Angeborene Fehlbildungen der Lunge – eine Übersicht

Zusammenfassung

Kongenitale pulmonale Malformationen stellen eine heterogene Gruppe seltener Erkrankungen dar, die auf Fehlentwicklungen während der embryonalen und fetalen Wachstumsphase basieren. Zu ihnen gehören der Trachealbronchus, die bronchiale Atresie, die bronchogene Zyste, die Lungensequestration, das kongenitale lobäre Emphysem sowie die sogenannte Congenital pulmonary Airway Malformation. Eines der Leitsymptome dieser Malformationen ist die durch ihren verdrängenden Effekt bedingte postnatale respiratorische Insuffizienz, welche eine rasche operative Versorgung erfordert. Auch bei asymptomatischen Malformationen wird aufgrund des erhöhten Infektrisikos die Resektion empfohlen.

In der folgenden Übersicht wird auf die Ursachen, das klinische Bild und die therapeutischen Optionen dieser angeborenen Fehlbildungen der Lunge und des Bronchialsystems eingegangen.

Abstract

Congenital pulmonary malformations comprise a heterogenous group of rare developmental diseases. The most common malformations are the tracheal bronchus, bronchial atresia, bronchogenic cyst, pulmonary sequestration, congenital lobar emphysema, and congenital pulmonary airway malformation. Due to their space-consuming effect, patients suffer early postnatal respiratory distress which generally requires immediate surgical resection. The management of asymptomatic lesions remains subject to debate, but early elective surgery is generally recommended to avoid respiratory and infectious complications at a later time point.

We here provide a comprehensive review in which we present causes, clinical presentation and therapeutic options for the most prominent congenital malformations of the airways and lung parenchyma.

Schlüsselwörter

Bronchialatresie - Bronchogene Zyste - Lungensequester - Kongenitales Lobäremphysem - Congenital Pulmonary Airway Malformation

Keywords

bronchial atresia - bronchogenic cyst - pulmonary sequestration - congenital lobar emphysema - congenital pulmonary airway malformation

Publication History

Received: 20 September 2021

Accepted: 13 October 2021

Article published online:
19 January 2022

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