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The Medical Management of Bronchiectasis
The Medical Management of Bronchiectasis FRANK M. MAcDONALD, M.D.*
BRONCHIECTASIS is a disease ,vith particularly great variation in severity. Before the antibiotic era these patients were prone to develop severe chronic infections and their sequelae, and progressive disease with a high eventual mortality was seen in a considerable number. Foul sputum, chronic toxicity, poor nutrition, amyloidosis, and other serious complications are now far less common. They are still seen in an occasional patient, especially where good medical management has not been carried out. Most reports of hospital experience previously emphasized the severe form with its attendant poor prognosis. The gloomy picture presented by these series was due to the selection of material. It is now well recognized that progressive bronchiectasis is quite unusual, and that life expectancy is not appreciably affected in most patients. Even before the antibiotic period, asymptomatic or mild forms of bronchiectasis were common in clinic and office practice. CASE I. This 57 year old clerical worker was admitted to the hospital because of an involutional depression. His only chest symptom was a chronic cough productive of about 31 ounce of mucopurulent sputum daily. He had received no treatment for his cough. He gave no history of pneumonia and had not had frequent chest colds. Physical examination revealed medium moist rales over the left lower lobe. A chest roentgenogram (Fig. 45, A) showed evidence of lower left lobe atelectasis. A bronchoscopic examination was normal, and bronchograms revealed saccular bronchiectasis involving the left lower lobe (Fig. 45, B).
The patient illustrates well the degree to which a severe anatomical change may be consistent with good health and freedom from infections. Two decades of chemotherapy have made a most marked change in the clinical management of bronchiectasis. Antibiotics have been only partly successful in controlling infections, however, and other modes of therapy are as important now as they ever have been. It is one of the purposes of this paper to emphasize that one cannot rely solely on anti-
* Assistant Professor of Medicine, University of Minnesota; Chief, Pulm(Jnary Disease Service, Veterans Administration Hospital, M inneapolis, Minnesota. 209
Frank M. M acDonald
210
A
B
Fig. 45 (Case I). A, Posteroanterior chest film, showing retrocardiac density due to left lower lobe disease with atelectasis. B, Bronchogram showing marked saccular bronchiectasis of basal segments of left lower lobe.
biotics, and that all available modes of treatment must be used for optimum results. The pathogenesis of bronchiectasis is not understood, but it is of some interest to examine briefly the major clinical features found in association with the disease. Congenital predisposition to this disease may occur in several ways. Situs inversus with dextrocardia (Kartagener's syndrome) is associated with bronchiectasis in many families. It is less well known that some individuals in such families may have bronchiectasis without exhibiting situs inversus. In other families, bronchiectasis may occur, without situs. inversus or other anomalies, in multiple siblings. It is interesting that the disease in these families is not different in natlire, distribution, or response to therapy from the common, nonfamilial type. Many instances of the familial disease have nevertheless been noted to begin with an acute pneumonia. 3 Agammaglobulinemia, congenital or acquired, is associated with a high rate of respiratory tract disease, including bronchiectasis and sinusitis. All patients with bronchiectasis should be screened for globulin deficiency. Chemical determination of serum albumin and globulin is helpful, but paper electrophoresis of serum is most accurate. The zinc turbidity test is the most commonly available screening test. All patients with bronchiectasis have a bronchoscopy as a part of their initial diagnostic study, since obstructive lesions occasionally are com-
The M edical M anagement of Bronchiectasis
211
