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Bronchiectasis
BRONCHIECTASIS MARTIN J.
SOKOLOFF,
M.D., F.A.C.P.
(I<
BRONCHIECTASIS is a disease characterized by irreversible pathological changes in the bronchial tree and its adjacent parenchyma. Modern diagnostic procedures reveal that it is much more common than was formerly believed; among chronic pulmonary diseases it is probably exceeded in frequency only by pulmonary tuberculosis. As is true of many other chronic conditions, it may exert a profound psychological effect in addition to the physical impairment it produces. Advances in medical science, particularly in the fields of radiology, bronchoscopy and respiratory physiology, have greatly increased our knowledge of bronchiectasis. As a result, we now have a much clearer understancling of the factors responsible for the development of the disease and are better able to recognize its presence in the early stages and to evaluate the degree of resulting respiratory impairment by means of pulmonary function tests. Similarly, antibiotic therapy and improved technics in thoracic surgery have dramatically changed the methods of managing bronchiectasis. Until recently, no specific therapeutic measures were available. Heretofore treatment afforded only symptomatic relief and did nothing to impede the development of new lesions. The introduction of newer methods of treatment not only provides a very definite plan of attack but has made it possible to dispel, to a great degree, the extreme pessimism which formerly prevailed among both physicians and patients concerning the prognosis in bronchiectasis. By reason of these advances, hope of a complete and lasting cure may be offered even to patients with advanced disease, who, only a few years ago, would probably have been doomed to a life of chronic invalidism. ETIOLOGY
Bronchiectasis may be congenital or acquired. Although there is considerable controversy concerning the congenital origin of bronchiectasis, it is generally believed that such a form does occur. The occasional case found in young infants and the larger number found in older children suggests the possibility of some congenital defect as a cause of the bronchial dilatations. Cystic bronchiectasis or cystic disease of the lung is consillered in this category. Acquired bronchiectasis is much more COIlllI10n and is the for111 which produces the classical clinical picture of the disease. It may be secondo Assistant Professor of Medicine, Jefferson Medical College; Chief, Medical Section, Barton Memorial Division, Jefferson Medical College Hospital; Chief, Division of Tuberculosis, Department of Public Health, Philadelphia.
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ary to a wide variety of conditions. Bronchopneumonia is generally recognized as the most common precursor of bronchiectasis. In contrast, it is generally believed that pneumococcic lobar pneumonia is rarely associ8rted with clinical bronchiectasis and plays a negligible part in its production. Measles and pertussis frequently antedate bronchiec.. tasis. In these conditions it is probably the accompanying bronchial and pulmonary infections which are responsible for the subsequent bronchial ectasia. Primary atypical pneumonia may, according ,to Blades and Dugan,! cause temporary dilatations of the bronchi, a condition known as pseudobronchiectasis. These dilatations persist for several. months but with their spontaneous disappearance the normal. contour' of the bronchial wall is restored. There is, however, no conclusive evidence that virus pneumonia can .produce true anatomical bronchiectasis. Chronic bronchitis, asthma and influenza are considered by some to be important antecedents of bronchiectasis. Lung abscess, bronchial and mediastinal neoplasm, foreign bodies, granulations and cicatrices of the bronchial mucosa such as occur in tuberculous bronchitis, enlarged mediastinal lymph glands, empyema and aortic aneurysm may produce changes in either the lumen of the bronchi or the contour of the bronchial wall with subsequent ectasia. Sinusitis is regarded by many as an important cause of bronchiectasis, principally because of the frequency with which these conditions are found in the same patient. In a series of bronchiectatic patients studied by Farrell,2 86 per cent were found to have sinus infection; similarly high incidence rat~s have been reported from other sources. During recent years, however, the role of sinusitis in the production of bronchiectasis has been questioned by many observers who maintain that the frequent association of these conditions is the result of widespread respiratory tract infection and that their coexistence proves no causal relationship. The various conditions which precede bronchiectasis favor its development by causing changes in the bacterial flora and in the structure of the bronchopulmonary tree. Usually three factors combine to produce the lesions which constitute bronchiectatic disease. They are (1) partial occlusion, which interferes with drainage and causes retention of infected material in the bronchi distal to the point of occlusion; (2) infection, which weakens the bronchial wall; (3) a mechanical force which produces the dilatations-this force may be intrinsic, such as a cough, which widens the previously weakened bronchus, or extrinsic, such as. traction exerted externally on the bronchial wall by puImorrary fibrosis, particularly as it occurs in tuberculosis. Tannenberg and Pinner3 have shown that infection is an absolute..prereqQ)site for the production of bronchiectasis in experimental anittlals, and It is generally agreed that this applies also to the disease as it occurs in man. The destructive processes in the bronchi and surrounding tis-
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sues which impair the integrity of the bronchial wall permitting it to dilate are due to this infection. Bacteriological studies of secretions obtained from bronchiectatic lesions reveal the presence of a variety of organisms. These may be either aerobic or anaerobic, and, according to Boyd,4 may vary from time to time in the same patient as a result of secondary infection. The organisms most commonly associated with bronchiectasis are Streptococcus hemolyticusand nonhemolyticus, Streptococcus viridans, Staphylococcus albus and aureus, Micrococcus catarrhalis, pneumococcus, Hemophilus influenzae and Micrococcus tetragenus and flavus. Occasionally fusiform bacilli, Friedlander bacilli, spirochetes and monilias are found. PATHOLOGY While bronchiectasis is primarily a disease of the bronchial tree, the peribronchial tissues and adjacent alveoli are also involved. The pathological features of bronchiectasis are dependent upon the antecedent condition, the type of organism present and the extent of involvement. Chronic inflammatory changes in the bronchial mucosa and submucosa, with extension of the process into the adjacent parenchyma, characterize the early stage of the disease. A serous exudate, which later becomes purulent, separates the epithelium from its basement membrane. As the process extends there is desquamation of the epithelium and destruction of the muscular and elastic elements and occasionally the cartilage of the bronchial wall. This necrotic inflammation which extends into the' surrounding pulmonary tissue is responsible for the characteristic bronchial dilatations. Reparative changes follow the stage of necrosis and dilatation. These are characterized by replacement of the mucous membrane by granulation tissue and replacement of the muscle and elastica of the bronchial wall by fibrous tissue. Regeneration of the bronchial epithelium frequently occurs, the ciliated cells being replaced mainly by nonciliated columnar and cuboidal cells. The parenchyma reveals evidence of fibrous change and usually varying degrees of emphysema. The disease is classified as cylindrical or saccular, depending on the shape assumed by the bronchial dilatations. Combinations of these forms are frequent. Cystic bronchiectasis, in which dilatatioQ.s are thinwalled, air-containing sacs of varying size, is thought to be of congenital origin. This form usually does not produce the same clinical picture as that seen in acquired bronchiectasis. Bronchiectasis may be unilateral or bilateral and it may involve por.tions of one or more lobes. When the lesions are bilateral one lung is usually nlore involved than the other. It occurs most frequently in the lower lobes, the left lower being affected more often tha.n the right. Involvement of the lingula of the left upper lobe is a frequent
