Table 18.1 Nomenclature of bone tumors

Bronchiectasis

BASIC INFORMATION
DEFINITION
Bronchiectases are rare but important chronic airway diseases. Bronchiectases are characterized by a lack of tapering and irreversible dilatation of the airways. Typical symptoms are chronic cough, usually accompanied by sputum production.
Bronchiectasis is the abnormal dilation and destruction of bronchial walls, which may be congenital or acquired.
EPIDEMIOLOGY & DEMOGRAPHICS
• Cystic fibrosis is responsible for nearly 55% of all cases of bronchiectasis.
• Acquired primary bronchiectasis is uncommon because of rapid diagnosis of pulmonary infections and frequent use of antibiotics.
• Effective childhood immunizations have lead to a significant decrease in the incidence of bronchiectasis due to pertussis.

DIAGNOSIS
The leading symptoms are chronic cough and sputum. Copious amounts of sputum, particularly in the morning, are typical for severe forms. If no sputum is produced bronchiectasis is not excluded. Major hemoptysis is a feared complication, which is caused by a ruptured bronchial artery.
     On auscultation coarse rales are a typical finding, particularly if localized. If the small airways are involved by the underlying inflammation an obstructive ventilatory defect may cause symptoms and can be diagnosed by spirometry. In advanced cases, the surrounding lung parenchyma is involved by chronic inflammation and causes a restrictive component.
     The sputum contains a mixed flora consisting of Hemophilus influenzae, Diplococcus pneumoniae, or Staphylococcus aureus. In chronic bronchiectasis, Pseudomonas aeruginosa or other Gram-negative bacteria (e. g., Stenotrophomonas maltophilia, Burkholderia cepacia, Achromobacter xylosoxidans) may be present. Mycobacteria, particularly Mycobacterium avium-intracellulare (MAI) need to be looked for at regular intervals.
     In most cases the chest radiograph shows increased linear markings, tubular structures (“tram lines”), or cystic formations suggestive for the presence of bronchiectasis. Localized bronchiectasis is most commonly found in the posterior segments of both lower lobes, in the middle lobe, and in the lingula. Diffuse bronchiectasis is usually more prominent in the upper than in the lower lobes. A normal chest radiograph does not exclude mild, usually tubular, bronchiectasis! Bronchography has been replaced by high-resolution CT (Fig. 7.14). A bronchus is called bronchiectatic if its outer diameter is larger then the diameter of the accompanying pulmonary artery. The bronchial wall is usually thickened. The lack of tapering may be clearly visible if the bronchus runs in the plane of the CT slice.
DIFFERENTIAL DIAGNOSIS
• TB
• Asthma
• Chronic bronchitis or chronic sinusitis
• Interstitial fibrosis
• Chronic lung abscess
• Foreign body aspiration
• Cystic fibrosis
• Lung carcinoma
WORKUP
• Sputum for Gram stain and C&S, chest x-ray examination, bronchoscopy, spirometry
• Spirometry reveals reduced ration of FEV1 to FVC, normal or slightly reduced FVC, and a reduced FEV1
LABORATORY TESTS
• Sputum for Gram stain, C&S, and acid-fast bacteria (AFB)
• CBC with differential (leukocytosis with left shift, anemia)
• Serum protein electrophoresis to evaluate for hypogammaglobulinemia
• Antibody test for aspergillosis
• Sweat test in patients with suspected cystic fibrosis
IMAGING STUDIES
• Chest x-ray examination: hyperinflation, crowded lung markings, small cystic spaces at the base of the lungs
• High-resolution CT scan of the chest has become the best tool to detect cystic lesions and exclude underlying obstruction from neoplasm. The CT study should be a noncontrast study with the use of 1 to 1.5 mm window every 1 cm with acquisition time of 1 sec. Typical findings on CT include dilation of airway lumen, lack of tapering of an airway toward periphery, ballooned cysts at the end of bronchus, and varicose constrictions along airways.
• Bronchography is rarely used and may be considered only when surgery is contemplated.
• Pulmonary function tests generally reveal obstructive or mixed ventilatory defect.
• Bronchoscopy may be helpful to evaluate hemoptysis, rule out obstructive lesions, and remove mucus plugs.
TREATMENT
NONPHARMACOLOGIC THERAPY
• Postural drainage (reclining prone on a bed with the head down on the side) and chest percussion with use of inflatable vests or mechanical vibrators applied to the chest may enhance removal of respiratory secretions
• Adequate hydration
• Supplemental oxygen for hypoxemia
ACUTE GENERAL Rx
• Antibiotic therapy is based on the results of sputum, Gram stain, and C&S; in patients with inadequate or inconclusive results, empiric therapy with amoxicillin/clavulanate 500 mg to 875 mg q12h, TMP-SMX q12h, doxycycline 100 mg bid, or cefuroxime 250 mg bid for 10 to 14 days is recommended.
• Bronchodilators are useful in patients with demonstrable air flow obstruction.
CHRONIC Rx
• Avoidance of tobacco
• Maintenance of proper nutrition and hydration
• Prompt identification and treatment of infections
• Pneumococcal vaccination and annual influenza vaccination
DISPOSITION
Prognosis is variable with severity of the disease and underlying etiology of bronchiectasis.

PHYSICAL FINDINGS & CLINICAL PRESENTATION
• Moist crackles at lung bases
• Cough with expectoration of large amount of purulent sputum
• Fever, night sweats, generalized malaise, weight loss
• Hemoptysis
• Halitosis, skin pallor
• Clubbing (infrequent)
ETIOLOGY
• Cystic fibrosis
• Lung infections (pneumonia, lung abscess, TB, fungal infections, viral infections)
• Abnormal host defense (panhypogammaglobulinemia, Kartagener’s syndrome, AIDS, chemotherapy)
• Localized airway obstruction (congenital structural defects, foreign bodies, neoplasms)
• Inflammation (inflammatory pneumonitis, granulomatous lung disease, allergic aspergillosis)

Fig. 7.14 Advanced bronchiectases in the lower lobe, partially filled with secretion.