FLASHPATHH A Z E M A L I

BRONCHIAL ATRESIA

H A Z E M A L I

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

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

GROSSSubtypes:• Isolated• Associated with abnormal systemic arterial connection

(intralobar sequestration)• Associated with abnormal communication to GI tract

(bronchopulmonary foregut malformation)

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

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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