Bronchiectasis was first described by Laennec (Paris, France) in the 1800 s as a bronchial dilatation. This definition is still valid, but it must be added that it is always associated with an alteration of the structural layers of the bronchial wall. The surgical management of bronchiectasis is presented in Chapter 94.
Various congenital and acquired disease processes can lead to bronchiectasis. During pulmonary infections, obstructive endobronchial processes localized to the peripheral lung may cause bronchial dilatation. Repeated infections damage the epithelial cilia, mucoelastic tissue, and even cartilage. Healing and replacement of these tissues with fibrous tissue results in loss of elasticity with contraction of the peribronchial tissues and traction on the bronchial tree leading to bronchial dilatation.15 True bronchiectasis is distinct from pseudobronchiectasis (bronchocele). This latter form of bronchial dilatation is reversible and lasts only a few weeks or months after an episode of bronchopneumonia. The epithelial damage is not severe and is without necrosis.
Sometimes bronchiectasis is caused by bronchial obstruction. The obstruction is created by tiny aspirated endoluminal foreign bodies,16 benign lymphoadenomatoid-bronchial syndromes, or slow-growth tumorlets such as carcinoids.17
Bronchial obstruction also can be caused by extrinsic compression from the peribronchial lymph nodes (middle lobe or lingula) in the case of neoplasms or by chronic infections (e.g., histoplasmosis, coccidioidomycosis, aspergillosis, tuberculosis, and AIDS-related infections); see Chapters 102 and 103.18 Three steps are implicated in the pathophysiology of extrinsic obstruction, namely, secretion retention, chronic infection, and bronchiectasis, as seen before.
Congenital and familial diseases are an uncommon cause of bronchiectasis (Table 90-1). The most important is Kartagener syndrome, or primary ciliary dyskinesia, a rare autosomal recessive disorder involving the combination of situs inversus, bronchiectasis, and sinusitis. A dynein deficiency leads to ciliary dyskinesia. The yellow nail syndrome is a rare clinical entity that combines three main features: yellow discoloration of the nails, chronic lymphedema, and pleural effusion. This syndrome is often complicated by bronchiectasis.19 The classification of bronchiectasis is morphologic, consisting of three types: (1) cylindrical or tubular, (2) saccular or cystic, and (3) varicose. Cylindrical bronchiectasis has a uniform caliber of dilatation and often is associated with tuberculosis. Saccular bronchiectasis is characterized by an evident dilatation at the distal end, often associated with postinfectious and postobstructive bronchiectasis. Varicose bronchiectasis is a mixed form and presents with alternating areas of saccular and cylindrical dilatation. The subclassification of bronchiectasis is based on blood perfusion. Two types of bronchiectasis are recognized: perfused and nonperfused. Whereas perfused bronchiectasis has intact pulmonary artery flow and cylindrical bronchiectatic changes, the nonperfused type is characterized by a lack of pulmonary artery flow, retrograde filling of the pulmonary artery through the systemic circulation, and cystic bronchiectatic changes.
Table 90-1Congenital and Familial Diseases That Cause Bronchiectasis ||Download (.pdf) Table 90-1Congenital and Familial Diseases That Cause Bronchiectasis
|ETIOLOGY ||SYNDROMES AND DISEASES |
|Bronchial malformations ||Primitive tracheomalacia |
|Vascular and lymphatic malformations ||Sequestration |
Yellow nails syndrome
|Chest malformations ||Kyphoscoliosis |
|Systemic diseases ||Cystic fibrosis |
|Bronchiectasis in other complex syndromes ||Kartagener syndrome (primary ciliary dyskinesia) |
The location of the bronchiectasis may reveal its pathogenesis. Bronchiectasis related to congenital and familial diseases is always bilateral and involves different segments of the lung. Bronchiectasis related to tuberculosis is located primarily in the upper lobes. Obstructive and inflammatory bronchiectasis is found in the lower lobes. The middle lobe syndrome is a selective localized form of bronchiectasis. The middle lobe bronchus arises with a 30-degree downward open angle from the intermediate bronchus and runs for approximately 2 cm. Lymph node hypertrophy of the angle can easily compress the middle bronchus, leading to atelectasis and bronchiectasis.20
Bronchiectasis may be symptomatic or asymptomatic.21 Bronchiectasis should be considered in all adults who have persistent productive cough (>90% of patients).
Factors favoring further investigation are any one of the following:
Young age at presentation
History of symptoms over many years
Absence of smoking history
Daily expectoration of sputum (the volume of sputum produced may vary widely with mean/median daily volumes of 300, 567, and 200 mL)
Sputum colonization with Pseudomonas aeruginosa
Nonproductive cough or unexplained hemoptysis. This latter symptom, even if rare, may occur and has been reported as a symptom of the type of bronchiectasis associated with coronary fistula.22
Physical examination of the chest may reveal coarse expiratory rhonchi and dullness over localized areas, especially during periods of acute infectious exacerbation.
