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

Prenatal diagnosis and management of congenital pulmonary airway malformation

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


Congenital lung masses include congenital pulmonary airway malformations (CPAMs), bronchopulmonary sequestration (BPS), and congenital lobar emphysema with bronchial atresia. Hybrid lesions have also been described, suggesting these masses represent a spectrum of abnormalities [1].

Prenatal diagnosis of these disorders has increased with widespread use of prenatal ultrasonography [2]. In addition, use of prenatal ultrasound and magnetic resonance (MR) imaging has improved understanding of the natural history of fetal lung lesions. Ultrasound evaluation is sensitive for the diagnosis of many prenatal lung lesions, but has low specificity [3]. Fetal MR imaging improves evaluation of pulmonary anatomy, which can help to confirm or exclude the suspected diagnosis, and provides additional information useful for counseling and obstetrical management [4-6]. A definitive diagnosis is usually not possible without surgical resection and histopathological evaluation [1,7,8].


Ultrasound is the primary modality for imaging the fetal chest and detecting fetal chest masses [9], but MR imaging can be a useful adjunct for confirming the presence of a mass, further characterizing normal and abnormal anatomy, and providing assessment of residual lung volume. Assessment of lung parenchyma sonographically can be limited due to maternal obesity, oligohydramnios, overlying ribs, and fetal lie; MR is less limited by these factors.

Ultrasound — On ultrasound, the normal fetal chest is oval or round, with the heart positioned in the anterior half of the left chest and bordered by lung parenchyma (image 1A-B). Inferiorly, the diaphragms are dome-shaped and hypoechoic. The fetal airway is not well delineated by ultrasound. Lung parenchyma appears homogeneous and slightly hyperechogenic compared to the fetal liver, and its echogenicity increases with gestational age [10].

Fetal lung volume increases with gestational age with the right lung typically measuring 56 percent of total lung volume. Three-dimensional (3D) and four-dimensional (4D) ultrasound imaging appear to be more useful than two-dimensional ultrasound for estimating lung volume [11].