plicated by bronchiectasis. Bronchial obstruction due to a foreign body may cause bronchiectasis in the obstructed segments. This may be most difficult to recognize clinically, since patients may not remember aspiration. The disease may present as bronchiectasis, or as an apparent intrabronchial tumor. Other obstructive lesions may cause bronchiectasis. It has been noted distal to bronchial adenoma, benign stricture, and very rarely in association with prolonged partial obstruction by a bronchial carcinoma. In general, obstruction is far more often found to produce atelectasis or obstructive pneumonitis than bronchiectatic changes. Many patients with bronchiectasis have some degree of generalized bronchitis. Although bronchitis is not frequently complicated by bronchiectasis, it may contribute to its development through increased tendency to infection, and through impaired bronchial drainage. Cigarette smoking, often heavy in these patients, very often increases the ~mount of disability and the frequency and severity of infections in established bronchiectasis. A history of pneumonia is obtained in most patients with bronchiectasis. It is, of course, a frequent complication of already established bronchial disease, but there 1s no doubt that bronchial injury in patients with certain pneumonias may produce a bronchiectatic area. It is not yet possible to state the incidence of this bronchial damage for pneumonias of all etiologies. However, it is clear that in young children, measles and pertussis are particularly apt to cause bronchial injury. Whitwel1 6 has shown that these are characterized often by lymphoid aggregates in bronchial walls and lung ("follicular bronchiectasis"), or by segmental collapse ("atelectatic bronchiectasis"). It is of particular interest that measles and pertussis are not associated with the common form of saccular bronchiectasis. Bronchiectasis has been an occasional complication during certain epidemics of respiratory disease, particularly among military recruits. Postoperative atelectasis and pneumonia may lead to bronchiectasis. This is especially likely following surgery in bronchiectatic individuals, and may be a cause of new disease appearing after resection in such patients.! THERAPY DURING EXACERBATIONS
Although some patients have no symptoms, or very little difficulty, most have recurrent bouts of infection. These range in severity from mild infections of the ectatic segments to severe pneumonias. It is very important that therapy be started as soon as possible in such infections. Treatment must include measures to promote drainage as well as specific antibiotics. Antibiotic therapy is employed in all instances, beginning as soon as
212
Frank M. M acDonald
possible. Unless bacteriologic information is already available, tetracycline 250 mg. four times daily is begun. Sputum studies, including stained smears and cultures and indicated sensitivity tests, are done immediately. The antibiotic regimen is changed if a significant organism is found which is not sensitive to tetracycline. The common pathogens are the pneumococcus, Hemophilus influenzae, and staphylococci. Anaerobic organisms and gram-negative bacilli are also found, the latter with increasing frequency. Antibiotics should be continued until maximum improvement has been obtained, usually for at least seven to ten days. Bronchial drainage must be vigorously carried out. Isuprel (0.5 per cent), and Alevaire, are given by aerosol beforehand in order to facilitate drainage by widening the airways and liquefying secretions. Tergemist may be effective over a longer period in liquefying secretions. Oral bronchodilators are added to this regimen if bronchial asthma or asthmatic bronchitis are present. Postural drainage is carried out several times daily for 20-minute periods with an attendant assisting and instructing the patient. It is essential that the doctor prescribe the exact position to be used, from his knowledge of the location of the segments to be drained. rrhe trachea should be tipped downward, 20° to 45° from the horizontal-it is of no value for a patient merely to hang his head over the side of the bed. In addition, it is necessary to keep the principal involved segments uppermost. Postural drainage may be done in several ways: 1. The knee lift of the hospital bed may be elevated, and the patient may lie with his head at the foot of the bed, his hips elevated, resting head and arms against a pillo\v. 11'or drainage of lateral portions of the chest, it may be necessary to support the legs at a higher level. This procedure is best used in the hospital, since it requires a hospital bed, but the patient readily learns the principle involved and may be prepared to use other means on returning to his home. 2. The commonly used method is with arms and head on a bedside chair, with hips and legs on the bed, the affected segments uppermost. While this position is effective, it may be difficult for some, especially the older patients. 3. Elevation of the foot of the bed (about ]2 inches) may be feasible and effective. This may be better tolerated than the above method. It does limit the angle which may be obtained, but this amount of elevation is often sufficient to obtain good bronchial drainage. 4. The knee-chest position may be used, if necessary. After the drainage period, the patient is encouraged to cough up all possible remaining secretions. It is obvious that postural drainage may not be possible for a few patients, who may be too debilitated or ill vvith other serious disease to tolerate the head-down position.
