Fetal intervention for congenital chylothorax is associated with improved outcomes in early life

Abstract Background Congenital chylothorax (CC) can have devastating consequences for neonates. We sought to determine the outcomes of cases treated at our institution and evaluate the role of fetal intervention. Materials and methods With Institutional Review Board approval, patients treated at our institution 09/2006-04/2016 with CC were reviewed. History and outcomes were compared between patients undergoing fetal intervention (fetal group) and patients who did not (control group). Results Twenty-three patients were identified. Mean gestational age at birth was 35 wk. Overall mortality was 30% (7 patients). Nineteen patients (83%) were prenatally diagnosed, and 10 patients (43%) underwent fetal intervention. Birth weight was significantly lower in the fetal group compared to the control group (median interquartile range [IQR]; 2.5 [2.3-3.0] versus 3.3 [2.6-3.7] kg, P  = 0.02). Apgar scores were significantly higher in the fetal group than the control group at 1 and 5 min (median [IQR]; 6 [4-8] versus 1 [1-2], P  = 0.005 and 8 [7-9] versus 2 [2-6], P  = 0.008, respectively). For those patients with prenatal diagnosis of CC and hydrops fetalis, thrombosis and lymphopenia were both improved in the fetal group (thrombosis 0% versus 40%, P  = 0.03; lymphocyte nadir [median {IQR}] 1.5 [0.6-2.9] versus 0.1 [0.05-0.2], P  = 0.02). Duration of support with mechanical ventilation was significantly shorter in the fetal group (median [IQR]; 1 [0-40] versus 41 [29-75] d, P  = 0.04). Conclusions Fetal intervention for CC is associated with improved Apgar scores and decreased ventilator days and complications in patients with hydrops fetalis. Fetuses with chylothorax, especially those with hydrops, should be referred to a fetal center for evaluation.