Skip to main content
SpringerLink
Log in

Congenital Lung Malformations

  • Reference work entry
  • First Online:
Airway Diseases

  • 689 Accesses

Abstract

Congenital lung malformations (CLMs) consist of congenital developmental abnormalities of airways, lung parenchyma, and pulmonary blood vessels [1]. There is a controversy about the developmental mechanism of these malformations, and thereby there are different classifications for these malformations defined in the literature [1–5]. Main reason of the presence of these varying classifications is the uncertainty of the embryological mechanism of formation of these lesions. Another reason is that the viewpoints of different specialties vary widely. Pathologist, perinatologist, radiologist, and finally surgeons have considerable different approaches to these malformations. Another point is that, while some of these classifications are aimed for academic definitions, some others are for daily practical use. Nowadays, it is generally accepted that congenital lung malformations are parts of a continuum of developmental abnormalities.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 949.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 949.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Langston C. New concepts in the pathology of congenital lung malformations. Semin Pediatr Surg. 2003;12:17–37.

    PubMed  Google Scholar 

  2. Clements BS, Warner JO. Pulmonary sequestration and related congenital bronchopulmonary-vascular malformations: nomenclature and classification based on anatomical and embryological considerations. Thorax. 1987;42(6):401–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  3. Bush A. Congenital lung disease: a plea for clear thinking and clear nomenclature. Pediatr Pulmonol. 2001;32:328–37.

    CAS  PubMed  Google Scholar 

  4. Seear M, Townsend J, Hoepker A, et al. A review of congenital lung malformations with a simplified classification system for clinical and research use. Pediatr Surg Int. 2017;33:657–64.

    PubMed  Google Scholar 

  5. Achiron R, Hegesh J, Yagel S. Fetal lung lesions: a spectrum of disease. New classification based on pathogenesis, two dimensional and color doppler ultrasound. Ultrasound Obstet Gynecol. 2004;24:107–14.

    CAS  PubMed  Google Scholar 

  6. Mullassery D. Lung development. Semin Pediatr Sugr. 2015;24:152–5.

    Google Scholar 

  7. Wong KKY, Flake AW, Tibboel D, et al. Congenital pulmonary airway malformation: advances and controversies. Lancet Child Adolesc Health. 2018;2(4):290–7.

    PubMed  Google Scholar 

  8. Adzick NS, Harrison MR, Glick PL, et al. Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg. 1985;20:483–8.

    CAS  PubMed  Google Scholar 

  9. Riedlinger W, Vargas S, Jennings R, et al. Bronchial atresia is common to extralobar sequestration, intralobar sequestration, congenital cystic adenomatoid malformation, and lobar emphysema. Pediatr Dev Pathol. 2006;9:361–73.

    PubMed  Google Scholar 

  10. Kunisaki SM, Fauza DO, Nemes LP, et al. Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses. J Pediatr Surg. 2006;41:61–5.

    PubMed  Google Scholar 

  11. Watanabe T, Ohno M, Tahara K, et al. An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia. J Pediatr Surg. 2018;53:2390–3.

    PubMed  Google Scholar 

  12. Stocker JT. Cystic lung disease in infants and children. Fetal Pediatr Pathol. 2009;28:155–84.

    PubMed  Google Scholar 

  13. Burge D, Wheeler R. Increasing incidence of detection of congenital lung lesions. Pediatr Pulmonol. 2010;45:103.

    PubMed  Google Scholar 

  14. Lima JS, Camargos PA, Aguiar RA, et al. Pre and perinatal aspects of congenital cystic adenomatoid malformation of the lung. J Matern Fetal Neonatal Med. 2014;27:228–32.

    PubMed  Google Scholar 

  15. Ehrenberg-Buchner S, Stapf AM, Berman DR, et al. Fetal lung lesions: can we start to breathe easier? Am J Obstet Gynecol. 2013;208:151–7.

    Google Scholar 

  16. Macardle CA, Ehrenberg-Buchner S, Smith EA, et al. Surveillance of fetal lung lesions using the congenital pulmonary airway malformation volume ratio: natural history and outcomes. Prenat Diagn. 2016;36:282–9.

    PubMed  Google Scholar 

  17. Kunisaki SM, Saito JM, Fallat ME, et al. Development of a multi-institutional registry for children with operative congenital lung malformations. J Pediatr Surg. 2019;55:1313–8.