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accompaniment' of disease in the lower lobe of the same side, as shown by Churchill and Belsey.5 In their series of 55 lobectomies for disease in the left lower lobe, the lingula was found involved in 44 (80 per cent). The preponderance of left lung involvement is believed by Duken and von den Steiner6 to be due to the fact that the right bronchus drains more easily because it is a continuation of the trachea, because of the narrower lumen of the left bronchus and also because the left bronchus is constricted at the point where it is crossed by the pulmonary artery. ' Recent studies indicate that bronchiectasis is not a progressive dis.. e~se and that once established it does not extend to other areas. The development of additional lesions is believed to be the result of subsequent infection which produces changes in previously uninvolved portions of the lung. This infection may be a pneumonitis, occurring in the affected lobe and terminating in a suppurative lesion in the sur.. rounding healthy parenchyma or in extension of the preexisting parenchymal lesion. It may also occur as a bronchopneumonia involving a previously unaffected lobe, thereby creating a new area of bronchiectasis. These superimposed infections are responsible, in part, for the acute exacerbations so characteristic of the clinical phase of bronchiectasis. INCIDENCE The nature of bronchiectasis is such that its true incidence is difficult to deter~ mine. Because of the lack of characteristic symptoms in many patients and the the fact that the clinical picture is dominated by symptoms of the primary condition in others, the existence of the process may escape detection. During recent years there has been a definite increase in the number of patients in whom this diagnosis has been established. This rising incidence, however, is probably apparent rather than real, being the result of the widespread use of specific diagnostic procedures, particularly bronchoscopy and bronchography. Statistics relating to bronchiectasis are meager and therefore not altogether reliable as an indication of the prevalence of the disease. Depending upon the source from which they emanate they show considerable variation. Thus, Lisa and Rosenblatt, 7 in a review of the records of 92,235 patients discharged from the New York City Hospital during the ten year period 1932-1941, found that only seventy patients (0.08 per cent) had a primary diagnosis of bronchiectasis. In contrast, these observers call attention to the fact that 70 new patients with bronchiectasis were admitted to the Out-Patient Department of the same institution in the two year period 1940-1941. There can be no doubt that many cases of bronchiectasis in the general population remain undiagnosed because so many of the patients do not present typical symptoms. It seems reasonable to assume. that the apparent wide variation in incidence is a reflection of the intensity with which the search for the disease was conducted by various groups of physicians. The disease is found with equal frequency in both sexes. Its occurrence, however, varies considerably in different age groups. It is generally agreed that in the majority of cases bronchiectasis develops in childhood or early adult life. This is in accord with the observations of Perry and King,S who found that in a series of 400 patients, 42 per cent were under 10 years of age, 27 per cent were between the ages of 11 and 20, 24 per cent were between 21 and 40 and only 7 per cent
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were over 40 years of age. It is also generally recognized that bronchiectasis is not a frequent finding in older age groups. Extensive or untreated bronchiectasis in older individuals is a serious disorder, often accompanied by severe cardiac and metabolic disturbances. In addition, the acute respiratory tract infections· wl1ich are so common in this condition tend to be of more serious consequence witli advancing years. Because of these complications the life expectancy in this group of patients is sharply reduced.
CLINICAL ASPECTS
The symptoms of bronchiectasis depend upon the severity of the existin~ infection, the ~ype of predominating organisms and, to a lesser extent, the, amount of bronchopulmonary involvement. If the infection is! mild the clinical picture is not characteristic. The patient may complain only of recurrent attacks of bronchitis, especially during the winter months. These differ from ordinary attacks of acute ., bronchitis in that they are longer in duration and usually do not respond readily to treatment. The sputum during these periods. may be streaked with blood and occasionally a dram or two of unmixed, bright-red blood may be expectorated. Other patients complain only of morning cough and expectoration. This bout of coughing occurs upon first arising, and after expectoration of a small amount of sputum symptoms may be absent until the next morning, when the procedure is repeated. These individuals nearly always attribute their symptoms to sinusitis or smoking. In those with extreme degrees of infection or extensive lesions the clinical picture may vary considerably. Cough is the most constant symptom, being present to some extent in almost every instance. It is paroxysmal, usually worse in the morning upon arising and nearly always productive. These paroxysms may be severe enough to cause vomiting or attacks of faintness or dizziness. In the intervening periods some patients may be entirely free from cough, while others have an irritation of the throat or a wheeze in the chest which induces attacks of cou'ghing. The coughing attacks are associated with the production of variable amounts of purulent sputum. Whereas the average patient expectorates about 1 or 2 ounces daily, those with severe infection may raise as much as SO ounces of sputum in twenty-four hours. If small quantities of sputum are expectorated the material has no distinguishing characteristics. Occasionally, but not always, large amounts of the sputum will separate into three layers if allowed to stand for several hours, a lower layer, grayish in color and opaque, a turbid middle layer and a frothy brownish material on top. The expectorated material may be fetid if anerobic organisms are present. Hemoptysis is an important symptom in bronchiectasis. The bleeding may vary from minute traces of blood, barely visible in the sputum, to frank hemorrhage of a pint or more. These more copious hem-
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orrhages occur in episodes during which the bleeding is repeated at intervals over periods lasting from several days to a week or' more. Occasionally the hemorrhage may be fatal. It is estimated that hemoptysis occurs in about 50 per cent of bronchiectatic patients. The importance of this symptom is emphasized by Clerf,9 who states that it is probably more often observed in bronchiectasis than in any other condition, including tuberculosis. Dry, hemorrhagic bronch,iectasis is the term applied to a form of the disease in which the predominant and sometimes the only symptom is hemoptysis. Its course is characterized by pulmonary hemorrhages repeated at varying intervals and often massive in extent. The paroxysmal cough and abundant sputum generally associated with bronchiectasis are lacking, and in the interval between the ,episodes of bleeding most of these patients are symptom-free. Others have a mild chronic cough with a small amount of sputum. This is more in the nature of a clearing of the throat and may be so insigni£tcant that the patient fails to make mention of it. Eventually this variety may be converted into typical putrid bronchiectasis by superimposed infection. Despite the severity of the local manifestations, constitutional symptoms may be mild or entirely absent for long periods after the onset of the disease. The nutrition may remain unimpaired, and apart from the annoyance of the cough and expectoration the patient is fairly comfortable. With increase in infection and· additional changes in the bronchopulmonary structures there occur loss of weight, easy fatigability, secondary anemia and febrile episodes of varying intensity. If there is inadequate drainage with retention of secretions, sepsis results. This is manifested by continuous fever, chills, drenching sweats and extreme weakness. Recurrent attacks of pneumonitis are ('ommon and are characterized by acute febrile reactions wit4~ exagr4eration of the local symptoms. These are usually of brief dqration Dyspnea is not a prominent symptom except in the prese~ce of extensive bilateral disease or during the attacks of acute pneumonitis If there is severe impairment of respiratory function, right ventricular strain may occur with the subsequent development of cor pulmonale Amyloidosis is an infrequent complication. Its development is dependent upon the degree and type of infection present. Clubbing of the fingers, and to a lesser extent of the toes, occurs in about one third of the patients. It is usually associated with an incurv.. ing Qf the nails. These changes represent either simple clubbing due to hypertrophy and hyperplasia of the tissues about the terminal phalanges, or hypertrophic osteo-arthropathy in which there is bone and joint hyperplasia. Physical examination may reveal few abnormal signs. Usually these are present only when there are extensive changes in the bronchopul-
