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

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Abstract

Purpose

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.

Methods

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.

Results

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.

Conclusions

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.

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Acknowledgement

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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Authors and Affiliations

  1. Division of Pediatric Surgery, Hiram C. Polk, Jr., MD Department of Surgery, University of Louisville, 315 East Broadway, Suite 565, Louisville, KY, 40202, USA

    Cynthia D. Downard

  2. Division of Pediatric General and Thoracic Surgery, Department of Surgery, The Medical College of Wisconsin, Milwaukee, WI, USA

    Casey M. Calkins

  3. Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Memphis, TN, USA

    Regan F. Williams & Tim Jancelewicz

  4. Division of Pediatric Surgery, Department of Surgery, Albany Medical Center, Albany, NY, USA

    Elizabeth J. Renaud

  5. Division of Pediatric Surgery, Department of Surgery, Ann and Robert Lurie Children’s Hospital of Chicago, Chicago, IL, USA

    Julia Grabowski

  6. Division of Pediatric General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Roshni Dasgupta

  7. Pediatric Surgery, Phoenix, AZ, USA

    Milissa McKee

  8. Division of Pediatric General and Thoracic Surgery, McGill University Health Center, Montreal Children’s Hospital, Glen Site, Montreal, QC, Canada

    Robert Baird

  9. Departments of Surgical Oncology and Division of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Mary T. Austin

  10. Division of Pediatric Surgery, Department of Surgery, University of Michigan CS Mott Children’s Hospital, Ann Arbor, MI, USA

    Meghan A. Arnold

  11. Division of Pediatric General and Thoracic Surgery, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, WA, USA

    Adam B. Goldin

  12. Division of Pediatric Surgery, Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, USA

    Julia Shelton

  13. Division of Pediatric Surgery, University of Florida, Gainesville, FL, USA

    Saleem Islam

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  1. Cynthia D. Downard
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Downard, C.D., Calkins, C.M., Williams, R.F. et al. Treatment of congenital pulmonary airway malformations: a systematic review from the APSA outcomes and evidence based practice committee. Pediatr Surg Int 33, 939–953 (2017). https://doi.org/10.1007/s00383-017-4098-z

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