Congenital Lung Malformations

  • Jörg Fuchs


Congenital lung lesions are mostly diagnosed by a routine antenatal ultrasound scanning. Many remain asymptomatic immediately after birth but are likely to present later in life with symptoms of pneumonic infection or a persistent lesion in adults can be confused with malignancy. Controversy amongst the surgeons is to resect these asymptomatic lesions electively or to resect only if presented with infection on follow-up. With the advent of thoracoscopic resections and reduced morbidity with minimally invasive resections, elective resection has become more attractive. There is definitely increased morbidity once the lesion becomes infected with need for blood transfusion, wider resection, post-operative air leaks and risk of conversion to open thoracotomy. The subspecialization in Pediatric thoracic surgery may improve overall outcome of this and many other Pediatric thoracic surgical conditions.


Antenatal diagnosis Congenital cystic lung lesions Congenital pulmonary airway malformations Congenital cystic adenomatoid malformations Pulmonary sequestrations Lung agenesis Bronchopulmonary foregut cysts Congenital lobar emphysema Hydrops fetalis VATS Lobectomy Segmental resection Partial lobectomy 


  1. 1.
    Adzick NS. Management of fetal lung lesions. Clin Perinatol. 2003;30(3):481–92.CrossRefPubMedGoogle Scholar
  2. 2.
    Azizkhan RG, Crombleholme TM. Congenital cystic lung disease: contemporary antenatal and postnatal management. Pediatr Surg Int. 2008;24(6):643–57.CrossRefPubMedGoogle Scholar
  3. 3.
    Baird R, Puligandla PS, Laberge JM. Congenital lung malformations: informing best practice. Semin Pediatr Surg. 2014;23(5):270–7.CrossRefPubMedGoogle Scholar
  4. 4.
    Aspirot A, Puligandla PS, Bouchard S, Su W, Flageole H, Laberge JM. A contemporary evaluation of surgical outcome in neonates and infants undergoing lung resection. J Pediatr Surg. 2008;43(3):508–12.CrossRefPubMedGoogle Scholar
  5. 5.
    Nazir Z, Qazi SH, Ahmed N, Atiq M, Billoo AG. Pulmonary agenesis—vascular airway compression and gastroesophageal reflux influence outcome. J Pediatr Surg. 2006;41(6):1165–9.CrossRefPubMedGoogle Scholar
  6. 6.
    Page DV, Stocker JT. Anomalies associated with pulmonary hypoplasia. Am Rev Respir Dis. 1982;125(2):216–21.PubMedGoogle Scholar
  7. 7.
    Backer CL, Kelle AM, Mavroudis C, Rigsby CK, Kaushal S, Holinger LD. Tracheal reconstruction in children with unilateral lung agenesis or severe hypoplasia. Ann Thorac Surg. 2009;88(2):624–30.CrossRefPubMedGoogle Scholar
  8. 8.
    Berrocal T, Madrid C, Novo S, Gutierrez J, Arjonilla A, Gomez-Leon N. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 2004;24(1):e17.CrossRefPubMedGoogle Scholar
  9. 9.
    Lal R, Lama TK, Bhatnagar V. Congenital lung cyst. Eur J Pediatr Surg. 1999;9(5):334–6.CrossRefPubMedGoogle Scholar
  10. 10.
    Mattioli G, Buffa P, Granata C, et al. Lung resection in pediatric patients. Pediatr Surg Int. 1998;13(1):10–3.CrossRefPubMedGoogle Scholar
  11. 11.
    Rothenberg SS. Thoracoscopic lung resection in children. J Pediatr Surg. 2000;35(2):271–4.CrossRefPubMedGoogle Scholar
  12. 12.
    Tsao K, Hawgood S, Vu L, et al. Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy. J Pediatr Surg. 2003;38(3):508–10.CrossRefPubMedGoogle Scholar
  13. 13.
    Libretti L, Ciriaco P, Casiraghi M, Arrigoni G, Zannini P. Pleuropulmonary blastoma in the area of a diagnosed congenital lung cyst. Ann Thorac Surg. 2008;85(2):658–60.CrossRefPubMedGoogle Scholar
  14. 14.
    Priest JR, Williams GM, Hill DA, Dehner LP, Jaffe A. Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol. 2009;44(1):14–30.CrossRefPubMedGoogle Scholar
  15. 15.
    Puligandla PS, Laberge JM. Congenital lung lesions. Clin Perinatol. 2012;39(2):331–47.CrossRefPubMedGoogle Scholar
  16. 16.
    Laberge JM, Puligandla P, Flageole H. Asymptomatic congenital lung malformations. Semin Pediatr Surg. 2005;14(1):16–33.CrossRefPubMedGoogle Scholar
  17. 17.
    Beres A, Aspirot A, Paris C, et al. A contemporary evaluation of pulmonary function in children undergoing lung resection in infancy. J Pediatr Surg. 2011;46(5):829–32.CrossRefPubMedGoogle Scholar
  18. 18.
