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19.10.2017 | Review

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

verfasst von: Claire Leblanc, Marguerite Baron, Emilie Desselas, Minh Hanh Phan, Alexis Rybak, Guillaume Thouvenin, Clara Lauby, Sabine Irtan

Erschienen in: European Journal of Pediatrics | Ausgabe 12/2017

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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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Titel: Congenital pulmonary airway malformations: state-of-the-art review for pediatrician’s use
verfasst von: Claire Leblanc
Marguerite Baron
Emilie Desselas
Minh Hanh Phan
Alexis Rybak
Guillaume Thouvenin
Clara Lauby
Sabine Irtan
Publikationsdatum: 19.10.2017
Verlag: Springer Berlin Heidelberg
Erschienen in: European Journal of Pediatrics / Ausgabe 12/2017
Print ISSN: 0340-6199
Elektronische ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-017-3032-7

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

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