Airway Disease
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Case Based Review H. Page McAdams, MD Duke University Medical Center Durham, NC 27710 [email protected] 21 year-old, cough, fever Courtesy Phil Boiselle, MD Courtesy of P. Boiselle, MD 21 year-old, cough, fever 21 year-old, cough, fever What is the most likely diagnosis? 1. Tuberculosis 2. Sequestration 3. Tracheal bronchus 4. Carcinoid tumor 5. Accessory cardiac bronchus What is the most likely diagnosis? 1. Tuberculosis 2. Sequestration 3. Tracheal bronchus 4. Carcinoid tumor 5. Accessory cardiac bronchus What is the most likely diagnosis? 1. Tuberculosis 2. Sequestration 3. Tracheal bronchus 4. Carcinoid tumor 5. Accessory cardiac bronchus Tracheal Bronchus • 0.1 - 2% pop. • R >> L • Apical segment • Asymptomatic • Infection, atelectasis Tracheal Bronchus • 0.1 - 2% pop. • R >> L • Apical segment • Asymptomatic • Infection, atelectasis Tracheal Bronchus “Bronchus Suis” Courtesy Santiago Jimenez, MD Accessory Cardiac Bronchus 45-year-old, cough, hemoptysis What is the most likely diagnosis? 1. Lung cancer 2. Histoplasmosis 3. Amyloidosis 4. Relapsing polychondritis 5. Wegener granulomatosis What is the most likely diagnosis? 1. Lung cancer 2. Histoplasmosis 3. Amyloidosis RMB RUL 4. Relapsing polychondritis BI 5. Wegener granulomatosis RLL DDx: Long Segment (> 1-cm) Narrowing • Inflammatory • Wegener granulomatosis • relapsing polychondritis • infection • amyloidosis 2.5 cm DDx: Long Segment (> 1-cm) Narrowing • Inflammatory • Neoplasm • lung cancer • esophageal cancer 2.5 cm What is the most likely diagnosis? 1. Lung cancer 2. Histoplasmosis 3. Amyloidosis 4. Relapsing polychondritis 5. Wegener granulomatosis Fibrosing Mediastinitis Wegener Granulomatosis Wegener Granulomatosis Wegener Granulomatosis • Airway disease • subglottic stenosis most common • lower airway disease less common • malacia • mass(es) • fistula 67-year-old, asymptomatic 67-year-old, asymptomatic What is the most likely diagnosis? 1. Thyroid cancer 2. ETT injury 3. Trauma 4. Relapsing polychondritis 5. Amyloidosis DDx: Short Segment (< 1-cm) Narrowing • Iatrogenic • tracheostomy • ET tube • lung transplant • Trauma • Extrinsic mass What is the most likely diagnosis? What is the most likely diagnosis? What is the most likely diagnosis? 1. Thyroid cancer 2. ETT injury 3. Trauma 4. Relapsing polychondritis 5. Amyloidosis Endotracheal Tube Overinflation 32-year-old, stridor What is the most likely diagnosis? 1. Carcinoid 2. Hamartoma 3. Metastasis 4. Adenoid cystic carcinoma 5. Amyloidosis DDx: Solitary Mass • Malignant (>90%) • primary • metastatic • Benign (<10%) • hamartoma • papilloma • aspirated material • broncholith(s) Primary Malignancies: Location • Trachea • squamous cell • adenoid cystic • metastases Primary Malignancies: Location • Trachea • Bronchi • lung cancer • carcinoid tumor • mucoepidermoid • metastases What is the most likely diagnosis? 1. Carcinoid 2. Hamartoma 3. Metastasis 4. Adenoid cystic carcinoma 5. Amyloidosis Adenoid Cystic Carcinoma • Young adults • Focal, smooth • Lateral wall Adenoid Cystic Carcinoma • Young adults • Focal, smooth • Lateral wall • Infiltrative • Slow growing • Good prognosis • Late metastases 25 year-old, history of “Asthma” 25 year-old, history of “Asthma” Adenoid Cystic Carcinoma Squamous Carcinoma of Trachea • Older adults • Lower-third trachea • Invasive • Poor prognosis 21-year-old, cough, fever What is the most likely diagnosis? 