Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations.
OBJECTIVE
Congenital pulmonary malformations (CPM) are mostly recognized on prenatal ultrasound scans. In a minority of cases, they may impair breathing at birth. The factors predictive of neonatal respiratory distress are not well defined, but an understanding of these factors is essential for decisions concerning the need for the delivery to take place in a tertiary care center. The aim of this study was to identify potential predictors of respiratory distress in neonates with CPM.


METHODS
We selected cases of prenatal diagnosis of hyperechoic and/or cystic lung lesions from RespiRare, the French prospective multicenter registry for liveborn children with rare respiratory diseases (2008-2013). Prenatal parameters were correlated with neonatal respiratory outcome.


RESULTS
Data were analyzed for 89 children, 22 (25%) of whom had abnormal breathing at birth. Severe respiratory distress, requiring oxygen supplementation or ventilatory support, was observed in 12 neonates (13%). Respiratory distress at birth was significantly associated with the following prenatal parameters: mediastinal shift (P = .0003), polyhydramnios (P = .05), ascites (P = .0005), maximum prenatal malformation area (P = .001), and maximum congenital pulmonary malformation volume ratio (CVR) (P = .001). Severe respiratory distress, requiring oxygen at birth, was best predicted by polyhydramnios, ascites, or a CVR >0.84.


CONCLUSIONS
CVR >0.84, polyhydramnios, and ascites increased the risk of respiratory complications at birth in fetuses with CPM, and especially of severe respiratory distress, requiring oxygen supplementation or more intensive intervention. In such situations, the delivery should take place in a tertiary care center.
Pneumologie Pédiatrique, Necker, AP-HP, France; Centre de Référence des Maladies Respiratoires Rares, Paris, France;
Isabelle
I
Ruchonnet-Metrailler
Pneumologie Pédiatrique, Necker, AP-HP, France; Centre de Référence des Maladies Respiratoires Rares, Paris, France;
Elise
E
Leroy-Terquem
Gynécologie-Obstétrique, Necker, AP-HP, France;
Julien
J
Stirnemann
Pneumologie Pédiatrique, CHU Brest, France;
Pierrick
P
Cros
Pneumologie Pédiatrique, CH Lens, France;
Héloïse
H
Ducoin
Pneumologie Pédiatrique, Necker, AP-HP, France; Centre de Référence des Maladies Respiratoires Rares, Paris, France; Université Paris-Descartes, Paris, France;
Alice
A
Hadchouel
Université Paris-Descartes, Paris, France; Chirurgie Pédiatrique, Necker, AP-HP, France;
Naziha
N
Khen-Dunlop
Pneumologie Pédiatrique, CHU Clermont-Ferrand, France;
André
A
Labbé
Pneumologie Pédiatrique, CHU Toulouse, France;
Géraldine
G
Labouret
Pneumologie Pédiatrique, Robert Debré, AP-HP, France;
Marie-Noëlle
MN
Lebras
Pneumologie Pédiatrique, Necker, AP-HP, France; Centre de Référence des Maladies Respiratoires Rares, Paris, France; Université Paris-Descartes, Paris, France;
Guillaume
G
Lezmi
Centre de Référence des Maladies Respiratoires Rares, Paris, France; Pneumologie Pédiatrique, CHI Créteil, France;
Fouad
F
Madhi
Gynécologie-Obstétrique, Necker, AP-HP, France; Université Paris-Descartes, Paris, France;
Laurent J
LJ
Salomon
Centre de Référence des Maladies Respiratoires Rares, Paris, France; Pneumologie Pédiatrique, Trousseau, AP-HP, France; and.
Guillaume
G
Thouvenin
Pneumologie Pédiatrique, CHRU Lille, France.
Caroline
C
Thumerelle
Pneumologie Pédiatrique, Necker, AP-HP, France; Centre de Référence des Maladies Respiratoires Rares, Paris, France; Université Paris-Descartes, Paris, France; christophe.delacourt@nck.aphp.fr.
Christophe
C
Delacourt
D001201:Ascites / Q000000981:diagnostic imaging; D001998:Bronchopulmonary Sequestration / Q000000981:diagnostic imaging*; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000000981:diagnostic imaging*; D005260:Female; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D008297:Male; D006831:Polyhydramnios / Q000000981:diagnostic imaging; D011247:Pregnancy; D011256:Pregnancy Outcome; D011262:Pregnancy Trimester, Second; D011446:Prospective Studies; D012042:Registries; D012127:Respiratory Distress Syndrome, Newborn / Q000000981:diagnostic imaging* / Q000209:etiology*; D012307:Risk Factors; D013223:Statistics as Topic; D016216:Ultrasonography, Prenatal
congenital cystic adenomatoid malformation; lung development; sequestration