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monary at:phitecture. They are not pathognomonic ,and are subject to considerable variation.. The most constant sign is the presence of fine or medium crackling rales over the base of one or both lungs. In some patients they are heard only after cough~ng. In advanced disease there may be limitation of motion of the chest over the affected area, dullness to percussion and distant breath sounds. DIAGNOSIS
In the diagnosis of bronchiectasis the medical history of the patient must be r,eviewedcarefully, particularly as it relates to the respiratory tract. A history of susceptibility to colds, pneumonia, tuberculosis or inhalation of a foreign body into the bronchus may indicate the need for further _study. In addition, consideration of the symptoms must be supplemented by ph.,Ysical, laboratory,_ bronchoscopic and x-ray examinations. Chronic cough" no matter how insignificant, is suggestive of bronchiectasis _and .must be investigated from that standpoint. It must be remembered that minimal bronchiectatic lesions do not, as a rule, produce the characteristic clinical picture usually associated with that condition. The morning cough often' attributed by patient~ to excessive smoking or sinusitis may be, the only manifestation of bronchial ectasia before severe infection supervenes. Hemoptysis, however slight, is significant. If there are severe 'paroxysms of cough accompanied by abundant quantities .of sputum, evidence of sepsis and clubbing. of the fingers, the diagnosis is almost self-evident and needs, only confirmatory studies. . The inadequaGY of physical examination in the diagnosis of bronchiectasis should be emphasized. Bronchiectasis is' a disease of symptoms rather th~n signs. Usually abnormal signs can be elicited only in the presence 'Of advanced disease. The ,most significant abnormality is the presence of crackling rales over the affected are,as, in many instances heard only after cough. The absolute diagnosis of bronchiectasis is based upon the x-ray appearance of'the chest, particularly after the introduction of a contrast medIum into the bronchial tree. Conventional x-ray films do not supply sufficient information to warrant a diagnosis of bronchiectasis even in the presence of advanced disease. If the changes are minimal the x-ray picture may be within ~ormallimits. Usually, however, there is intensification of thehilar shadows extending to the base of the lungs, resembling those seen in tracheo-bronchitis. In advanced dis.. ease the appearance of the chest'varies considerably. In 'addition to theprominent,hilar' markings, there maybe evidence of bronchopneumonia, atelectasis of a lobe or 'portion ofa lobe, areas of mottled densities, or radiolucent areas suggesting honeycombs (Fig. 204). Visualization of the bronchial tree by use 'of 'a radiopaq.ue substance is ,the only relia~le method of establishing the diagnosis
B
Fig.· 204.-A, Plain film of chest showing mottling at both bases consistent with pneumonitis assocIated .with bronchiectasis. B, Bronchogram in the· same case, proving bilateral bronchiectasis.
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(Fig. 204). This procedure, known as bronchography, reveals even the smallest dilatation. Introduction of the contrast medium, iodized oil, is a relatively simple procedure and there are several methods by which it can be done. It may be introduced by dire~t vision through a bronchoscope but, an easier and more widely used method is that of instillation, by the supraglottic route, through 'an intratracheal cathetet. The oil may be instilled under fluoroscopic guidance so that its How into the diseased area can be controlled, thus making it possible to. visualize any portion of the bronchial' tree independently
Fig. 205.-Bronchogram showing bilateral lower lobe bronch1ectasis, combined cylindrical and saccular types. Note the dilated bronchi shown behind the heart shadow on left.
(Fig. 205). Bronchographi.c studies are indicated for' all patients who complain of chronic cough for which·, no cause can be found. Even though plain films of the chest show no evidence of disease, broncho, grams may reveal dilatations which otherwise would have been unrecognized. this is particularly true of bronchial dilatations situated in that portion of the 'lung which lies behind the heart, an area not visible on conventional chest x-ray films. Direct inspection of the bronchial orifices by bronchoscopy often reveals the source of the secretions and furnishes some idea of the relative amounts originating in various lobes. This information is
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hIghly important when there is bilateral disease and resection is being considered or when there' is recurrent hemoptysis and the site of its origin must be determined. TREATMENT
A plan of treatment based on sound therapeutic principles is now available for the" patient suffering from bronchiectasis. Because of the number of unrelated conditi9Ds which may be associated with the disease and also' because of its diverse pathological and clinical manifestations, careful study of all its phases are necessary to determine the proper therapeutic approach. Treatment is dictated by the stage of the, disease, the number of lobes involved, the severity of the symptoms and the presence or absence of complications. In general, relief of individual symptoms may be obtained fro'm those 'therapeutic agents used for the alleviation of similar symptoms in 'other' diseases of the respiratory tract. The successful'management of the disease, however, is dependent upon the application of measures directed towards its specific features. ' Prophylaxis.-As a result of our increased understanding of its etiology and pathogenesis it is possible now, more than ever before, to prevent the dev-elopment of bronchiectasis. Residence in milder climates for those individual~~usceptible to respiratpry tract infeQtions may be a, means' of prevep.ting subsequent' irreparable bronchopulmonary damage. Whethel;, pr not one is in agreement with the theory that sinus infection is 'a,· 'precursor of bronchiectasis, control of sinus infection is important. Sill(je bronchopneumonia is the most com.. mon antecedent, of bronchiectasjs, its effective treatment by chemo: therapeutic and antibiotic ageQts may have a definite, bearing on the subsequent health of the patient. Prompt removal' of inhaled foreign bodies, endothoracic new growths and bronchial strictures wherever possible may prevent interference with bronchial drainage and lessen the danger of bronchial ectasia. . Medical Treatment.-Although bronchiectasis is a disease of the respiratory tract, in its advanced stages it produces severe constitutional disturbances. Medical measures, therefore, must be directed towards relief of the systemic effects as well as of the local symptoms. Rest in bed is of value not only in conserving energy but also because it tends to reduce the cough. The diet should be of high caloric, high vitamin con~ent. Since vitamin "requirements are higher in disease than in health, supplemental vitamin feedings are indicated. Climatic' conditions have no effect on the disease itself, although warm, dry climates may lessen 'the incidence of acute respiratory infections.' Sulfonamide therapy, alone or in combination with allergy' management, has been recommended by Thomas 'and his associates10 as being of definite value. This regimen, how~ver, has not been generally accepted
