Medscape is available in 5 Language Editions – Choose your Edition here.


Pediatric Pulmonary Sequestration Workup

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

Laboratory Studies

No laboratory studies are needed in pulmonary sequestration.


Imaging Studies

See the list below:

  • Chest radiography is indicated.[14]
    • Chest radiography findings vary depending on the size of the sequestered lung tissue and whether infection is present. If no communication between sequestration and normal lung tissue is present, radiography usually reveals a dense opacity in the posterior basal segment of the lower lobe. A cystic appearance may also be observed.[15]
      The multicystic lesion in the left lower lobe seenThe multicystic lesion in the left lower lobe seen on the chest radiograph of this patient was causing repeated respiratory infections.
    • Lesion density often increases with secondary infection and appears as a uniform consolidation. When this area fails to clear after a course of appropriate medical therapy, the presence of a malformation, such as sequestration, should be considered, particularly if the lesion is localized to the posterior basal segment of the left lower lobe.
    • Distinguishing an intrapulmonary sequestration from extrapulmonary sequestration is difficult using plain radiography.[16]
      • Intrapulmonary lesions tend to be heterogeneous and are not well defined.
      • Extrapulmonary masses are usually observed as solid, well defined, and retrocardiac.
  • Bronchography and arteriography are unnecessary because of current noninvasive imaging available.[17]
  • Presence of systemic arteries revealed by chest imaging is the major diagnostic feature of pulmonary sequestration. CT scanning with contrast[18] or magnetic resonance angiography (MRA) have been very useful.[19] The arterial supply and venous drainage both should be outlined because of the unpredictability of vascular connections. CT angiography is helpful in identifying aberrant systemic arterial supply, and the 3-dimensional rendering of multidetector row CT scanning can reveal venous drainage.[20, 21]
    The sequestration (S) is linked by an aberrant vesThe sequestration (S) is linked by an aberrant vessel (arrows).
  • Real-time ultrasonography and Doppler imaging are reliable methods of demonstrating systemic origin or blood supply, as well.[22] On prenatal ultrasonography, a sequestration usually appears as well-defined echodense, homogeneous mass. Detection by color flow Doppler of a systemic artery from the aorta to the fetal lung lesion is a pathognomonic feature.

Other Tests

Upper GI contrast examination may be useful if communication with the GI tract is considered.



See the list below:

  • Bronchoscopy: Bronchoscopy is not necessary unless an alternative cause of the radiographic abnormalities, such as an inhaled foreign body, is suspected.
  • Balloon occlusion
    • Consider balloon occlusion or embolization of the aberrant systemic arteries at the time of catheterization.
    • Some patients may have a considerable shunt through this anomalous circuit to the extent that, once the segment has been removed, the improvement in cardiovascular status may be striking.
    • If surgical resection is necessary, risk of vascular complications is greatly reduced with this procedure.

Histologic Findings

See the list below:

  • Because no communication with the bronchial tree occurs, sequestration appears to have loose, spongy tissue with numerous small cystic spaces containing clear, mucoid fluid.
  • Structures that may resemble bronchi are present near the center.
  • Dilated subpleural lymphatics may also be present.[5]
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.

  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].

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).
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.