eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Pulmonary Sequestration

Author: Bruce M Schnapf, DO, Chief, Division of Pulmonology, Department of Pediatrics, Associate Professor, University of South Florida College of Medicine
Contributor Information and Disclosures

Updated: Jun 11, 2009

Introduction

Background

Pulmonary sequestration is a cystic or solid mass composed of nonfunctioning primitive tissue that does not communicate with the tracheobronchial tree and has anomalous systemic blood supply.  It is a type of congenital thoracic malformation. It may present as a lung infection on physical examination and chest imaging. Its blood supply is from systemic circulation rather than the pulmonary circulation. Multiple feeding vessels may be present in 15-20% of cases. The 2 forms of pulmonary sequestration are intrapulmonary, which is surrounded by normal lung tissue, and extrapulmonary, which has its own pleural investment. Other congenital malformations may be present.1

Pathophysiology

The most frequently supported theory of sequestration formation involves an accessory lung bud that develops from the ventral aspect of the primitive foregut. The pluripotential tissue from this additional lung bud migrates in a caudal direction with the normally developing lung. It receives its blood supply from vessels that connect to the aorta and cover the primitive foregut. These attachments to the aorta remain to form the systemic arterial supply of the sequestration.2  

Early embryologic development of the accessory lung bud results in formation of the sequestration within normal lung tissue. The sequestration is encased within the same pleural covering. This is the intrapulmonary variant. In contrast, later development of the accessory lung bud results in the extrapulmonary type that may give rise to communication with the GI tract. Both types of sequestration usually have arterial supply from the thoracic or abdominal aorta. Rarely, the celiac axis, internal mammary, subclavian, or renal artery may be involved.3

Intrapulmonary sequestration occurs within the visceral pleura of normal lung tissue. Usually, no communication with the tracheobronchial tree occurs. The most common location is in the posterior basal segment, and nearly two thirds of pulmonary sequestrations appear in the left lung. Venous drainage is usually via the pulmonary veins.4  Foregut communication is very rare, and associated anomalies are uncommon.

Extrapulmonary sequestration is completely enclosed in its own pleural sac. It may occur above, within, or below the diaphragm, and nearly all appear on the left side. No communication with the tracheobronchial tree occurs. Venous drainage is usually via the systemic venous system. Foregut communication and associated anomalies, such as diaphragmatic hernia, are more common.

Frequency

United States

Pulmonary sequestration represents approximately 6% of all congenital pulmonary malformations.

5  Intrapulmonary sequestrations are the most common form, and 60% of these are found in the posterior basal segment of the left lower lobe. Overall, 98% occur in the lower lobes. Bilateral involvement is uncommon. About 10% of cases may be associated with other congenital anomalies.6 A case of intrapulmonary sequestration associated with a bronchogenic cyst has been reported.7  

Extrapulmonary sequestrations occur on the left in 95% of cases. Of these, 75% are found in the costophrenic sulcus on the left side. They may also be found in the mediastinum, pericardium, and within or below the diaphragm. They are associated with other congenital malformations in more than 50% of cases, such as congenital diaphragmatic hernias, congenital cystic adenomatoid malformation (CCAM) type II (hybrid lesions), and congenital heart disease.8

Mortality/Morbidity

The morbidity and mortality rates are exceedingly low if resection of the mass precedes repeated infection. Postoperative results are uniformly good.9

Sex

In the extrapulmonary form, males are affected approximately 4 times more often than females. Incidence is equal in males and females in the intrapulmonary type.10

Age

More than one half of intrapulmonary sequestrations are diagnosed in later childhood or even in adulthood. Neonates and infants are usually asymptomatic.11 In contrast, more than one half of extrapulmonary sequestrations are diagnosed in patients younger than 1 year. Often, this is because other congenital anomalies are present, including congenital diaphragmatic hernia, cardiac malformations, and GI malformations.

Clinical

History

  • 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.

Physical

  • 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.

Causes

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

More on Pulmonary Sequestration

Overview: Pulmonary Sequestration
Differential Diagnoses & Workup: Pulmonary Sequestration
Treatment & Medication: Pulmonary Sequestration
Follow-up: Pulmonary Sequestration
Multimedia: Pulmonary Sequestration
References
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References

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  2. Corbett HJ, Humphrey GM. Pulmonary sequestration. Paediatr Respir Rev. Mar 2004;5(1):59-68. [Medline].

  3. Abel RM, Bush A, Chitty LS, Harcourt J, Nicholson AG. Congenital lung disease. In: Kendig's Disorders of the Respiratory Tract in Children. 7th ed. Philadelphia, Pa: WB Saunders; 2006:301.

  4. Alivizatos P, Cheatle T, de Leval M, Stark J. Pulmonary sequestration complicated by anomalies of pulmonary venous return. J Pediatr Surg. Feb 1985;20(1):76-9. [Medline].

  5. DeParedes CG, Pierce WS, Johnson DG, Waldhausen JA. Pulmonary sequestration in infants and children: a 20-year experience and review of the literature. J Pediatr Surg. Apr 1970;5(2):136-47. [Medline].

  6. Gustafson RA, Murray GF, Warden HE, et al. Intralobar sequestration. A missed diagnosis. Ann Thorac Surg. Jun 1989;47(6):841-7. [Medline].

  7. Wilson RL, Lettieri CJ, Fitzpatrick TM, Shorr AF. Intralobular bronchopulmonary sequestrations associated with bronchogenic cysts. Respir Med. Apr 2005;99(4):508-10. [Medline].

