Zusammenfassung
Trotz verbesserter Therapien stellt die kongenitale zystadenomatoide Malformation der Lunge für den Feten weiterhin eine potenziell lebensbedrohliche Missbildung dar. Pränatal sind insbesondere ein Hydrops fetalis sowie der Spontanverlauf von prognostischer und therapeutischer Relevanz. Postnatal kommt der Lungenhypoplasie eine entscheidende Bedeutung zu. Aufgrund der hohen Verfügbarkeit und Sensitivität bleibt die Primärdiagnostik weiterhin dem Ultraschall vorbehalten. Die fetale MRT hingegen hat sich insbesondere bei unklarer sonographischer Befundlage als Methode der Wahl etabliert. Neben der Diagnosesicherung erleichtert sie durch eine übersichtliche Darstellung der Befundausdehnung häufig die Abschätzung einer postnatalen Intensivtherapie. Mit zunehmender Erfahrung beim Einsatz fetalchirurgischer Verfahren kommt ihr ferner eine wachsende Bedeutung in der Indikationsstellung und Planung pränataler Therapien zu. Beides erfordert u. U. die zeitnahe Zuweisung in eine Spezialklinik.
Abstract
Despite advancing therapeutic strategies, congenital cystadenomatoid malformation of the fetal lung is still a potentially life-threatening anomaly. Antenatally, the development of hydrops as well as the natural history of the malformation is of particular therapeutic and prognostic importance. Postnatally, respiratory distress due to pulmonary hypoplasia counts as a crucial limiting factor. Owing to its feasibility and practicability, as well as a high sensitivity, antenatal ultrasound is still the screening method of choice for the detection of fetal thoracic malformations. However, particularly in cases of indistinguishable sonographic findings, fetal MRI is the modality of choice for proving the diagnosis and preliminary appraisal of intensive care therapy and extracorporal membrane oxygenation postnatally. Furthermore, fetal MRI often facilitates assessment and planning of intrauterine surgical procedures. These two features frequently require a close transfer to an expert neonatal centre.
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Büsing, K.A., Kilian, A.K., Schaible, T. et al. Fetale Magnetresonanztomographie. Radiologe 46, 133–138 (2006). https://doi.org/10.1007/s00117-005-1311-1
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DOI: https://doi.org/10.1007/s00117-005-1311-1