The kIedical Management of Bronchiectasis
213
MAINTENANCE THERAPY
Early and adequate treatment of acute infections has been the most important factor in the improved clinical state of most bronchiectasis patients. They must be told to report immediately to their physician when even a mild lower respiratory infection develops. However, it is very important that the patient continue on preventive medical care, with regular visits to his physician often enough to be certain of full cooperation. If chronic infection has again become established, evidenced by increased cough and sputum of purulent nature, and diminished strength and well-being, the patient should again receive an adequate course of an antibiotic, with all adjuvant measures. In this area, as in most other types of infection, prophylactic antibiotics have not proved satisfactory. On all regimens used to date, infections may develop and may be difficult to treat. Some physicians believe that an adequate prophylactic regimen may yet be developed. However, at present it is generally felt that such prophylactic use of antibiotics is not warranted. Whether the patient is symptomatic or not, he should continue to do postural drainage regularly two or more times daily. Retention even of small amounts of secretion daily may lead to obstruction and reinfection. The patient must be persuaded to stop smoking completely. This requires conviction and enthusiastic support on the physician's part; it is common to note that the physician who is convinced of the importance of this succeeds in helping most of his patients to stop, while the unenthusiastic routine recommendation of others has far less effect. CASE 11. This 32 year old carpenter had a chronic cough for many years, productive of small amounts of clear mucoid sputum in the mornings. At the age of 2, he had pneumonia. Acute bronchitis was diagnosed eight months before admission, but cleared rapidly. He had not had repeated bouts of bronchitis or other respiratory infections, and had not missed work because of illness. His weight was well maintained. Eight days before admission he noted hemoptysis of a few rol. daily. Five days before admission the hemoptysis increased to about 20 rol. daily. He had noted no previous hemoptysis. He smoked 20 cigarettf-s daily. Physical examination revealed a few medium rales over the left lower lobe. Chest roentgenograms showed some infiltration in the region of the lateral basal segment (Fig. 46). Bronchoscopy was normal except for a moderate generalized bronchitis, and bronchograms revealed cylindrical bronchiectasis of the left lower lobe basal segments and some saccular change in the lingula. (Fig. 47). Conservative therapy was elected, and the patient stopped smoking completely. He has continued asymptomatic and in good health for two years.
This case demonstrates the importance of smoking in producing an irritant bronchitis and in causing symptoms in a bronchiectatic area which has done well since the patient stopped smoking. Hemoptysis of this degree, occurring for the first time, was not thought an indication for surgical resection.
Frank M. M acDonald
214
Fig. 46 (Case 11). Posteroanterior chest film showing increased markings in the left midlung field, and increased radiability suggestive of emphysema at the left base.
A
B
Fig. 47 (Case 11). Bronchograms. A, Oblique film, demonstrating moderate cylindrical bronchiectasis of the basal segments, and more severe involvement of the lingula. B, Lateral film of same area.
The control of other airborne irritants (especially in certain occupations) is important in some instances. Similarly, if allergy is recognized, anything possible in desensitization or avoidance of allergens is indicated. SURGERY OF BRONCHIECTASIS
With proper selection of patients, surgical resection of bronchiectatic segments has proved of great value. In planning the treatment of every
The Medical M anagement of Bronchiectasis
215
bronchiectatic patient, the physician must consider whether an operation is indicated. To help him in the important decision, he must have certain basic data. Bronchoscopy and adequate bronchographic mapping are necessary. Clinical evaluation of pulmonary function (including timed vital capacity or maximum breathing capacity and observation of exer-cise tolerance), as well as detailed knowledge of present and past history, are also necessary. If dyspnea Or abnormal screening tests of ventilation are found, further physiologic investigation may be in order. I t is obvious that the patient with mild or no symptoms does not, need surgery. It is also clear that there are some patients with far too extensive involvement to permit operation. In the remaining group the indicatIons commonly employed are·: 1. Symptoms of productive cough and recurrent'infections sufficient to interfere with general health or to decrease capacity to work and carry out other normal activities, in spite of an adequate trial of medical therapy. Foul sputum, formerly a social problem, is no longer an important indication for surgery, since this is nearly always well controlled medically. 