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Bronchiectasis and Bronchopulmonary Sequestration
Symposium on Noncardiac Thoracic Surgery
Bronchiectasis and Bronchopulmonary Sequestration
R. Morton BoZman, III, M.D.,* and Walter G. Wolfe, M.D.t
BRONCHIECTASIS ·Bronchiectasis is one of the group of suppurative diseases of the lungs characterized by dilatation of the bronchi. This affliction, which was first described in 1819 by Laennec, was an important clinical entity, particularly in the years preceding the availability of antibiotics. Although its overall incidence has declined significantly as a result of effective antimicrobial therapy, bronchiectasis remains an entity with which the thoracic surgeon should be familiar.
Etiology The development of bronchiectasis is a multifactorial phenomenon. Although acquired infection of the lung is in all likelihood the major contributor, congenital factors may also play a role (Table 1). Congenital cystic bronchiectasis, an example of which is shown in Figure 1, is a disorder of infants and young children. This entity is thought to arise from an arrest in development of the bronchial tree. 1 Various immune deficiency states have been found to be associated with the formation of bronchiectasis in recent years. Patients with selective immunoglobulin A (IgA) deficiency or with primary or acquired hypogammaglobulinemia have been found to be susceptible to the development of bronchiectasis. 5 • 14 Alpharantitrypsin deficiency, though usually associated with panacinar emphysema, occasionally will be discovered as a contributing factor in cases of bronchiectasis. V arpela studied 60 patients with bronchographically proven bronchiectasis and found six (10 per cent) with the genotype Pimz who possessed less than 66 per cent of the normal level of alpha 1-antitrypsin. This substance is a very potent protease inhibitor, and patients with diminished levels would appear to be susceptible to airway damage from otherwise relatively benign infections. The disease seen in these patients with alpharantitrypsin *Chief Resident in Surgery, Duke University Medical Center, Durham, North Carolina. tProfessor of Surgery, Duke University Medical Center, Durham, North Carolina
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Table 1. Causative Factors of Importance in Development of Bronchiectasis Congenital Congenital cystic bronchiectasis Selective IgA deficiency Primary hypogammaglobulinemia Alpha,-antitrypsin deficiency Cystic fibrosis Congenital deficiency of bronchial cartilage Kartagener's syndrome: situs inversus, sinusitis, and bronchiectasis Bronchopulmonary sequestration Acquired Infection-bacterial, viral Bronchial obstruction Intrinsic: neoplasm, foreign body, mucus plug Extrinsic: enlarged lymph nodes "Middle lobe syndrome" Scarring secondary to tuberculosis Acquired hypogammaglobulinemia
deficiency is extensive, and it appears that deficiency of this inhibitor of proteolytic enzyme activity predisposes to development of bronchiectasis.24 As mentioned earlier, infection is felt to be critical to the development of bronchiectasis. The history frequently dates to the childhood years, with pneumonia developing as a sequel to measles, pertussis, or influenza. This association, though less prominent now, is nonetheless still observed. Bacterial pneumonia, with destruction of alveoli and bronchial walls, leads to scarring, and the resultant shrinkage of surrounding lung caused by scar contracture places traction on the bronchi, leading to bronchiectasis. 8 • 11 Other etiologic agents of importance include intrinsic bronchial obstruction from a foreign body, neoplasm, or mucus plug; extrinsic bronchial obstruction from enlarged hilar nodes; and bronchial dilatation following healing of tuberculous lesions or as a residual effect of chronic lung abscess. 8 • 23 Bradham described a series of 23 patients with so-called "middle lobe syndrome" and suggested that the presence or absence of complete fissures between the right upper and right middle lobes might have a bearing on the development of chronic infection and bronchiectasis. Patients with incomplete fissures would have improved collateral ventilation, allowing aeration of the pulmonary tissue, whereas those with complete fissures would not possess this alternative route and therefore would be predisposed to atelectasis and bronchiectasis in the middle lobe. Eleven of these 23 patient with bronchiectasis had complete fissures, and 8 of 11 had no other apparent etiology for their bronchiectasis. 2 Finally, a small number of cases can be ascribed to a congenital defect of a structural nature. Patients with cystic fibrosis and congenital deficiency of bronchial cartilage fall into this category. Also, there is an
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C.n·r:r!'·r:r Figure 1. A and B, Posteroanterior and lateral chest roentgenograms of a five-year-old boy with congenital cystic bronchiectasis demonstrate a "honeycomb" pattern of bronchiectasis with rarefaction in the left upper lobe. The patient had sustained an episode of necrotizing pneumonia at the age of two years. C, Marked cystic and saccular changes are noted throughout the left lung of this patient with very little uninvolved parenchyma.
association between situs inversus, sinusitis, and bronchiectasis. Together these disorders constitute Kartagener's syndrome, 4 in which there has been identified a congenital defect in respiratory cilia. 6
Pathology Reid divided bronchiectasis into three groupings: (1) cylindrical, with dilated bronchi of regular outline; (2) varicose, with greater dilatation, irregularity in outline, and absent peripheral filling; and (3) saccular or cystic, with bronchial dilatation increasing toward the periphery, balloon outline of the bronchi, and no communication with the peripheral bronchial tree. 19 The bronchiectatic changes typically involve
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the second to fourth order bronchi, and the distribution in the lungs corresponds to common sites of occurrence of necrotizing pneumonia, that is, the basal segments of the lower lobes. The superior segments are usually spared. 11 Figure 2 shows a representative pathologic specimen from a patient with cystic bronchiectasis of the lower lung. In patients with immune deficiency states, the disease may affect any or all pulmonary segments and may or may not be localized. Disease is bilateral in approximately 30 per cent of patients.
Clinical Presentation Typically, the patient with bronchiectasis presents a clinical picture characterized by recurrent cough productive of purulent sputum, which layers on standing; fever; and hemoptysis. Hemoptysis occurs in roughly 50 per cent of adult patients and rarely in pediatric patients. In 10 per cent it is severe, though rarely lethal. The hemoptysis is thought to be due to bleeding from anastomoses between hypertrophied bronchial arteries and the pulmonary circulation in diseased segments. 26 Other symptoms, though less common, may prove troublesome, These include fetor oris, which is produced by fetid sputum and which in years past constituted a major social and psychological problem but is now less common because of antibiotic therapy; anorexia; pleurisy; and arthralgias. In addition, these patients can manifest the findings of hyper-
Figure 2. Resection was performed on the left lung of a patient with severe cystic bronchiectasis of the lower lobe and the lingular segment of the upper lobe. Note multiple cysts of various sizes with destruction of intervening pulmonary parenchyma.
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trophic pulmonary osteoarthropathy, namely, clubbing of fingers, joint swelling, and shin tenderness. Interestingly, these symptoms abate following surgical extirpation of diseased lung. Physical examination reveals the signs of chronic illness, and occasionally the stigmata of pulmonary osteoarthropathy will be seen. Auscultatory findings are characterized by variation from day to day but commonly include diminished breath sounds over the lower chest and the presence of wet rales in the area of the involved lung.
