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.

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.

Intraluminal low-dose rate brachytherapy for malignant endobronchial obstruction.

From October 1985 through October 1989, 87 patients underwent 105 intraluminal brachytherapy treatments for endobronchial or endotracheal malignant tumors. Low-dose rate iridium-192 seeds were used. Of the 60 patients treated for primary lung carcinoma, 52 patients (87%) had previously received full-course external beam radiotherapy to the tumor sites. Ten patients were treated for symptomatic metastatic disease, and one patient had extension of tumor into the trachea from carcinoma of the cervical esophagus. Clinical or bronchoscopic improvement was noted in 42 patients (59%). No significant difference in the response rate was observed between various types of tumor. Patients who were treated with a radiation dose larger than 2500 cGy at a 2 cm radius had a significantly greater response rate (77%) than patients treated with a dose less than 2500 cGy (38%) (p = 0.001). A trend toward better results was apparent in patients who had undergone Nd:YAG laser bronchoscopy in the 2 weeks before brachytherapy. The treatments were well tolerated, and the incidence of serious complications was low and acceptable.

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.

Teaching models for Nd:YAG laser bronchoscopy.

A number of groups have demonstrated the efficacy of neodymium:yttrium-aluminum-garnet (Nd:YAG) laser bronchoscopy for treatment of patients with selected tracheobronchial disorders. This therapy involves a new technology, the laser, and new adaptations of conventional rigid and flexible bronchoscopy that are unfamiliar to many bronchoscopists. We describe two teaching models for laser bronchoscopy that have been employed successfully in our laser courses. Using these models, bronchoscopists can become familiar with laser bronchoscopic equipment and appreciate the interaction of laser and soft tissue in a controlled setting in the laboratory before applying this therapy to patients.

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.

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.

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.

Metastatic coccidioidal endophthalmitis.

A severe granulomatous iridocyclitis developed in association with a cavitary pulmonary lesion in a 29-year-old man. The initial diagnosis and treatment was for pulmonary tuberculosis with tuberculous uveitis. Although the pulmonary lesion improved with antituberculous therapy, the condition of the eye deteriorated. An anterior-chamber tap was positive for Coccidioides immitis, and the patient was treated with intravenous and two intracameral injections of amphotericin B. The eye was ultimately enucleated three weeks after the initial intracameral injection, and yet was culture-positive for the organism. Histopathologic examination disclosed diffuse involvement of the anterior segment, with multiple spherules present within the iris and limbus.

Changing epidemiology of lung cancer. Increasing incidence in women.

Recent worldwide reports show a large increase in the incidence of lung cancer in both men and women. To detail changes in the epidemiology of lung cancer relating to the incidence in men and women, we reviewed the patterns of diagnosis of 1145 patients with lung cancer seen at the Lahey Clinic between 1956 and 1972, during which time the proportion of all men and women seen was unchanged. The total number of women with lung cancer increased greatly and has almost doubled during this period. Lung cancer in women is now increasing at a faster rate than in men so that the male to female incidence has decreased from 6.8/1 (1957 to 1960) to 2.4/1 (1969 to 1972). We reviewed in detail the case histories and pathology of 231 women with lung cancer. No significant change was evident in cell type distribution during the study years. The most frequently seen tumors in women were adenocarcinoma (31 per cent), undifferentiated large cell cancer (22 per cent), epidermoid carcinoma (16 per cent), and undifferentiated small cell carcinoma (12 per cent). Among those women with known smoking histories, the group most responsible for the recent increase in women with lung cancer was comprised of smoking women in whom Kreyberg group 1 (smoking-related) tumors developed.

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.

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.

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.

Safety precautions for bronchoscopic Nd-YAG laser surgery.