2. Progressive, severe disease, with apparent poor prognosis where good medical therapy has been ineffective. ~n these rather rare instances, it is felt that even a fairly extensive resection may be the safest course for the patient. 3. Recurrent hemorrhage. Mild hemoptysis due to granulations in the bronchi is not considered here. Serious bleeding, due to erosion of an enlarged bronchial artery, is potentially dangerous. The segmental source of the bleeding should be determined bronchoscopically. If hemorrhage recurs, resection is strongly advised. 2 4. Question of diagnosis. In certain cases, differentiation of bronchial adenoma, foreign body, or carcinoma may not be clear from clinical studies, and diagnostic thoracotomy with resection should be done. Since nearly all surgery in this disease is elective, one must attempt to weigh the severity of symptoms and the expected extent of resection against the probable loss of pulmonary function, and the risk for each patient. It is especially important to realize that most patients who have resections do not remain completely asymptomatic. Nevertheless, many patients have. been greatly improved after resection of bronchiectatic segments. CASE Ill. This 28 year old auto mechanic had had a chronic cough and occasional chest discomfort since 1941. Mild hemoptysis occurred in 1943 and 1944. After a pneumonia in 1945 bronchiectasis was diagnosed. Since then he had a cough productive of 15 to 60 ml. of purulent sputum daily, often foul, and frequently difficult to raise because of its thick viscous nature. He was followed for several years by a Veterans Administration Clinic, and antibiotics and1Postural drainage were given an adequate trial, without much lasting benefit. He smoked 5 small cigars daily, never cigarettes. His chest roentgenogram is shown in Figure
Frank M. M acDonald
216 A
B C Fig. 48 (Case Ill). A, Posteroanterior chest film showing left lower lung field infiltrate. B, Bronchogram (oblique), obtained 5 years before resection, showing saccular involvement of the left lower lobe basal segments. C, Bronchogram (lateral), shortly before resection, revealing marked saccular changes. Moderately severe disease of the lingula has increased since earlier studies.
48, A. Because of his severe persistent productive cough and evidence of extension of the disease as demonstrated on bronchograms (Fig. 48, B, C), resection was advised. Several bronchographic attempts were necessary to secure reliable information about all segments of the left lung, particular difficulty being experienced in demonstrating the superior segment initially. Resection of the lingula and basal segments, lower left lobe, was carried out uneventfully, but a postoperative atelectasis occurred 2 weeks after the surgery. This was relieved by bronchoscopy. Follow-up has revealed that he considers himself much improved since surgery, but still has a chronic productive cough. He is in good health generally, but experiences recurrent bouts of otitis media and sinusitis.
The Medical Management of Bronchiectasis
217
CONCLUSION
The prognosis of the bronchiectasis patient has improved greatly. This change has occurred even though our understanding of the nature of the disease is very limited and our treatment in the main palliative. The impressive but limited success of the antibiotics in this disease makes it of continuing importance that all physicians managing these patients emphasize bronchial drainage, control of allergens and irritants and the complete avoidance of smol{ing. Segmental resection plays a limited but important role, particularly in the more serious forms of the disease which respond poorly to medical therapy. With these measures, the health of the bronchiectatic patient may be brought close to normal. REFERENCES 1. Ginsberg, R. L., Cooley, J. C., Olsen, A. M. and Kirklin, J. W.: An Analysis of
2. 3. 4. 5. 6. 7.
Unfavorable Results in the Surgical Therapy of Bronchiectasis. J. Thoracic Surg.30:331, 1953. Jackson, B. A. and Lynn, R. B.: Massive Pulmonary Haemorrhage from Bronchiectasis. Canada J. Surge 1: 42, 1957. Kartagener, M. and Mully, K.: Familial Incidence of Bronchiectasis. Schweiz. Ztschr. Tuberk. 13: 221, 1956. McKim, A.: Bronchiectasis As Seen in an Ambulant Clinic Service. Am. Rev. Tuberc. 66: 457, 1952. Stuart-Harris, C. H., Pownall, M., Scothorne, C. M. and Franks, Z.: The Factor of Infection in Chronic Bronchitis. Quart. J. Med. 22: 121, 1953. Whitwell, F.: A Study of the Pathology and Pathogenesis of Bronchiectasis. Thorax 7: 213, 1952. Wynn-Williams, N.: Observations on the Treatment of Bronchiectasis and Its Relation to Prognosis. Tubercle 38: 133, 1957.
