Pediatric Pulmonary Sequestration

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

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]

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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.

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Epidemiology

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 pulmonary airway malformation (CPAM) 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.

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Contributor Information and Disclosures
Author

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.

References
  1. Flye MW, Conley M, Silver D. Spectrum of pulmonary sequestration. Ann Thorac Surg. 1976 Nov. 22(5):478-82. [Medline].

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

  3. Abel RM, Bush A, Chitty LS, Harcourt J, Nicholson AG. Congenital lung disease. 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. 1985 Feb. 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. 1970 Apr. 5(2):136-47. [Medline].

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

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

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

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

  10. Clement BS. Congenital malformations of the lungs and airways. 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. 2007 Apr. 133(4):955-9. [Medline].

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

  13. Telander RL, Lennox C, Sieber W. Sequestration of the lung in children. Mayo Clin Proc. 1976 Sep. 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. 2009 Feb. 53(1):22-31. [Medline].

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

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

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

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

  19. Deguchi E, Furukawa T, Ono S, et al. Intralobar pulmonary sequestration diagnosed by MR angiography. Pediatr Surg Int. 2005 Jul. 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. 2006 Nov-Dec. 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. 2004 Jul. 183(1):183-8. [Medline].

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

  23. Laberge JM, Bratu I, Flageole H. The management of asymptomatic congenital lung malformations. Paediatr Respir Rev. 2004. 5 Suppl A:S305-12. [Medline].

  24. Albanese CT, Rothenberg SS. Experience with 144 consecutive pediatric thoracoscopic lobectomies. J Laparoendosc Adv Surg Tech A. 2007 Jun. 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. 1998 Mar. 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. 1982 Nov. 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. 2004 Mar. 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. 1998 Jan. 101(1 Pt 1):120-4. [Medline].

 
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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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