Congenital Lung Lesion: Prenatal Course, Therapy and Predictors of Perinatal Outcome

Abstract

Purpose To describe the prenatal course and perinatal outcome, and to define prognostic markers for fetuses with congenital pulmonary airway malformation (CPAM) or bronchopulmonary sequestration (BPS).

Materials and Methods A retrospective study was performed at the University Hospital Zurich including pregnancies with either fetal CPAM (n = 26) or BPS (n = 11) between 2000 and 2013.

Results Three patients decided for termination of pregnancy. Two intrauterine deaths (CPAM) occurred at 25 weeks. Minimally invasive interventions were performed in 9/37 (24 %) fetuses, post-interventional survival was 8/9 (89 %). Mean gestational age at delivery was 38.1 +/–2.8 and 39.1 +/–2.5 weeks in fetuses with CPAM or BPS, respectively. In fetuses with CPAM the perinatal mortality rate was 4/24 (17 %); the rate of invasive interventions or surgery during the early neonatal period (neonatal morbidity) was 9/22 (41 %). Prenatal diagnosis of hydrothorax and/or increasing cystic volume ratio (CVR) until delivery preceded perinatal death in 3/5 (60 %). Absent mediastinal shift showed a neonatal morbidity rate of 1/8 (13 %) without any perinatal mortality. In fetuses with BPS the perinatal morbidity and mortality were both 1/10 (10 %). Hydrops predicted morbidity and mortality in 100 % of cases. Absent hydrops was followed by uncomplicated perinatal outcome.

Conclusion Fetuses with CPAM or BPS have a good outcome under optimal perinatal care including the possibility to perform minimally invasive prenatal interventions. CPAM without mediastinal shift and BPS without hydrops have an excellent prognosis. Hydrothorax, increasing CVR or hydrops indicates a high risk for perinatal morbidity and mortality.

Zusammenfassung

Ziel Analyse des pränatalen Verlaufes und perinatalen Outcome sowie die Identifikation von Prognosefaktoren für Feten mit Congenital pulmonary airway malformation (CPAM) und bronchopulmonale Sequester (BPS).

Material und Methoden Retrospektive Analyse aller Schwangerschaften (SS) mit fetaler CPAM (n = 26) bzw. BPS (n = 11) abgeklärt am Universitätsspital Zürich von 2000 bis 2013.

Ergebnisse Drei Schwangere entschieden sich zur Abruptio, in zwei SS kam es mit intrauterinem Fruchttod in der 25. SSW (CPAM). Minimal-invasive Interventionen wurden bei 9/37 (24 %) Feten durchgeführt, postinterventionell überlebten 8/9 (89 %). Die Geburt von Feten mit CPAM oder BPS fand bei 38,1 +/–2,8 und 39,1 +/–2,5 SSW statt. Bei Neugeborenen mit CPAM betrug die perinatale Mortalität 4/24 (17 %); Die Rate invasiver Interventionen oder Operationen in der frühen neonatalen Periode (neonatale Morbidität) war 9/22 (41 %). Nach Hydrothorax oder steigender CVR bis zur Geburt kam es zum perinatalen Versterben in 3/5 (60 %). Fehlende Mediastinalverlagerung war gefolgt von neonataler Morbidität in 1/8 (13 %), keine perinatale Mortalität. Bei Feten mit BPS betrug die perinatale Morbidität und Mortalität jeweils 1/10 (10 %). In beiden Fällen wurde zuvor ein Hydrops diagnostiziert. Fehlender Hydrops war gefolgt von unkompliziertem perinatalen Outcome.

Schlussfolgerung Feten mit CPAM oder BPS haben ein gutes Outcome bei optimaler perinatologischer Betreuung mit der Möglichkeit minimal-invasiver pränataler Interventionen. Feten mit CPAM ohne Mediastinalverschiebung und BPS ohne Hydrops haben eine exzellente Prognose. Hydrothorax, steigende CVR oder Hydrops sind Zeichen eines hohen Risikos für perinatale Morbidität und Mortalität.

Key words

malformation - obstetrics - treatment effects - outcomes analysis

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