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.^[14]

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 Examination

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.

Differential Diagnoses

Flye MW, Conley M, Silver D. Spectrum of pulmonary sequestration. Ann Thorac Surg. 1976 Nov. 22(5):478-82. [QxMD MEDLINE Link].
Telander RL, Lennox C, Sieber W. Sequestration of the lung in children. Mayo Clin Proc. 1976 Sep. 51(9):578-84. [QxMD MEDLINE Link].
Corbett HJ, Humphrey GM. Pulmonary sequestration. Paediatr Respir Rev. 2004 Mar. 5(1):59-68. [QxMD MEDLINE Link].
Abel RM, Bush A, Chitty LS, Harcourt J, Nicholson AG. Congenital lung disease. Kendig's Disorders of the Respiratory Tract in Children. 8th ed. Philadelphia, Pa: WB Saunders; 2012. 317-357.
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Gustafson RA, Murray GF, Warden HE, et al. Intralobar sequestration. A missed diagnosis. Ann Thorac Surg. 1989 Jun. 47(6):841-7. [QxMD MEDLINE Link].
Wilson RL, Lettieri CJ, Fitzpatrick TM, Shorr AF. Intralobular bronchopulmonary sequestrations associated with bronchogenic cysts. Respir Med. 2005 Apr. 99(4):508-10. [QxMD MEDLINE Link].
Hadley GP, Egner J. Gastric duplication with extralobar pulmonary sequestration: an uncommon cause of "colic". Clin Pediatr (Phila). 2001 Jun. 40(6):364. [QxMD MEDLINE Link].
Fowler DJ, Gould SJ. The pathology of congenital lung lesions. Semin Pediatr Surg. 2015. 24:176-182. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Trabalza Marinucci B, Maurizi G, Vanni C, et al. Surgical treatment of pulmonary sequestration in adults and children: long-term results. Interact Cardiovasc Thorac Surg. 2020 Jul 1. 31 (1):71-7. [QxMD MEDLINE Link].
Collin PP, Desjardins JG, Khan AH. Pulmonary sequestration. J Pediatr Surg. 1987 Aug. 22(8):750-3. [QxMD MEDLINE Link].
[Guideline] Rosen MJ. Chronic cough due to bronchiectasis: ACCP evidence-based clinical practice guidelines. Chest. 2006 Jan. 129(1 Suppl):122S-131S. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link].
Torreggiani WC, Logan PM, McElvaney NG. Persistant right lower lobe consolidation. Chest. 2000 Feb. 117(2):588-90. [QxMD MEDLINE Link].
Ko SF, Ng SH, Lee TY, et al. Noninvasive imaging of bronchopulmonary sequestration. AJR Am J Roentgenol. 2000 Oct. 175(4):1005-12. [QxMD MEDLINE Link].
Chowdhury MM, Chakraborty S. Imaging of congenital lung malformations. Semin Pediatr Surg. 2015. 24:168-175. [QxMD MEDLINE Link].
Ikezoe J, Murayama S, Godwin JD, et al. Bronchopulmonary sequestration: CT assessment. Radiology. 1990 Aug. 176(2):375-9. [QxMD MEDLINE Link].
Deguchi E, Furukawa T, Ono S, et al. Intralobar pulmonary sequestration diagnosed by MR angiography. Pediatr Surg Int. 2005 Jul. 21(7):576-7. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Adzick NS, Harrison MR, Crombleholme TM, et al. Fetal lung lesions: management and outcome. Am J Obstet Gynecol. 1998 Oct. 179(4):884-9. [QxMD MEDLINE Link].
Laberge JM, Bratu I, Flageole H. The management of asymptomatic congenital lung malformations. Paediatr Respir Rev. 2004. 5 Suppl A:S305-12. [QxMD MEDLINE Link].
Albanese CT, Rothenberg SS. Experience with 144 consecutive pediatric thoracoscopic lobectomies. J Laparoendosc Adv Surg Tech A. 2007 Jun. 17(3):339-41. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].

Media Gallery

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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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

Girish D Sharma, MD, FCCP, FAAP Professor of Pediatrics, Rush Medical College; Director, Section of Pediatric Pulmonology and Rush Cystic Fibrosis Center, Rush Children's Hospital, Rush University Medical Center

Girish D Sharma, MD, FCCP, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, Royal College of Physicians of Ireland

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.

Sections Pediatric Pulmonary Sequestration
Overview
Presentation
DDx
Workup
Treatment
Media Gallery
References

Pediatric Pulmonary Sequestration Clinical Presentation

History

Intrapulmonary sequestration

Extrapulmonary sequestration

Physical Examination

References

Tables

Contributor Information and Disclosures

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