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Pediatric Surgery International
Published in: Pediatric Surgery International 9/2017

01-09-2017 | Original Article

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

Authors: Cynthia D. Downard, 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

Published in: Pediatric Surgery International | Issue 9/2017

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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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Metadata
Title
Treatment of congenital pulmonary airway malformations: a systematic review from the APSA outcomes and evidence based practice committee
Authors
Cynthia D. Downard
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
Publication date
01-09-2017
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Surgery International / Issue 9/2017
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-017-4098-z

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