Diagnosis A history of chronic cough, recurrent pneumonic infection with suppuration, fever, and often hemoptysis will strongly suggest the diagnosis in most instances. The plain chest roentgenogram is also useful and may reveal evidence of the underlying pathology. Such evidence includes increased markings in diseased segments, volume loss, and cystic spaces. In patients with severe cases, a "honeycomb" pattern may also be seen and is caused by rarefied areas of destroyed lung and compensatory overinflation of the remaining normal lung (Fig. 1). 8 Bronchography in three dimensions is the mainstay in the evaluation of bronchiectasis and must be performed to obtain a definitive diagnosis. This study should be done when the patient is in optimal condition, that is, after postural drainage and antibiotics have controlled any acute exacerbations and have decreased secretions to a minimum. Each lung should be studied separately. There is a reversible condition very much resembling bronchiectasis that occurs in the wake of an acute pneumonia. For this reason bronchography should be postponed for four to six weeks after an acute infectious episode. Figures 3 through 5 demonstrate the roentgenographic and bronchographic findings in two patients with bronchiectasis. Pulmonary function tests show no specific pattern in patients with bronchiectasis but should be performed to assess the tolerance of the individual patient for lung resection. Bronchoscopy is very helpful and should be performed to rule out endobronchial disease or bronchial obstruction caused by a foreign body or a neoplasm and to obtain good material for culture. In addition, the segmental anatomy can be studied and the procedure may prove to be therapeutic by providing good tracheobronchial toilet. Treatment
The initial treatment of bronchiectasis is always conservative, consisting of medical measures to control superimposed bronchitis or pneumonia. A long trial of medical therapy may be appropriate for determination of the need for surgery. 11 Antibiotics, the choice of which is based on culture and sensitivity data, should be continued until production of sputum is minimal. An aggressive regimen of postural drainage and chest physiotherapy should be combined with chemotherapy. The avoidance of tobacco and polluted air should be strongly encouraged, and all patients should receive pneumococcal vaccine and yearly influenza vaccine. 15
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Figure 3. UppeT TOw: posteroanterior and lateral chest roentgenograms and a lung scan in a patient with chronic saccular bronchiectasis of the left lower lobe. Note the volume loss and the cystic changes in the left lower lobe on the chest roentgenogram as well as the lack of perfusion on the scan seen at the far right. LoweT TOw: posteroanterior and lateral bronchograms in this patient reveal saccular dilatation of the bronchi of the left lower lobe and the lingular segment of the left upper lobe.
Figure 4. Posteroanterior chest roentgenogram of a patient with bronchiectasis of the right lower lobe. Note the increased markings in the right lower medial lung zone.
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Figure 5. Posteroanterior (A) and lateral (B) bronchographic views of the patient shown in Figure 4. This patient has localized saccular bronchiectasis of the right lower lobe, as seen in these bronchograms, with cystic dilatation of third and fourth order bronchi.
This regimen will suffice in the majority of patients with bronchiectasis. In patients in whom medical management fails after an adequate trial and in whom symptoms persist, with sputum production, recurrent pneumonitis, or frequent hemoptysis, surgical therapy should be considered. This is especially true in instances in which the disease is localized to one area or one segment of a lung. Surgical treatment is less feasible in patients in whom the disease is diffuse and multisegmental. Surgery for bronchiectasis dates to the late 1800s. Heuer in 1934 reported on 56 cases treated by resection between 1880 and 1920 and reported a 50 per cent mortality rate. The mortality remained high until the 1930s when Churchill reported on 84 cases of bronchiectasis treated by lobectomy with a mortality rate of only 4.6 per cent. Today, mortality following these resections is less than 1 per cent. Kent and Blades in 1942 demonstrated the technique of lobectomy with individual ligation of hilar structures. They were also among the earliest to utilize the technique of segmental resection in order to preserve normal lung tissue.16 In patients in whom surgery is being entertained as a therapeutic alternative, it is essential to have good quality three-dimensional bronchograms to fully outline the extent of disease. The ideal candidate has localized disease, preferably in one lobe or in contiguous segments of two lobes. 11 Sealy reported the experience at Duke University Medical Center in 140 patients with bronchiectasis, 70 with localized disease and 70 with diffuse disease. 22 In the group with localized disease, the onset could be traced to a single initiating event, such as pneumonia or aspiration of a foreign body, whereas in the group with diffuse involvement, an immune deficit was more likely to be the primary etiologic factor. The
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patients in this series were operated on after an intensive period of medical preparation, and the emphasis at surgery was on preservation of uninvolved lung by careful segmental resection. Mortality was less than 1 per cent and follow-up showed 95 per cent of patients with good-toimproved results. In general, the patients with localized disease had better results after surgery, and the incidence of recurrent disease was quite low. The ongoing immune deficiency status in the multisegmented group prevented their being uniformly asymptomatic after surgery. Overall, however, 36 per cent in this group were "cured," and an additional 53 per cent were improved and able to resume normal activity. In contrast, 80 per cent of the group with localized disease were symptom free and an additional 10 per cent were improved. 22 As alluded to in this series, and confirmed by George, bilateral disease need not be a contraindication to surgery. 9 George et al. reported on 99 patients undergoing 216 resections with an operative mortality of 1.4 per cent. The foci were mostly in dependent areas of the lungs, namely, the right middle lobe, the lingula of the left upper lobe, and basal segments of the lower lobes. The principle advanced by these authors is to attack the side with the most severe disease first and to stage the opposite side, if necessary, two to three months later. Also, preservation of normal pulmonary parenchyma becomes particularly critical in this group of patients, who constitute approximately 30 per cent of all patients with bronchiectasis. Five of the patients in this report developed cor pulmonale 5 to 20 years after resection. The mean number of segments removed in this group was 11, significantly greater than the average for the series. Overall, 83 of 90 patients showed improvement, 9 showed no change, and 4 actually had progression of disease after surgery. 9 Sanderson reviewed 393 patients treated for bronchiectasis at one institution over a 15-year period. 21 Roughly, two thirds received surgical treatment and one third medical treatment. All surgery was preceded by a two-year period of medical treatment. Patients with chronic infection, frequent hemoptysis, recurrent pneumonia, and persistent production of foul sputum were offered surgery. In patients with bilateral disease, after the worst side was operated on first, these authors noted that nothing further was necessary in the majority of individuals. Surgical management must include meticulous anesthetic management, especially the use of selective endotracheal intubation with the Carlens tube to allow the diseased lung to be removed from the respiratory circuit during the procedure and to prevent spillage of purulent secretions into the normal lung. With proper selection and preparation of patients, careful anesthetic management, and intraoperative management principles emphasizing extirpation of disease with preservation of normal lung, surgery has much to offer in these patients.
BRONCHOPULMONARY SEQUESTRATION Bronchopulmonary sequestration is an uncommon congenital malformation of the lung wherein a portion oflung is separated from normal,
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functioning lung early in embryologic life. The arterial supply to this portion of lung is abnormal, arising in almost all patients from the aorta or a branch of the aorta somewhere between the base of the neck and the upper abdomen. 7 • 17 1fthe abnormal pulrp~mary tissue is incorporated into the normal lung and shares a common covering of visceral pleura, it is called an intralobular sequestration. If the anomalous lung has its own pleural covering, it is known as an extralobar sequestration. Aberrant blood supply to the lung was first described by Huber in 1777, and the extralobar variety of sequestration was reported independently in 1861 by Rektorzik and Rokitansky. This form was therefore known as "Rokitansky's lobe" for many years. 3 Pryce introduced the term "sequestration" to describe a condition wherein an ectopic pulmonary mass was associated with an abnormal artery .18 The disorder thus gained recognition, and much has been written regarding this entity in the intervening years.
