Congenital lobar emphysema is a rare condition where one or more lung lobes are abnormally enlarged due to partial obstruction of the bronchus supplying the lobe. It usually presents within the first 6 months of life with respiratory distress. Grossly, the affected lobe is hyperinflated and can compress other lobes. Microscopically, the alveoli are distended without destruction of walls. It must be differentiated from other congenital lung diseases and pneumothorax.

1. FLASHPATH
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2. INFANTILE
(CONGENITAL)
LOBAR EMPHYSEMA
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3. CLINICAL
• Rare, idiopathic hyperinflation of one or more lobes of the lung
– May be due to Partial obstruction of lobar bronchus supplying the
developing lobe
• Intrinsic obstruction: by mucus plug or cartilage hypoplasia
• Extrinsic obstruction: by cyst or tumor
• Usually present within first 6 months of life, males > females
• Present with respiratory distress (tachypnea, cyanosis, wheezing, difficult
breathing), recurrent pneumonia and failure to thrive
• Associated with cardiovascular anomalies in 14% of cases

4. GROSS
• Commonly affect upper lobes “ especially the left one”
• Hyperinflated lobe cause compression to other lobes and mediastinal
shift

5. MICROSCOPY
• Alveolar distension without actual wall destruction
– So it is not “true” emphysema

6. 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:
– Pulmonary sequestration
– Congenital pulmonary cysts
– Congenital pulmonary airway malformation
– Congenital lobar emphysema
• Acquired:
– Emphysema
– Healed abscess
– Honeycombing
• Mixed:
– Cystic fibrosis

7. DIFFERENTIAL DIAGNOSIS
Also can be misdiagnosed clinically as “pneumothorax”
• Pneumothorax lacks the linear bronchovascular and alveolar markings
seen radiologically in congenital lobar emphysema
• Treatments aimed at pneumothorax can worsen patient’s actual
congenital lobar emphysema

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