Angeborene pulmonale Malformationen

Der Beitrag stellt Ursachen, Verlauf, diagnostische und therapeutische Möglichkeiten anhand der bronchogenen Zyste, der bronchopulmonalen Sequestration, des kongenitalen lobären Emphysems und der Congenital pulmonary Airway Malformation (CPAM) dar. Die angeborenen pulmonalen Malformationen sind zwar selten, bergen aber ein erhebliches Risiko für respiratorische Probleme beim Neugeborenen, für Infektionen und maligne Transformation im Verlauf.

Kernaussagen

• Angeborene pulmonale Malformationen sind mit einer Prävalenz der verschiedenen Entitäten zwischen 1 : 1000 und 1 : 35 000 zwar selten, bergen aber ein erhebliches Risiko für respiratorische Probleme beim Neugeborenen sowie für Infektionen und maligne Transformation im Verlauf.

• Deshalb ist die Kenntnis der Ursachen, des Verlaufs, der diagnostischen Möglichkeiten und therapeutischen Optionen der Schlüssel für die Wahl der Diagnostik, der chirurgischen Therapie und des optimalen Zeitpunkts der Operation.

• Dargestellt wird dies an den typischen angeborenen Fehlbildungen der Lunge: bronchogene Zyste, bronchopulmonale Sequestration, kongenitales lobäres Emphysem und Congenital pulmonary Airway Malformation (CPAM).

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