    Azizkhan RG, Grimmer DL, Askin FB, Lacey SR, Merten DF, Wood RE. Acquired lobar emphysema (overinflation): clinical and pathological evaluation of infants requiring lobectomy. J Pediatr Surg. 1992;27(8):1145–51.CrossRefPubMedGoogle Scholar
  19. 19.
    Cataneo DC, Rodrigues OR, Hasimoto EN, Schmidt AF Jr, Cataneo AJ. Congenital lobar emphysema: 30-year case series in two university hospitals. J Bras Pneumol. 2013;39(4):418–26.CrossRefPubMedPubMedCentralGoogle Scholar
  20. 20.
    Alivizatos P, Cheatle T, de Leval M, Stark J. Pulmonary sequestration complicated by anomalies of pulmonary venous return. J Pediatr Surg. 1985;20(1):76–9.CrossRefPubMedGoogle Scholar
  21. 21.
    Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung lesions: management and outcome. Am J Obstet Gynecol. 1998;179(4):884–9.CrossRefPubMedGoogle Scholar
  22. 22.
    Saeed A, Kazmierski M, Khan A, McShane D, Gomez A, Aslam A. Congenital lung lesions: preoperative three-dimensional reconstructed CT scan as the definitive investigation and surgical management. Eur J Pediatr Surg. 2013;23(1):53–6.CrossRefPubMedGoogle Scholar
  23. 23.
    Vaos G, Zavras N, Antypas G. Bronchogenic cyst of the lung mimicking a pulmonary hydatid cyst in a child. Pediatr Surg Int. 2005;21(5):383–5.CrossRefPubMedGoogle Scholar
  24. 24.
    Rothenberg SS. Thoracoscopic pulmonary surgery. Semin Pediatr Surg. 2007;16(4):231–7.CrossRefPubMedGoogle Scholar
  25. 25.
    Rothenberg SS, Shipman K, Kay S, et al. Thoracoscopic segmentectomy for congenital and acquired pulmonary disease: a case for lung-sparing surgery. J Laparoendosc Adv Surg Tech A. 2014;24(1):50–4.CrossRefPubMedGoogle Scholar
  26. 26.
    Vu LT, Farmer DL, Nobuhara KK, Miniati D, Lee H. Thoracoscopic versus open resection for congenital cystic adenomatoid malformations of the lung. J Pediatr Surg. 2008;43(1):35–9.CrossRefPubMedGoogle Scholar
  27. 27.
    Bishay M, Giacomello L, Retrosi G, et al. Hypercapnia and acidosis during open and thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia: results of a pilot randomized controlled trial. Ann Surg. 2013;258(6):895–900.CrossRefPubMedGoogle Scholar
  28. 28.
    Dingemann C, Zoeller C, Bataineh Z, Osthaus A, Suempelmann R, Ure B. Single- and double-lung ventilation in infants and children undergoing thoracoscopic lung resection. Eur J Pediatr Surg. 2013;23(1):48–52.PubMedGoogle Scholar
  29. 29.
    Rothenberg SS. First decade’s experience with thoracoscopic lobectomy in infants and children. J Pediatr Surg. 2008;43(1):40–4.CrossRefPubMedGoogle Scholar
  30. 30.
    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(5):423–8.CrossRefPubMedGoogle Scholar
  31. 31.
    Albanese CT, Sydorak RM, Tsao K, Lee H. Thoracoscopic lobectomy for prenatally diagnosed lung lesions. J Pediatr Surg. 2003;38(4):553–5.CrossRefPubMedGoogle Scholar
  32. 32.
    Seitz G, Warmann SW, Szavay PO, et al. Thoracoscopic lobectomy as a treatment option for persistent middle lobe syndrome in children. Pediatr Int. 2010;52(2):e79–81.CrossRefPubMedGoogle Scholar
  33. 33.
    Fuchs J, Kirschner HJ, Warmann SW, Schellinger K, Baden W, Szavay P. Thoracoscopic anatomical lung resection in children. Zentralbl Chir. 2007;132(3):247–50.CrossRefPubMedGoogle Scholar
  34. 34.
    Johnson SM, Grace N, Edwards MJ, Woo R, Puapong D. Thoracoscopic segmentectomy for treatment of congenital lung malformations. J Pediatr Surg. 2011;46(12):2265–9.CrossRefPubMedGoogle Scholar
  35. 35.
    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(2):261–70.CrossRefPubMedGoogle Scholar
  36. 36.
    Mattioli G, Asquasciati C, Castagnetti M, Bellodi S, Rossi G, Jasonni V. Muscle-sparing thoracotomy combined with mechanically stapled lung resection for benign lung disorders: functional results and quality of life. Pediatr Surg Int. 2006;22(6):491–5.CrossRefPubMedGoogle Scholar
  37. 37.
    Adzick NS, Harrison MR, Glick PL, et al. Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg. 1985;20(5):483–8.CrossRefPubMedGoogle Scholar
  38. 38.
    Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol. 1977;8(2):155–71.CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag London Ltd., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Pediatric Surgery and Pediatric UrologyUniversity of TuebingenTuebingenGermany

Personalised recommendations