Case of the Week - Patient Summary 6529Peer Reviewed and Certified -
CT: There is an area of increased lucency in the inferior left lower lobe, with multiple dilated abnormal blood vessels. The area is supplied predominantly by a large artery arising from the aorta just above the aortic hiatus. Drainage is to the left inferior pulmonary vein (not shown).
Cystic Congenital Adenomatoid Malformation
Bronchiectasis
Tuberculosis
Intralobar Pulmonary Sequestration Pathology: Lung, left lower lobe, partial lobe resection. Lung parenchyma and pleura with mucus-filled cyst like bronchi, lymphoid hyperplasia, numerous pulmonary macrophages, thick-walled vessels, and fibrosis consistent with clinical history of pulmonary sequestration.
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In comparison, intralobar sequestrations usually become manifest during adolescence or adulthood, whereas extralobar sequestrations present at birth or soon thereafter (or may be suspected based upon prenatal ultrasonography). Intralobar sequestrations are three times more common with a male predominance, and a much lower incidence of associated anomalies (10% with associated skeletal, foregut, or diaphragmatic anomalies). Extralobar sequestrations may have associated anomalies in up to 65% of patients, including diaphragmatic defects, pulmonary hypoplasia, bronchogenic cysts, and cardiac anomalies. Intralobar sequestrations present with recurrent pneumonias, chronic cough, or dyspnea, or may be completely asymptomatic. Other complications of intralobar sequestrations include hemoptysis, and intrathoracic hemorrhage, and where there is marked vascular shunting, cardiac decompensation. Extralobar sequestrations, presenting at birth or infancy, may manifest as cyanosis, respiratory distress, feeding difficulties, failure to thrive, and recurrent pneumonias. Both predominantly are located in the left base in 60-90%. Treatment of both is resection.
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Animation showing origin of pulmonary vessel from descending thoracic aorta:
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