BRONCHIECTASIS is a disease ,vith particularly great variation in severity. Before the antibiotic era these patients were prone to develop severe chronic infections and their sequelae, and progressive disease with a high eventual mortality was seen in a considerable number. Foul sputum, chronic toxicity, poor nutrition, amyloidosis, and other serious complications are now far less common. They are still seen in an occasional patient, especially where good medical management has not been carried out. Most reports of hospital experience previously emphasized the severe form with its attendant poor prognosis. The gloomy picture presented by these series was due to the selection of material. It is now well recognized that progressive bronchiectasis is quite unusual, and that life expectancy is not appreciably affected in most patients. Even before the antibiotic period, asymptomatic or mild forms of bronchiectasis were common in clinic and office practice. CASE I. This 57 year old clerical worker was admitted to the hospital because of an involutional depression. His only chest symptom was a chronic cough productive of about 31 ounce of mucopurulent sputum daily. He had received no treatment for his cough. He gave no history of pneumonia and had not had frequent chest colds. Physical examination revealed medium moist rales over the left lower lobe. A chest roentgenogram (Fig. 45, A) showed evidence of lower left lobe atelectasis. A bronchoscopic examination was normal, and bronchograms revealed saccular bronchiectasis involving the left lower lobe (Fig. 45, B).
The patient illustrates well the degree to which a severe anatomical change may be consistent with good health and freedom from infections. Two decades of chemotherapy have made a most marked change in the clinical management of bronchiectasis. Antibiotics have been only partly successful in controlling infections, however, and other modes of therapy are as important now as they ever have been. It is one of the purposes of this paper to emphasize that one cannot rely solely on anti-
* Assistant Professor of Medicine, University of Minnesota; Chief, Pulm(Jnary Disease Service, Veterans Administration Hospital, M inneapolis, Minnesota. 209
Frank M. M acDonald
210
A
B
Fig. 45 (Case I). A, Posteroanterior chest film, showing retrocardiac density due to left lower lobe disease with atelectasis. B, Bronchogram showing marked saccular bronchiectasis of basal segments of left lower lobe.
biotics, and that all available modes of treatment must be used for optimum results. The pathogenesis of bronchiectasis is not understood, but it is of some interest to examine briefly the major clinical features found in association with the disease. Congenital predisposition to this disease may occur in several ways. Situs inversus with dextrocardia (Kartagener's syndrome) is associated with bronchiectasis in many families. It is less well known that some individuals in such families may have bronchiectasis without exhibiting situs inversus. In other families, bronchiectasis may occur, without situs. inversus or other anomalies, in multiple siblings. It is interesting that the disease in these families is not different in natlire, distribution, or response to therapy from the common, nonfamilial type. Many instances of the familial disease have nevertheless been noted to begin with an acute pneumonia. 3 Agammaglobulinemia, congenital or acquired, is associated with a high rate of respiratory tract disease, including bronchiectasis and sinusitis. All patients with bronchiectasis should be screened for globulin deficiency. Chemical determination of serum albumin and globulin is helpful, but paper electrophoresis of serum is most accurate. The zinc turbidity test is the most commonly available screening test. All patients with bronchiectasis have a bronchoscopy as a part of their initial diagnostic study, since obstructive lesions occasionally are com-
The M edical M anagement of Bronchiectasis
211
plicated by bronchiectasis. Bronchial obstruction due to a foreign body may cause bronchiectasis in the obstructed segments. This may be most difficult to recognize clinically, since patients may not remember aspiration. The disease may present as bronchiectasis, or as an apparent intrabronchial tumor. Other obstructive lesions may cause bronchiectasis. It has been noted distal to bronchial adenoma, benign stricture, and very rarely in association with prolonged partial obstruction by a bronchial carcinoma. In general, obstruction is far more often found to produce atelectasis or obstructive pneumonitis than bronchiectatic changes. Many patients with bronchiectasis have some degree of generalized bronchitis. Although bronchitis is not frequently complicated by bronchiectasis, it may contribute to