Standard chest radiography is often not useful because it shows only nonspecific inflammatory radiographic signs (Fig. 90-3). In the past, the ideal radiographic method was, and in some cases still is, a bronchogram. This procedure provides information about the morphology, localization, and extent of the bronchiectasis (Fig. 90-4). High-resolution, fine-cut CT scan is the procedure of choice for the diagnosis of bronchiectasis.21
Chest radiograph showing bronchiectasis.
Bronchograms of bronchiectasis of the lower lobe.
CT is able to show the extent of the disease bilaterally and gives information about the walls of the bronchioles, including the presence of peribronchial inflammation and parenchymal disease (Fig. 90-5).
High-resolution spiral CT scan of diffuse cylindrical bronchiectasis. (Courtesy of Dr. Nunzio Calia.)
Flexible fiberoptic bronchoscopy is useful for evaluating bronchial neoplasms and endoluminal foreign bodies or obtaining samples for bacterial culture to start specific antibiotic therapy. Bronchoscopy permits biopsy of the respiratory mucosa for the diagnosis of hereditary mucociliary disorders.
The treatment aims in adult care are to control symptoms and thus enhance quality of life, reduce exacerbations, and maintain pulmonary function. The evidence is clear that patients with bronchiectasis who have more frequent exacerbations have a worse quality of life.21
Postural drainage is the most effective treatment for mobilizing the thick bronchial secretions but it requires the skills of a trained respiratory physiotherapist. To treat infections, a sputum culture should be performed. Targeted antibiotic therapy is started if an acute infection of the lung is present or for a preoperative workup. Patients with primary or secondary immune deficiency should be under joint care with a clinical immunologist.21
The conservative therapeutic approach should be continued for several months. Annual vaccination against influenza and pneumococcus is recommended.
Indications for surgery reported in the literature have included failure of medical/conservative therapy, recurrent respiratory infections, persistent sputum production, hemoptysis, chronic cough, and persistent lung abscess.21,23–26
Complete excision of the bronchiectatic parenchyma is the goal of surgery. Patients considered candidates for surgical resection must meet the following criteria: (1) localized bronchiectasis that is completely resectable, (2) adequate respiratory reserve, (3) irreversible process versus an early treatable condition, (4) significant symptoms with a continued chronic productive cough, significant hemoptysis, major recurring episodes of pneumonia sufficiently severe to warrant surgery, and (5) failed prolonged attempts at medical therapy.27
For massive pulmonary hemorrhage, endobronchial suction with balloon tamponade, embolization of the bleeding vessel (usually a bronchial artery), or emergency resection can be lifesaving. The surgical approach involves anatomic resection of all targeted areas. The anatomic resection depends on the origin of the bronchiectasis. Tuberculous bronchiectasis typically requires resection of the upper lobes, whereas postinfective bronchiectasis usually involves resection of the lower lobes or lingula (Fig. 90-6). Abundant mucopurulent secretions must be treated with particular attention to prevent contamination of the normal airways. Lung transplantation is available for end-stage cardiopulmonary disease in children and adults, although there is a paucity of literature on bronchiectasis specifically. As a general guideline, patients are evaluated for lung transplantation if the FEV1 is <30% or if there is rapid, progressive respiratory deterioration despite optimal medical management. The following additional factors should lower the threshold for considering referral for transplantation assessment: massive hemoptysis, severe secondary pulmonary hypertension, multiple ICU admissions, or respiratory failure (particularly if requiring noninvasive ventilation). It should be noted that antibody deficiency is not an absolute contraindication to transplantation.21,28–29
Resected lingular lobe with bronchiectasis.
Surgical treatment of bronchiectasis is more effective in patients with localized disease. Excision in such patients is satisfactory with an acceptable rate of morbidity and mortality. Mortality and morbidity rates range from 1.7% to 3.5% in different case series (Table 90-2).
Table 90-2Results of Surgical Resection for Bronchiectasis ||Download (.pdf) Table 90-2Results of Surgical Resection for Bronchiectasis
|REFERENCE ||PATIENTS ||MORTALITY RATE (%) ||MORBIDITY RATE (%) |
|Kutlay et al.26 |
Agasthian et al.41
Dogan et al.42
Surgical treatment of bronchiectasis is used widely, but there appear to be no randomized, controlled trials. It is not possible to provide an unbiased estimate of its benefit compared with conservative therapy.30 Addition of a vascularized muscle flap should be considered to prevent postoperative infection.