Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Prenatal diagnosis and management of bronchopulmonary sequestration

Dorothy I Bulas, MD
Alexia Egloff, MD
Section Editors
Deborah Levine, MD
Louise Wilkins-Haug, MD, PhD
Deputy Editor
Vanessa A Barss, MD, FACOG


Bronchopulmonary sequestration (BPS) is a congenital anomaly consisting of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its blood supply from the systemic (rather than the pulmonary) circulation [1]. The feeding vessel most commonly originates from the aorta, although other origins, such as the splenic artery or gastric artery, have been described [2]. BPS accounts for only 0.15 to 6.4 percent of congenital lung malformations [3]. Eighty percent of cases occur on the left [4].

BPS is classified anatomically as intralobar or extralobar:

In intralobar pulmonary sequestration (ILS, also known as intrapulmonary sequestration), the mass is located within the normal lung and is covered by the visceral pleura of the lung. Postnatally, ILS accounts for about 75 percent of BPS and is almost always located in the lower lobes, most commonly in the left medial or posterior segment [5,6]. Venous drainage is usually via the pulmonary vein to the left atrium, although connections to the vena cava, azygous vein, or right atrium also occur. ILS is distributed equally between sexes.

In extralobar pulmonary sequestration (ELS, also known as extrapulmonary sequestration), the mass is located outside the normal lung and has its own visceral pleura. Postnatally, about 25 percent of BPS is extralobar [7]. An extralobar lesion can occur between the neck and diaphragm, within the diaphragm, or infradiaphragmatically, where it can mimic a suprarenal neuroblastoma [5,7,8]. The most common location is between the left lower lobe and hemidiaphragm (80 percent) [6,9]. Ten to 15 percent are subdiaphragmatic, usually in the suprarenal space and left-sided (90 percent) [10]. A case report described ELS with combined gastric and intradiaphragmatic locations [11].

