Treatment of congenital pulmonary airway malformations: a systematic review from the APSA outcomes and evidence based practice committee
- 1.2k Downloads
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.
KeywordsPulmonary 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.
- 4.Liberati A, Altman DG, Tetzlaff J, Mulrow C, Gotzsche PC, Ioannidis JP, Clarke M, Devereaux PJ, Kleijnen J, Moher D (2009) The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate health care interventions: explanation and elaboration. PLoS Med 6(7):e1000100. doi: 10.1371/journal.pmed.1000100 PubMedPubMedCentralCrossRefGoogle Scholar
- 15.Feinberg A, Hall NJ, Williams GM, Schultz KA, Miniati D, Hill DA, Dehner LP, Messinger YH, Langer JC (2016) Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features? J Pediatr Surg 51(1):33–37. doi: 10.1016/j.jpedsurg.2015.10.019 PubMedCrossRefGoogle Scholar
- 28.van Leeuwen K, Teitelbaum DH, Hirschl RB, Austin E, Adelman SH, Polley TZ, Marshall KW, Coran AG, Nugent C (1999) Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history. Journal of Pediatric Surgery 34(5):794–798 (discussion 798–799) PubMedCrossRefGoogle Scholar
- 30.Doros L, Schultz KA, Stewart DR, Bauer AJ, Williams G, Rossi CT, Carr A, Yang J, Dehner LP, Messinger Y, Hill DA (1993) DICER1-related disorders. In: Pagon RA, Adam MP, Ardinger HH et al (eds) GeneReviews(R). University of Washington, SeattleGoogle Scholar
- 55.Yong PJ, Von Dadelszen P, Carpara D, Lim K, Kent N, Tessier F, Delisle MF, Wong T, Blair G, Skarsgard ED (2012) Prediction of pediatric outcome after prenatal diagnosis and expectant antenatal management of congenital cystic adenomatoid malformation. Fetal Diagn Ther 31(2):94–102PubMedCrossRefGoogle Scholar