Pulmonary Board Review

1. Pulmonary Board Review January 24, 2011

2. Physical Exam • Always look for clubbing • Suprasternal and intercostal retractions may be normal following exercise • Subcostal retractions always pathologic • Accessory muscles • Respiratory alternans- alternating abdominal and chest expansion • Respiratory muscle fatigue

3. Chlamydia trachomatis pneumonia 1-2 months of age Afebrilepneumonitis Congestion, wheezing, fine diffuse crackles Paroxysmal, staccato cough Inclusion conjunctivitis in 50% CBC may have eosinophil predominance

4. Question 1 • Which CBC results support the diagnosis of Bordetellapertussis? A. High white count with lymphocytosis B. High white count with eosinophilia C. High white count with high bands D. Low white count

5. Bordetellapertussis • 3 Phases • Catarrhal • Paroxysmal • Severe paroxysmal coughing episodes • Followed by cyanosis and apnea • Inspiratory whoop • Convalescent • Apnea may be presenting sign • CXR nonspecific • Elevated WBCs with lymphocytosis

6. Bordetellapertussis • Dx with PCR • Treat only to reduce spread • Macrolide • (treat contacts also) • Tdap vaccine • Adults 19-64 years old, one Tdap • At least 2 years after Td

7. Question 2 You diagnose a 4 year old patient with 3rd episode of pneumonia. A dense, opaque area has been seen on CXR in same location all three times. What is most likely diagnosis? A Pulmonary Sequestration B Bronchogenic cyst C Congenital Cystic Adenomatoid Malformation D Retained foreign body

8. Recurrent consolidated PNA of same location Think of underlying structural defect!!

9. Recurrent consolidated PNA of same location Bronchopulmonary Sequestration Bronchogenic Cyst Congenital Cystic Adenomatoid Malformation

10. Pulmonary sequestration • Portion of lungs perfused by systemic arteries • Lacks normal connection with tracheobronchial tree • Intralobar: • Most common lower lobes • Anywhere in thorax • Extralobar: • Subdiaphragmatic or retroperitoneal • Associated with other anomalies

11. Pulmonary sequestration

12. Bronchogenic Cyst • Occur any point along the tracheobronchial tree • Centrally located • Does not communicate, fluid fill with wall composed of tissue resembling large airways • Typically present 2nd decade of life with recurrent wheezing, cough, pneumonia

13. Bronchogenic Cyst

14. Congenital Pulmonary Airway Malformation (CPAM) • Previously known as Congenital Cystic Adenomatoid Malformation (CCAM) • Hamartomatous lesion • Cystic and adenomatous elements • Resp distress, recurrent PNA, asymptomatic • Surgical resection is symptomatic • Controversial if asymptomatic

15. Congenital Pulmonary Airway Malformation (CPAM)

16. Question 3 • A 21year old patient is in the ER for pneumonia, which has been recurrent. He has also been treated multiple times for chronic sinusitis. He has required multiple sets of ear tubes for OM, even as an adolescent. He has been married for a year, and has been trying unsuccessfully to have a child. On review of systems, he has had normal growth and development, no bowel or bladder complaints. On exam: bibasilar crackles and ronchi, nasal discharge, nasal polyps. CV exam normal. What is the most likely underlying diagnosis • A. Cystic Fibrosis • B. Kartagener Syndrome (Primary ciliarydyskinesia) • C. Aquiredciliarydyskinesia • D. Lymphocytic interstitial pneumonitis

17. KartagenerPrimary CiliaryDyskinesia • Triad: situsinversus, sinusitis, bronchiectasis • Situsinversus in only 50% • Recurrent OM • Nasal polyposis • Recurrent Pneumonia • Infertility

18. Acquired ciliarydyskinesia • Far more common • May follow LRTI • Adeno • Mycoplasma • RSV • Influenza • Need bx of resp epithelium for confirmation

19. Pulmonary Hemosiderosis • Potentially fatal • Associated with: • Cardiac dz • Glomerulonephritis (Goodpasture syndrome) • Infantile hypersensitivity to cow’s milk • Collagen vascular diseases • Idiopathic

20. Idiopathic Pulmonary Hemosiderosis • Dyspnea, cough, hemoptysis, cyanosis, fever, iron-def anemia • Hematemasis or melena • May develop jaundice or clubbing • CXR: variable • Transient infiltrates • Similar to miliary TB

21. Idiopathic Pulmonary Hemosiderosis • Dx: • hemosiderin-laden macrophages (suggestive) • Lung bx definitive

22. Mycoplasmapneumoniae • School age children and adults • May imitate viral URI • Progress to lower resp tract • Persistent • Cough variable, may become productive • May progress to consolidation • Lower lobes • Physical findings minimal (crackles/wheezing) • Mycoplasma PCR available • Primarily clinical diagnosis

23. Psychogenic or Habit Cough • Usually following LRTI • May persist for weeks or months • Loud “honking” “barking” • Disturbing to family members, classmates • Always disappears with sleep • Worse with: • Stress • Attention to cough • Extensive evaluation may exacerbate problem