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as a satisfactory form of treatment. In the past a vast number of therapeutic measures have been recommended as beneficial in bronchiectasis. Of these, bronchial lavage using hypertonic salt solutions, iodized oil instillations, vaccine therapy and roentgen-ray therapy have been most widely used, but they are of doubtful value and in recent years are being used with decreasing frequency. Postural drainage is elnployed to assist the patient in getting rid of the thick, viscid sputum. In this procedure the patient assumes various positions in which emptying of the bronchialqilatations is facilitated by gravity. The simplest method and the one most widely used is continuous elevation of the foot of the bed. Hanging from the hips over the side of the bed with the head almost touching the Hoor or bending over a stool in a similar manner are usually more effective. No particular posture is applicable in. all cases, but most patients soon learn by experience which position gives them the best results. The procedure should be repeated at least twice daily, at night before retiring and in the morning before arising. Usually fifteen to twenty minutes will suffice to loosen the secretions which are expectorated in large quantities soon after resuming normal posture. When postural drainage is first attempted many patients complain of headache or vertigo, but these symptoms always disappear after several treatments. Expectorant drugs may be helpful in decreasing the viscosity of the secretions when postural drainage is being performed. Bronchoscopic aspirations, repeated at intervals, are of considerable palliative value. They are particularly useful in patients with advanced bilateral disease who are considered unsuitable for surgery. Removal of secretions in this way not only affords great relief but may prevent the spread of disease by lessening damage due to stagnated exudate. Antibiotic Therapy.-Antibiotic agents are of definite value inbronchiectasis but their effects are only temporary. Since no permanent benefits may be expected from any medical procedure in this disease, prolonged use of antibiotics is unnecessary. They should be administered only when indicated and discontinued as soon as they have served their purpose. Their most important field of usefulness lies in combating the superimposed acute respiratory tract infections, particularly the attacks of pneumonitis, which are such a prominent feature of the disease. Their use during these periods results in reduction in the volume and offensive character of the sputum. There is usually some improvement in the general condition and some limitation of sepsis. In addition, they are of inestimable value in the preparation of patients for pulmonary resection and in decreasing the postoperative complications. For the treatment of bronchiectasis antibiotics may be administered intratracheally, by nebulization in the form of an aerosol, or parenterally. Intratracheal instillations have now been generally supplanted
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by aerosols. The best results are obtained by simultaneous use of nebulization and intramuscular injections. Penicillin and streptomycin Inay be used either alone or in combination. Penicillin aerosols are mists or sprays in which are suspended fine particles of penicillin. The aerosol is prepared by dissolving 20,000 or 25,000 units of penicillin in 1 cc. sterile isotonic solution of sodium ch.loride. This solution is converted into a spray by a stream of compressed oxygen as described by Barach and his co-workers. 11 The fluid is completely vaporized in from ten to fifteen minutes and the treatments should be repeated every three or four hours during the day and once or twice during the night, if necessary. This procedure is quite simple and apparatus is now available for home use. Aerosol therapy has the advantage of depositing high concentrations of penicillin directly at the site of bacterial invasion and at the same time producing adequate blood levels. Use of a .hand-operated bulb atomizer has been recommended. This method of administration is of doubtful value, not only because of the coarseness of the spray produced, but also because so much energy is expended in the frequently repeated treatments that patients become fatigued. Penicillin should be given parenterally in conjunction with aerosol. Intramuscular injections of 25,000 units of penicillin every three hours will augment the effect of the inhalations and give better results than if either method is used alone. Streptomycin is of value in bronchiectasis, particularly in eradicating penicillin-resistant organisms. Olsen12 noted striking improvement after the administration of streptomycin aerosol, while Nichols and Herrel118 obtained favorable results following parenteral injections. Daily nebulization of 500,000 units of streptomycin in 20 cc. isotonic saline solution is recommended. For intramuscular injections, % dram (500,000 units) of streptomycin dissolved in 1 cc. of sterile isotonic saline solution may be given once or twice daily. Administration of streptomycin by both routes is sometimes advisable. Nichols and Herrell have pointed out that the elimination of gram-negative organisms from the secretions by the use of streptomycin may favor the growth of organisms which are sensitive to penicillin. They suggest the combined administration of penicillin and streptomycin in an effort to eliminate pathogens sensitive to both antibiotics, particularly since there is no contraindication to their use simultaneously. For this purpose solutions containing 200,000 units of penicillin and 500,000 units of ~treptomycin in 20 to 30 cc. saline solution should be nebulized every 24 hours. Surgical Treatment.-Many surgical procedures have been recommended for the treatment of bronchiectasis but most of them have failed to stand the test of time. During recent years, phrenicectomy, thoracoplasty and artificial pneumothorax have been given extensive trial
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and all have been found unsatisfactory. Objections to these measures are that they fail to remove the source of infection and that they have a tendency to interfere with the drainage of ·secretions from the bronchial tree. Since the changes in bronchiectasis are irreversible, permanent cure of the disease can be obtained only by surgical removal of the affected lobe or lobes. Provided there are no other contraindications to surgical treatment, lobectomy should be recommended for all patients except those with minimal disease who can be treated conservatively and those with involvement of all lobes. When the disease is bilateral, removal of the lobe which is most extensively involved usually gives considerable symptomatic relief. In these patients preliminary bronchospirometric studies should be made to make certain that the functional capacity of the contralateral lung is not too greatly impaired. The amazing results obtained following pulmonary resection for bronchiectasis make it imperative that every patient be given the opportunity to obtain a complete cure by this form of treatment. During recent years reports from various sources have shown a mortality rate in pulmonary resection of from 3 to 5 per cent. The most recent reports, however, are even more encouraging. Thus Kay, Mead and Hughes,14 in a series of 258 consecutive lobectomies had only one operative death, a mortality rate of 0.35 per cent, while Adams and Fioarra,15 report fifty consecutive operative cases, including one pneumonectomy, with no deaths either at operation or during the follow-up period. In the light of these statistics, pulmonary resection must be regarded as a comparatively safe procedure which should be recommended without hesitation in selected patients. The postoperative course is often extremely mild and recovery rapid. By means of this method of treatment, chronic invalids may be restored to health and returned to their normal place in society free from the unpredictable paroxysms of cough, the foul sputum and other offensive features of the disease. REFERENCES 1. Blades, B. a.nd Dugan, D. J.: Pseudobronchiectasis Following Atypical Pneumonia. Bull. U. S. Army M. Dept., 70:60-67 (Nov.) 1943. 2. Farrell, J. T. Jr.: The Importance of Early Diagnosis in Bronchiectasis. J.A.M.A., 106:92-96 (Jan. 11) 1936. 3. Tannenberg, G., and Pinner, M.: Atelectasis and Bronchiectasis: Experimental Study Concerning Their Relationship. J. Thoracic Surg., 11 :571-615 (Aug.) 1942. 4. Boyd, G. L.: Bronchiectasis in Children. Canad. M. A. J., 25:174-182 (Aug.) 1931. 5. Churchill, E. D. and Belsey, R.: Segmented, Pneumonectomy in Bronchiectasis. Ann. Surg., 109:481-499 (April) 1939~", 6. Duken, J. and von den Steiner, R.: Das Krankheitsbild der Bronchietasie im Kindesalter. Ergebn. d. imm. Med. u. Kinderh. 84:457-566, 1928.
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7. Lisa, J. R. and Rosenblatt, M. B.: Bronchiectasis. New York, Oxford University Press, 1943. 8. Perry, K. and King, D.: Bronchiectasis. Am. Rev. Tuberc., 41:531-548 (May) 1940. 9. Clerf, L. H.: Clinical Signi6cance of Certain Respiratory Symptoms. Pennsylvania M. J., 49:520-522 (Feb.) 1946. 10. Thomas, J. W., Van Ordstrand, H. S. and Tomlinson, C.: The Treatment of Bronchiectasis with Chemotherapy and Allergy Management. Ann. Int. Med., 23:405--413 (Sept.) 1945. 11. Barach, A. L., et al.: Inhalation of Penicillin Aerosol in Patients with Bronchial Asthma, Chronic· Bronchitis, Bronchiectasis and Lung Abscess: Preliminary Report. Ann. Int. Med., 22:485-509 (Apr.) 1945. 12. Olsen, A. M.: Streptomycin Aerosol in the Treatment of Chronic Bronchiectasis: Preliminary Report. Proc. Staff Meet., Mayo Clin., 21:53-54 (Feb. 6) 1946. 13. Nichols, D. R. and Herrell, W. E.: Streptomycin. J.A.M.A., 132:200-206 (Sept. 28) 1946. 14. Kay, E. B., Meade, R. H. Jr., and Hughes, F. A. Jr.: Surgical Treatment of Bronchiectasis. Ann. Int. Med., 26:1-12 (Jan.) 1947. 15. Adams, R. and Ficarra, B. J.: Appraisal of Surgery in the Treatment of Bron.. chiectasis. J.A.M.A., 184:240-245 (May 17) 1947.