1. Carcinoid 2. Hamartoma 3. Metastasis 4. Adenoid cystic carcinoma 5. Amyloidosis What is the most likely diagnosis? 1. Carcinoid 2. Hamartoma 3. Metastasis 4. Adenoid cystic carcinoma 5. Amyloidosis Carcinoid Tumor • Young adults • Variable malignant potential • Bronchi >> trachea • 80 – 85% central • 15 – 20% distal to segmental airway Carcinoid Tumor • Young adults • Variable malignant potential • Bronchi >> trachea • 80 – 85% central • 15 – 20% distal to segmental airway • “Iceberg” sign Carcinoid Tumor • Young adults • Variable malignant potential • Bronchi >> trachea • 80 – 85% central • 15 – 20% distal to segmental airway • “Iceberg” sign Carcinoid Tumor • Young adults • Variable malignant potential • Bronchi >> trachea • 80 – 85% central • 15 – 20% distal • “Iceberg” sign • 37% calcify 67-year-old, NSCLC, baseline CT 9 months, cough, fever What is the most likely diagnosis? 1. Carcinoid 2. NSCLC 3. Metastasis 4. Broncholithiasis 5. Aspiration What is the most likely diagnosis? 1. Carcinoid 2. NSCLC 3. Metastasis 4. Broncholithiasis 5. Aspiration Aspirated Cashew Nut Foreign Body Aspiration • Right > left • 75% vegetable matter • Signs: • air-trapping (children) • atelectasis, pneumonia (adults) Recurrent Pneumonias X 2 years Recurrent Pneumonias X 2 years Recurrent Pneumonias X 2 years Aspirated Spoon Broncholithiasis • Histoplasmosis • Tuberculosis • Signs: • atelectasis, pneumonia • calcified nodes • calcific focus within airway Broncholithiasis • Histoplasmosis • Tuberculosis • Signs: • atelectasis, pneumonia • calcified nodes • calcific focus within airway Trauma: best diagnosis? 1. Carcinoid 2. Broncholithiasis 3. Tooth 4. Amyloidosis 5. Cashew nut Trauma: best diagnosis? 1. Carcinoid 2. Broncholithiasis 3. Tooth 4. Amyloidosis 5. Cashew nut Trauma: best diagnosis? 1. Carcinoid 2. Broncholithiasis 3. Tooth 4. Amyloidosis 5. Cashew nut 26-year-old, chronic cough 26-year-old, chronic cough What is the most likely diagnosis? 1. Papillomatosis 2. Mucus 3. Tracheopathia Osteochondoplastica 4. Amyloidosis 5. Metastases DDx: Multiple Masses • Metastases • renal cell carcinoma • breast cancer • melanoma • Papillomatosis • Tracheopathia Osteochondroplastica • Amyloidosis What is the most likely diagnosis? 1. Papillomatosis 2. Mucus 3. Tracheopathia Osteochondoplastica 4. Amyloidosis 5. Metastases What is the most likely diagnosis? 1. Papillomatosis 2. Mucus 3. Tracheopathia Osteochondoplastica 4. Amyloidosis 5. Metastases Papillomatosis Courtesy M Rosado-de-Christensen, MD Papillomatosis Papillomatosis Papillomatosis Courtesy M Rosado-de-Christensen, MD 67-year-old, chronic cough INSP EXP What is the most likely diagnosis? 1. Papillomatosis 2. Wegener granulomatosis 3. Relapsing polychondritis 4. Amyloidosis 5. Tracheobronchomalacia EXP What is the most likely diagnosis? 