title: Congenital pulmonary airway malformations: from prenatal diagnosis to postnatal outcome.
abstract: Congenital pulmonary airway malformations (CPAMs) include cystic and non-cystic lung lesions. These represent about 30-40% of developmental lung bud anomaly lesions mainly diagnosed during pregnancy or in newborn infants; or sometimes they remain undetected until adult life. The malformation usually presents as a sporadic, non-hereditary lung abnormality, with no predilection for the right or left lung, sex or race. CPAMs vary in their histological features, epidemiological and clinical presentation, severity and prognosis, supporting the embryologic hypothesis of arrested lung growth during branching morphogenesis. The existence of "hybrid" forms underline the possible common pathogenic mechanism involved in the development of different lesion types; a genetic role has also been proposed in abnormal lung development. Influence of the natural history on pre and postnatal management is relevant. Surgical resection is the standard of therapy for symptomatic CPAMs, while the management of asymptomatic cases remains controversial. The potential risk of infection and malignancy in CPAMs justifies complete surgical resection in the first year of life; while long term follow-up is required in children who do not undergo surgery. A multidisciplinary team including gynecologists, neonatologists, radiologists, pediatricians and pediatric surgeons is recommended in pre, postnatal management and in the postsurgical follow-up of all children with CPAMs.
authors.affiliation: Pediatric Surgery Unit, Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Pavia, Italy - g.pelizzo@smatteo.pv.it.
authors.forename: Gloria
authors.initials: G
authors.lastname: Pelizzo
authors.forename: Federico
authors.initials: F
authors.lastname: Costanzo
authors.forename: Erika
authors.initials: E
authors.lastname: Andreatta
authors.forename: Valeria
authors.initials: V
authors.lastname: Calcaterra
mesh_terms: D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities* / Q000601:surgery; D008171:Lung Diseases / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery; D010348:Patient Care Team / Q000458:organization & administration; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000379:methods*; D011379:Prognosis