    PubMed  Google Scholar 

  18. Kunisaki SM, Ehrenberg-Buchner S, Dillman JR, et al. Vanishing fetal lung malformations: prenatal sonographic characteristics and postnatal outcomes. J Pediatr Surg. 2015;50:978–82.

    PubMed  Google Scholar 

  19. Crombleholme TM, Coleman B, Hedrick H, et al. Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg. 2002;37:331–8.

    PubMed  Google Scholar 

  20. Mon RA, Johnson KN, Ladino-Torres M, et al. Diagnostic accuracy of imaging studies in congenital lung malformations. Arch Dis Child Fetal Neonatal Ed. 2019;104:372–7.

    Google Scholar 

  21. Cass DL, Olutoye OO, Cassady CI, et al. Prenatal diagnosis and outcome of fetal lung masses. J Pediatr Surg. 2011;46:292–8.

    PubMed  Google Scholar 

  22. Adzick NS. Management of fetal lung lesions. Clin Perinatol. 2009;36:363–76.

    PubMed  Google Scholar 

  23. Cass DL, Olutoye OO, Ayres NA, et al. Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors. J Pediatr Surg. 2012;47:40–5.

    PubMed  Google Scholar 

  24. Kunisaki SM, Saito JM, Fallat ME, et al. Fetal risk stratification and outcomes in children with prenatally diagnosed lung malformations: results from a multi-institutional research collaborative. Ann Surg; 2020.

    Google Scholar 

  25. Peranteau WH, Wilson RD, Liechty KW, et al. Effect on maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival. Fetal Diagn Ther. 2007;22:365–71.

    PubMed  Google Scholar 

  26. Morris LM, Lim FY, Livingston JC, et al. High-risk fetal congenital pulmonary airway malformations have a variable response to steroids. J Pediatr Surg. 2009;44:60–5.

    PubMed  Google Scholar 

  27. Stocker JT, Madewll JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol. 1977;8:155–71.

    CAS  PubMed  Google Scholar 

  28. Tsai AY, Liechty KW, Hedrick HL, et al. Outcomes after postnatal resection of prenatally diagnosed asymptomatic cystic lung lesions. J Pediatr Surg. 2008;43:513–7.

    PubMed  Google Scholar 

  29. Cass DL, Crombleholme TM, Howell IJ, et al. Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg. 1997;32:986–90.

    CAS  PubMed  Google Scholar 

  30. Langston C. Intralobar sequestration, revisited. Pediatr Dev Pathol. 2003;6:283.

    PubMed  Google Scholar 

  31. Feinberg A, Hall NJ, Williams GM, et al. Can congenital pulmonary airway malformation be distinguished from type I pleuropulmonary blastoma based on clinical and radiological features? J Pediatr Surg. 2016;51:33–7.

    PubMed  Google Scholar 

  32. Kunisaki SM, Leys CM. Surgical pulmonary and pleural diseases in children: lung malformations, empyema, and spontaneous pneumothorax. Adv Pediatr. 2020;67:145–69.

    PubMed  Google Scholar 

  33. Fievet L, D’Journo XB, Guys JM, et al. Bronchogenic cyst: best time for surgery? Ann Thorac Surg. 2012;94:1695–9.

    PubMed  Google Scholar 

  34. St-Georges R, Deslauriers J, Duranceau A, et al. Clinical spectrum of bronchogenic cysts of the mediastinum and lung in the adult. Ann Thorac Surg. 1991;52:6–13.

    CAS  PubMed  Google Scholar 

  35. Limaïem F, Ayadi-Kaddour A, Djilani H, et al. Pulmonary and mediastinal bronchogenic cysts: a clinicopathologic study of 33 cases. Lung. 2008;186:55–61.

    PubMed  Google Scholar 

  36. Kunisaki SM, Saito JM, Fallat ME, et al. Current operative management of congenital lobar emphysema in children: a report from the Midwest Pediatric Surgery Consortium. J Pediatr Surg. 2019;54:1138–42.

    PubMed  Google Scholar 

  37. Tapper D, Schuster S, McBride J, et al. Polyalveolar lobe: anatomic and physiologic parameters and their relationship to congenital lobar emphysema. J Pediatr Surg. 1980;15:931–7.

