Perinatal features of congenital cystic lung diseases: results of a nationwide multicentric study in Japan.

Abstract

The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD). Of the 874 CCLD patients identified in a nationwide survey, 428 patients born between 1992 and 2012 and treated at 10 high-volume centers, were retrospectively reviewed. Fetal hydrops was visualized using MRI in 9.2% of the patients. Prenatal interventions were described for 221 of the 428 patients, including the maternal administration of steroid and pleuro-amniotic shunting. Postnatally, a right-to-left shunt flow through a persistent ductus arteriosus was observed in 7.8% of the patients. The fetal lung lesion volume ratio (LVR) was significantly higher among these symptomatic patients (2.04±1.71 vs. 0.98±0.50, P<0.00071), and decreased to a greater degree in non-CCAM patients compared with CCAM patients during the late gestational period (from 1.37±1.28 to 1.14±0.84 in CCAM and from 1.08±0.47 to 0.46±0.64 in non-CCAM). An estimated 8-9% of prenatally diagnosed patients carry the highest risk of perinatal respiratory distress. Fetal LVR remaining at a high level during the late gestational period seems to predict a high risk.