eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Pulmonary Sequestration: Differential Diagnoses & Workup

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

Differential Diagnoses

Atelectasis, Pulmonary
Bronchiectasis
Pneumonia

Other Problems to Be Considered

Primary lung abscess
Recurrent pneumonia
Congenital lung mass

Workup

Laboratory Studies

  • No laboratory studies are needed in pulmonary sequestration.

Imaging Studies

  • 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 see...

      The multicystic lesion in the left lower lobe seen on the chest radiograph of this patient was causing repeated respiratory infections.

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      The multicystic lesion in the left lower lobe see...

      The 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 contrast18  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
  • Real-time ultrasonography and Doppler imaging are reliable methods of demonstrating systemic origin or blood supply, as well.22

Other Tests

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

Procedures

  • 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

  • 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

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

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

  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].

  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. 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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