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DOI: 10.1055/s-0042-124361
Primary Fetal Lung Hypoplasia (PFLH): Imaging and Clinical Characteristics
Primäre fetale Lungenhypoplasie (PFLH): Bildgebung und klinische MerkmalePublikationsverlauf
27. Mai 2016
02. Dezember 2016
Publikationsdatum:
14. Juni 2017 (online)
![](https://www.thieme-connect.de/media/ultraschall/201703/lookinside/thumbnails/uim-2196_10-1055-s-0042-124361-1.jpg)
Abstract
Purpose To describe in utero and postnatal imaging and clinical characteristics of primary fetal lung hypoplasia (PFLH).
Methods A retrospective review of fetuses and neonates diagnosed in one academic tertiary center during an eleven-year period.
Results 12 cases of PFLH were identified. 4 were bilateral and 8 had unilateral involvement. Prenatal sonographic characteristics, postnatal magnetic resonance imaging (MRI), computerized tomographic angiography (CTA), and histologic findings are described. 3 of the 4 bilateral cases were evaluated during fetal live. 2 were terminated and 2 died shortly after delivery. Among the 8 cases with unilateral PFLH, 7 involved the right lung and 1 the left lung. In fetuses with right hypoplasia, 5 showed characteristic features of Scimitar syndrome, while associated gastrointestinal tract (GIT) anomalies were presented in 2 cases. In this group 3 were born alive and the other 5 were terminated.
Conclusion Primary PFLH is a rare anomaly that lethal in its bilateral form and with variable prognosis in its unilateral variant. Targeted evaluation of lung vascularity and exclusion of associated anomalies, especially of the GIT, are important prognostic factors.
Zusammenfassung
Ziel Beschreibung der intrauterinen und postnatalen Bildgebung und der klinischen Merkmale bei primärer fetaler Lungenhypoplasie (PFLH).
Methoden Eine retrospektive Überprüfung von Feten und Neugeborenen, die in einem einzigen akademischen Tertiärzentrum über einen Zeitraum von 11 Jahren diagnostiziert wurden.
Ergebnisse Es wurden 12 Fälle mit PFLH diagnostiziert. Davon waren 4 bilateral und 8 zeigten unilaterale Beteiligung. Drei der 4 bilateralen Fälle wurden in der Fetalzeit bewertet. Die pränatalen sonografischen Merkmale, die postnatale Magnetresonanztomografie (MRT), die computertomografische Angiografie (CTA) und die histologischen Befunde werden beschrieben. Zwei der Fälle endeten mit Abbruch und 2 Kinder verstarben kurz nach der Geburt. In 7 von 8 Fällen mit unilateraler PFLH war die rechte und bei einem der Fälle die linke Lunge beteiligt. Bei Feten mit rechtsseitiger Hypoplasie zeigten 5 die charakteristischen Merkmale des Scimitar-Syndroms, während assoziierte Anomalien des Gastrointestinaltrakts (GIT) in 2 Fällen auftraten. In dieser Gruppe wurden 3 Kinder lebend geboren, die anderen 5 Schwangerschaften wurden abgebrochen.
Schlussfolgerung Die primäre PFLH ist eine seltene Anomalie, die in ihrer bilateralen Ausprägung letal verläuft und bei der die unilaterale Variante eine sehr variable Prognose hat. Die gezielte Bewertung der Vaskularität der Lunge und der Ausschluss von beteiligten Anomalien, insbesondere des GIT sind wichtige Prognosefaktoren.
* Both authors contributed equally
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