The application of the neodymium-yttrium aluminum garnet (Nd-YAG) laser in bronchoscopy originated in Europe in 1981 and is now widespread in the United States. Transmissible through flexible fiberoptic quartz fibers and an efficient coagulator of tissue, the Nd-YAG laser deeply penetrates tissue not readily predictable by the endoscopist. Dangers associated with the Nd-YAG laser include complications secondary to inadvertent exposure to normal tissues or structures, tracheobronchial accidents (perforation, hemorrhage, fire), and complications related to anesthetic technique (respiratory depression). Hypoxemia related to persistent hemorrhage, accumulation of secretions or debris or both, or anesthesia-induced respiratory depression is the common denominator of most intraoperative and postoperative complications. Careful selection of patients, intraoperative monitoring of ventilation and blood gases, and techniques of rigid bronchoscopy are stressed to avoid complications.

A new rigid bronchoscope for laser fiber application.

A new fiberoptic rigid bronchoscope system, designed especially for laser fiber delivery, provides the endoscopist with an excellent conduit for application of the flexible fiberoptic bronchoscope, while still allowing maintenance of adequate ventilation. This system is designed for treatment of benign and malignant obstructing tumors in the trachea and mainstem bronchi. The bronchoscope will be available in sizes ranging from 6 mm to 10 mm in diameter.

Endoscopic treatment of tracheobronchial malignancy. Experience with Nd-YAG and CO2 lasers in 506 operations.

Laser technology and the endoscope have been combined for the palliation of obstructive tracheobronchial malignant lesions. The neodymium-yttrium-aluminum-garnet (Nd-YAG) laser was used to treat 249 patients (447 operations), and the CO2 laser was used on 34 patients (59 operations). Hemorrhage, the major complication in both groups of patients, was more easily controlled with the Nd-YAG laser. One patient in the CO2 laser group died, and one patient in the group being treated with Nd-YAG laser bronchoscopy died. The Nd-YAG laser can be applied more efficiently through a fiber system, with better optic control and secure hemostasis. The commonest indications for treatment were dyspnea, obstructive pneumonia, and hemoptysis. Extrinsic compression was the most frequent reason for failure. The Nd-YAG laser, most often applied through open rigid bronchoscopes under general anesthesia, has become our treatment of choice for the palliation of tracheobronchial malignancy.

Universal ventilation laser tracheoscope.

A universal ventilation laser fiberoptic tracheoscope has been developed for use in endoscopic treatment of patients with obstructing benign and malignant lesions of the subglottis-cervicotrachea and laryngectomy tracheal stoma. This instrument features a ventilation connector for either side port or Venturi jet ventilation and a proximal connector with a sheath for delivery of a 0 degrees telescope, laser fiber, and suction catheter. Difficulties encountered with exposure and ventilation during laser treatment of the subglottis-cervicotrachea and laryngectomy stoma are overcome with this new instrument.

Endoscopic laser therapy for obstructing tracheobronchial lesions.

The Lahey Clinic experience using laser bronchoscopy for relief of obstructive tracheobronchial lesions during a 7-year period from 1982 to 1989 involves 269 patients treated with 400 procedures. The carbon dioxide (CO2) laser was used for tracheal stenosis and granulation tissue. The neodymium:yttrium-aluminum-garnet (Nd:YAG) laser was used for all obstructing endobronchial neoplasms. Indications for therapy included severe dyspnea, hemoptysis, and postobstructive pneumonitis. All patients had relatively central lesions. A rigid bronchoscope was used to treat 88% of patients, and 12% of patients were treated with a flexible bronchoscope. One death occurred during the intraoperative period. Eleven deaths occurred within 1 week of therapy and were related to the presence of extensive malignant lesions or to coronary artery disease. Our experience indicates that bronchoscopic application of the CO2 or Nd:YAG laser affords effective palliation for patients with obstructive tracheobronchial lesions. The Nd:YAG laser is recommended for patients with bulky vascular endobronchial neoplasms, and the CO2 laser is best reserved for patients with benign tracheal stenosis and granulation tissue.