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Kongenitale Lungenfehlbildungen

Diagnostik und Therapie

Congenital pulmonary malformations

Diagnosis and treatment

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Zusammenfassung

Leistungsfähigkeit

Kongenitale Lungenfehlbildungen (KL) sind selten und können mit einer hohen Morbidität einhergehen. Klinik, diagnostisches Vorgehen, Bildmorphologie und Therapie werden diskutiert.

Bewertung

Die meisten kongenitalen Lungenfehlbildungen werden heutzutage pränatal durch Ultraschall diagnostiziert. Postnatal sind respiratorische Symptome bis zum respiratorischen Versagen und rezidivierende Infektionen typisch. Wegen der geringen diagnostischen Aussagekraft der konventionellen Röntgenuntersuchung sollten alle Kinder mit pränatal diagnostizierter oder postnatal vermuteter kongenitaler Lungenfehlbildung eine Schnittbildgebung des Thorax erhalten.

Empfehlung für die Praxis

Anhand der Bildgebung können die verschiedenen Unterformen der kongenitalen Lungenfehlbildungen nicht mit Sicherheit abgegrenzt werden, so dass eine histologische Diagnosesicherung der Goldstandard ist. Kongenitale Lungenfehlbildungen werden meistens reseziert, wobei zunehmend minimal-invasive Verfahren zum Einsatz kommen. Auch ein Watch-and-wait-Ansatz ist unter bestimmten Voraussetzungen vertretbar.

Abstract

Performance

Congenital pulmonary malformations (CPM) are rare and can be associated with high morbidity. Clinical presentation, diagnostic procedures, imaging, and therapy of CPM are discussed.

Achievements

Today, most CPM can be diagnosed prenatally by ultrasound. Postnatally, respiratory symptoms up to respiratory failure and recurrent lower respiratory tract infection are typical findings. Due to low diagnostic accuracy of chest x‑ray in CPM, all children with prenatal diagnosis of CPM or postnatally suspected CPM should undergo cross-sectional imaging.

Practical recommendations

Based on imaging alone, the various subtypes of CPM cannot be definitively differentiated, which is why histological confirmation remains the gold standard. Surgical resection is the standard of care with minimally invasive procedures increasingly being employed. In certain situations, a watch-and-wait approach is possible.

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Danksagung

Die Autoren danken Frau Dr. Rim Kiblawi für die Bereitstellung der Abb. 1 (erstmals veröffentlicht in „Kinder- und Jugendarzt“, Ausgabe 09/2022, S. 592–600, Hansisches Verlagskontor GmbH, Lübeck.) sowie Herrn PD Dr. Nikolaus Schwerk für die wertvollen klinischen Hinweise.

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Authors and Affiliations

  1. Institut für diagnostische und interventionelle Radiologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    M. Dohna & J. Dingemann

  2. Klinik für diagnostische und interventionelle Radiologie, Universitätsklinikum Bonn, Venusberg-Campus 1, 53127, Bonn, Deutschland

    M. Dohna

  3. Universitätsklinikum Leipzig, Leipzig, Deutschland

    W. F. Hirsch & D. Gräfe

  4. Klinik für Kinderchirurgie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    J. Dingemann

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  1. M. Dohna
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  2. W. F. Hirsch
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Correspondence to M. Dohna.

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M. Dohna, W. F. Hirsch, J. Dingemann und D. Gräfe geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Der Verlag bleibt in Hinblick auf geografische Zuordnungen und Gebietsbezeichnungen in veröffentlichten Karten und Institutsadressen neutral.

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Dohna, M., Hirsch, W.F., Dingemann, J. et al. Kongenitale Lungenfehlbildungen. Radiologie (2024). https://doi.org/10.1007/s00117-024-01291-3

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