Etiology The mechanism of formation of bronchopulmonary sequestration has been the subject of much debate and research over the years. There is general agreement that these are congenital anomalies since they have been diagnosed in infants as young as two weeks of age. 25 The various theories have been nicely summarized elsewhere, and evidence points to congenital foregut anomalies as the basis of this disorder 3 • 10• 17 and the closely related entities of lobar emphysema, bronchogenic cyst, and cystic adenomatoid malformation. 12 Gerle and coworkers proposed the term "congenital bronchopulmonary-foregut malformation" to encompass the varieties of sequestration with or without gastrointestinal tract communications. 10 These authors propose that anomalous vessels pinch off and separate a portion of the developing lung and ultimately supply the sequestered segment. The communication with primitive foregut may either persist or, more usually, will outgrow its blood supply and atrophy. Klebs in 1874 first described the communication of accessory lung with the esophagus and stomach. 10 Other congenital anomalies may accompany bronchopulmonary sequestration, particularly the extralobar variety, in up to 50 per cent of patients. These include congenital diaphragmatic hernia in approximately 30 per cent of patients, 10 pectus excavatum, congenital heart defects (ventricular septal defect, atrial septal defect, anomalous pulmonary venous drainage/ and so forth), pericardia! cyst, and congenital absence of the pericardium. More rare associated anomalies include vertebral malformations, pulmonary arteriovenous malformation, foregut duplication, achalasia, and duplication of the colon (hindgut). 17 Location and Vascular Supply Both forms of sequestration are commonly located in the lower lung fields and more usually on the left than on the right. Intralobar sequestration typically is found in the posterior basal aspect of the lower lo .... e with, roughly, a 2:1left-to-right predilection. Extralobar sequestration is most often found in the posterior mediastinum at the costophrenic sulcus or in the pleural space and also occurs about twice as often in the left
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chest. Both types may be found rarely in the upper chest, and extralobar sequestration may occur in the peritoneum or pericardium. 17 Sade et al. described the "spectrum of pulmonary sequestration" as extremely broad and variable. 20 This group pointed out that the vascular supply to an intralobar sequestration is usually from a systemic artery with venous drainage to the inferior pulmonary vein. Extralobar sequestrations are usually supplied by a systemic arterial branch from the aorta and drain into the azygous or hemiazygous system. Either type, however, can be supplied by systemic or pulmonary arteries, or both, and can have venous drainage to pulmonary or systemic veins, or both. 20 The typical patient will have blood supplied to the sequestered area by one or more branches from the aorta. The significance of this lies in the fact that the thoracic surgeon, in performing resections, may inadvertently divide one of these anomalous vessels with potentially disastrous results, as documented in the case of fatal hemorrhage during lobectomy in a five-year-old boy reported by Harris and Lewis. 13 The arterial supply usually enters the sequestrum at the posteroinferior and medial corner of the lower lobe and ascends in the pulmonary ligament. The vessels are 0.5 to 2.0 em in diameter, may be multiple in approximately 20 per cent of patients, and are supradiaphragmatic in 80 to 90 per cent of patients. 3 Pathology The sequestered segments are made up of multiple cysts of varying size. Histologically, they contain normal lung elements in an abnormal and disorderly arrangement. There may be cartilage, bronchial glands, and alveolar parenchyma present as well, and the usual cyst is lined with ciliated respiratory epithelium. With infection, fibrosis and inflammation will be present. Seventeen per cent of patients will demonstrate bronchial communication in cases of intralobar sequestration, usually due to erosion from infection in adjacent lungY The aberrant arteries show features characteristic of pulmonary arteries, being predominantly elastic rather than muscular, and roughly 75 per cent of patients, even very young patients, will demonstrate changes of atherosclerosis. 3 Symptoms Flye divided patients with sequestration into three clinical categories, those with respiratory symptoms, those with no symptoms, and those with cardiovascular symptoms. 7 The presence or absence of respiratory symptoms is determined by whether or not there is a bronchial communication to the sequestered segment. Bronchial communication is very rare in extralobar sequestration and is initially absent in intralobar cases as well. With secretion of mucus in the intralobar sequestrum, cystic swelling develops and the surrounding lung is compressed. This leads to pneumonitis, which can easily extend to the adjacent sequestrum, and with capsular erosion an airway communication is formed. Owing to the poor drainage from the sequestered segment, a cycle of recurrent episodes of fever, chills, hemoptysis, and cough with production of purulent sputum is established, often with bronchiectatic changes in the sequestrum. Symptoms typically appear in the first two decades of life, and onset is rare after the
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age of 40, though the disease may present in the seventh or eighth decade. Asymptomatic patients are those with extralobar sequestration whose disease is detected on routine chest roentgenogram (approximately 15 per cent of patients) and those with intralobar sequestration without bronchial communication. The third category of patients is small but very critical, namely, those with symptoms of heart failure due to shunting of blood through the sequestrum, that is, a left-to-right shu.nt from systemic artery to pulmonary vein. Attention was drawn to this presentation by White, who described six patients in the pediatric age group who had intralobar sequestration, two with severe congenital heart disease and four with arteriovenous shunts in the sequestrum. 25 The patients with significant shunts had continuous murmurs noted on auscultation over the ohest and axilla on the involved side. Children with arteriovenous shunts can improve dramatically following resection of the sequestrum or simple ligation of the anomalous vessel. 25 As an index of the amount of blood flowing through these anomalous channels, cases of, massive intrapleural hemorrhage and massive hemoptysis have been reported. 17 A rare symptom complex occurs in the patient who has a gastrointestinal tract communication with a sequestration. This patient may develop severe infectious problems in the sequestrum that are associated with hemoptysis or hematemesis.
Diagnosis A plain chest roentgenogram will almost always reveal some abnormality in patients with bronchopulmonary sequestration. In those with intralobar sequestration, the roentgenogram usually demonstrates a
Figure 6. Posteroanterior chest roentgenogram ·is of a patient with right lower lobe intralobar bronchopulmonary sequestration. An air-fluid level in the midst of the right lower lobe mass attests to the probable development of airway communication in this sequestrum.
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dense mass or infiltrate in the posterior basal segments ofthe lower lobe, usually on the left side. The appearance of cystic structures with or without air-fluid levels may or may not be present (Fig. 6). Patients with extralobar sequestration typically demonstrate a triangular density near the costophrenic sulcus adjacent to the mediastinum (Figs. 7 and 8). Bronchoscopy is of little benefit in these cases. Bronchograms usually reveal a normal complement of bronchi with displacement of terminal branches by the space-occupying sequestrum. It is uncommon to fill the sequestered segment even in the presence of a bronchial communication. Upper gastrointestinal tract roentgenograms with the use of con-
Figure 7. Posteroanterior and lateral chest roentgenograms and arteriogram of a fiveweek-old baby boy with extralobar bronchopulmonary sequestration. The triangular density seen in the left lower chest on plain roentgenogram is supplied by the aberrant branch of the aorta seen on the arteriogram.
