Propranolol: A novel therapy for neonatal chylothorax secondary to congenital lymphatic anomaly

Introduction: Neonatal chylothorax, a common cause of pleural effusion in neonates, can occur secondary to trauma or from primary congenital causes. Congenital chylothorax due to chromosomal anomalies or lymphatic malformations can result in refractory chylothorax. Medical management using octreotide, sildenafil, and sirolimus has shown variable success, particularly with congenital chylothorax. These pharmacotherapeutic options have different adverse effect profiles that must be balanced against their effectiveness. Case Presentation: A late preterm infant was born to a consanguineous couple with antenatal suspicion of bilateral pleural effusion. After birth, the infant experienced respiratory distress, and bilateral chest tubes were placed, which drained significant amounts of chylous effusion over the subsequent 4 weeks. Whole genome sequencing revealed a PIEZO-1 mutation associated with congenital lymphatic anomaly. Conventional medical management, including intercostal drainage, medium-chain triglyceride-enriched formula, parenteral nutrition, prednisolone, and octreotide, was continued for an 8-week period without resolution. Propranolol was then initiated, leading to complete resolution of chylothorax. The infant was discharged home on propranolol with no recurrence noted on follow-up. Conclusion: This case report indicates that propranolol can be used as an effective therapy to treat moderate to severe chylothorax, especially in the context of congenital lymphatic anomaly, mainly because of its anti-vascular endothelial growth factor action.