1. Papillomatosis 2. Wegener granulomatosis 3. Relapsing polychondritis 4. Amyloidosis 5. Tracheobronchomalacia EXP Tracheobronchomalacia • Diagnosis • controversial • abnormal reduction in cross- sectional area on expiration • > 50%, >70% • abnormal flow-volume loop • symptoms Tracheobronchomalacia Normal Intrathoracic Obstruction Insp Exp Flattened Exp Insp Tracheobronchomalacia 䇾Frown䇿 EXP Tracheobronchomalacia INSP EXP Tracheobronchomalacia • Diagnosis • Associations • COPD • chronic corticosteroid therapy • relapsing polychondritis EXPEXP 16 year-old, chest trauma initial 16 year-old, chest trauma 24 hours 16 year-old, chest trauma What is the most likely diagnosis? 1. Malfunctioning chest tube 2. Mucus plug 3. Bronchial fracture 4. Contusion 5. Esophageal rupture 24 hours What is the most likely diagnosis? 1. Malfunctioning chest tube 2. Mucus plug 3. Bronchial fracture 4. Contusion 5. Esophageal rupture 24 hours CT Fallen Lung Sign Right Bronchial Fracture Blunt Airway Trauma • Location • most 2.5-cm carina • 80% main bronchi • 20% trachea • right > left Blunt Airway Trauma • Diagnosis • persistent ptx 24 hours Blunt Airway Trauma • Diagnosis • persistent ptx • severe pneumo- mediastinum Blunt Airway Trauma • Diagnosis • persistent ptx • severe pneumo- mediastinum Blunt Airway Trauma • Diagnosis • persistent ptx • severe pneumomediastinum • “fallen” lung sign • CT not 100% sensitive 37 year-old, chronic cough 37 year-old, chronic cough What is the most likely diagnosis? 1. Cystic fibrosis 2. Williams Campbell syndrome 3. Lady Windermere syndrome 4. Ciliary dyskinesia 5. Yellow nail syndrome DDx: Bronchiectasis • Congenital • ciliary dyskinesia • cystic fibrosis • immune deficiency disorders • Mounier Kuhn (tracheobronchomegaly) • Williams Campbell • Yellow nail syndrome DDx: Bronchiectasis • Acquired • infection • toxic fume inhalation • recurrent aspiration • central obstruction • fibrosis (traction) What is the most likely diagnosis? 1. Cystic fibrosis 2. Williams Campbell syndrome 3. Lady Windermere syndrome 4. Ciliary dyskinesia 5. Yellow nail syndrome Primary Ciliary Dyskinesia • AKA: immotile cilia syndrome • Autosomal recessive Primary Ciliary Dyskinesia • AKA: immotile cilia syndrome • Autosomal recessive • Abnormal dynein arms Primary Ciliary Dyskinesia • AKA: immotile cilia syndrome • Sinusitis • Bronchiectasis • basal > apical Primary Ciliary Dyskinesia • AKA: immotile cilia syndrome • Sinusitis • Bronchiectasis • basal > apical • ≈ 50% situs inversus • Kartagener syndrome Immunodeficiency • Recurrent infection • Large number • agammaglobulinemia • CVID • IgG deficiency • hyper IgE (Job syn) • No correlation with CT findings/etiology “Lady Windermere” Syndrome • Chronic MAC infection • Elderly women • Low body weight • Chronic, nagging cough • Associated with pectus, mitral valve prolapse • ≈ CTFR mutation “Lady Windermere” Syndrome • CT findings • cylindrical bronchiectasis • middle lobe, lingula • centrilobular nodules • cavitation uncommon • slow progression “Lady Windermere” Syndrome • CT findings • cylindrical bronchiectasis • middle lobe, lingula • centrilobular nodules • cavitation uncommon • slow progression 77 year-old, chronic cough What is the most likely diagnosis? 1. Bronchial atresia 2. Lung cancer 3. ABPA 4. Ciliary dyskinesia 5. Foreign body aspiration What is the