24. Stridor

25. Never forget foreign body!!

26. Laryngotracheobronchitis(croup) • Most common cause of inspiratorystridor kids • Acute viral illness • Parainfluenza • Almost any viral resp illness • Clinical findings • Coryza 1-2 days • Harsh cough • Hoarseness • stridor

27. Laryngotracheobronchitis(croup) • Mostly self limited • Airway obstruction possible • Treatment • Dexamethasone • RacemicEpi nebs (vaponeb) • Warm or cool humidified air: • no significant benefit

28. What now? • Bacterial Tracheitis Child treated for croup returns 3 days later looking toxic, stridor has returned

29. Question 4 An 12 month old has been having daily stridor and intermittent ronchi. Mom was told that her child has laryngomalacia. What is your diagnosis? A. Laryngomalacia B. Laryngeal web C. Hemangioma D. Chronic aspiration E. Vascular ring

30. Laryngomalacia • Congenitally floppy glottis • Symptoms • Stridor usually by 1st week of life • Varies with activity • May be biphasic • Worse supine • Usually self limited • Improvement at 6-10 months of age

31. Laryngomalacia • Diagnosis • Clinical • Airway flouro or direct visualization if concerned • Treat for GE Reflux • Refer to ENT if failure to thrive or other complications

32. SubglotticStenosis • Congenital • Improves with age • Acquired • Endotracheal intubation • Problematic • May require tracheostomy

33. Congenital laryngeal/pharyngeal masses • Laryngeal papillomatosis • Rare • Life threatening • Presents in first decade • Vocal cords or entire tracheobronchial tree • Clinical features • Hoarseness • Stridor • Brassy cough

34. Congenital laryngeal/pharyngeal masses • Hemangiomas • Stridor, brassy cough • Cutaneoushemangiomas may offer clues • Now treated with beta-blockers

35. Congenital laryngeal/pharyngeal masses • Laryngeal webs, cysts, laryngoceles • Uncommon • Clinical manifestations • Resp distress • Feeding difficulties • Stridor • Cyanosis

36. Vocal Cord Paralysis • Clinical Findings • Weak or absent cry • Hoarseness • Inspstridor with/without distress • Feeding difficulties • Diagnosis • Flexible bronchoscopy

37. Vocal Cord Paralysis • Unilateral • Causes • Cardiomegaly • Vascular ring • May be subltle • Bilateral • Causes • Hydrocephalus • Myelomeningocele • Arnold-chiari malformation

38. wheezing

39. Question 5 A 13 year old athlete has had wheezing and shortness of breath at multiple track meets this season. A. PFT’s consistent with asthma B. PFT’s consistent with restrictive lung disease C. PFT’s consistent with vocal cord dysfunction D. Normal PFTs

40. Vocal Cord Dysfunction • Often mistaken for exercise-induced asthma • Many have BOTH • Vocal cords adduct during inspiration • Psychogenic origin • Highly pressured adolescent female athlete • Competitive event • Sensation of dyspnea • “trouble getting air IN” • Localized to throat

41. Vocal Cord Dysfunction • Diagnosis • Clinical suspicion • PFT’s • Flexible nasolaryngoscopy • Treatment • Speech therapists, breathing exercises • Psychology may be needed

42. Allergic BronchopulmonaryAspergillosis (ABPA) • Increased wheezing in previously well controlled asthmatic or CF patient • Insidious presentation • Low-grade fevers • Fatigue • Weight loss • Productive cough • Physical • Wheeze • Bibasilar crackles • clubbing

43. Allergic BronchopulmonaryAspergillosis (ABPA) • Imaging • Consolidation or atelectasis • Dilated bronchi radiating from hila • Diagnosis • Skin prick to Aspergillusfumigatusantigen • Elevated total and specific IgE • Isolation of A.fumigatus from sputum • Worsened PFT’s (obstruction)

44. Vascular Ring • Recurrent wheezing or stridor • CXR: Right sided aortic arch • Tracheal deviation to the Left • Barium swallow • Inexpensive screening test • MRI • Delineates airway and vascular anatomy • Necessary prior to surgery

45. Tracheal compression • Don’t forget about • Goiter • Mediastinal mass

46. Never forget foreign body!!

47. Other lung pathology

48. Congenital or acquired lobar emphysema • CXR: • Large, hyperlucent area • Few bronchovascular markings • Compression atelectasis of adjacent lobes • LUL most common • Middle lobe also seen • Treatment • Conservative if infant thriving • Resection for prolonged resp distress, recurrent infxn

49. Bronchopulmonary Dysplasia • Any resp infection may cause severe illness • May develop • Chronic resp insufficiency • Pulmonary HTN • Corpulmonale • Most continue with reactive airways

50. Bronchiolitisobliterans • Bronchioles become inflamed/fibrosed • Non-reversible obstructive lung disease • Causes • Idiopathic • Post adenoviral • Toxic inhalation • Conjunction with other diseases • (rheumatoid arthritis) • Post-transplant