FlashPath - Lung - Bronchial Atresia
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Bronchial atresia is a congenital disorder where a portion of the bronchial tree has no connection to the main bronchial tree. It usually affects a segmental bronchus and presents as an ovoid hilar opacity on imaging with branches radiating into a distal area of hyperinflation. Pathology shows a mucocele or "bronchocele" distal to the atretic segment, filled with mucus and macrophages. The distal lung is hyperinflated due to collateral ventilation around the obstruction. Bronchial atresia is usually an incidental finding in asymptomatic adults, though some cases present with recurrent pneumonias or respiratory symptoms.
FlashPath - Lung - Bronchial Atresia
- 1. FLASHPATH H A Z E M A L I
- 2. BRONCHIAL ATRESIA H A Z E M A L I
- 3. CLINICAL • Portion of bronchial tree that have NO connection to the main bronchial tree, but with normal subsequent generations of bronchi – Usually affect a segmental bronchus (but can also affect lober or subsegmental bronchui) – Apico-posterior segment of left upper lobe is the most common affected segment • Usually presents incidentally in asymptomatic young male adults – Some cases presents with recurrent pneumonias, dyspnea and cough – Although it is a congenital disorder, it is rarely diagnosed in children • The radiology is diagnostic – An ovoid hilar opacity with branches radiating out into a distal area of hyperinflation
- 4. GROSS • Atretic bronchus: – Usually segmental bronchus – Mucocele “bronchocele” distal to site of obstruction • Cystic mass • Blind-ended • Mucus-filled – The atretic bronchus is isolated from the proximal bronchial tree – The subsequent branching pattern distally to it is usually normal • Distal lung parenchyma is hyperinflated – Because of the “collateral ventilation” in one-way direction – via pores of Kohn, the bronchoalveolar channels of Lambert or via interbronchiolar channels
- 5. GROSS Subtypes: • Isolated • Associated with abnormal systemic arterial connection (intralobar sequestration) • Associated with abnormal communication to GI tract (bronchopulmonary foregut malformation)
- 6. MICROSCOPY • Mucocele “Bronchocele” • Dilated large airway • Just distal to the atretic segment • Filled with mucus and mucin-filled macrophages • Distal lung parenchyma – Alveolar overdistention – Contain abundant mucus • Features of infection / fibrosis
- 7. DIFFERENTIAL DIAGNOSIS “ O t h e r c o n g e n i t a l / c y s t i c l u n g d i s e a s e s ” • Congenital: – Bronchogenic cyst – Pulmonary sequestration – Congenital pulmonary cysts – Congenital pulmonary airway malformation – Congenital lobar emphysema • Acquired: – Emphysema – Healed abscess – Honeycombing • Mixed: – Cystic fibrosis Affect the whole lobe (not focal) • Not connected to distal bronchi • Not cause hyperinflation of distal lung
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