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Pediatric Pulmonary Sequestration Clinical Presentation

  • Author: Bruce M Schnapf, DO; Chief Editor: Michael R Bye, MD  more...
Updated: May 01, 2014


See the list below:

  • 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.[12]
    • 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.


See the list below:

  • 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.


Pulmonary sequestration is believed to result from abnormal diverticulation of foregut and aberrant lung buds.[13]

Contributor Information and Disclosures

Bruce M Schnapf, DO Associate Professor of Pediatrics, Division Chief, Pulmonology, University of South Florida College of Medicine

Bruce M Schnapf, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Charles Callahan, DO Professor, Chief, Department of Pediatrics and Pediatric Pulmonology, Tripler Army Medical Center

Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, Christian Medical and Dental Associations

Disclosure: Nothing to disclose.

Chief Editor

Michael R Bye, MD Professor of Clinical Pediatrics, State University of New York at Buffalo School of Medicine; Attending Physician, Pediatric Pulmonary Division, Women's and Children's Hospital of Buffalo

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

Susanna A McColley, MD Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Director of Cystic Fibrosis Center, Head, Division of Pulmonary Medicine, Children's Memorial Medical Center of Chicago

Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, American Thoracic Society

Disclosure: Received honoraria from Genentech for speaking and teaching; Received honoraria from Genentech for consulting; Partner received consulting fee from Boston Scientific for consulting; Received honoraria from Gilead for speaking and teaching; Received consulting fee from Caremark for consulting; Received honoraria from Vertex Pharmaceuticals for speaking and teaching.

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The multicystic lesion in the left lower lobe seen on the chest radiograph of this patient was causing repeated respiratory infections.
The sequestration (S) is linked by an aberrant vessel (arrows).
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