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Figure 8. A and B, Medial and lateral views of the extralobar sequestration from the patient in Figure 7. The separate visceral pleural investment can be appreciated from these views.
trast material should be performed to exclude the possibility of esophageal or gastric communication. The definitive procedure is arteriography to demonstrate the aberrant arterial supply (Figs. 7 and 9). Plain roentgenograms, upper gastrointestinal tract films, and arteriography, then, are the most useful examinations to perform. The most critical element, however, is a high index of suspicion for the diagnosis of bronchopulmonary sequestration. 17 Treatment Therapy for symptomatic bronchopulmonary sequestration is resection of the involved area. Intralobar sequestration is far more common than the extralobar variety and when symptomatic should be treated by excision in a quiescent phase. The majority of patients require lobectomy because recqrrent infection destroys segmental planes and prevents segmentectomy. Segmental resection has, however, been possible in 15 to 20 per cent of cases without additional morbidity. 25 The treatment of extralobar sequestration is excision to prevent complications and to exclude other, more serious lesions. Some authors, however, advocate observation for these lesions if the diagnosis is established with certainty. 27 Since there is difficulty in differentiating these lesions preoperatively and since the intralobar type is both more common and also quite likely to become symptomatic with time, most advocate extirpation of these lesions when diagnosed. In those patients with cardiovascular symptoms secondary to repeated infection in a sequestrum in conjunction with severe congenital heart disease or in those patients with heart failure due to shunting of blood through the sequestrated segment, either resection, if appropriate,
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Figure 9. An arteriog ram of the patient in Figure 6 shows the aberrant branch of the aorta that supplies the right lower lobe sequestr ation.
or simple ligatio n of the anoma lous artery can be curativ e. 7 25 • These proced ures can be perform ed in very small infants if the sympto ms are sufficie ntly severe. Arterio graphy is very helpful . Extrem e care is necessary in perform ing pulmo nary resecti on in young patient s in order to avoid transec tion of an anoma lous vessel with potenti ally catastr ophic results . Since these aberra nt arterie s typical ly travers e the pulmo nary ligame nt, this structu re should be closely inspec ted prior to divisio n.
REFERENCES 1. Aliabadi , P., and Shafiepo or, H.: Broncho graphy in the recognit ion of congenit al cystic bronchie ctasis. Am. J. Roentgen ol., 131 :255, 1978. 2. Bradham , R. R., Sealy, W. C., and Young, W. G.: Chronic middle lobe infection . Ann. Thorac. Surg., 2:612, 1966. 3. Carter, R.: Pulmona ry sequestr ation. Ann. Thorac. Surg., 7:68, 1969. 4. Chang, K. H. R., Niguidu la, F. N., and Ramos, A.: Ka:rtage ner's triad. J. Thorae. Ca:rdiovasc. Surg., 43:127, 1962. 5. Chipps, B. E., Talamo, R. C., and Winkels tein, J. A.: lgA deficien cy, recurren t pneumo nias and bronchie ctasis, Chest, 73:419, 1978. 6. Eliasson , R., Mossber g, B., Camner, P., et al.: The immotile -cilia syndrom e. A congenit al ciliary abnorma lity as an etiologic factor in chronic airway infection s and male sterility. New Engl. J. Med., 297:1, 1977. 7. Flye, M. W., Conley, M., and Silver, D.: Spectrum of pulmona ry sequestr ation. Ann. Thorac. Surg., 22:480, 1976. 8. Fraser, R. G., and Pa:re, J. A. P.: Diagnos is of Diseases of the Chest. Philadel phia, W. B. Saunder s Compan y, 1972.
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9. George, S. A., Leonardi, H. K., and Overholt, R. A.: Bilateral pulmonary resection for bronchiectasis: A 40-year experience. Ann. Thorac. Surg., 28:48, 1979. 10. Gerle, R., Jaretzki, A., Ashley, C., et al.: Congenital bronchopulmonary-foregut malformation. Pulmonary sequestration communicating with the gastrointestinal tract. New Engl. J. Med., 278:1413, 1968. 11. Glenn, W. W. L., Liebow, A. A., and Lindskog, G. E.: Thoracic and Cardiovascular Surgery with Related Pathology. New York, Appleton-Century-Crofts, 1971. 12. Haller, J. A., Golladay, E. S., Pickard, L. R., et al.: Surgical management of lung bud anomalies: Lobar emphysema, bronchogenic cyst, cystic adenomatoid malformation and intralobular pulmonary sequestration. Ann. Thorac. Surg., 28:33, 1979. 13. Harris, H. A., and Lewis, I.: Anomalies of the lungs with special reference to the danger of abnormal vessels in lobectomy. J. Thorac. Surg., 9:666, 1940. 14. Hilton, A.M., and Doyle, L.: Immunological abnormalities in bronchiectasis with chronic bronchial suppuration. Br. J. Dis. Chest, 72:207, 1978. 15. Hinshaw, H. C., and Murray, J. F.: Diseases of the Chest, 4th ed. Philadelphia, W. B. Saunders Company, 1980. 16. Ochsner, A.: The development of pulmonary surgery, with special emphasis on carcinoma and bronchiectasis. Am. J. Surg., 135:732, 1978. 17. O'Mara, C. S., Baker, R., and Jeyasingham, K.: Pulmonary sequestration. Surg. Gynecol. Obstet., 147:609, 1978. 18. Pryce, D. M., Sellors, T. H., and Blair, L. G.: Intralobular sequestration oflung associated with an abnormal pulmonary artery. Br. J. Surg., 35:18, 1947. 19. Reid, L.: Reduction in bronchial subdivision in bronchiectasis. Thorax, 5:233, 1950. 20. Sade, R. M., Clouse, M., and Ellis, F. H., Jr.: The spectrum of pulmonary sequestration. Ann. Thorac. Surg., 18:644, 1974. 21. Sanderson, J. M., Kennedy, M. C. S., Johnson, M. F., et al.: Bronchiectasis: Results of surgical and conservative management. A review of 393 cases. Thorax, 29:407, 1974. 22. Sealy, W. C., Bradham, R. R., and Young, W. G.: The surgical treatment ofmultisegmental and localized bronchiectasis. Surg. Gynecol. Obstet., 123:80, 1966. 23. Takaro, T.: Lung infections and interstitial pneumonopathies. In Sabiston, D. C., Jr. and Spencer, F. C. (eds) Gibbon's Surgery of the Chest. Philadelphia, W. B. Saunders Company, 1976. 24. Varpela, E., Koistinen, J., Korhola, 0., et al.: Deficiency of alpha-1 antitrypsin and bronchiectasis. Ann. Clin. Res., 10:79, 1978. 25. White, J. J., Donahoo, J, S., Ostrow, P. T., et al.: Cardiovascular and respiratory manifestations of pulmonary sequestration in childhood. Ann. Thorac. Surg., 18:286, 1974. 26. Wolfe, W. G., Anderson, R. W., and Sealy, W. C.: Hyperlucent lung. Pathophysiology and surgical management. Ann. Thorac. Surg., 18:172, 1974. 27. Zumbro, G. L., Treasure, R. L., Seitter, G., et al.: Pulmonary sequestration: A broad spectrum of bronchopulmonary foregut abnormalities. Ann. Thorac. Surg., 20:161, 1975.
