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 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. cancer 2. Histoplasmosis 3. Amyloidosis 4. 5. Wegener granulomatosis What is the most likely diagnosis? 1. 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 • • 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 “” 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: • Congenital • ciliary dyskinesia • cystic fibrosis • immune deficiency disorders • Mounier Kuhn () • 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 most likely diagnosis? 1. Bronchial atresia 2. Lung cancer 3. ABPA 4. Ciliary dyskinesia 5. Foreign body aspiration DDx: Mucoid Impaction • ABPA (allergic bronchopulmonary aspergillosis • Segmental bronchial atresia • Asthma • Bronchiectasis • Central obstruction What is the most likely diagnosis? 1. Bronchial atresia 2. Lung cancer 3. ABPA 4. Ciliary dyskinesia 5. Foreign body aspiration 67 year-old, asymptomatic What is the most likely diagnosis? 1. Bronchial atresia 2. Lung cancer 3. ABPA 4. Ciliary dyskinesia 5. Foreign body aspiration What is the most likely diagnosis? 1. Bronchial atresia 2. Lung cancer 3. ABPA 4. Ciliary dyskinesia 5. Foreign body aspiration Segmental Bronchial Atresia

* Segmental Bronchial Atresia

Anterior Apical Segment Segment *

Posterior Segment Segmental Bronchial Atresia • Proximal atresia • Upper > middle, lower lobes • CT findings • central mucus plug (*) • hyperlucent segment * Allergic BronchoPulmonary Aspergillosis • Airway infection/allergy • Severe, refractory asthma • A. fumigatus most common • CT findings • large mucus plugs Allergic BronchoPulmonary Aspergillosis • Airway infection/allergy • Severe, refractory asthma • A. fumigatus most common • CT findings • large mucus plugs • high attenuation ≈ 40% Allergic BronchoPulmonary Aspergillosis • Airway infection/allergy • Severe, refractory asthma • A. fumigatus most common • CT findings • large mucus plugs • high attenuation ≈ 40% • central bronchiectasis • upper lobe, asymmetric Outline - Summary • Congenital • tracheal bronchus Outline - Summary • Congenital • Long-segment narrowing • histoplasmosis Outline - Summary • Congenital • Long-segment narrowing • Short-segment narrowing • ETT stricture

Outline - Summary • Congenital • Long-segment narrowing • Short-segment narrowing • Solitary mass • adenocystic carcinoma Outline - Summary • Congenital • Long-segment narrowing • Short-segment narrowing • Solitary mass • Multiple masses • papillomatosis Outline - Summary • Congenital • Long-segment narrowing • Short-segment narrowing • Solitary mass • Multiple masses • Airway trauma • right bronchial fracture Outline - Summary • Congenital • Long-segment narrowing • Short-segment narrowing • Solitary mass • Multiple masses • Airway trauma • Bronchiectasis • ciliary dyskinesia Outline - Summary • Congenital • Long-segment narrowing • Short-segment narrowing • Solitary mass • Multiple masses • Airway trauma • Bronchiectasis • Mucoid Impaction • lung cancer Case Based Review H. Page McAdams, MD Duke University Medical Center Durham, NC 27710 [email protected]