EP09.09: The importance of malformation counting method in patients with more malformed organs for the diagnostic accuracy in the tertiary referral centre

Abstract

Objectives: To describe the antenatal ultrasound findings and outcome of fetuses with echogenic lung masses. Methods: Ten fetuses with echogenic lung masses suspected prenatally by ultrasound were observed in a total of 2916 examinations performed in our unit from 2011 up to 2014. Eleven cases of congenital diaphragmatic hernia were excluded. Results: There were three cases of congenital pulmonary airway malformation (CPAM). Five cases of pulmonary sequestration (PS), three of them were considered to have also CPAM. One case of congenital high airway obstruction syndrome (CHAOS) and one case of unilateral bronchial obstruction (UBO). In all cases, a displacement of the heart and abnormal lung was observed and presence of an aberrant vessel rising from the descending aorta was seen in all PS cases. In 8 cases, magnetic resonance imaging (MRI) was performed, in 7/8 helped to clarify the diagnosis. The prenatal management in all cases was expectant and in cases of CPAM and PS + CPAM, a course of corticosteroids was administered. We observed prenatal remission of the lung mass in 6 cases (complete: 4 and partial: 2). One case of PS had hydrothorax, which decreased completely along gestation. The fetus with CHAOS develop hydrops and died in utero. The fetus with UBO had a right aortic arch and during pregnancy the obstruction decreases and the lung recovered to its normal size. Conclusions: The fetuses with echogenic lung masses is a rare congenital abnormality (1/1,000 birth). Clinical diagnosis can be suspected by US, but the final classification may be difficult and others diagnostic tools (MRI and histology) should be employed. In most cases prognosis is good (even in a case with PS and hydrothorax), except in CHAOS. Corticosteroid therapy should be offered in cases of CPAM and PS (mixed). EP09.09 The importance of malformation counting method in patients with more malformed organs for the diagnostic accuracy in the tertiary referral centre