R. Morton Bolman, III, M.D. Department of Surgery Box 3507 Duke University Medical Center Durham, North Carolina 27710
Bronchiectasis and Bronchopulmonary Sequestration
R. Morton BoZman, III, M.D.,* and Walter G. Wolfe, M.D.t
BRONCHIECTASIS ·Bronchiectasis is one of the group of suppurative diseases of the lungs characterized by dilatation of the bronchi. This affliction, which was first described in 1819 by Laennec, was an important clinical entity, particularly in the years preceding the availability of antibiotics. Although its overall incidence has declined significantly as a result of effective antimicrobial therapy, bronchiectasis remains an entity with which the thoracic surgeon should be familiar.
Etiology The development of bronchiectasis is a multifactorial phenomenon. Although acquired infection of the lung is in all likelihood the major contributor, congenital factors may also play a role (Table 1). Congenital cystic bronchiectasis, an example of which is shown in Figure 1, is a disorder of infants and young children. This entity is thought to arise from an arrest in development of the bronchial tree. 1 Various immune deficiency states have been found to be associated with the formation of bronchiectasis in recent years. Patients with selective immunoglobulin A (IgA) deficiency or with primary or acquired hypogammaglobulinemia have been found to be susceptible to the development of bronchiectasis. 5 • 14 Alpharantitrypsin deficiency, though usually associated with panacinar emphysema, occasionally will be discovered as a contributing factor in cases of bronchiectasis. V arpela studied 60 patients with bronchographically proven bronchiectasis and found six (10 per cent) with the genotype Pimz who possessed less than 66 per cent of the normal level of alpha 1-antitrypsin. This substance is a very potent protease inhibitor, and patients with diminished levels would appear to be susceptible to airway damage from otherwise relatively benign infections. The disease seen in these patients with alpharantitrypsin *Chief Resident in Surgery, Duke University Medical Center, Durham, North Carolina. tProfessor of Surgery, Duke University Medical Center, Durham, North Carolina
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Table 1. Causative Factors of Importance in Development of Bronchiectasis Congenital Congenital cystic bronchiectasis Selective IgA deficiency Primary hypogammaglobulinemia Alpha,-antitrypsin deficiency Cystic fibrosis Congenital deficiency of bronchial cartilage Kartagener's syndrome: situs inversus, sinusitis, and bronchiectasis Bronchopulmonary sequestration Acquired Infection-bacterial, viral Bronchial obstruction Intrinsic: neoplasm, foreign body, mucus plug Extrinsic: enlarged lymph nodes "Middle lobe syndrome" Scarring secondary to tuberculosis Acquired hypogammaglobulinemia
deficiency is extensive, and it appears that deficiency of this inhibitor of proteolytic enzyme activity predisposes to development of bronchiectasis.24 As mentioned earlier, infection is felt to be critical to the development of bronchiectasis. The history frequently dates to the childhood years, with pneumonia developing as a sequel to measles, pertussis, or influenza. This association, though less prominent now, is nonetheless still observed. Bacterial pneumonia, with destruction of alveoli and bronchial walls, leads to scarring, and the resultant shrinkage of surrounding lung caused by scar contracture places traction on the bronchi, leading to bronchiectasis. 8 • 11 Other etiologic agents of importance include intrinsic bronchial obstruction from a foreign body, neoplasm, or mucus plug; extrinsic bronchial obstruction from enlarged hilar nodes; and bronchial dilatation following healing of tuberculous lesions or as a residual effect of chronic lung abscess. 8 • 23 Bradham described a series of 23 patients with so-called "middle lobe syndrome" and suggested that the presence or absence of complete fissures between the right upper and right middle lobes might have a bearing on the development of chronic infection and bronchiectasis. Patients with incomplete fissures would have improved collateral ventilation, allowing aeration of the pulmonary tissue, whereas those with complete fissures would not possess this alternative route and therefore would be predisposed to atelectasis and bronchiectasis in the middle lobe. Eleven of these 23 patient with bronchiectasis had complete fissures, and 8 of 11 had no other apparent etiology for their bronchiectasis. 2 Finally, a small number of cases can be ascribed to a congenital defect of a structural nature. Patients with cystic fibrosis and congenital deficiency of bronchial cartilage fall into this category. Also, there is an
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C.n·r:r!'·r:r Figure 1. A and B, Posteroanterior and lateral chest roentgenograms of a five-year-old boy with congenital cystic bronchiectasis demonstrate a "honeycomb" pattern of bronchiectasis with rarefaction in the left upper lobe. The patient had sustained an episode of necrotizing pneumonia at the age of two years. C, Marked cystic and saccular changes are noted throughout the left lung of this patient with very little uninvolved parenchyma.
association between situs inversus, sinusitis, and bronchiectasis. Together these disorders constitute Kartagener's syndrome, 4 in which there has been identified a congenital defect in respiratory cilia. 6
Pathology Reid divided bronchiectasis into three groupings: (1) cylindrical, with dilated bronchi of regular outline; (2) varicose, with greater dilatation, irregularity in outline, and absent peripheral filling; and (3) saccular or cystic, with bronchial dilatation increasing toward the periphery, balloon outline of the bronchi, and no communication with the peripheral bronchial tree. 19 The bronchiectatic changes typically involve
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the second to fourth order bronchi, and the distribution in the lungs corresponds to common sites of occurrence of necrotizing pneumonia, that is, the basal segments of the lower lobes. The superior segments are usually spared. 11 Figure 2 shows a representative pathologic specimen from a patient with cystic bronchiectasis of the lower lung. In patients with immune deficiency states, the disease may affect any or all pulmonary segments and may or may not be localized. Disease is bilateral in approximately 30 per cent of patients.
Clinical Presentation Typically, the patient with bronchiectasis presents a clinical picture characterized by recurrent cough productive of purulent sputum, which layers on standing; fever; and hemoptysis. Hemoptysis occurs in roughly 50 per cent of adult patients and rarely in pediatric patients. In 10 per cent it is severe, though rarely lethal. The hemoptysis is thought to be due to bleeding from anastomoses between hypertrophied bronchial arteries and the pulmonary circulation in diseased segments. 26 Other symptoms, though less common, may prove troublesome, These include fetor oris, which is produced by fetid sputum and which in years past constituted a major social and psychological problem but is now less common because of antibiotic therapy; anorexia; pleurisy; and arthralgias. In addition, these patients can manifest the findings of hyper-
Figure 2. Resection was performed on the left lung of a patient with severe cystic bronchiectasis of the lower lobe and the lingular segment of the upper lobe. Note multiple cysts of various sizes with destruction of intervening pulmonary parenchyma.
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trophic pulmonary osteoarthropathy, namely, clubbing of fingers, joint swelling, and shin tenderness. Interestingly, these symptoms abate following surgical extirpation of diseased lung. Physical examination reveals the signs of chronic illness, and occasionally the stigmata of pulmonary osteoarthropathy will be seen. Auscultatory findings are characterized by variation from day to day but commonly include diminished breath sounds over the lower chest and the presence of wet rales in the area of the involved lung.