its development through increased tendency to infection, and through impaired bronchial drainage. Cigarette smoking, often heavy in these patients, very often increases the ~mount of disability and the frequency and severity of infections in established bronchiectasis. A history of pneumonia is obtained in most patients with bronchiectasis. It is, of course, a frequent complication of already established bronchial disease, but there 1s no doubt that bronchial injury in patients with certain pneumonias may produce a bronchiectatic area. It is not yet possible to state the incidence of this bronchial damage for pneumonias of all etiologies. However, it is clear that in young children, measles and pertussis are particularly apt to cause bronchial injury. Whitwel1 6 has shown that these are characterized often by lymphoid aggregates in bronchial walls and lung ("follicular bronchiectasis"), or by segmental collapse ("atelectatic bronchiectasis"). It is of particular interest that measles and pertussis are not associated with the common form of saccular bronchiectasis. Bronchiectasis has been an occasional complication during certain epidemics of respiratory disease, particularly among military recruits. Postoperative atelectasis and pneumonia may lead to bronchiectasis. This is especially likely following surgery in bronchiectatic individuals, and may be a cause of new disease appearing after resection in such patients.! THERAPY DURING EXACERBATIONS
Although some patients have no symptoms, or very little difficulty, most have recurrent bouts of infection. These range in severity from mild infections of the ectatic segments to severe pneumonias. It is very important that therapy be started as soon as possible in such infections. Treatment must include measures to promote drainage as well as specific antibiotics. Antibiotic therapy is employed in all instances, beginning as soon as
212
Frank M. M acDonald
possible. Unless bacteriologic information is already available, tetracycline 250 mg. four times daily is begun. Sputum studies, including stained smears and cultures and indicated sensitivity tests, are done immediately. The antibiotic regimen is changed if a significant organism is found which is not sensitive to tetracycline. The common pathogens are the pneumococcus, Hemophilus influenzae, and staphylococci. Anaerobic organisms and gram-negative bacilli are also found, the latter with increasing frequency. Antibiotics should be continued until maximum improvement has been obtained, usually for at least seven to ten days. Bronchial drainage must be vigorously carried out. Isuprel (0.5 per cent), and Alevaire, are given by aerosol beforehand in order to facilitate drainage by widening the airways and liquefying secretions. Tergemist may be effective over a longer period in liquefying secretions. Oral bronchodilators are added to this regimen if bronchial asthma or asthmatic bronchitis are present. Postural drainage is carried out several times daily for 20-minute periods with an attendant assisting and instructing the patient. It is essential that the doctor prescribe the exact position to be used, from his knowledge of the location of the segments to be drained. rrhe trachea should be tipped downward, 20° to 45° from the horizontal-it is of no value for a patient merely to hang his head over the side of the bed. In addition, it is necessary to keep the principal involved segments uppermost. Postural drainage may be done in several ways: 1. The knee lift of the hospital bed may be elevated, and the patient may lie with his head at the foot of the bed, his hips elevated, resting head and arms against a pillo\v. 11'or drainage of lateral portions of the chest, it may be necessary to support the legs at a higher level. This procedure is best used in the hospital, since it requires a hospital bed, but the patient readily learns the principle involved and may be prepared to use other means on returning to his home. 2. The commonly used method is with arms and head on a bedside chair, with hips and legs on the bed, the affected segments uppermost. While this position is effective, it may be difficult for some, especially the older patients. 3. Elevation of the foot of the bed (about ]2 inches) may be feasible and effective. This may be better tolerated than the above method. It does limit the angle which may be obtained, but this amount of elevation is often sufficient to obtain good bronchial drainage. 4. The knee-chest position may be used, if necessary. After the drainage period, the patient is encouraged to cough up all possible remaining secretions. It is obvious that postural drainage may not be possible for a few patients, who may be too debilitated or ill vvith other serious disease to tolerate the head-down position.