SOKOLOFF,
M.D., F.A.C.P.
(I<
BRONCHIECTASIS is a disease characterized by irreversible pathological changes in the bronchial tree and its adjacent parenchyma. Modern diagnostic procedures reveal that it is much more common than was formerly believed; among chronic pulmonary diseases it is probably exceeded in frequency only by pulmonary tuberculosis. As is true of many other chronic conditions, it may exert a profound psychological effect in addition to the physical impairment it produces. Advances in medical science, particularly in the fields of radiology, bronchoscopy and respiratory physiology, have greatly increased our knowledge of bronchiectasis. As a result, we now have a much clearer understancling of the factors responsible for the development of the disease and are better able to recognize its presence in the early stages and to evaluate the degree of resulting respiratory impairment by means of pulmonary function tests. Similarly, antibiotic therapy and improved technics in thoracic surgery have dramatically changed the methods of managing bronchiectasis. Until recently, no specific therapeutic measures were available. Heretofore treatment afforded only symptomatic relief and did nothing to impede the development of new lesions. The introduction of newer methods of treatment not only provides a very definite plan of attack but has made it possible to dispel, to a great degree, the extreme pessimism which formerly prevailed among both physicians and patients concerning the prognosis in bronchiectasis. By reason of these advances, hope of a complete and lasting cure may be offered even to patients with advanced disease, who, only a few years ago, would probably have been doomed to a life of chronic invalidism. ETIOLOGY
Bronchiectasis may be congenital or acquired. Although there is considerable controversy concerning the congenital origin of bronchiectasis, it is generally believed that such a form does occur. The occasional case found in young infants and the larger number found in older children suggests the possibility of some congenital defect as a cause of the bronchial dilatations. Cystic bronchiectasis or cystic disease of the lung is consillered in this category. Acquired bronchiectasis is much more COIlllI10n and is the for111 which produces the classical clinical picture of the disease. It may be secondo Assistant Professor of Medicine, Jefferson Medical College; Chief, Medical Section, Barton Memorial Division, Jefferson Medical College Hospital; Chief, Division of Tuberculosis, Department of Public Health, Philadelphia.
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ary to a wide variety of conditions. Bronchopneumonia is generally recognized as the most common precursor of bronchiectasis. In contrast, it is generally believed that pneumococcic lobar pneumonia is rarely associ8rted with clinical bronchiectasis and plays a negligible part in its production. Measles and pertussis frequently antedate bronchiec.. tasis. In these conditions it is probably the accompanying bronchial and pulmonary infections which are responsible for the subsequent bronchial ectasia. Primary atypical pneumonia may, according ,to Blades and Dugan,! cause temporary dilatations of the bronchi, a condition known as pseudobronchiectasis. These dilatations persist for several. months but with their spontaneous disappearance the normal. contour' of the bronchial wall is restored. There is, however, no conclusive evidence that virus pneumonia can .produce true anatomical bronchiectasis. Chronic bronchitis, asthma and influenza are considered by some to be important antecedents of bronchiectasis. Lung abscess, bronchial and mediastinal neoplasm, foreign bodies, granulations and cicatrices of the bronchial mucosa such as occur in tuberculous bronchitis, enlarged mediastinal lymph glands, empyema and aortic aneurysm may produce changes in either the lumen of the bronchi or the contour of the bronchial wall with subsequent ectasia. Sinusitis is regarded by many as an important cause of bronchiectasis, principally because of the frequency with which these conditions are found in the same patient. In a series of bronchiectatic patients studied by Farrell,2 86 per cent were found to have sinus infection; similarly high incidence rat~s have been reported from other sources. During recent years, however, the role of sinusitis in the production of bronchiectasis has been questioned by many observers who maintain that the frequent association of these conditions is the result of widespread respiratory tract infection and that their coexistence proves no causal relationship. The various conditions which precede bronchiectasis favor its development by causing changes in the bacterial flora and in the structure of the bronchopulmonary tree. Usually three factors combine to produce the lesions which constitute bronchiectatic disease. They are (1) partial occlusion, which interferes with drainage and causes retention of infected material in the bronchi distal to the point of occlusion; (2) infection, which weakens the bronchial wall; (3) a mechanical force which produces the dilatations-this force may be intrinsic, such as a cough, which widens the previously weakened bronchus, or extrinsic, such as. traction exerted externally on the bronchial wall by puImorrary fibrosis, particularly as it occurs in tuberculosis. Tannenberg and Pinner3 have shown that infection is an absolute..prereqQ)site for the production of bronchiectasis in experimental anittlals, and It is generally agreed that this applies also to the disease as it occurs in man. The destructive processes in the bronchi and surrounding tis-
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sues which impair the integrity of the bronchial wall permitting it to dilate are due to this infection. Bacteriological studies of secretions obtained from bronchiectatic lesions reveal the presence of a variety of organisms. These may be either aerobic or anaerobic, and, according to Boyd,4 may vary from time to time in the same patient as a result of secondary infection. The organisms most commonly associated with bronchiectasis are Streptococcus hemolyticusand nonhemolyticus, Streptococcus viridans, Staphylococcus albus and aureus, Micrococcus catarrhalis, pneumococcus, Hemophilus influenzae and Micrococcus tetragenus and flavus. Occasionally fusiform bacilli, Friedlander bacilli, spirochetes and monilias are found. PATHOLOGY While bronchiectasis is primarily a disease of the bronchial tree, the peribronchial tissues and adjacent alveoli are also involved. The pathological features of bronchiectasis are dependent upon the antecedent condition, the type of organism present and the extent of involvement. Chronic inflammatory changes in the bronchial mucosa and submucosa, with extension of the process into the adjacent parenchyma, characterize the early stage of the disease. A serous exudate, which later becomes purulent, separates the epithelium from its basement membrane. As the process extends there is desquamation of the epithelium and destruction of the muscular and elastic elements and occasionally the cartilage of the bronchial wall. This necrotic inflammation which extends into the' surrounding pulmonary tissue is responsible for the characteristic bronchial dilatations. Reparative changes follow the stage of necrosis and dilatation. These are characterized by replacement of the mucous membrane by granulation tissue and replacement of the muscle and elastica of the bronchial wall by fibrous tissue. Regeneration of the bronchial epithelium frequently occurs, the ciliated cells being replaced mainly by nonciliated columnar and cuboidal cells. The parenchyma reveals evidence of fibrous change and usually varying degrees of emphysema. The disease is classified as cylindrical or saccular, depending on the shape assumed by the bronchial dilatations. Combinations of these forms are frequent. Cystic bronchiectasis, in which dilatatioQ.s are thinwalled, air-containing sacs of varying size, is thought to be of congenital origin. This form usually does not produce the same clinical picture as that seen in acquired bronchiectasis. Bronchiectasis may be unilateral or bilateral and it may involve por.tions of one or more lobes. When the lesions are bilateral one lung is usually nlore involved than the other. It occurs most frequently in the lower lobes, the left lower being affected more often tha.n the right. Involvement of the lingula of the left upper lobe is a frequent
BRONCillECTASIS
