Case of the Week - Patient Summary 4253Peer Reviewed and Certified -
1.Bronchiectasis
2.Multifocal bronchioloalveolar carcinoma
3.Metastatic adenocarcinoma
Bronchiectasis defined as: localized, mostly irreversible, dilatation of bronchi often with thickening of the bronchial wall.
Bronchiectasis can result from:
A.Congenital
1.Bronchial atresia, Williams-Campbell syndrome
2.Abnormal mucociliary transport: Kartagener syndrome
3.Abnormal secretions: mucoviscidosis = cystic fibrosis
B.Congenital / acquired immune deficiency (IgG)
chronic granulomatous disease of childhood
alpha 1-antitrypsin deficiency
C.Postinfectious: measles, whooping cough, Swyer-James syndrome, allergic bronchopulmonary aspergillosis, chronic granulomatous infection (TB)
D.Bronchial obstruction: neoplasm, inflammatory nodes, foreign body
E.Aspiration / inhalation: gastric contents / inhaled fumes (late complication)
F.Pulmonary fibrosis: "traction bronchiectasis" due to increased elastic recoil with bronchial dilatation mechanical distortion of bronchi by fibrosis
Bronchiectasis Post-infectious: Measles, whooping cough, TB and allergic
bronchopulmonary aspergillosis)
Inflammatory: Association with Crohn’s & Ulcerative Colitis
Congenital: Bronchial atresia, Williams-Campbell syndrome, Cystic Fibrosis
Obstruction: Primary neoplasm, Foreign body, Enlarged lymph nodes
Impaired Clearance: Kartagener’s syndrome (Dysmotile cilia syndrome + situs)
Immunodeficiencies: Hypogammaglobulinemia, alpha-1 antitrypsin deficiency
Pulmonary Fibrosis: Traction bronchiectasis
Although findings consistent with bronchiectasis can be observed on routine chest radiographs and moreso on chest CT, dedicated high-resolution CT (HRCT) remains the modality of choice for the evaluation of bronchiectasis. Although some authors report up to 7% normal chest radiographs in patients with bronchiectasis, even conventional CT may not delineate the etiology in a small percentage of patients. Therefore, not only does HRCT delineate the location of the lesions in the lung, but remains the most sensitive and specific examination available.
Findings on HRCT include:
-Lack of bronchial tapering -wall thickening
- bronchial dilatation -mucoid filling
-“signet ring” sign with adjacent pulm artery as stone and bronchi as ring ie: relative enlargement of the bronchial
-“tram lines” sign
Three classifications have been adopted as standard which include:
1. Cylindrical: bronchi have fusiform dilation and lack of tapering and presence of abrupt termination.-ABPA
2. Varicose: usually has a more dilated bronchi than cylindrical, however will have bronchial constrictions that produce an irregular outline to the bronchi. – Cystic Fibrosis
3. Cystic: thick-walled saccular dilatations that can have air-fluid levels secondary to retained secretions-Bronchial stenosis
Although various disease processes have some regularity, there is some overlap and variablity of the type and location of the involved bronchi.
Treatment:
Antibiotics and respiratory therapy are the mainstays of conservative therapy. Surgical resection is an option of last resort for advanced disease. Episodes of massive hemoptysis have been known to occur requiring emergent embolization of involved bronchial artery. In the case of bronchiectasis in the setting of inflammatory bowel disease however the mainstay of therapy remains steroids as they lesions can undergo striking resolution once proper therapy is initiated.