The kIedical Management of Bronchiectasis
213
MAINTENANCE THERAPY
Early and adequate treatment of acute infections has been the most important factor in the improved clinical state of most bronchiectasis patients. They must be told to report immediately to their physician when even a mild lower respiratory infection develops. However, it is very important that the patient continue on preventive medical care, with regular visits to his physician often enough to be certain of full cooperation. If chronic infection has again become established, evidenced by increased cough and sputum of purulent nature, and diminished strength and well-being, the patient should again receive an adequate course of an antibiotic, with all adjuvant measures. In this area, as in most other types of infection, prophylactic antibiotics have not proved satisfactory. On all regimens used to date, infections may develop and may be difficult to treat. Some physicians believe that an adequate prophylactic regimen may yet be developed. However, at present it is generally felt that such prophylactic use of antibiotics is not warranted. Whether the patient is symptomatic or not, he should continue to do postural drainage regularly two or more times daily. Retention even of small amounts of secretion daily may lead to obstruction and reinfection. The patient must be persuaded to stop smoking completely. This requires conviction and enthusiastic support on the physician's part; it is common to note that the physician who is convinced of the importance of this succeeds in helping most of his patients to stop, while the unenthusiastic routine recommendation of others has far less effect. CASE 11. This 32 year old carpenter had a chronic cough for many years, productive of small amounts of clear mucoid sputum in the mornings. At the age of 2, he had pneumonia. Acute bronchitis was diagnosed eight months before admission, but cleared rapidly. He had not had repeated bouts of bronchitis or other respiratory infections, and had not missed work because of illness. His weight was well maintained. Eight days before admission he noted hemoptysis of a few rol. daily. Five days before admission the hemoptysis increased to about 20 rol. daily. He had noted no previous hemoptysis. He smoked 20 cigarettf-s daily. Physical examination revealed a few medium rales over the left lower lobe. Chest roentgenograms showed some infiltration in the region of the lateral basal segment (Fig. 46). Bronchoscopy was normal except for a moderate generalized bronchitis, and bronchograms revealed cylindrical bronchiectasis of the left lower lobe basal segments and some saccular change in the lingula. (Fig. 47). Conservative therapy was elected, and the patient stopped smoking completely. He has continued asymptomatic and in good health for two years.
This case demonstrates the importance of smoking in producing an irritant bronchitis and in causing symptoms in a bronchiectatic area which has done well since the patient stopped smoking. Hemoptysis of this degree, occurring for the first time, was not thought an indication for surgical resection.
Frank M. M acDonald
214
Fig. 46 (Case 11). Posteroanterior chest film showing increased markings in the left midlung field, and increased radiability suggestive of emphysema at the left base.
A
B
Fig. 47 (Case 11). Bronchograms. A, Oblique film, demonstrating moderate cylindrical bronchiectasis of the basal segments, and more severe involvement of the lingula. B, Lateral film of same area.
The control of other airborne irritants (especially in certain occupations) is important in some instances. Similarly, if allergy is recognized, anything possible in desensitization or avoidance of allergens is indicated. SURGERY OF BRONCHIECTASIS
With proper selection of patients, surgical resection of bronchiectatic segments has proved of great value. In planning the treatment of every
The Medical M anagement of Bronchiectasis
215
bronchiectatic patient, the physician must consider whether an operation is indicated. To help him in the important decision, he must have certain basic data. Bronchoscopy and adequate bronchographic mapping are necessary. Clinical evaluation of pulmonary function (including timed vital capacity or maximum breathing capacity and observation of exer-cise tolerance), as well as detailed knowledge of present and past history, are also necessary. If dyspnea Or abnormal screening tests of ventilation are found, further physiologic investigation may be in order. I t is obvious that the patient with mild or no symptoms does not, need surgery. It is also clear that there are some patients with far too extensive involvement to permit operation. In the remaining group the indicatIons commonly employed are·: 1. Symptoms of productive cough and recurrent'infections sufficient to interfere with general health or to decrease capacity to work and carry out other normal activities, in spite of an adequate trial of medical therapy. Foul sputum, formerly a social problem, is no longer an important indication for surgery, since this is nearly always well controlled medically. 