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Virtual Pediatric Hospital: Paediapaedia: Sequestration Paediapaedia: Chest and Airway Diseases

Sequestration

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Usually has symptoms of recurrent respiratory tract infections or respiratory distress if the sequestration is large enough to compress adjacent lung.

Etiology/Pathophysiology:
Congenital mass of dysplastic lung tissue without normal tracheobronchial or vascular connections. The arterial blood supply usually comes from an aortic vessel beneath the diaphragm.

There are two types: (1) Intralobar - the mass is within the pulmonary visceral pleura and drains via pulmonary veins. (2) Extra lobar - the mass is separate with its own pleural investment and drains via systemic veins. It is thought to come from an accessory lung bud. If this bud develops early before the pleura develops it becomes an intralobar sequestration, if this bud develops later, after the pleura develops, it becomes an extralobar sequestration.

Pathology:
Dysplastic lung tissue

Imaging Findings:
The posterior basal segment of the lung is most frequently involved with a slight left sided predominance. Can look like a solid mass, a cystic lesion with and without air fluid levels, or an inflammatory infiltrate.

DDX:

References:

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