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Congenital pulmonary airway malformations: state-of-the-art review for pediatrician’s use

European Journal of Pediatrics volume 176, pages 1559–1571 (2017)Cite this article

Abstract

Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are diagnosed prenatally, improving the prenatal and immediate postnatal care and ultimately the knowledge on CPAM pathophysiology. CPAM natural evolution can lead to infections or malignancies, whose exact prevalence is still difficult to assess. The aim of this “state-of-the-art” review is to cover the recently published literature on CPAM management whether the pulmonary lesion was detected during pregnancy or after birth, the current indications of surgery or surveillance and finally its potential evolution to pleuro-pulmonary blastoma.

Conclusion: Surgery remains the cornerstone treatment of symptomatic lesions but the postnatal management of asymptomatic CPAM remains controversial. There are pros and cons of surgical resection, as increasing rate of infections over time renders the surgery more difficult after months or years of evolution, as well as risk of malignancy, though exact incidence is still unknown.

What is known:
• Congenital pulmonary airway malformations (CPAM) are rare developmental lung malformations mainly antenatally diagnosed.
• While the neonatal management of symptomatic CPAM is clear and includes prompt surgery, controversies remain for asymptomatic CPAM due to risk of infections and malignancies.
What is new:
• Increased rate of infection over time renders the surgery more difficult after months or years of evolution and pushes for recommendation of early elective surgery.
• New molecular or pathological pathways may help in the distinction of type 4 CPAM from type I pleuropulmonary blastoma.

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Abbreviations

BAC:

bronchio-alveolar carcinoma

BC:

bronchogenic cysts

BMP4:

bone morphogenic protein 4

BPS:

bronchopulmonary sequestration

CCAM:

congenital cystic adenomatoid malformation

CC10:

clara cell 10

CLE:

congenital lobar emphysema

CPAM:

Congenital pulmonary airway malformation

CT:

computer tomography

CVR:

CPAM volume ratio

EFS:

event free survival

EXIT:

ex utero intrapartum treatment

FABP7:

fat acid binding protein 7

FEV1:

forced expiratory volume in 1 s

FGF10:

fibroblast growth factor-10

FGFR2b:

fibroblast growth factor receptor

FVC:

forced vital capacity (FVC)

MRI:

magnetic resonance imaging

PDGF-B:

platelet-derived growth factor B

PPB:

pleuro-pulmonary blastoma

OS:

overall survival

SPRY2:

Sprouty 2

SSH:

Sonic Hedghog

TGF β:

growth factor β

US:

ultrasound scan

YY1:

Yin Yang 1

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Acknowledgements

The authors want to gratefully thank Emily Barrios for her kind review of English editing.

Funding

The authors have no funding to declare.

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Affiliations

  1. Department of Pediatric Pulmonology, APHP Hôpital Armand Trousseau, Paris, France

    Claire Leblanc, Marguerite Baron, Emilie Desselas, Minh Hanh Phan, Alexis Rybak, Guillaume Thouvenin & Clara Lauby

  2. UPMC Univ Paris 06, Centre de Recherche St Antoine Inserm UMRS.938, Sorbonne Universités, Paris, France

    Guillaume Thouvenin & Sabine Irtan

  3. Department of Pediatric Surgery, APHP Hôpital Armand Trousseau, 26 avenue du Dr Arnold Netter, 75012, Paris, France

    Sabine Irtan

Authors
  1. Claire Leblanc
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  2. Marguerite Baron
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  7. Clara Lauby
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  8. Sabine Irtan
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Contributions

Claire Leblanc: collect data, write the draft, approve final manuscript.

Marguerite Baron: collect data, write the draft, approve final manuscript.

Emilie Desselas: collect data, write the draft, approve final manuscript.

Minh Hanh Phan: collect data, write the draft, approve final manuscript.

Alexis Rybak: collect data, write the draft, approve final manuscript.

Guillaume Thouvenin : critical analysis of the manuscript, approve final manuscript.

Clara Lauby: collect data, write the draft, approve final manuscript.

Sabine Irtan: design the study, critical analysis of the manuscript, approve final manuscript.

Corresponding author

Correspondence to Sabine Irtan.

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This article does not contain any studies with human participants or animals performed by any of the authors.

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Communicated by Peter de Winter

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Leblanc, C., Baron, M., Desselas, E. et al. Congenital pulmonary airway malformations: state-of-the-art review for pediatrician’s use. Eur J Pediatr 176, 1559–1571 (2017). https://doi.org/10.1007/s00431-017-3032-7

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  • DOI: https://doi.org/10.1007/s00431-017-3032-7

Keywords

  • CPAM
  • State of the art
  • Pleuropulmonary blastoma