2. Progressive, severe disease, with apparent poor prognosis where good medical therapy has been ineffective. ~n these rather rare instances, it is felt that even a fairly extensive resection may be the safest course for the patient. 3. Recurrent hemorrhage. Mild hemoptysis due to granulations in the bronchi is not considered here. Serious bleeding, due to erosion of an enlarged bronchial artery, is potentially dangerous. The segmental source of the bleeding should be determined bronchoscopically. If hemorrhage recurs, resection is strongly advised. 2 4. Question of diagnosis. In certain cases, differentiation of bronchial adenoma, foreign body, or carcinoma may not be clear from clinical studies, and diagnostic thoracotomy with resection should be done. Since nearly all surgery in this disease is elective, one must attempt to weigh the severity of symptoms and the expected extent of resection against the probable loss of pulmonary function, and the risk for each patient. It is especially important to realize that most patients who have resections do not remain completely asymptomatic. Nevertheless, many patients have. been greatly improved after resection of bronchiectatic segments. CASE Ill. This 28 year old auto mechanic had had a chronic cough and occasional chest discomfort since 1941. Mild hemoptysis occurred in 1943 and 1944. After a pneumonia in 1945 bronchiectasis was diagnosed. Since then he had a cough productive of 15 to 60 ml. of purulent sputum daily, often foul, and frequently difficult to raise because of its thick viscous nature. He was followed for several years by a Veterans Administration Clinic, and antibiotics and1Postural drainage were given an adequate trial, without much lasting benefit. He smoked 5 small cigars daily, never cigarettes. His chest roentgenogram is shown in Figure
Frank M. M acDonald
216 A
B C Fig. 48 (Case Ill). A, Posteroanterior chest film showing left lower lung field infiltrate. B, Bronchogram (oblique), obtained 5 years before resection, showing saccular involvement of the left lower lobe basal segments. C, Bronchogram (lateral), shortly before resection, revealing marked saccular changes. Moderately severe disease of the lingula has increased since earlier studies.
48, A. Because of his severe persistent productive cough and evidence of extension of the disease as demonstrated on bronchograms (Fig. 48, B, C), resection was advised. Several bronchographic attempts were necessary to secure reliable information about all segments of the left lung, particular difficulty being experienced in demonstrating the superior segment initially. Resection of the lingula and basal segments, lower left lobe, was carried out uneventfully, but a postoperative atelectasis occurred 2 weeks after the surgery. This was relieved by bronchoscopy. Follow-up has revealed that he considers himself much improved since surgery, but still has a chronic productive cough. He is in good health generally, but experiences recurrent bouts of otitis media and sinusitis.
The Medical Management of Bronchiectasis
217
CONCLUSION
The prognosis of the bronchiectasis patient has improved greatly. This change has occurred even though our understanding of the nature of the disease is very limited and our treatment in the main palliative. The impressive but limited success of the antibiotics in this disease makes it of continuing importance that all physicians managing these patients emphasize bronchial drainage, control of allergens and irritants and the complete avoidance of smol{ing. Segmental resection plays a limited but important role, particularly in the more serious forms of the disease which respond poorly to medical therapy. With these measures, the health of the bronchiectatic patient may be brought close to normal. REFERENCES 1. Ginsberg, R. L., Cooley, J. C., Olsen, A. M. and Kirklin, J. W.: An Analysis of
2. 3. 4. 5. 6. 7.
Unfavorable Results in the Surgical Therapy of Bronchiectasis. J. Thoracic Surg.30:331, 1953. Jackson, B. A. and Lynn, R. B.: Massive Pulmonary Haemorrhage from Bronchiectasis. Canada J. Surge 1: 42, 1957. Kartagener, M. and Mully, K.: Familial Incidence of Bronchiectasis. Schweiz. Ztschr. Tuberk. 13: 221, 1956. McKim, A.: Bronchiectasis As Seen in an Ambulant Clinic Service. Am. Rev. Tuberc. 66: 457, 1952. Stuart-Harris, C. H., Pownall, M., Scothorne, C. M. and Franks, Z.: The Factor of Infection in Chronic Bronchitis. Quart. J. Med. 22: 121, 1953. Whitwell, F.: A Study of the Pathology and Pathogenesis of Bronchiectasis. Thorax 7: 213, 1952. Wynn-Williams, N.: Observations on the Treatment of Bronchiectasis and Its Relation to Prognosis. Tubercle 38: 133, 1957.