FLASHPATHH A Z E M A L I

INFANTILE (CONGENITAL

) LOBAR EMPHYSEMA

H A Z E M A L I

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

GROSS

• Commonly affect upper lobes “ especially the left one”

• Hyperinflated lobe cause compression to other lobes and mediastinal shift

MICROSCOPY

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

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

DIFFERENTIAL DIAGNOSISAlso 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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