L. Renata Thronson, HMS III Gillian Lieberman, MD Oct/Nov 2003 Radiologic Findings in Bronchiectasis L. Renata Thronson, Harvard Medical School Year III Gillian Lieberman, MD L. Renata Thronson, HMS III Gillian Lieberman, MD Bronchiectasis Bronchiectasis is an irreversible, chronic dilatation of diseased airways. 2 L. Renata Thronson, HMS III Gillian Lieberman, MD A 64 year-old male with recurrent pneumonia A 44 year-old woman with cough and rales and PACS, BIDMC PACS, BIDMC 3 L. Renata Thronson, HMS III Gillian Lieberman, MD Pathogenesis of Bronchiectasis Environmental insult Defective mucociliary clearance Microbes persist in bronchi Genetic susceptibility Tissue damage Colonization Progressive lung damage Chronic inflammation Adapted from Hansell DM. Bronchiectasis. Radiologic Clinics of North America 1998; 36:107-126. 4 L. Renata Thronson, HMS III Gillian Lieberman, MD Menu of tests • Bronchography – The “gold standard” • High-resolution CT – 1-2 mm collimation – Grenier et al: Se 96%; Sp 93% – Young et al: Se 98%; Sp 99% • Chest x-ray – Inferior sensitivity and specificity 5 L. Renata Thronson, HMS III Gillian Lieberman, MD Bronchography • Requires local anesthetic and bronchographic medium potential for allergic reaction • Exam limited to airways http://radiology.rsnajnls.org 6 L. Renata Thronson, HMS III Gillian Lieberman, MD CXR: Findings • Indistinct vessel margins • Tram lines • Ring shadows • Volume loss – Elevated left hemidiaphragm PACS, BIDMC 7 L. Renata Thronson, HMS III Gillian Lieberman, MD HRCT: Findings • Dilatation – Signet ring sign – Non-tapering airways • Indirect signs – Mucous plugging – Lobar volume loss – Wall thickening PACS, BIDMC 8 L. Renata Thronson, HMS III Gillian Lieberman, MD Signet Ring Sign Airway Pulmonary artery PACS, BIDMC 9 L. Renata Thronson, HMS III Gillian Lieberman, MD Non-tapering airways • Airways visible within 1 cm of the visceral pleura • Tram lines • Flaring PACS, BIDMC 10 L. Renata Thronson, HMS III Gillian Lieberman, MD Mucous plugging Hansell DM. Bronchiectasis. Radiologic Clinics of North America 1998; 36:107-126. 11 L. Renata Thronson, HMS III Gillian Lieberman, MD Morphologies of Bronchiectasis • Cylindrical • Cystic • Varicoid 12 L. Renata Thronson, HMS III Gillian Lieberman, MD Morphologies of Bronchiectasis • Cylindrical • Cystic • Varicoid PACS, BIDMC 13 L. Renata Thronson, HMS III Gillian Lieberman, MD Morphologies of Bronchiectasis • Cylindrical • Cystic • Varicoid PACS, BIDMC 14 L. Renata Thronson, HMS III Gillian Lieberman, MD Morphologies of Bronchiectasis • Cylindrical • Cystic • Varicoid PACS, BIDMC 15 L. Renata Thronson, HMS III Gillian Lieberman, MD DDX of Bronchiectasis • Postinfectious – – – – Bacterial Mycobacterial Viral Fungal • Post-transplantation – Chronic rejection – GVHD 16 L. Renata Thronson, HMS III Gillian Lieberman, MD DDX of Bronchiectasis • Postinflammatory – Aspiration – Toxic inhalation • Postobstructive – Tumor – Foreign body – Stricture 17 L. Renata Thronson, HMS III Gillian Lieberman, MD DDX of Bronchiectasis • Impaired mucociliary clearance – Dyskinetic cilia syndromes – Cystic Fibrosis • Inherited cellular or molecular defects – Alpha-1-antitrypsin deficiency – Cystic Fibrosis 18 L. Renata Thronson, HMS III Gillian Lieberman, MD DDX of Bronchiectasis • Immune deficiency – Inherited – Acquired • Collagen Vascular Disease – – – – – Rheumatoid arthritis Sjogren’s syndrome Ankylosing spondylitis Marfan syndrome IBD 19 L. Renata Thronson, HMS III Gillian Lieberman, MD DDX of Bronchiectasis • Congenital bronchial abnormalities – Mounier-Kuhn syndrome – Williams-Campbell syndrome – Bronchopulmonary sequestration • Miscellaneous – Asthma – Yellow-nail syndrome – Sarcoidosis 20 L. Renata Thronson, HMS III Gillian Lieberman, MD Diagnostic Pitfalls Bronchiectasis