    CAS  PubMed  Google Scholar 

  38. Mei-Zahav M, Konen O, Manson D, et al. Is congenital lobar emphysema a surgical disease? J Pediatr Surg. 2006;41:1058–61.

    PubMed  Google Scholar 

  39. Haller JA Jr, Golladay ES, Pickard LR, et al. Surgical management of lung bud anomalies: lobar emphysema, bronchogenic cyst, cystic adenomatoid malformation, and intralobar pulmonary sequestration. Ann Thorac Surg. 1979;28:33–43.

    PubMed  Google Scholar 

  40. Glenski JA, Thibeault DW, Hall FK, et al. Selective bronchial intubation in infants with lobar emphysema: indications, complications, and long term outcome. Am J Perinatol. 1986;3:199–204.

    CAS  PubMed  Google Scholar 

  41. Moideen I, Nair SG, Cherian A, et al. Congenital lobar emphysema associated with congenital heart disease. J Cardiothorac Vasc Anesth. 2006;20:239–41.

    PubMed  Google Scholar 

  42. Kulaylat AN, Engbrecht BW, Hollenbeak CS, et al. Comparing 30-day outcomes between thoracoscopic and open approaches for resection of pediatric congenital lung malformations: evidence from NSQIP. J Pediatr Surg. 2015;50:1716–21.

    PubMed  Google Scholar 

  43. Rahman N, Lakhoo K. Comparison between open and thoracoscopic resection of congenital lung lesions. J Pediatr Surg. 2009;44:333–6.

    CAS  PubMed  Google Scholar 

  44. Singh R, Davenport M. The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg. 2015;24:187–95.

    PubMed  Google Scholar 

  45. Rothenberg SS, Middlesworth W, Kadennhe-Chiweshe A, et al. Two decades of experience with thoracoscopic lobectomy in infants and children: standardizing techniques for advanced thoracoscopic surgery. J Laparoendosc Adv Surg Tech A. 2015;25:423–8.

    PubMed  Google Scholar 

  46. Kunisaki SM, Powelson IA, Haydar B, et al. Thoracoscopic vs open lobectomy in infants and young children with congenital lung malformations. J Am Coll Surg. 2014;218:261–70.

    PubMed  Google Scholar 

  47. Hall NJ, Chiu PPL, Langer J. Morbidity after elective resection of prenatally diagnosed asymptomatic congenital pulmonary airway malformations. Pediatr Pulmonol. 2016;51:525–30.

    PubMed  Google Scholar 

  48. Criss CN, Musili N, Matusko N, et al. Asymptomatic congenital lung malformations: is nonoperative management a viable alternative? J Pediatr Surg. 2018;53:1092–7.

    PubMed  Google Scholar 

  49. Ng C, Stanwell J, Burge DM, et al. Conservative management of antenatally diagnosed cystic lung malformations. Arch Dis Child. 2014;99:432–7.

    PubMed  Google Scholar 

  50. Stanton M, Njere I, Ade-Ajayi N, et al. Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions. J Pediatr Surg. 2009;44:1027–33.

    PubMed  Google Scholar 

  51. Kapralik J, Wayne C, Chan E, et al. Surgical versus conservative management of congenital pulmonary airway malformation in children: a systematic review and meta-analysis. J Pediatr Surg. 2016;51:508–12.

    PubMed  Google Scholar 

  52. Jelin EB, O’Hare EM, Jancelewicz T, et al. Optimal timing for elective resection of asymptomatic congenital pulmonary airway malformations. J Pediatr Surg. 2018;53:1001–5.

    PubMed  Google Scholar 

  53. Style CC, Cass DL, Verla MA, et al. Early vs late resection of asymptomatic congenital lung malformations. J Pediatr Surg. 2019;54:70–4.

    PubMed  Google Scholar 

  54. Duron V, Zenilman A, Griggs C, et al. Asymptomatic congenital lung malformations: timing of resection does not affect adverse surgical outcomes. Front Pediatr. 2020;14:35–.

    Google Scholar 

  55. Rothenberg SS, Kuenzler KA, Middlesworth W, et al. Thoracoscopic lobectomy in infants less than 10 kg with prenatally diagnosed cystic lung disease. J Laparoendosc Adv Surg Tech A. 2011;21:181–4.

