Pediatric Pulmonary Sequestration Clinical Presentation

Updated: Oct 27, 2021
  • Author: Bruce M Schnapf, DO; Chief Editor: Girish D Sharma, MD, FCCP, FAAP  more...
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Intrapulmonary sequestration

Although an intrapulmonary sequestration is usually diagnosed later in childhood or adolescence, symptoms may begin early in childhood with multiple episodes of pneumonia. A chronic or recurrent cough is common. Intrapulmonary sequestration shares the visceral pleura that covers the adjacent lung tissue and is usually located in the posterobasal segment of the lower lobes. The thoracic or abdominal aorta often provides the arterial blood supply. Venous drainage is commonly provided to the left atrium via the pulmonary veins.

An elemental communication with other bronchi or lung parenchyma may be present, allowing infection to occur. Rarely, an esophageal bronchus may be present. Resolution of infection is usually slow and incomplete because of inadequate bronchial drainage.

Overdistension of the cystic mass with air can result in compression of normal lung tissue with impairment of cardiorespiratory function. Aeration probably occurs through the pores of Kohn.

Other congenital anomalies may appear in 10% of cases.

Extrapulmonary sequestration

Many patients present in infancy with respiratory distress and chronic cough. The American College of Chest Physicians has established guidelines for the evaluation of chronic cough. [14]

Lesions are commonly diagnosed coincidentally during investigation of, or surgery for, an associated congenital anomaly. Therefore, clinical symptoms may be absent or minor.

Extrapulmonary sequestration may manifest as GI symptoms if communication with the GI tract is present. As a result, infants may have feeding difficulties. In addition, extrapulmonary sequestration may manifest as recurrent lung infection, similar to the intrapulmonary form. This type of sequestration does not contain air unless communication with the foregut is present.


Physical Examination

The lung examination may reveal signs consistent with consolidation.

Occasionally, patients may have a systolic bruit or continuous murmur over the affected area. This is related to flow through the sequestration from the large systemic arterial supply.