Perinatale aanpak bij congenitale luchtwegmalformaties

This review on congenital pulmonary airway malformations intends to illustrate the positive impact of prenatal diagnosis on their postnatal management. For the gynaecologist these fetal malformations present as uni- or bilateral hyperdense or so-called "bright" lungs on fetal ultrasound. The underlying cause for bilateral hyperdense fetal lungs is mostly related to a Congenital High Airway Obstruction Syndrome (CHAOS) and exceptionally to a bilateral form of Congenital Cystic Adenomatoid Malformation (CCAM). Outcome is globally poor, although specific therapeutic procedures may be considered for CHAOS if a reasonable chance for integral repair of the airways exists. On the other hand, overall outcome for most of the unilateral types of congenital pulmonary airway malformation should be considered as fairly good to excellent. Therefore reticence upon termination of pregnancy is a major message. It is also important to underscore that in utero regression of these unilateral bright lungs is rather the rule and not the exception. Still, these "vanishing lesions" should be thoroughly evaluated after birth. The newborn infant with congenital pulmonary airway malformation is often asymptomatic at birth. For this reason prenatal diagnosis will promote an elective and timely postnatal management. Postnatal surgery - mostly not urgent - is the rule for CCAM, as well as for pulmonary sequestration, bronchogenic cyst and for most cases of congenital lobar emphysema (CLE). When the baby is symptom-free, elective surgery is generally advised between 3 and 6 months of age. Conservative segmentectomy should be avoided because of a higher risk of recurrence. Therefore lobectomy is the suitable intervention. In case of asymptomatic CLE a conservative policy can be considered, although this holds the objection that in later life final diagnosis can be proven to be incorrect.