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accompaniment' of disease in the lower lobe of the same side, as shown by Churchill and Belsey.5 In their series of 55 lobectomies for disease in the left lower lobe, the lingula was found involved in 44 (80 per cent). The preponderance of left lung involvement is believed by Duken and von den Steiner6 to be due to the fact that the right bronchus drains more easily because it is a continuation of the trachea, because of the narrower lumen of the left bronchus and also because the left bronchus is constricted at the point where it is crossed by the pulmonary artery. ' Recent studies indicate that bronchiectasis is not a progressive dis.. e~se and that once established it does not extend to other areas. The development of additional lesions is believed to be the result of subsequent infection which produces changes in previously uninvolved portions of the lung. This infection may be a pneumonitis, occurring in the affected lobe and terminating in a suppurative lesion in the sur.. rounding healthy parenchyma or in extension of the preexisting parenchymal lesion. It may also occur as a bronchopneumonia involving a previously unaffected lobe, thereby creating a new area of bronchiectasis. These superimposed infections are responsible, in part, for the acute exacerbations so characteristic of the clinical phase of bronchiectasis. INCIDENCE The nature of bronchiectasis is such that its true incidence is difficult to deter~ mine. Because of the lack of characteristic symptoms in many patients and the the fact that the clinical picture is dominated by symptoms of the primary condition in others, the existence of the process may escape detection. During recent years there has been a definite increase in the number of patients in whom this diagnosis has been established. This rising incidence, however, is probably apparent rather than real, being the result of the widespread use of specific diagnostic procedures, particularly bronchoscopy and bronchography. Statistics relating to bronchiectasis are meager and therefore not altogether reliable as an indication of the prevalence of the disease. Depending upon the source from which they emanate they show considerable variation. Thus, Lisa and Rosenblatt, 7 in a review of the records of 92,235 patients discharged from the New York City Hospital during the ten year period 1932-1941, found that only seventy patients (0.08 per cent) had a primary diagnosis of bronchiectasis. In contrast, these observers call attention to the fact that 70 new patients with bronchiectasis were admitted to the Out-Patient Department of the same institution in the two year period 1940-1941. There can be no doubt that many cases of bronchiectasis in the general population remain undiagnosed because so many of the patients do not present typical symptoms. It seems reasonable to assume. that the apparent wide variation in incidence is a reflection of the intensity with which the search for the disease was conducted by various groups of physicians. The disease is found with equal frequency in both sexes. Its occurrence, however, varies considerably in different age groups. It is generally agreed that in the majority of cases bronchiectasis develops in childhood or early adult life. This is in accord with the observations of Perry and King,S who found that in a series of 400 patients, 42 per cent were under 10 years of age, 27 per cent were between the ages of 11 and 20, 24 per cent were between 21 and 40 and only 7 per cent
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were over 40 years of age. It is also generally recognized that bronchiectasis is not a frequent finding in older age groups. Extensive or untreated bronchiectasis in older individuals is a serious disorder, often accompanied by severe cardiac and metabolic disturbances. In addition, the acute respiratory tract infections· wl1ich are so common in this condition tend to be of more serious consequence witli advancing years. Because of these complications the life expectancy in this group of patients is sharply reduced.
CLINICAL ASPECTS
The symptoms of bronchiectasis depend upon the severity of the existin~ infection, the ~ype of predominating organisms and, to a lesser extent, the, amount of bronchopulmonary involvement. If the infection is! mild the clinical picture is not characteristic. The patient may complain only of recurrent attacks of bronchitis, especially during the winter months. These differ from ordinary attacks of acute ., bronchitis in that they are longer in duration and usually do not respond readily to treatment. The sputum during these periods. may be streaked with blood and occasionally a dram or two of unmixed, bright-red blood may be expectorated. Other patients complain only of morning cough and expectoration. This bout of coughing occurs upon first arising, and after expectoration of a small amount of sputum symptoms may be absent until the next morning, when the procedure is repeated. These individuals nearly always attribute their symptoms to sinusitis or smoking. In those with extreme degrees of infection or extensive lesions the clinical picture may vary considerably. Cough is the most constant symptom, being present to some extent in almost every instance. It is paroxysmal, usually worse in the morning upon arising and nearly always productive. These paroxysms may be severe enough to cause vomiting or attacks of faintness or dizziness. In the intervening periods some patients may be entirely free from cough, while others have an irritation of the throat or a wheeze in the chest which induces attacks of cou'ghing. The coughing attacks are associated with the production of variable amounts of purulent sputum. Whereas the average patient expectorates about 1 or 2 ounces daily, those with severe infection may raise as much as SO ounces of sputum in twenty-four hours. If small quantities of sputum are expectorated the material has no distinguishing characteristics. Occasionally, but not always, large amounts of the sputum will separate into three layers if allowed to stand for several hours, a lower layer, grayish in color and opaque, a turbid middle layer and a frothy brownish material on top. The expectorated material may be fetid if anerobic organisms are present. Hemoptysis is an important symptom in bronchiectasis. The bleeding may vary from minute traces of blood, barely visible in the sputum, to frank hemorrhage of a pint or more. These more copious hem-
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orrhages occur in episodes during which the bleeding is repeated at intervals over periods lasting from several days to a week or' more. Occasionally the hemorrhage may be fatal. It is estimated that hemoptysis occurs in about 50 per cent of bronchiectatic patients. The importance of this symptom is emphasized by Clerf,9 who states that it is probably more often observed in bronchiectasis than in any other condition, including tuberculosis. Dry, hemorrhagic bronch,iectasis is the term applied to a form of the disease in which the predominant and sometimes the only symptom is hemoptysis. Its course is characterized by pulmonary hemorrhages repeated at varying intervals and often massive in extent. The paroxysmal cough and abundant sputum generally associated with bronchiectasis are lacking, and in the interval between the ,episodes of bleeding most of these patients are symptom-free. Others have a mild chronic cough with a small amount of sputum. This is more in the nature of a clearing of the throat and may be so insigni£tcant that the patient fails to make mention of it. Eventually this variety may be converted into typical putrid bronchiectasis by superimposed infection. Despite the severity of the local manifestations, constitutional symptoms may be mild or entirely absent for long periods after the onset of the disease. The nutrition may remain unimpaired, and apart from the annoyance of the cough and expectoration the patient is fairly comfortable. With increase in infection and· additional changes in the bronchopulmonary structures there occur loss of weight, easy fatigability, secondary anemia and febrile episodes of varying intensity. If there is inadequate drainage with retention of secretions, sepsis results. This is manifested by continuous fever, chills, drenching sweats and extreme weakness. Recurrent attacks of pneumonitis are ('ommon and are characterized by acute febrile reactions wit4~ exagr4eration of the local symptoms. These are usually of brief dqration Dyspnea is not a prominent symptom except in the prese~ce of extensive bilateral disease or during the attacks of acute pneumonitis If there is severe impairment of respiratory function, right ventricular strain may occur with the subsequent development of cor pulmonale Amyloidosis is an infrequent complication. Its development is dependent upon the degree and type of infection present. Clubbing of the fingers, and to a lesser extent of the toes, occurs in about one third of the patients. It is usually associated with an incurv.. ing Qf the nails. These changes represent either simple clubbing due to hypertrophy and hyperplasia of the tissues about the terminal phalanges, or hypertrophic osteo-arthropathy in which there is bone and joint hyperplasia. Physical examination may reveal few abnormal signs. Usually these are present only when there are extensive changes in the bronchopul-
