先天性乳糜胸（CC）是新生儿期胸腔积液最常见病因，其围产期病死率为15%~57%，当伴随有胎儿水肿时，病死率可高达98%。目前对于CC尚无标准化治疗指南。本研究回顾性分析2例以难治性胸腔积液为主要表现的先天性乳糜胸新生儿的临床诊治及随访情况。并以关键词检索国内外数据库，检索时间为建库至2024年9月，总结难治性先天性乳糜胸新生儿的临床特点和治疗进展。2例患儿均为近足月早产儿，胎儿超声示双侧胸腔积液，生后呼吸困难、口唇发绀，立即转入新生儿重症监护病房，胸腔积液示三酰甘油浓度升高，明确为先天性乳糜胸，经气管插管机械通气、胸腔穿刺闭式引流、饮食调节、静脉营养和奥曲肽静脉输注保守治疗>4周无效，予普萘洛尔口服。例1胸腔积液完全吸收出院，随访至6月龄，无复发。例2胸腔积液好转出院，停用普萘洛尔后复发，予动态观察，1周后自行吸收，随访至7月龄，无复发。本研究提示，对于保守治疗无效的难治性先天性乳糜胸，普萘洛尔可能是一种有效且安全的治疗选择。

Congenital chylothorax (CC) is the most common cause of pleural effusion during the neonatal period, with a reported perinatal mortality rate ranging from 15% to 57%. When associated with fetal hydrops, this mortality rate can increase dramatically, reaching as high as 98%. Currently, there are no universally accepted standardized treatment guidelines for CC. This study retrospectively reviewed the clinical presentation, diagnosis, treatment, and follow-up outcomes of two neonates diagnosed with refractory congenital chylothorax. Additionally, a comprehensive literature search was conducted using relevant keywords in both domestic and international databases from their inception to September 2024, aiming to summarize the clinical features and recent advances in the management of refractory congenital chylothorax in neonates. Both infants were born as near-term preterm babies and had prenatal ultrasound findings of bilateral pleural effusion. Following birth, they exhibited signs of respiratory distress and cyanosis of the lips, necessitating immediate transfer to the neonatal intensive care unit (NICU). The presence of elevated triglyceride levels in the pleural fluid confirmed the diagnosis of congenital chylothorax. Despite more than four weeks of conservative management—including endotracheal intubation with mechanical ventilation, closed thoracic drainage, dietary modifications, parenteral nutrition, and intravenous octreotide infusion—clinical improvement was not observed. Subsequently, oral propranolol was initiated. In case 1, complete resolution of pleural effusion was achieved before discharge, and no recurrence was observed during follow-up until six months of age. In case 2, the effusion showed partial improvement at discharge. However, after discontinuation of propranolol, recurrence occurred. The infant was managed with close observation, and the effusion resolved spontaneously within one week. No recurrence was noted during follow-up until seven months of age. This study suggests that propranolol may serve as a potentially effective and safe therapeutic option for refractory congenital chylothorax when conventional conservative treatments fail.