Diagnosis A history of chronic cough, recurrent pneumonic infection with suppuration, fever, and often hemoptysis will strongly suggest the diagnosis in most instances. The plain chest roentgenogram is also useful and may reveal evidence of the underlying pathology. Such evidence includes increased markings in diseased segments, volume loss, and cystic spaces. In patients with severe cases, a "honeycomb" pattern may also be seen and is caused by rarefied areas of destroyed lung and compensatory overinflation of the remaining normal lung (Fig. 1). 8 Bronchography in three dimensions is the mainstay in the evaluation of bronchiectasis and must be performed to obtain a definitive diagnosis. This study should be done when the patient is in optimal condition, that is, after postural drainage and antibiotics have controlled any acute exacerbations and have decreased secretions to a minimum. Each lung should be studied separately. There is a reversible condition very much resembling bronchiectasis that occurs in the wake of an acute pneumonia. For this reason bronchography should be postponed for four to six weeks after an acute infectious episode. Figures 3 through 5 demonstrate the roentgenographic and bronchographic findings in two patients with bronchiectasis. Pulmonary function tests show no specific pattern in patients with bronchiectasis but should be performed to assess the tolerance of the individual patient for lung resection. Bronchoscopy is very helpful and should be performed to rule out endobronchial disease or bronchial obstruction caused by a foreign body or a neoplasm and to obtain good material for culture. In addition, the segmental anatomy can be studied and the procedure may prove to be therapeutic by providing good tracheobronchial toilet. Treatment
The initial treatment of bronchiectasis is always conservative, consisting of medical measures to control superimposed bronchitis or pneumonia. A long trial of medical therapy may be appropriate for determination of the need for surgery. 11 Antibiotics, the choice of which is based on culture and sensitivity data, should be continued until production of sputum is minimal. An aggressive regimen of postural drainage and chest physiotherapy should be combined with chemotherapy. The avoidance of tobacco and polluted air should be strongly encouraged, and all patients should receive pneumococcal vaccine and yearly influenza vaccine. 15
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Figure 3. UppeT TOw: posteroanterior and lateral chest roentgenograms and a lung scan in a patient with chronic saccular bronchiectasis of the left lower lobe. Note the volume loss and the cystic changes in the left lower lobe on the chest roentgenogram as well as the lack of perfusion on the scan seen at the far right. LoweT TOw: posteroanterior and lateral bronchograms in this patient reveal saccular dilatation of the bronchi of the left lower lobe and the lingular segment of the left upper lobe.
Figure 4. Posteroanterior chest roentgenogram of a patient with bronchiectasis of the right lower lobe. Note the increased markings in the right lower medial lung zone.
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Figure 5. Posteroanterior (A) and lateral (B) bronchographic views of the patient shown in Figure 4. This patient has localized saccular bronchiectasis of the right lower lobe, as seen in these bronchograms, with cystic dilatation of third and fourth order bronchi.
This regimen will suffice in the majority of patients with bronchiectasis. In patients in whom medical management fails after an adequate trial and in whom symptoms persist, with sputum production, recurrent pneumonitis, or frequent hemoptysis, surgical therapy should be considered. This is especially true in instances in which the disease is localized to one area or one segment of a lung. Surgical treatment is less feasible in patients in whom the disease is diffuse and multisegmental. Surgery for bronchiectasis dates to the late 1800s. Heuer in 1934 reported on 56 cases treated by resection between 1880 and 1920 and reported a 50 per cent mortality rate. The mortality remained high until the 1930s when Churchill reported on 84 cases of bronchiectasis treated by lobectomy with a mortality rate of only 4.6 per cent. Today, mortality following these resections is less than 1 per cent. Kent and Blades in 1942 demonstrated the technique of lobectomy with individual ligation of hilar structures. They were also among the earliest to utilize the technique of segmental resection in order to preserve normal lung tissue.16 In patients in whom surgery is being entertained as a therapeutic alternative, it is essential to have good quality three-dimensional bronchograms to fully outline the extent of disease. The ideal candidate has localized disease, preferably in one lobe or in contiguous segments of two lobes. 11 Sealy reported the experience at Duke University Medical Center in 140 patients with bronchiectasis, 70 with localized disease and 70 with diffuse disease. 22 In the group with localized disease, the onset could be traced to a single initiating event, such as pneumonia or aspiration of a foreign body, whereas in the group with diffuse involvement, an immune deficit was more likely to be the primary etiologic factor. The
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patients in this series were operated on after an intensive period of medical preparation, and the emphasis at surgery was on preservation of uninvolved lung by careful segmental resection. Mortality was less than 1 per cent and follow-up showed 95 per cent of patients with good-toimproved results. In general, the patients with localized disease had better results after surgery, and the incidence of recurrent disease was quite low. The ongoing immune deficiency status in the multisegmented group prevented their being uniformly asymptomatic after surgery. Overall, however, 36 per cent in this group were "cured," and an additional 53 per cent were improved and able to resume normal activity. In contrast, 80 per cent of the group with localized disease were symptom free and an additional 10 per cent were improved. 22 As alluded to in this series, and confirmed by George, bilateral disease need not be a contraindication to surgery. 9 George et al. reported on 99 patients undergoing 216 resections with an operative mortality of 1.4 per cent. The foci were mostly in dependent areas of the lungs, namely, the right middle lobe, the lingula of the left upper lobe, and basal segments of the lower lobes. The principle advanced by these authors is to attack the side with the most severe disease first and to stage the opposite side, if necessary, two to three months later. Also, preservation of normal pulmonary parenchyma becomes particularly critical in this group of patients, who constitute approximately 30 per cent of all patients with bronchiectasis. Five of the patients in this report developed cor pulmonale 5 to 20 years after resection. The mean number of segments removed in this group was 11, significantly greater than the average for the series. Overall, 83 of 90 patients showed improvement, 9 showed no change, and 4 actually had progression of disease after surgery. 9 Sanderson reviewed 393 patients treated for bronchiectasis at one institution over a 15-year period. 21 Roughly, two thirds received surgical treatment and one third medical treatment. All surgery was preceded by a two-year period of medical treatment. Patients with chronic infection, frequent hemoptysis, recurrent pneumonia, and persistent production of foul sputum were offered surgery. In patients with bilateral disease, after the worst side was operated on first, these authors noted that nothing further was necessary in the majority of individuals. Surgical management must include meticulous anesthetic management, especially the use of selective endotracheal intubation with the Carlens tube to allow the diseased lung to be removed from the respiratory circuit during the procedure and to prevent spillage of purulent secretions into the normal lung. With proper selection and preparation of patients, careful anesthetic management, and intraoperative management principles emphasizing extirpation of disease with preservation of normal lung, surgery has much to offer in these patients.
BRONCHOPULMONARY SEQUESTRATION Bronchopulmonary sequestration is an uncommon congenital malformation of the lung wherein a portion oflung is separated from normal,
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functioning lung early in embryologic life. The arterial supply to this portion of lung is abnormal, arising in almost all patients from the aorta or a branch of the aorta somewhere between the base of the neck and the upper abdomen. 7 • 17 1fthe abnormal pulrp~mary tissue is incorporated into the normal lung and shares a common covering of visceral pleura, it is called an intralobular sequestration. If the anomalous lung has its own pleural covering, it is known as an extralobar sequestration. Aberrant blood supply to the lung was first described by Huber in 1777, and the extralobar variety of sequestration was reported independently in 1861 by Rektorzik and Rokitansky. This form was therefore known as "Rokitansky's lobe" for many years. 3 Pryce introduced the term "sequestration" to describe a condition wherein an ectopic pulmonary mass was associated with an abnormal artery .18 The disorder thus gained recognition, and much has been written regarding this entity in the intervening years.