  8. Hadley GP, Egner J. Gastric duplication with extralobar pulmonary sequestration: an uncommon cause of "colic". Clin Pediatr (Phila). Jun 2001;40(6):364. [Medline].

  9. Collin PP, Desjardins JG, Khan AH. Pulmonary sequestration. J Pediatr Surg. Aug 1987;22(8):750-3. [Medline].

  10. Clement BS. Congenital malformations of the lungs and airways. In: Pediatric Respiratory Medicine. St Louis, Mo: Mosby; 1999:1124-5.

  11. Gezer S, Tastepe I, Sirmali M, et al. Pulmonary sequestration: a single-institutional series composed of 27 cases. J Thorac Cardiovasc Surg. Apr 2007;133(4):955-9. [Medline].

  12. [Guideline] Rosen MJ. Chronic cough due to bronchiectasis: ACCP evidence-based clinical practice guidelines. Chest. Jan 2006;129(1 Suppl):122S-131S. [Medline]. [Full Text].

  13. Telander RL, Lennox C, Sieber W. Sequestration of the lung in children. Mayo Clin Proc. Sep 1976;51(9):578-84. [Medline].

  14. Abbey P, Das CJ, Pangtey GS, Seith A, Dutta R, Kumar A. Imaging in bronchopulmonary sequestration. J Med Imaging Radiat Oncol. Feb 2009;53(1):22-31. [Medline].

  15. Torreggiani WC, Logan PM, McElvaney NG. Persistant right lower lobe consolidation. Chest. Feb 2000;117(2):588-90. [Medline].

  16. Ko SF, Ng SH, Lee TY, et al. Noninvasive imaging of bronchopulmonary sequestration. AJR Am J Roentgenol. Oct 2000;175(4):1005-12. [Medline].

  17. Paterson A. Imaging evaluation of congenital lung abnormalities in infants and children. Radiol Clin North Am. Mar 2005;43(2):303-23. [Medline].

  18. Ikezoe J, Murayama S, Godwin JD, et al. Bronchopulmonary sequestration: CT assessment. Radiology. Aug 1990;176(2):375-9. [Medline].

  19. Deguchi E, Furukawa T, Ono S, et al. Intralobar pulmonary sequestration diagnosed by MR angiography. Pediatr Surg Int. Jul 2005;21(7):576-7. [Medline].

  20. Kang M, Khandelwal N, Ojili V, Rao KL, Rana SS. Multidetector CT angiography in pulmonary sequestration. J Comput Assist Tomogr. Nov-Dec 2006;30(6):926-32. [Medline].

  21. Lee EY, Siegel MJ, Sierra LM, Foglia RP. Evaluation of angioarchitecture of pulmonary sequestration in pediatric patients using 3D MDCT angiography. AJR Am J Roentgenol. Jul 2004;183(1):183-8. [Medline].

  22. Adzick NS, Harrison MR, Crombleholme TM, et al. Fetal lung lesions: management and outcome. Am J Obstet Gynecol. Oct 1998;179(4):884-9. [Medline].

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  24. Albanese CT, Rothenberg SS. Experience with 144 consecutive pediatric thoracoscopic lobectomies. J Laparoendosc Adv Surg Tech A. Jun 2007;17(3):339-41. [Medline].

  25. Ko SC, Chang YC, Liaw YS, et al. Diagnosis of pulmonary sequestration by magnetic resonance imaging. J Formos Med Assoc. Mar 1998;97(3):220-3. [Medline].

  26. Levine MM, Nudel DB, Gootman N, et al. Pulmonary sequestration causing congestive heart failure in infancy: a report of two cases and review of the literature. Ann Thorac Surg. Nov 1982;34(5):581-5. [Medline].

  27. Sersar Sameh I, El Diasty M, Ibrahim Hammad R, et al. Lower lobe segments and pulmonary sequestrations. J Thorac Cardiovasc Surg. Mar 2004;127(3):898-9. [Medline].

  28. Spinella PC, Strieper MJ, Callahan CW. Congestive heart failure in a neonate secondary to bilateral intralobar and extralobar pulmonary sequestrations. Pediatrics. Jan 1998;101(1 Pt 1):120-4. [Medline].

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Further Reading

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Keywords

pulmonary sequestration, extrapulmonary sequestration, intrapulmonary sequestration, sequestrum, lung bud, congenital lung malformation, pneumonia, lung injury, respiratory distress, chronic cough, feeding difficulties, treatment, diagnosis

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Contributor Information and Disclosures

Author

Bruce M Schnapf, DO, Chief, Division of Pulmonology, Department of Pediatrics, Associate Professor, 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, and American Thoracic Society
Disclosure: Nothing to disclose.

Medical Editor

Susanna A McColley, MD, Director of Cystic Fibrosis Center; Head, Division of Pulmonary Medicine; Associate Professor, Department of Pediatrics, Children's Memorial Medical Center of Chicago, Northwestern University
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, and American Thoracic Society
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Consulting fee Consulting; Novartis Consulting fee Consulting; Altus Consulting fee Consulting; Axcan Scandi Consulting fee Consulting; Boston Scientific Consulting fee Consulting

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Charles Callahan, DO, Professor, Deputy Chief of Clinical Services, Walter Reed 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, and Christian Medical & Dental Society
Disclosure: Nothing to disclose.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD, Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching

 
 
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