European Journal of Pediatrics

, Volume 176, Issue 12, pp 1559–1571 | Cite as

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

  • Claire Leblanc
  • Marguerite Baron
  • Emilie Desselas
  • Minh Hanh Phan
  • Alexis Rybak
  • Guillaume Thouvenin
  • Clara Lauby
  • Sabine IrtanEmail author


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.


CPAM State of the art Pleuropulmonary blastoma 



bronchio-alveolar carcinoma


bronchogenic cysts


bone morphogenic protein 4


bronchopulmonary sequestration


congenital cystic adenomatoid malformation


clara cell 10


congenital lobar emphysema


Congenital pulmonary airway malformation


computer tomography


CPAM volume ratio


event free survival


ex utero intrapartum treatment


fat acid binding protein 7


forced expiratory volume in 1 s


fibroblast growth factor-10


fibroblast growth factor receptor


forced vital capacity (FVC)


magnetic resonance imaging


platelet-derived growth factor B


pleuro-pulmonary blastoma


overall survival


Sprouty 2


Sonic Hedghog


growth factor β


ultrasound scan


Yin Yang 1



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

Authors’ 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.


The authors have no funding to declare.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

No informed consent was needed for this article.

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


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Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  1. 1.Department of Pediatric PulmonologyAPHP Hôpital Armand TrousseauParisFrance
  2. 2.UPMC Univ Paris 06, Centre de Recherche St Antoine Inserm UMRS.938Sorbonne UniversitésParisFrance
  3. 3.Department of Pediatric SurgeryAPHP Hôpital Armand TrousseauParisFrance

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