Congenital Pulmonary Airway Malformations: State-of-the-art Review for Pediatrician's Use

Overview

Journal Eur J Pediatr

Specialty Pediatrics

Date 2017 Oct 20

PMID 29046943

Citations 43

Authors

Claire LeBlanc

Marguerite Baron

Emilie Desselas

Minh Hanh Phan

Alexis Rybak

Guillaume Thouvenin

Clara Lauby

Sabine Irtan

Affiliations

Soon will be listed here.

Abstract

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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