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Key Features

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  • Defined as nonfunctional pulmonary tissue that does not communicate with the tracheobronchial tree and receives its blood supply from one or more anomalous systemic arteries

  • Classified as either extralobar or intralobar

    • Extralobar sequestration

      • A mass of pulmonary parenchyma anatomically separate from the normal lung, with a distinct pleural investment

      • Appears as a solitary thoracic lesion near the diaphragm

      • Size varies from 0.5 cm to 12 cm

    • Intralobar sequestration

      • An isolated segment of lung within the normal pleural investment that often receives blood from one or more arteries arising from the aorta or its branches

      • Usually found within the lower lobes (98%), two-thirds are found on the left side, and it is rarely associated with other congenital anomalies (< 2% vs 50% with extralobar sequestration)

      • Rarely presents in the newborn period (unlike extralobar sequestration)

      • Some researchers have hypothesized that intralobar sequestration is an acquired lesion secondary to chronic infection

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Clinical Findings

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  • Chronic cough

  • Wheezing

  • Recurrent pneumonias

  • Hemoptysis in patients with intralobar sequestration (rarely)

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Diagnosis

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Diagnosis is often suggested by CT imaging. Definitive diagnosis can be made by angiography, which shows large systemic arteries perfusing the lesion. Spiral CT scans with contrast or magnetic resonance angiography have proved useful in identifying anomalous systemic arterial supply to the lung.

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Treatment

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Surgical resection

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