Etiology The mechanism of formation of bronchopulmonary sequestration has been the subject of much debate and research over the years. There is general agreement that these are congenital anomalies since they have been diagnosed in infants as young as two weeks of age. 25 The various theories have been nicely summarized elsewhere, and evidence points to congenital foregut anomalies as the basis of this disorder 3 • 10• 17 and the closely related entities of lobar emphysema, bronchogenic cyst, and cystic adenomatoid malformation. 12 Gerle and coworkers proposed the term "congenital bronchopulmonary-foregut malformation" to encompass the varieties of sequestration with or without gastrointestinal tract communications. 10 These authors propose that anomalous vessels pinch off and separate a portion of the developing lung and ultimately supply the sequestered segment. The communication with primitive foregut may either persist or, more usually, will outgrow its blood supply and atrophy. Klebs in 1874 first described the communication of accessory lung with the esophagus and stomach. 10 Other congenital anomalies may accompany bronchopulmonary sequestration, particularly the extralobar variety, in up to 50 per cent of patients. These include congenital diaphragmatic hernia in approximately 30 per cent of patients, 10 pectus excavatum, congenital heart defects (ventricular septal defect, atrial septal defect, anomalous pulmonary venous drainage/ and so forth), pericardia! cyst, and congenital absence of the pericardium. More rare associated anomalies include vertebral malformations, pulmonary arteriovenous malformation, foregut duplication, achalasia, and duplication of the colon (hindgut). 17 Location and Vascular Supply Both forms of sequestration are commonly located in the lower lung fields and more usually on the left than on the right. Intralobar sequestration typically is found in the posterior basal aspect of the lower lo .... e with, roughly, a 2:1left-to-right predilection. Extralobar sequestration is most often found in the posterior mediastinum at the costophrenic sulcus or in the pleural space and also occurs about twice as often in the left
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chest. Both types may be found rarely in the upper chest, and extralobar sequestration may occur in the peritoneum or pericardium. 17 Sade et al. described the "spectrum of pulmonary sequestration" as extremely broad and variable. 20 This group pointed out that the vascular supply to an intralobar sequestration is usually from a systemic artery with venous drainage to the inferior pulmonary vein. Extralobar sequestrations are usually supplied by a systemic arterial branch from the aorta and drain into the azygous or hemiazygous system. Either type, however, can be supplied by systemic or pulmonary arteries, or both, and can have venous drainage to pulmonary or systemic veins, or both. 20 The typical patient will have blood supplied to the sequestered area by one or more branches from the aorta. The significance of this lies in the fact that the thoracic surgeon, in performing resections, may inadvertently divide one of these anomalous vessels with potentially disastrous results, as documented in the case of fatal hemorrhage during lobectomy in a five-year-old boy reported by Harris and Lewis. 13 The arterial supply usually enters the sequestrum at the posteroinferior and medial corner of the lower lobe and ascends in the pulmonary ligament. The vessels are 0.5 to 2.0 em in diameter, may be multiple in approximately 20 per cent of patients, and are supradiaphragmatic in 80 to 90 per cent of patients. 3 Pathology The sequestered segments are made up of multiple cysts of varying size. Histologically, they contain normal lung elements in an abnormal and disorderly arrangement. There may be cartilage, bronchial glands, and alveolar parenchyma present as well, and the usual cyst is lined with ciliated respiratory epithelium. With infection, fibrosis and inflammation will be present. Seventeen per cent of patients will demonstrate bronchial communication in cases of intralobar sequestration, usually due to erosion from infection in adjacent lungY The aberrant arteries show features characteristic of pulmonary arteries, being predominantly elastic rather than muscular, and roughly 75 per cent of patients, even very young patients, will demonstrate changes of atherosclerosis. 3 Symptoms Flye divided patients with sequestration into three clinical categories, those with respiratory symptoms, those with no symptoms, and those with cardiovascular symptoms. 7 The presence or absence of respiratory symptoms is determined by whether or not there is a bronchial communication to the sequestered segment. Bronchial communication is very rare in extralobar sequestration and is initially absent in intralobar cases as well. With secretion of mucus in the intralobar sequestrum, cystic swelling develops and the surrounding lung is compressed. This leads to pneumonitis, which can easily extend to the adjacent sequestrum, and with capsular erosion an airway communication is formed. Owing to the poor drainage from the sequestered segment, a cycle of recurrent episodes of fever, chills, hemoptysis, and cough with production of purulent sputum is established, often with bronchiectatic changes in the sequestrum. Symptoms typically appear in the first two decades of life, and onset is rare after the
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age of 40, though the disease may present in the seventh or eighth decade. Asymptomatic patients are those with extralobar sequestration whose disease is detected on routine chest roentgenogram (approximately 15 per cent of patients) and those with intralobar sequestration without bronchial communication. The third category of patients is small but very critical, namely, those with symptoms of heart failure due to shunting of blood through the sequestrum, that is, a left-to-right shu.nt from systemic artery to pulmonary vein. Attention was drawn to this presentation by White, who described six patients in the pediatric age group who had intralobar sequestration, two with severe congenital heart disease and four with arteriovenous shunts in the sequestrum. 25 The patients with significant shunts had continuous murmurs noted on auscultation over the ohest and axilla on the involved side. Children with arteriovenous shunts can improve dramatically following resection of the sequestrum or simple ligation of the anomalous vessel. 25 As an index of the amount of blood flowing through these anomalous channels, cases of, massive intrapleural hemorrhage and massive hemoptysis have been reported. 17 A rare symptom complex occurs in the patient who has a gastrointestinal tract communication with a sequestration. This patient may develop severe infectious problems in the sequestrum that are associated with hemoptysis or hematemesis.
Diagnosis A plain chest roentgenogram will almost always reveal some abnormality in patients with bronchopulmonary sequestration. In those with intralobar sequestration, the roentgenogram usually demonstrates a
Figure 6. Posteroanterior chest roentgenogram ·is of a patient with right lower lobe intralobar bronchopulmonary sequestration. An air-fluid level in the midst of the right lower lobe mass attests to the probable development of airway communication in this sequestrum.
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dense mass or infiltrate in the posterior basal segments ofthe lower lobe, usually on the left side. The appearance of cystic structures with or without air-fluid levels may or may not be present (Fig. 6). Patients with extralobar sequestration typically demonstrate a triangular density near the costophrenic sulcus adjacent to the mediastinum (Figs. 7 and 8). Bronchoscopy is of little benefit in these cases. Bronchograms usually reveal a normal complement of bronchi with displacement of terminal branches by the space-occupying sequestrum. It is uncommon to fill the sequestered segment even in the presence of a bronchial communication. Upper gastrointestinal tract roentgenograms with the use of con-
Figure 7. Posteroanterior and lateral chest roentgenograms and arteriogram of a fiveweek-old baby boy with extralobar bronchopulmonary sequestration. The triangular density seen in the left lower chest on plain roentgenogram is supplied by the aberrant branch of the aorta seen on the arteriogram.