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monary at:phitecture. They are not pathognomonic ,and are subject to considerable variation.. The most constant sign is the presence of fine or medium crackling rales over the base of one or both lungs. In some patients they are heard only after cough~ng. In advanced disease there may be limitation of motion of the chest over the affected area, dullness to percussion and distant breath sounds. DIAGNOSIS
In the diagnosis of bronchiectasis the medical history of the patient must be r,eviewedcarefully, particularly as it relates to the respiratory tract. A history of susceptibility to colds, pneumonia, tuberculosis or inhalation of a foreign body into the bronchus may indicate the need for further _study. In addition, consideration of the symptoms must be supplemented by ph.,Ysical, laboratory,_ bronchoscopic and x-ray examinations. Chronic cough" no matter how insignificant, is suggestive of bronchiectasis _and .must be investigated from that standpoint. It must be remembered that minimal bronchiectatic lesions do not, as a rule, produce the characteristic clinical picture usually associated with that condition. The morning cough often' attributed by patient~ to excessive smoking or sinusitis may be, the only manifestation of bronchial ectasia before severe infection supervenes. Hemoptysis, however slight, is significant. If there are severe 'paroxysms of cough accompanied by abundant quantities .of sputum, evidence of sepsis and clubbing. of the fingers, the diagnosis is almost self-evident and needs, only confirmatory studies. . The inadequaGY of physical examination in the diagnosis of bronchiectasis should be emphasized. Bronchiectasis is' a disease of symptoms rather th~n signs. Usually abnormal signs can be elicited only in the presence 'Of advanced disease. The ,most significant abnormality is the presence of crackling rales over the affected are,as, in many instances heard only after cough. The absolute diagnosis of bronchiectasis is based upon the x-ray appearance of'the chest, particularly after the introduction of a contrast medIum into the bronchial tree. Conventional x-ray films do not supply sufficient information to warrant a diagnosis of bronchiectasis even in the presence of advanced disease. If the changes are minimal the x-ray picture may be within ~ormallimits. Usually, however, there is intensification of thehilar shadows extending to the base of the lungs, resembling those seen in tracheo-bronchitis. In advanced dis.. ease the appearance of the chest'varies considerably. In 'addition to theprominent,hilar' markings, there maybe evidence of bronchopneumonia, atelectasis of a lobe or 'portion ofa lobe, areas of mottled densities, or radiolucent areas suggesting honeycombs (Fig. 204). Visualization of the bronchial tree by use 'of 'a radiopaq.ue substance is ,the only relia~le method of establishing the diagnosis
B
Fig.· 204.-A, Plain film of chest showing mottling at both bases consistent with pneumonitis assocIated .with bronchiectasis. B, Bronchogram in the· same case, proving bilateral bronchiectasis.
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(Fig. 204). This procedure, known as bronchography, reveals even the smallest dilatation. Introduction of the contrast medium, iodized oil, is a relatively simple procedure and there are several methods by which it can be done. It may be introduced by dire~t vision through a bronchoscope but, an easier and more widely used method is that of instillation, by the supraglottic route, through 'an intratracheal cathetet. The oil may be instilled under fluoroscopic guidance so that its How into the diseased area can be controlled, thus making it possible to. visualize any portion of the bronchial' tree independently
Fig. 205.-Bronchogram showing bilateral lower lobe bronch1ectasis, combined cylindrical and saccular types. Note the dilated bronchi shown behind the heart shadow on left.
(Fig. 205). Bronchographi.c studies are indicated for' all patients who complain of chronic cough for which·, no cause can be found. Even though plain films of the chest show no evidence of disease, broncho, grams may reveal dilatations which otherwise would have been unrecognized. this is particularly true of bronchial dilatations situated in that portion of the 'lung which lies behind the heart, an area not visible on conventional chest x-ray films. Direct inspection of the bronchial orifices by bronchoscopy often reveals the source of the secretions and furnishes some idea of the relative amounts originating in various lobes. This information is
BRONcau:crASIS /0
; (
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hIghly important when there is bilateral disease and resection is being considered or when there' is recurrent hemoptysis and the site of its origin must be determined. TREATMENT
A plan of treatment based on sound therapeutic principles is now available for the" patient suffering from bronchiectasis. Because of the number of unrelated conditi9Ds which may be associated with the disease and also' because of its diverse pathological and clinical manifestations, careful study of all its phases are necessary to determine the proper therapeutic approach. Treatment is dictated by the stage of the, disease, the number of lobes involved, the severity of the symptoms and the presence or absence of complications. In general, relief of individual symptoms may be obtained fro'm those 'therapeutic agents used for the alleviation of similar symptoms in 'other' diseases of the respiratory tract. The successful'management of the disease, however, is dependent upon the application of measures directed towards its specific features. ' Prophylaxis.-As a result of our increased understanding of its etiology and pathogenesis it is possible now, more than ever before, to prevent the dev-elopment of bronchiectasis. Residence in milder climates for those individual~~usceptible to respiratpry tract infeQtions may be a, means' of prevep.ting subsequent' irreparable bronchopulmonary damage. Whethel;, pr not one is in agreement with the theory that sinus infection is 'a,· 'precursor of bronchiectasis, control of sinus infection is important. Sill(je bronchopneumonia is the most com.. mon antecedent, of bronchiectasjs, its effective treatment by chemo: therapeutic and antibiotic ageQts may have a definite, bearing on the subsequent health of the patient. Prompt removal' of inhaled foreign bodies, endothoracic new growths and bronchial strictures wherever possible may prevent interference with bronchial drainage and lessen the danger of bronchial ectasia. . Medical Treatment.-Although bronchiectasis is a disease of the respiratory tract, in its advanced stages it produces severe constitutional disturbances. Medical measures, therefore, must be directed towards relief of the systemic effects as well as of the local symptoms. Rest in bed is of value not only in conserving energy but also because it tends to reduce the cough. The diet should be of high caloric, high vitamin con~ent. Since vitamin "requirements are higher in disease than in health, supplemental vitamin feedings are indicated. Climatic' conditions have no effect on the disease itself, although warm, dry climates may lessen 'the incidence of acute respiratory infections.' Sulfonamide therapy, alone or in combination with allergy' management, has been recommended by Thomas 'and his associates10 as being of definite value. This regimen, how~ver, has not been generally accepted