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: Feb 07, 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. Ankermann T, Oppermann HC, Engler S, et al. Congenital masses of the lung, cystic adenomatoid malformation versus congenital lobar emphysema: prenatal diagnosis and implications for postnatal treatment. J Ultrasound Med 2004; 23:1379.
  2. Shanmugam G, MacArthur K, Pollock JC. Congenital lung malformations--antenatal and postnatal evaluation and management. Eur J Cardiothorac Surg 2005; 27:45.
  3. Kline-Fath BM. Is prenatal sonography accurate in identification of Congenital lung lesions? Scientific paper presented at SPR, Boston, MA. April 15, 2010.
  4. 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.
  5. Quinn TM, Hubbard AM, Adzick NS. Prenatal magnetic resonance imaging enhances fetal diagnosis. J Pediatr Surg 1998; 33:553.
  6. Aite L, Zaccara A, Trucchi A, et al. When uncertainty generates more anxiety than severity: the prenatal experience with cystic adenomatoid malformation of the lung. J Perinat Med 2009; 37:539.
  7. Harmath A, Csaba A, Hauzman E, et al. Congenital lung malformations in the second trimester: prenatal ultrasound diagnosis and pathologic findings. J Clin Ultrasound 2007; 35:250.
  8. 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.
  9. Bulas D. Fetal magnetic resonance imaging as a complement to fetal ultrasonography. Ultrasound Q 2007; 23:3.
  10. Hubbard AM, Adzick NS, Crombleholme TM, et al. Congenital chest lesions: diagnosis and characterization with prenatal MR imaging. Radiology 1999; 212:43.
  11. Ruano R, Joubin L, Aubry MC, et al. A nomogram of fetal lung volumes estimated by 3-dimensional ultrasonography using the rotational technique (virtual organ computer-aided analysis). J Ultrasound Med 2006; 25:701.
  12. Levine D, Barnewolt CE, Mehta TS, et al. Fetal thoracic abnormalities: MR imaging. Radiology 2003; 228:379.
  13. Curran PF, Jelin EB, Rand L, et al. Prenatal steroids for microcystic congenital cystic adenomatoid malformations. J Pediatr Surg 2010; 45:145.
  14. Daltro P, Werner H, Gasparetto TD, et al. Congenital chest malformations: a multimodality approach with emphasis on fetal MR imaging. Radiographics 2010; 30:385.
  15. Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol 1977; 8:155.
  16. Adzick NS, Harrison MR, Crombleholme TM, et al. Fetal lung lesions: management and outcome. Am J Obstet Gynecol 1998; 179:884.
  17. 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.
  18. Azizkhan RG, Crombleholme TM. Congenital cystic lung disease: contemporary antenatal and postnatal management. Pediatr Surg Int 2008; 24:643.
  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.
  20. Mann S, Wilson RD, Bebbington MW, et al. Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med 2007; 12:477.
  21. Zeidan S, Gorincour G, Potier A, et al. Congenital lung malformation: evaluation of prenatal and postnatal radiological findings. Respirology 2009; 14:1005.
  22. 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.
  23. Schott S, Mackensen-Haen S, Wallwiener M, et al. Cystic adenomatoid malformation of the lung causing hydrops fetalis: case report and review of the literature. Arch Gynecol Obstet 2009; 280:293.
  24. Stocker, T, Dehner, LP. Pediatric Pathology, 2nd ed, Lippincott Williams and Wilkins, p.473.
  25. Bush A, Hogg J, Chitty LS. Cystic lung lesions - prenatal diagnosis and management. Prenat Diagn 2008; 28:604.
  26. Seo T, Ando H, Kaneko K, et al. Two cases of prenatally diagnosed congenital lobar emphysema caused by lobar bronchial atresia. J Pediatr Surg 2006; 41:e17.
  27. Kumar AN. Perinatal management of common neonatal thoracic lesions. Indian J Pediatr 2008; 75:931.
  28. Wilson RD. In utero therapy for fetal thoracic abnormalities. Prenat Diagn 2008; 28:619.
  29. 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.
  30. Mechoulan A, Leclair MD, Yvinec M, et al. [Pleuropulmonary blastoma: a case of early neonatal diagnosis through antenatal scan screening]. Gynecol Obstet Fertil 2007; 35:437.
  31. Dishop MK, McKay EM, Kreiger PA, et al. Fetal lung interstitial tumor (FLIT): A proposed newly recognized lung tumor of infancy to be differentiated from cystic pleuropulmonary blastoma and other developmental pulmonary lesions. Am J Surg Pathol 2010; 34:1762.
  32. Adzick NS. Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med 2010; 15:1.
  33. Kunisaki SM, Barnewolt CE, Estroff JA, et al. Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival. J Pediatr Surg 2007; 42:404.
  34. Kunisaki SM, Fauza DO, Barnewolt CE, et al. Ex utero intrapartum treatment with placement on extracorporeal membrane oxygenation for fetal thoracic masses. J Pediatr Surg 2007; 42:420.
  35. Cavoretto P, Molina F, Poggi S, et al. Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol 2008; 32:769.
  36. 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.
  37. Hellmund A, Berg C, Geipel A, et al. Prenatal Diagnosis and Evaluation of Sonographic Predictors for Intervention and Adverse Outcome in Congenital Pulmonary Airway Malformation. PLoS One 2016; 11:e0150474.
  38. Costanzo S, Filisetti C, Vella C, et al. Pulmonary Malformations: Predictors of Neonatal Respiratory Distress and Early Surgery. J Neonatal Surg 2016; 5:27.
  39. Raychaudhuri P, Pasupati A, James A, et al. Prospective study of antenatally diagnosed congenital cystic adenomatoid malformations. Pediatr Surg Int 2011; 27:1159.
  40. Zamora IJ, Sheikh F, Cassady CI, et al. Fetal MRI lung volumes are predictive of perinatal outcomes in fetuses with congenital lung masses. J Pediatr Surg 2014; 49:853.
  41. Hofstaetter C, Hansmann M, Eik-Nes SH, et al. A cardiovascular profile score in the surveillance of fetal hydrops. J Matern Fetal Neonatal Med 2006; 19:407.
  42. Coleman BG, Adzick NS, Crombleholme TM, et al. Fetal therapy: state of the art. J Ultrasound Med 2002; 21:1257.
  43. 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.
  44. Liechty KW. Ex-utero intrapartum therapy. Semin Fetal Neonatal Med 2010; 15:34.
  45. Hedrick HL, Flake AW, Crombleholme TM, et al. The ex utero intrapartum therapy procedure for high-risk fetal lung lesions. J Pediatr Surg 2005; 40:1038.
  46. Witlox RS, Lopriore E, Oepkes D. Prenatal interventions for fetal lung lesions. Prenat Diagn 2011; 31:628.
  47. Adzick NS. Management of fetal lung lesions. Clin Perinatol 2009; 36:363.
  48. 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.
  49. Peranteau WH, Wilson RD, Liechty KW, et al. Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival. Fetal Diagn Ther 2007; 22:365.
  50. Loh KC, Jelin E, Hirose S, et al. Microcystic congenital pulmonary airway malformation with hydrops fetalis: steroids vs open fetal resection. J Pediatr Surg 2012; 47:36.
  51. Derderian SC, Coleman AM, Jeanty C, et al. Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone. J Pediatr Surg 2015; 50:515.
  52. Peranteau WH, Boelig MM, Khalek N, et al. Effect of single and multiple courses of maternal betamethasone on prenatal congenital lung lesion growth and fetal survival. J Pediatr Surg 2016; 51:28.
  53. Peranteau WH, Adzick NS, Boelig MM, et al. Thoracoamniotic shunts for the management of fetal lung lesions and pleural effusions: a single-institution review and predictors of survival in 75 cases. J Pediatr Surg 2015; 50:301.
  54. Law BH, Bratu I, Jain V, Landry MA. Refractory tension pneumothorax as a result of an internally displaced thoracoamniotic shunt in an infant with a congenital pulmonary airway malformation. BMJ Case Rep 2016; 2016.
  55. 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.
  56. Fortunato S. Intrauterine laser ablation of a fetal cystic adenomatoid malformation with hydrops: the application of minimally invasive surgical techniques to fetal surgery. Am J Obstet Gynecol 1997; 176:S84.
  57. Ong SS, Chan SY, Ewer AK, et al. Laser ablation of foetal microcystic lung lesion: successful outcome and rationale for its use. Fetal Diagn Ther 2006; 21:471.
  58. 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.
  59. Bermúdez C, Pérez-Wulff J, Arcadipane M, et al. Percutaneous fetal sclerotherapy for congenital cystic adenomatoid malformation of the lung. Fetal Diagn Ther 2008; 24:237.
  60. Marshall KW, Blane CE, Teitelbaum DH, van Leeuwen K. Congenital cystic adenomatoid malformation: impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient. AJR Am J Roentgenol 2000; 175:1551.
  61. 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.e1.