Pediatric Surgery International

, Volume 33, Issue 9, pp 939–953 | Cite as

Treatment of congenital pulmonary airway malformations: a systematic review from the APSA outcomes and evidence based practice committee

  • Cynthia D. DownardEmail author
  • Casey M. Calkins
  • Regan F. Williams
  • Elizabeth J. Renaud
  • Tim Jancelewicz
  • Julia Grabowski
  • Roshni Dasgupta
  • Milissa McKee
  • Robert Baird
  • Mary T. Austin
  • Meghan A. Arnold
  • Adam B. Goldin
  • Julia Shelton
  • Saleem Islam
Original Article



Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients.


Questions regarding the management of a pediatric patient with a CPAM were generated. English language articles published between 1960 and 2014 were compiled after searching Medline and OvidSP. The articles were divided by subject area and by the question asked, then reviewed and included if they specifically addressed the proposed question.


1040 articles were identified on initial search. After screening abstracts per eligibility criteria, 130 articles were used to answer the proposed questions. Based on the available literature, resection of an asymptomatic CPAM is controversial, and when performed is usually completed within the first six months of life. Lobectomy remains the standard resection method for CPAM, and can be performed thoracoscopically or via thoracotomy. There is no consensus regarding a monitoring protocol for observing asymptomatic lesions, although at least one chest computerized tomogram (CT) should be performed postnatally for lesion characterization. An antenatally identified CPAM can be evaluated with MRI if fetal intervention is being considered, but is not required for the fetus with a lesion not at risk for hydrops. Prenatal consultation should be offered for infants with CPAM and encouraged for those infants in whom characteristics indicate risk of hydrops.


Very few articles provided definitive recommendations for care of the patient with a CPAM and none reported Level I or II evidence. Based on available information, CPAMs are usually resected early in life if at all. A prenatally diagnosed congenital lung lesion should be evaluated postnatally with CT, and prenatal counseling should be undertaken in patients at risk for hydrops.


Pulmonary Congenital Systematic review Adenomatoid CPAM Fetal Lung 



The authors would like to thank Michel Atlas, Reference and Acquisitions Librarian, Kornhauser Health Sciences Library, University of Louisville, for her assistance with the literature search and citation management. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Cynthia D. Downard
    • 1
    Email author
  • Casey M. Calkins
    • 2
  • Regan F. Williams
    • 3
  • Elizabeth J. Renaud
    • 4
  • Tim Jancelewicz
    • 3
  • Julia Grabowski
    • 5
  • Roshni Dasgupta
    • 6
  • Milissa McKee
    • 7
  • Robert Baird
    • 8
  • Mary T. Austin
    • 9
  • Meghan A. Arnold
    • 10
  • Adam B. Goldin
    • 11
  • Julia Shelton
    • 12
  • Saleem Islam
    • 13
  1. 1.Division of Pediatric Surgery, Hiram C. Polk, Jr., MD Department of SurgeryUniversity of LouisvilleLouisvilleUSA
  2. 2.Division of Pediatric General and Thoracic Surgery, Department of SurgeryThe Medical College of WisconsinMilwaukeeUSA
  3. 3.Division of Pediatric Surgery, Department of SurgeryUniversity of Tennessee Health Science CenterMemphisUSA
  4. 4.Division of Pediatric Surgery, Department of SurgeryAlbany Medical CenterAlbanyUSA
  5. 5.Division of Pediatric Surgery, Department of SurgeryAnn and Robert Lurie Children’s Hospital of ChicagoChicagoUSA
  6. 6.Division of Pediatric General and Thoracic SurgeryCincinnati Children’s Hospital Medical CenterCincinnatiUSA
  7. 7.Pediatric SurgeryPhoenixUSA
  8. 8.Division of Pediatric General and Thoracic SurgeryMcGill University Health Center, Montreal Children’s HospitalMontrealCanada
  9. 9.Departments of Surgical Oncology and Division of PediatricsThe University of Texas MD Anderson Cancer CenterHoustonUSA
  10. 10.Division of Pediatric Surgery, Department of SurgeryUniversity of Michigan CS Mott Children’s HospitalAnn ArborUSA
  11. 11.Division of Pediatric General and Thoracic Surgery, Seattle Children’s HospitalUniversity of Washington School of MedicineSeattleUSA
  12. 12.Division of Pediatric Surgery, Department of SurgeryUniversity of Iowa Hospitals and ClinicsIowa CityUSA
  13. 13.Division of Pediatric SurgeryUniversity of FloridaGainesvilleUSA

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