Perinatale aanpak bij congenitale luchtwegmalformaties

Abstract

AbstractPerinatal diagnosis and management ofcongenital pulmonary airway malformations This review on congenital pulmonary airwaymalformations intends to illustrate the positiveimpact of prenatal diagnosis on their postnatalmanagement. For the gynaecologist these fetalmalformations present as uni- or bilateral hyper-dense or so-called „bright” lungs on fetal ultra-sound. The underlying cause for bilateral hyper-dense fetal lungs is mostly related to a CongenitalHigh Airway Obstruction Syndrome (CHAOS)and exceptionally to a bilateral form of Congeni-tal Cystic Adenomatoid Malformation (CCAM).Outcome is globally poor, although specifictherapeutic procedures may be considered forCHAOS if a reasonable chance for integralrepair of the airways exists. On the other hand,overall outcome for most of the unilateral typesof congenital pulmonary airway malformationshould be considered as fairly good to excellent.Therefore reticence upon termination of preg-nancy is a major message. It is also importantto underscore that in utero regression of theseunilateral bright lungs is rather the rule and notthe exception. Still, these „vanishing lesions”should be thoroughly evaluated after birth.The newborn infant with congenital pulmo-nary airway malformation is often asymptomatic