Most ELS drains to the systemic circulation through the azygous or hemiazygous vein or vena cava [3]. ELS is more likely to affect males and to be associated with anomalies, including congenital diaphragmatic hernia or eventration, cardiac defects, and foregut anomalies.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Aug 15, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. PRYCE DM. Lower accessory pulmonary artery with intralobar sequestration of lung; a report of seven cases. J Pathol Bacteriol 1946; 58:457.
  2. Gomez L, Robert JA, Sepulveda W. Fetal retroperitoneal pulmonary sequestration with an atypical vascular pattern. Prenat Diagn 2009; 29:290.
  3. Vijayaraghavan SB, Rao PS, Selvarasu CD, Rao TM. Prenatal sonographic features of intralobar bronchopulmonary sequestration. J Ultrasound Med 2003; 22:541.
  4. Cruz-Martinez R, Méndez A, Dueñas-Riaño J, et al. Fetal laser surgery prevents fetal death and avoids the need for neonatal sequestrectomy in cases with bronchopulmonary sequestration. Ultrasound Obstet Gynecol 2015; 46:627.
  5. Azizkhan RG, Crombleholme TM. Congenital cystic lung disease: contemporary antenatal and postnatal management. Pediatr Surg Int 2008; 24:643.
  6. Abbey P, Das CJ, Pangtey GS, et al. Imaging in bronchopulmonary sequestration. J Med Imaging Radiat Oncol 2009; 53:22.
  7. Epelman M, Kreiger PA, Servaes S, et al. Current imaging of prenatally diagnosed congenital lung lesions. Semin Ultrasound CT MR 2010; 31:141.
  8. Nijagal A, Jelin E, Feldstein VA, et al. The diagnosis and management of intradiaphragmatic extralobar pulmonary sequestrations: a report of 4 cases. J Pediatr Surg 2012; 47:1501.
  9. Houda el M, Ahmed Z, Amine K, et al. Antenatal diagnosis of extralobar pulmonar sequestration. Pan Afr Med J 2014; 19:54.
  10. Maki E, Oh K, Rogers S, Sohaey R. Imaging and differential diagnosis of suprarenal masses in the fetus. J Ultrasound Med 2014; 33:895.
  11. Chouikh T, Berteloot L, Revillon Y, et al. Extralobar pulmonary sequestration with combined gastric and intradiaphragmatic locations. Pediatr Pulmonol 2014; 49:512.
  12. Maas KL, Feldstein VA, Goldstein RB, Filly RA. Sonographic detection of bilateral fetal chest masses: report of three cases. J Ultrasound Med 1997; 16:647.
  13. Hubbard AM, Adzick NS, Crombleholme TM, et al. Congenital chest lesions: diagnosis and characterization with prenatal MR imaging. Radiology 1999; 212:43.
  14. Sepulveda W. Perinatal imaging in bronchopulmonary sequestration. J Ultrasound Med 2009; 28:89.
  15. Zhang H, Tian J, Chen Z, et al. Retrospective study of prenatal diagnosed pulmonary sequestration. Pediatr Surg Int 2014; 30:47.
  16. Daltro P, Werner H, Gasparetto TD, et al. Congenital chest malformations: a multimodality approach with emphasis on fetal MR imaging. Radiographics 2010; 30:385.
  17. Pariente G, Aviram M, Landau D, Hershkovitz R. Prenatal diagnosis of congenital lobar emphysema: case report and review of the literature. J Ultrasound Med 2009; 28:1081.
  18. Zeidan S, Gorincour G, Potier A, et al. Congenital lung malformation: evaluation of prenatal and postnatal radiological findings. Respirology 2009; 14:1005.
  19. Bush A, Hogg J, Chitty LS. Cystic lung lesions - prenatal diagnosis and management. Prenat Diagn 2008; 28:604.
  20. Durell J, Thakkar H, Gould S, et al. Pathology of asymptomatic, prenatally diagnosed cystic lung malformations. J Pediatr Surg 2016; 51:231.
  21. Adzick NS, Harrison MR, Crombleholme TM, et al. Fetal lung lesions: management and outcome. Am J Obstet Gynecol 1998; 179:884.
  22. Breysem L, Bosmans H, Dymarkowski S, et al. The value of fast MR imaging as an adjunct to ultrasound in prenatal diagnosis. Eur Radiol 2003; 13:1538.
  23. Lecomte B, Hadden H, Coste K, et al. Hyperechoic congenital lung lesions in a non-selected population: from prenatal detection till perinatal management. Prenat Diagn 2009; 29:1222.
  24. Coleman AM, Merrow AC, Crombleholme TM, et al. Fetal MRI of Torsed Bronchopulmonary Sequestration with Tension Hydrothorax and Hydrops in a Twin Gestation. Fetal Diagn Ther 2016; 40:156.
  25. Grethel EJ, Wagner AJ, Clifton MS, et al. Fetal intervention for mass lesions and hydrops improves outcome: a 15-year experience. J Pediatr Surg 2007; 42:117.
  26. Gerle RD, Jaretzki A 3rd, Ashley CA, Berne AS. Congenital bronchopulmonary-foregut malformation. Pulmonary sequestration communicating with the gastrointestinal tract. N Engl J Med 1968; 278:1413.
  27. Pinto RM, Araujo Júnior E, Augusto LC, et al. Spontaneous regression of intralobar pulmonary sequestration during the pregnancy: report of two cases through relationships between mass and fetal biometry and review of the literature. J Matern Fetal Neonatal Med 2016; 29:1720.
  28. Flanagan S, Rubesova E, Hintz S, et al. Prenatal imaging of bronchopulmonary malformations: Is there a role for late third trimester fetal MRI? Pediatr Radiol 2013; 43:205.
  29. Knox EM, Kilby MD, Martin WL, Khan KS. In-utero pulmonary drainage in the management of primary hydrothorax and congenital cystic lung lesion: a systematic review. Ultrasound Obstet Gynecol 2006; 28:726.
  30. Bruner JP, Jarnagin BK, Reinisch L. Percutaneous laser ablation of fetal congenital cystic adenomatoid malformation: too little, too late? Fetal Diagn Ther 2000; 15:359.
  31. Mann S, Wilson RD, Bebbington MW, et al. Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med 2007; 12:477.
  32. Wilson RD. In utero therapy for fetal thoracic abnormalities. Prenat Diagn 2008; 28:619.
  33. Mychaliska GB, Bryner BS, Nugent C, et al. Giant pulmonary sequestration: the rare case requiring the EXIT procedure with resection and ECMO. Fetal Diagn Ther 2009; 25:163.
  34. Witlox RS, Lopriore E, Walther FJ, et al. Single-needle laser treatment with drainage of hydrothorax in fetal bronchopulmonary sequestration with hydrops. Ultrasound Obstet Gynecol 2009; 34:355.
  35. Oepkes D, Devlieger R, Lopriore E, Klumper FJ. Successful ultrasound-guided laser treatment of fetal hydrops caused by pulmonary sequestration. Ultrasound Obstet Gynecol 2007; 29:457.
  36. Ruano R, de A Pimenta EJ, Marques da Silva M, et al. Percutaneous intrauterine laser ablation of the abnormal vessel in pulmonary sequestration with hydrops at 29 weeks' gestation. J Ultrasound Med 2007; 26:1235.
  37. Mallmann MR, Geipel A, Bludau M, et al. Bronchopulmonary sequestration with massive pleural effusion: pleuroamniotic shunting vs intrafetal vascular laser ablation. Ultrasound Obstet Gynecol 2014; 44:441.
  38. Ruano R, da Silva MM, Salustiano EM, et al. Percutaneous laser ablation under ultrasound guidance for fetal hyperechogenic microcystic lung lesions with hydrops: a single center cohort and a literature review. Prenat Diagn 2012; 32:1127.
  39. Bermúdez C, Pérez-Wulff J, Bufalino G, et al. Percutaneous ultrasound-guided sclerotherapy for complicated fetal intralobar bronchopulmonary sequestration. Ultrasound Obstet Gynecol 2007; 29:586.
  40. Adzick NS. Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med 2010; 15:1.