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as a satisfactory form of treatment. In the past a vast number of therapeutic measures have been recommended as beneficial in bronchiectasis. Of these, bronchial lavage using hypertonic salt solutions, iodized oil instillations, vaccine therapy and roentgen-ray therapy have been most widely used, but they are of doubtful value and in recent years are being used with decreasing frequency. Postural drainage is elnployed to assist the patient in getting rid of the thick, viscid sputum. In this procedure the patient assumes various positions in which emptying of the bronchialqilatations is facilitated by gravity. The simplest method and the one most widely used is continuous elevation of the foot of the bed. Hanging from the hips over the side of the bed with the head almost touching the Hoor or bending over a stool in a similar manner are usually more effective. No particular posture is applicable in. all cases, but most patients soon learn by experience which position gives them the best results. The procedure should be repeated at least twice daily, at night before retiring and in the morning before arising. Usually fifteen to twenty minutes will suffice to loosen the secretions which are expectorated in large quantities soon after resuming normal posture. When postural drainage is first attempted many patients complain of headache or vertigo, but these symptoms always disappear after several treatments. Expectorant drugs may be helpful in decreasing the viscosity of the secretions when postural drainage is being performed. Bronchoscopic aspirations, repeated at intervals, are of considerable palliative value. They are particularly useful in patients with advanced bilateral disease who are considered unsuitable for surgery. Removal of secretions in this way not only affords great relief but may prevent the spread of disease by lessening damage due to stagnated exudate. Antibiotic Therapy.-Antibiotic agents are of definite value inbronchiectasis but their effects are only temporary. Since no permanent benefits may be expected from any medical procedure in this disease, prolonged use of antibiotics is unnecessary. They should be administered only when indicated and discontinued as soon as they have served their purpose. Their most important field of usefulness lies in combating the superimposed acute respiratory tract infections, particularly the attacks of pneumonitis, which are such a prominent feature of the disease. Their use during these periods results in reduction in the volume and offensive character of the sputum. There is usually some improvement in the general condition and some limitation of sepsis. In addition, they are of inestimable value in the preparation of patients for pulmonary resection and in decreasing the postoperative complications. For the treatment of bronchiectasis antibiotics may be administered intratracheally, by nebulization in the form of an aerosol, or parenterally. Intratracheal instillations have now been generally supplanted
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by aerosols. The best results are obtained by simultaneous use of nebulization and intramuscular injections. Penicillin and streptomycin Inay be used either alone or in combination. Penicillin aerosols are mists or sprays in which are suspended fine particles of penicillin. The aerosol is prepared by dissolving 20,000 or 25,000 units of penicillin in 1 cc. sterile isotonic solution of sodium ch.loride. This solution is converted into a spray by a stream of compressed oxygen as described by Barach and his co-workers. 11 The fluid is completely vaporized in from ten to fifteen minutes and the treatments should be repeated every three or four hours during the day and once or twice during the night, if necessary. This procedure is quite simple and apparatus is now available for home use. Aerosol therapy has the advantage of depositing high concentrations of penicillin directly at the site of bacterial invasion and at the same time producing adequate blood levels. Use of a .hand-operated bulb atomizer has been recommended. This method of administration is of doubtful value, not only because of the coarseness of the spray produced, but also because so much energy is expended in the frequently repeated treatments that patients become fatigued. Penicillin should be given parenterally in conjunction with aerosol. Intramuscular injections of 25,000 units of penicillin every three hours will augment the effect of the inhalations and give better results than if either method is used alone. Streptomycin is of value in bronchiectasis, particularly in eradicating penicillin-resistant organisms. Olsen12 noted striking improvement after the administration of streptomycin aerosol, while Nichols and Herrel118 obtained favorable results following parenteral injections. Daily nebulization of 500,000 units of streptomycin in 20 cc. isotonic saline solution is recommended. For intramuscular injections, % dram (500,000 units) of streptomycin dissolved in 1 cc. of sterile isotonic saline solution may be given once or twice daily. Administration of streptomycin by both routes is sometimes advisable. Nichols and Herrell have pointed out that the elimination of gram-negative organisms from the secretions by the use of streptomycin may favor the growth of organisms which are sensitive to penicillin. They suggest the combined administration of penicillin and streptomycin in an effort to eliminate pathogens sensitive to both antibiotics, particularly since there is no contraindication to their use simultaneously. For this purpose solutions containing 200,000 units of penicillin and 500,000 units of ~treptomycin in 20 to 30 cc. saline solution should be nebulized every 24 hours. Surgical Treatment.-Many surgical procedures have been recommended for the treatment of bronchiectasis but most of them have failed to stand the test of time. During recent years, phrenicectomy, thoracoplasty and artificial pneumothorax have been given extensive trial
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and all have been found unsatisfactory. Objections to these measures are that they fail to remove the source of infection and that they have a tendency to interfere with the drainage of ·secretions from the bronchial tree. Since the changes in bronchiectasis are irreversible, permanent cure of the disease can be obtained only by surgical removal of the affected lobe or lobes. Provided there are no other contraindications to surgical treatment, lobectomy should be recommended for all patients except those with minimal disease who can be treated conservatively and those with involvement of all lobes. When the disease is bilateral, removal of the lobe which is most extensively involved usually gives considerable symptomatic relief. In these patients preliminary bronchospirometric studies should be made to make certain that the functional capacity of the contralateral lung is not too greatly impaired. The amazing results obtained following pulmonary resection for bronchiectasis make it imperative that every patient be given the opportunity to obtain a complete cure by this form of treatment. During recent years reports from various sources have shown a mortality rate in pulmonary resection of from 3 to 5 per cent. The most recent reports, however, are even more encouraging. Thus Kay, Mead and Hughes,14 in a series of 258 consecutive lobectomies had only one operative death, a mortality rate of 0.35 per cent, while Adams and Fioarra,15 report fifty consecutive operative cases, including one pneumonectomy, with no deaths either at operation or during the follow-up period. In the light of these statistics, pulmonary resection must be regarded as a comparatively safe procedure which should be recommended without hesitation in selected patients. The postoperative course is often extremely mild and recovery rapid. By means of this method of treatment, chronic invalids may be restored to health and returned to their normal place in society free from the unpredictable paroxysms of cough, the foul sputum and other offensive features of the disease. REFERENCES 1. Blades, B. a.nd Dugan, D. J.: Pseudobronchiectasis Following Atypical Pneumonia. Bull. U. S. Army M. Dept., 70:60-67 (Nov.) 1943. 2. Farrell, J. T. Jr.: The Importance of Early Diagnosis in Bronchiectasis. J.A.M.A., 106:92-96 (Jan. 11) 1936. 3. Tannenberg, G., and Pinner, M.: Atelectasis and Bronchiectasis: Experimental Study Concerning Their Relationship. J. Thoracic Surg., 11 :571-615 (Aug.) 1942. 4. Boyd, G. L.: Bronchiectasis in Children. Canad. M. A. J., 25:174-182 (Aug.) 1931. 5. Churchill, E. D. and Belsey, R.: Segmented, Pneumonectomy in Bronchiectasis. Ann. Surg., 109:481-499 (April) 1939~", 6. Duken, J. and von den Steiner, R.: Das Krankheitsbild der Bronchietasie im Kindesalter. Ergebn. d. imm. Med. u. Kinderh. 84:457-566, 1928.
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7. Lisa, J. R. and Rosenblatt, M. B.: Bronchiectasis. New York, Oxford University Press, 1943. 8. Perry, K. and King, D.: Bronchiectasis. Am. Rev. Tuberc., 41:531-548 (May) 1940. 9. Clerf, L. H.: Clinical Signi6cance of Certain Respiratory Symptoms. Pennsylvania M. J., 49:520-522 (Feb.) 1946. 10. Thomas, J. W., Van Ordstrand, H. S. and Tomlinson, C.: The Treatment of Bronchiectasis with Chemotherapy and Allergy Management. Ann. Int. Med., 23:405--413 (Sept.) 1945. 11. Barach, A. L., et al.: Inhalation of Penicillin Aerosol in Patients with Bronchial Asthma, Chronic· Bronchitis, Bronchiectasis and Lung Abscess: Preliminary Report. Ann. Int. Med., 22:485-509 (Apr.) 1945. 12. Olsen, A. M.: Streptomycin Aerosol in the Treatment of Chronic Bronchiectasis: Preliminary Report. Proc. Staff Meet., Mayo Clin., 21:53-54 (Feb. 6) 1946. 13. Nichols, D. R. and Herrell, W. E.: Streptomycin. J.A.M.A., 132:200-206 (Sept. 28) 1946. 14. Kay, E. B., Meade, R. H. Jr., and Hughes, F. A. Jr.: Surgical Treatment of Bronchiectasis. Ann. Int. Med., 26:1-12 (Jan.) 1947. 15. Adams, R. and Ficarra, B. J.: Appraisal of Surgery in the Treatment of Bron.. chiectasis. J.A.M.A., 184:240-245 (May 17) 1947.