PACS, BIDMC Interstitial disease http://www.hospitalist.net/case1.html 21 L. Renata Thronson, HMS III Gillian Lieberman, MD Diagnostic Pitfalls Signet ring? Tram lines? Hansell DM. Bronchiectasis. Radiologic Clinics of North America 1998; 36:107-126 22 L. Renata Thronson, HMS III Gillian Lieberman, MD Diagnostic Pitfalls Branching airway Double shadow: heart and vessels Hansell DM. Bronchiectasis. Radiologic Clinics of North America 1998; 36:107-126 23 L. Renata Thronson, HMS III Gillian Lieberman, MD 64 year-old man with recurrent pneumonia PACS, BIDMC 24 L. Renata Thronson, HMS III Gillian Lieberman, MD 64 year-old man with recurrent pneumonia Ring shadow PACS, BIDMC Tram lines 25 L. Renata Thronson, HMS III Gillian Lieberman, MD 64 year-old man with recurrent pneumonia Airways visible at pleural margin Varicoid dilatation Mucous plugging PACS, BIDMC 26 L. Renata Thronson, HMS III Gillian Lieberman, MD 44 year-old woman with cough Varicoid dilatation PACS, BIDMC Cystic dilatation PACS, BIDMC 27 L. Renata Thronson, HMS III Gillian Lieberman, MD 44 year-old woman with cough Tracheal margins PACS, BIDMC Tracheal margins PACS, BIDMC 28 L. Renata Thronson, HMS III Gillian Lieberman, MD Mounier-Kuhn syndrome • Congenital bronchial abnormality • ?Autosomal recessive • Findings – Tracheomegaly – Bronchomegaly PACS, BIDMC 29 L. Renata Thronson, HMS III Gillian Lieberman, MD Bronchiectasis and Situs Inversus Courtesy of Phillip Boiselle, MD 30 L. Renata Thronson, HMS III Gillian Lieberman, MD Kartagener syndrome • Primary ciliary dismotility – Recurrent respiratory infections – Decreased fertility • Findings on CXR – Bronchiectasis – Situs inversus Courtesy of Phillip Boiselle, MD 31 L. Renata Thronson, HMS III Gillian Lieberman, MD Cystic Fibrosis • Upper lobe predilection • Panlobar disease • Marked cystic dilatation – Air-fluid levels may be seen within cysts Courtesy of Phillip Boiselle, MD 32 L. Renata Thronson, HMS III Gillian Lieberman, MD Cystic Fibrosis • Upper lobe predilection • Panlobar disease • Marked cystic dilatation – Air-fluid levels may be seen within cysts Courtesy of Phillip Boiselle, MD 33 L. Renata Thronson, HMS III Gillian Lieberman, MD Postinfectious Varicoid Bronchiectasis Focal bronchiectasis PACS, BIDMC 34 L. Renata Thronson, HMS III Gillian Lieberman, MD Summary • DDX of bronchiectasis is broad, but the diseases share a common pathogenesis • Morphologies reflect degrees of severity – Cylindrical – Cystic – Varicoid • Tests available – Bronchography – CXR – HRCT 35 L. Renata Thronson, HMS III Gillian Lieberman, MD Summary • Radiologic findings – CXR • Volume loss • Mucous plugging – “gloved finger” • Inflammation – Indistinct vessel margins – Tram lines – Ring shadows 36 L. Renata Thronson, HMS III Gillian Lieberman, MD Summary • Radiologic findings – HRCT • • • • Wall thickening Mucous plugging Crowded bronchilobar volume loss Airway dilatation – Signet ring sign – Non-tapering small airways 37 L. Renata Thronson, HMS III Gillian Lieberman, MD References • • • • • Hansell DM. Bronchiectasis. Radiologic Clinics of North America 1998; 36:107-126. McGuinness G, Naidich DP. CT of Airways Disease and Bronchiectasis. Radiologic Clinics of North America 2002; 40:1-19. Friedman, PJ. Chest Radiographic Findings in Adults with Cystic Fibrosis. Seminars in Roentgenology 1987; 22:114-124. Grenier P, Maurice R, Musset D, et al. Bronchiectasis: Assessment by thin-section CT. Radiology 1986; 161:95-99. Young K, Aspestrand F, Kolbenstvedt. High resolution CT and bronchography in the assessment of bronchiectasis. Acta Radiology 1991; 32:439-441. 38 L. Renata Thronson, HMS III Gillian Lieberman, MD Acknowledgements • • • • • • Andru Bageac, MD Eric Niendorf, MD Phillip Boiselle, MD Gillian Lieberman, MD Larry Barbaras Pamela Lepkowski 39