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Figure 8. A and B, Medial and lateral views of the extralobar sequestration from the patient in Figure 7. The separate visceral pleural investment can be appreciated from these views.
trast material should be performed to exclude the possibility of esophageal or gastric communication. The definitive procedure is arteriography to demonstrate the aberrant arterial supply (Figs. 7 and 9). Plain roentgenograms, upper gastrointestinal tract films, and arteriography, then, are the most useful examinations to perform. The most critical element, however, is a high index of suspicion for the diagnosis of bronchopulmonary sequestration. 17 Treatment Therapy for symptomatic bronchopulmonary sequestration is resection of the involved area. Intralobar sequestration is far more common than the extralobar variety and when symptomatic should be treated by excision in a quiescent phase. The majority of patients require lobectomy because recqrrent infection destroys segmental planes and prevents segmentectomy. Segmental resection has, however, been possible in 15 to 20 per cent of cases without additional morbidity. 25 The treatment of extralobar sequestration is excision to prevent complications and to exclude other, more serious lesions. Some authors, however, advocate observation for these lesions if the diagnosis is established with certainty. 27 Since there is difficulty in differentiating these lesions preoperatively and since the intralobar type is both more common and also quite likely to become symptomatic with time, most advocate extirpation of these lesions when diagnosed. In those patients with cardiovascular symptoms secondary to repeated infection in a sequestrum in conjunction with severe congenital heart disease or in those patients with heart failure due to shunting of blood through the sequestrated segment, either resection, if appropriate,
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Figure 9. An arteriog ram of the patient in Figure 6 shows the aberrant branch of the aorta that supplies the right lower lobe sequestr ation.
or simple ligatio n of the anoma lous artery can be curativ e. 7 25 • These proced ures can be perform ed in very small infants if the sympto ms are sufficie ntly severe. Arterio graphy is very helpful . Extrem e care is necessary in perform ing pulmo nary resecti on in young patient s in order to avoid transec tion of an anoma lous vessel with potenti ally catastr ophic results . Since these aberra nt arterie s typical ly travers e the pulmo nary ligame nt, this structu re should be closely inspec ted prior to divisio n.
REFERENCES 1. Aliabadi , P., and Shafiepo or, H.: Broncho graphy in the recognit ion of congenit al cystic bronchie ctasis. Am. J. Roentgen ol., 131 :255, 1978. 2. Bradham , R. R., Sealy, W. C., and Young, W. G.: Chronic middle lobe infection . Ann. Thorac. Surg., 2:612, 1966. 3. Carter, R.: Pulmona ry sequestr ation. Ann. Thorac. Surg., 7:68, 1969. 4. Chang, K. H. R., Niguidu la, F. N., and Ramos, A.: Ka:rtage ner's triad. J. Thorae. Ca:rdiovasc. Surg., 43:127, 1962. 5. Chipps, B. E., Talamo, R. C., and Winkels tein, J. A.: lgA deficien cy, recurren t pneumo nias and bronchie ctasis, Chest, 73:419, 1978. 6. Eliasson , R., Mossber g, B., Camner, P., et al.: The immotile -cilia syndrom e. A congenit al ciliary abnorma lity as an etiologic factor in chronic airway infection s and male sterility. New Engl. J. Med., 297:1, 1977. 7. Flye, M. W., Conley, M., and Silver, D.: Spectrum of pulmona ry sequestr ation. Ann. Thorac. Surg., 22:480, 1976. 8. Fraser, R. G., and Pa:re, J. A. P.: Diagnos is of Diseases of the Chest. Philadel phia, W. B. Saunder s Compan y, 1972.
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9. George, S. A., Leonardi, H. K., and Overholt, R. A.: Bilateral pulmonary resection for bronchiectasis: A 40-year experience. Ann. Thorac. Surg., 28:48, 1979. 10. Gerle, R., Jaretzki, A., Ashley, C., et al.: Congenital bronchopulmonary-foregut malformation. Pulmonary sequestration communicating with the gastrointestinal tract. New Engl. J. Med., 278:1413, 1968. 11. Glenn, W. W. L., Liebow, A. A., and Lindskog, G. E.: Thoracic and Cardiovascular Surgery with Related Pathology. New York, Appleton-Century-Crofts, 1971. 12. Haller, J. A., Golladay, E. S., Pickard, L. R., et al.: Surgical management of lung bud anomalies: Lobar emphysema, bronchogenic cyst, cystic adenomatoid malformation and intralobular pulmonary sequestration. Ann. Thorac. Surg., 28:33, 1979. 13. Harris, H. A., and Lewis, I.: Anomalies of the lungs with special reference to the danger of abnormal vessels in lobectomy. J. Thorac. Surg., 9:666, 1940. 14. Hilton, A.M., and Doyle, L.: Immunological abnormalities in bronchiectasis with chronic bronchial suppuration. Br. J. Dis. Chest, 72:207, 1978. 15. Hinshaw, H. C., and Murray, J. F.: Diseases of the Chest, 4th ed. Philadelphia, W. B. Saunders Company, 1980. 16. Ochsner, A.: The development of pulmonary surgery, with special emphasis on carcinoma and bronchiectasis. Am. J. Surg., 135:732, 1978. 17. O'Mara, C. S., Baker, R., and Jeyasingham, K.: Pulmonary sequestration. Surg. Gynecol. Obstet., 147:609, 1978. 18. Pryce, D. M., Sellors, T. H., and Blair, L. G.: Intralobular sequestration oflung associated with an abnormal pulmonary artery. Br. J. Surg., 35:18, 1947. 19. Reid, L.: Reduction in bronchial subdivision in bronchiectasis. Thorax, 5:233, 1950. 20. Sade, R. M., Clouse, M., and Ellis, F. H., Jr.: The spectrum of pulmonary sequestration. Ann. Thorac. Surg., 18:644, 1974. 21. Sanderson, J. M., Kennedy, M. C. S., Johnson, M. F., et al.: Bronchiectasis: Results of surgical and conservative management. A review of 393 cases. Thorax, 29:407, 1974. 22. Sealy, W. C., Bradham, R. R., and Young, W. G.: The surgical treatment ofmultisegmental and localized bronchiectasis. Surg. Gynecol. Obstet., 123:80, 1966. 23. Takaro, T.: Lung infections and interstitial pneumonopathies. In Sabiston, D. C., Jr. and Spencer, F. C. (eds) Gibbon's Surgery of the Chest. Philadelphia, W. B. Saunders Company, 1976. 24. Varpela, E., Koistinen, J., Korhola, 0., et al.: Deficiency of alpha-1 antitrypsin and bronchiectasis. Ann. Clin. Res., 10:79, 1978. 25. White, J. J., Donahoo, J, S., Ostrow, P. T., et al.: Cardiovascular and respiratory manifestations of pulmonary sequestration in childhood. Ann. Thorac. Surg., 18:286, 1974. 26. Wolfe, W. G., Anderson, R. W., and Sealy, W. C.: Hyperlucent lung. Pathophysiology and surgical management. Ann. Thorac. Surg., 18:172, 1974. 27. Zumbro, G. L., Treasure, R. L., Seitter, G., et al.: Pulmonary sequestration: A broad spectrum of bronchopulmonary foregut abnormalities. Ann. Thorac. Surg., 20:161, 1975.
R. Morton Bolman, III, M.D. Department of Surgery Box 3507 Duke University Medical Center Durham, North Carolina 27710