of lung re-expansion was obtained and the amount of fluid drained was < 150 ml/day, before tetracycline (1.5 g) was instilled. The chest tube was removed when the amount of fluid drained after instillation was < 150 ml/day. Patients in group 2 also had suction drainage, but the tetracycline (1.5 g) was instilled when the chest radiograph showed the lung to be re-expanded and the effusion drained, which was usually within 24 hours. The chest tube was removed the next day. RESULTS: The response to tetracycline sclerotherapy in the two groups was the same (80%) but the duration of chest tube drainage was significantly shorter for patients in group 2 (median two days) than for those in group 1 (median seven days). CONCLUSIONS: The duration of chest tube drainage before sclerotherapy for malignant pleural effusions need not be influenced by the amount of fluid drained daily but by radiographic evidence of fluid evacuation and lung re-expansion. Shorter duration of drainage will reduce the length of hospital stay without sacrificing the efficacy of pleurodesis.

Supported arm exercise vs unsupported arm exercise in the rehabilitation of patients with severe chronic airflow obstruction.

OBJECTIVE: Compare unsupported (UAEx) vs supported (SAEx) arm exercise in training of patients with severe chronic airflow obstruction (CAO). DESIGN: Randomized trial of UAEx vs SAEx training added to a 10-week outpatient program of lower extremity (LE) exercise training, respiratory muscle training, breathing retraining, psychological support, and teaching. SETTING: The Lahey Clinic Medical Center, a tertiary referral center. PATIENTS: Forty patients with CAO entered the rehabilitation program with 32 completing training and testing. INTERVENTIONS: All underwent progressive bicycle ergometer and treadmill training and respiratory muscle training using a threshold inspiratory pressure trainer. Patients were randomized to progressive SAEx training (arm cycle ergometer, n = 17) or UAEx training (raising weighted dowel, n = 18). MAIN OUTCOME MEASURES AND RESULTS: There was no significant difference in disease severity or exercise capacity between the two groups. Twelve-min walk test, bicycle ergometer power output, and respiratory muscle function improved with no significant difference in improvement between the two groups. Both groups showed similar improvements in arm ergometer testing while those trained with UAEx showed greater improvement in dowel testing (UAEx > SAEx, p = 0.002). In 17 patients VO2isotime (time at which patient performed pre-training and post-training tests) was measured during dowel testing. Only those trained with UAEx showed decreases in VO2isotime (UAEx trained, p = 0.02; SAEx, p = 0.18). VO2 during the last minute of a 2-min period of simple arm elevation was also measured in 17 patients. Only those trained with UAEx showed decreases in VO2 (UAEx, p = 0.02; SAEx, p = 0.20). CONCLUSION: We confirm that a pulmonary rehabilitation program incorporating exercise training improves LE and respiratory muscle function. Arm exercise training improved arm activity with greater increases in unsupported arm activity seen in those trained with unsupported arm training. Metabolic cost of UAEx decreased only in those trained with UAEx. As UAEx is typical of activities of daily living in patients with CAO, the changes seen with UAEx training may be of greater clinical significance. Arm training should be incorporated in exercise training and a simple program of UAEx appears the optimal format.

Laser bronchoscopy in respiratory failure from malignant airway obstruction.

OBJECTIVE: To examine the value of laser bronchoscopy in patients with inoperable carcinoma of the lung who required ventilatory assistance for acute respiratory failure. DESIGN: Retrospective review of the medical records of all patients undergoing laser bronchoscopy. SETTING: The Lahey Clinic Medical Center, a tertiary referral center. PATIENTS: The medical records of 311 patients undergoing laser bronchoscopy at the Lahey Clinic Medical Center between 1982 and 1990 were reviewed. The 17 patients who required mechanical ventilation at the time of laser surgery formed the study group. INTERVENTIONS: All patients underwent neodymium:yttrium-aluminum-garnet laser bronchoscopy through a rigid bronchoscope in the operating room with use of jet ventilation and general anesthesia throughout the procedure. Postoperative care was directed by the same physician for all patients. MEASUREMENTS AND MAIN RESULTS: The following data were extracted from medical records: age and sex of patient, cell type and location of tumor, length of time between diagnosis and laser therapy, treatment before and after laser therapy, number of days receiving mechanical ventilation before and after laser therapy, medical and operative complications, survival, and cause of death. The patients were divided into two groups on the basis of clinical improvement that permitted elective discontinuation of mechanical ventilation. Group 1 included patients who experienced clinical improvement permitting endotracheal extubation. Group 2 was composed of patients who died while still receiving mechanical ventilatory support or who were extubated before institution of comfort measures. The two groups were compared using nonparametric tests of significance, including the Mann-Whitney test. No significant differences were apparent between groups 1 and 2 regarding clinical characteristics before laser treatment. Patients in group 1 had significantly (p = .03) shorter requirements for mechanical ventilation after laser treatment than patients in group 2 (1 vs. 6 days). Similarly, patients in group 1 were more likely to receive additional treatment after laser therapy than patients in group 2 (seven of nine patients vs. two of eight patients; p = .048). Patients in group 1 had significantly (p = .0038) longer survival than patients in group 2 (98 vs. 8.5 days). A greater endobronchial component to airway obstruction appeared to exist in patients in group 1 (nine of nine patients) vs. patients in group 2 (three of eight patients; p = .009). Extrinsic compression and submucosal tumor were more commonly seen in patients in group 2. In addition, patients in group 2 appeared to have a greater number of postoperative medical complications than patients in group 1. Operative complications were minor in both groups. CONCLUSIONS: In nine of 17 patients, laser bronchoscopy appeared to improve the clinical status, permitting removal of mechanical ventilation and extubation and provided the opportunity for further treatment modalities in seven of the nine patients. Survival was improved in this subgroup, and requirement for mechanical ventilatory support was shortened. The improved outcome after laser therapy was related to the presence of obstructing endobronchial tumor as the cause of the respiratory insufficiency. Patients with inoperable carcinoma of the lung and respiratory failure should be evaluated for the presence of an endobronchial lesion that might be responsive to laser bronchoscopy.

Tracheobronchial obstruction from esophageal carcinoma: bronchoscopic treatment with neodymium: yttrium-aluminum-garnet laser.

Tracheobronchial obstruction resulting from esophageal carcinoma is uncommon. Patients with advanced esophageal carcinoma with tracheobronchial obstruction usually present with severe dyspnea or hemoptysis or both and may die of suffocation. The Lahey Clinic experience using laser bronchoscopy for the palliation of symptoms of airway obstruction in patients with esophageal carcinoma is presented. From 1982 to 1990, nine patients were treated in 13 procedures using the neodymium: yttrium-aluminum-garnet laser. Of the patients, seven had undergone previous treatment of the primary tumor. Tumors were located in the trachea in seven patients and in the main stem bronchi in three patients. Improvement of the airway caliber was achieved in all patients with relief of the dyspnea. The mean hospital stay was 2 days. One patient lived 4 years after laser treatment with no recurrence of tumor, and one patient died 1 week after treatment as a result of his poor general condition. The rest of the patients lived 3 to 41 weeks, with a median survival of 35 weeks. No complications were related to the procedures, and in particular, no tracheoesophageal fistulas developed. Our experience indicates that bronchoscopic application of this laser in conjunction with other treatment modalities can improve the quality and duration of life in selected patients with esophageal carcinoma that invades and obstructs the trachea.