    PubMed  Google Scholar 

  56. Johnson KN, Mon RA, Gadepalli SK, et al. Short term respiratory outcomes of neonates with symptomatic congenital lung malformations. J Pediatr Surg. 2019;54:1766–70.

    PubMed  Google Scholar 

  57. Rothenberg SS. Thoracoscopic pulmonary surgery. Seminars Pediatr Surg. 2007;16:231–7.

    Google Scholar 

  58. Fascetti-Leon F, Gobbi D, Pavia SV, et al. Sparing-lung surgery for the treatment of congenital lung malformations. J Pediatr Surg. 2013;48:1476–80.

    PubMed  Google Scholar 

  59. Krivchenya DU, Rudenko EO, Dubrovin AG. Congenital emphysema in children: segmental lung resection as an alternative to lobectomy. J Pediatr Surg. 2013;48:309–14.

    PubMed  Google Scholar 

  60. Adams S, Jobson M, Sangnawakij B, et al. Does thoracoscopy have advantages over open surgery for asymptomatic congenital lung malformations? An analysis of 1626 resections. J Pediatr Surg. 2017;52:247–51.

    PubMed  Google Scholar 

  61. Rothenberg SS. First decade’s experience with thoracoscopic lobectomy in infants and children. J Pediatr Surg. 2008;43:40–5.

    PubMed  Google Scholar 

  62. Nasr A, Bass J. Thoracoscopic vs open resection of congenital lung lesions: a meta-analysis. J Pediatr Surg. 2012;47:857–61.

    PubMed  Google Scholar 

  63. Tocchioni F, Lombardi E, Ghionzoli M, et al. Long-term lung function in children following lobectomy for congenital lung malformation. J Pediatr Surg. 2017;52:1891–7.

    PubMed  Google Scholar 

  64. Komori K, Kamagata S, Hirobe S, et al. Radionuclide imaging study of long-term pulmonary function after lobectomy in children with congenital cystic lung disease. J Pediatr Surg. 2009;44:2096–100.

    PubMed  Google Scholar 

  65. Hijkoop A, Schoonhoven MM, Rosmalen J, et al. Lung function, exercise tolerance, and physical growth of children with congenital lung malformations at 8 years of age. Pediatr Pulmonol. 2019;54:1326–34.

    PubMed  PubMed Central  Google Scholar 

  66. Keijzer R, Chiu PPL, Ratjen F, et al. Pulmonary function after early vs late lobectomy during childhood: a preliminary study. J Pediatr Surg. 2009;44:893–5.

    PubMed  Google Scholar 

  67. Nasr A, Himidan S, Pastor AC, et al. Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? J Pediatr Surg. 2010;45:1086–9.

    PubMed  Google Scholar 

  68. Casagrande A, Pederiva F. Association between congenital lung malformations and lung tumors in children and adults: a systematic review. J Thorac Oncol. 2016;11:1837–45.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Marmara University, School of Medicine, Department of Pediatric Surgery, Istanbul, Turkey

    Kıvılcım Karadeniz Cerit & Gürsu Kıyan

Authors
  1. Kıvılcım Karadeniz Cerit
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Gürsu Kıyan
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Department of Otorhinolaryngology, Eskişehir Osmangazi University, Eskisehir, Türkiye

    Cemal Cingi

  2. Department of Pulmonology, Celal Bayar University, Manisa, Türkiye

    Arzu Yorgancıoğlu

  3. Department of Otorhinolaryngology, Kırıkkale University, Kırıkkale, Türkiye

    Nuray Bayar Muluk

  4. Federal University of Bahia School of Medicine and ProAR Foundation, Salvador - Bahia, Brazil

    Alvaro A. Cruz

Section Editor information

  1. Pediatric Pulmonology, Marmara University Faculty of Medicine, Istanbul, Türkiye

    Bulent Karadag

Rights and permissions

Reprints and permissions

Copyright information

© 2023 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Cerit, K.K., Kıyan, G. (2023). Congenital Lung Malformations. In: Cingi, C., Yorgancıoğlu, A., Bayar Muluk, N., Cruz, A.A. (eds) Airway Diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-22483-6_100-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-031-22483-6_100-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-22482-9

  • Online ISBN: 978-3-031-22483-6

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics

Search

Navigation