Kongenitale Pulmonale Atemwegsmalformation (CPAM)

◆Diagnostische Schlüsselhinweise

• 1CT: Zystische oder gemischte Lungenmasse in einzelnem Lappen — Zysten luft- oder flüssigkeitsgefüllt
• 2Normale pulmonalarterielle Versorgung (keine systemische — nicht Sequestration)
• 3Pränataler US: Echogene oder zystische thorakale Masse — beim Anomaliescreening detektiert
• 4CXR: Multizystische oder solide Lungenverschattung — Einzellappenbeteiligung
• 5Stocker-Klassifikation: Typ 1 (>2 cm Zysten) am häufigsten, Typ 4 (Pleuropulmonales Blastomrisiko) am gefährlichsten

♦Pathophysiologie

Differentialdiagnostische Tipps

• →Pulmonale Sequestration: systemische arterielle Versorgung (Aorta), CPAM hat pulmonale arterielle Versorgung
• →CDH: Darmschlingen im Thorax, Zwerchfelldefekt, keine Lungenmasse
• →Kongenitales Lobaremphysem: überblähter Lappen, nicht zystisch, Air Trapping
• →Bronchogene Zyste: solitär, unilokulär, mediastinal oder intrapulmonal, meist Wasserdichte

●Klinische Bedeutung

Am häufigsten in der Pränatalperiode erkannte kongenitale Lungenanomalie. Große Läsionen können Hydrops fetalis und Lungenhypoplasie verursachen. CVR (CPAM-Volumen-Verhältnis) >1,6 erhöht das Hydrops-Risiko. Spontane Regression ist möglich, aber vollständiges Verschwinden ist selten (kann in der Bildgebung verborgen sein). Das Risiko maligner Transformation (pleuropulmonales Blastom, 1-4% bei Typ 1) macht die elektive Resektion kontrovers, aber allgemein empfohlen. Rezidivierende Pneumonien bei symptomatischen Fällen indizieren eine Operation.

Referenzen

• Stocker JT. Congenital pulmonary airway malformation — a new name and an expanded classification. Histopathology. 2002;41 Suppl 2:424-31.
• Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Laberge JM, et al. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation. Fetal Diagn Ther. 2001;16(3):178-86.
• ACR Appropriateness Criteria: Neonatal Respiratory Distress. 2020.
• Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Hill DA, et al. Type I pleuropulmonary blastoma: pathology of the DICER1 syndrome. Am J Surg Pathol. 2009;33(11):1578-88.
• Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Adzick NS. Management of fetal lung lesions. Clin Perinatol. 2003;30(3):481-92.