Congenital Lung Disease in Adults

Question 1

Bronchogenic cyst

Esophageal duplicaton cyst

Answer 1

• Can only be differentiated based on histology
• If asymtpomatic - leave along lesions

Question 2

Bronchial Atresia

Answer 2

• atresia of central bronchus, usually segmental
• thought to be due to ischemic insult
• mucocele (bronchocele)
• regional air-trapping (due to collateral air flow)
• asymptomatic - do not touch lesion
• can rarely get superinfected
• DDx: central obstructing lesion, e.g., carcinoid!

Question 3

Congenital lobar emphysema

a.k.a.

Congenital lobar Hyperinflation

Answer 3

• intrinsic - web/malacia
• extrinsic - airway obstruction
• most common - LUL

Question 4

Most common location of congenital lobar emphysema

Answer 4

LUL

Question 5

Congenital lobar emphysema

vs Swyer James (acquired bronchiolitis obliterans)

Answer 5

• Congenital lobar emphysema
  
  • MASS EFFECT
• Swyer James Syndrome
  
  • due to childhood infection
  • small airway air trapping
  • peribronchial thickening
  • decreased vasculature
  • NO mass effect

Question 6

Congenital Pulmonary Airway Malformation

a.k.a.

Cystic Adenomatoid Malformation - C-CAM

Answer 6

• disorganized lung tissue with bronchiolar proliferation
• NO airway communication; usually vasculature

Question 7

Classification of Congenital Pulmonary Airway Malformation (C-CAM)

a.k.a.

Stocker Classification

Answer 7

• Type 0 - solid with small firm lungs
• Type I - large cysts >2cm
• Type II - multiple small cysts <2cm
• Type III - solid, microcystic lesion
• Type IV - large cysts, peripheral

Question 8

Intralobar Sequestration

Answer 8

• Non-functioning tissue “sequestered” from the tracheobronchial tree
• No seprate pleural coverage
  
  • extarlobar - own pleural covering
• systemic arterial supply
• pulmonary venous drainage
  
  • extralobar sequestration - systemic drainage
• congenital vs acquired