目的 探讨新生儿先天性乳糜胸（CC）的临床特点及影响其预后的高危因素。方法 回顾性分析2012年1月至2022年5月在新生儿重症监护室收治的CC患儿的临床资料。结果 最终纳入CC患儿60例，男36例、女24例，胎龄36.7（34.7~38.8）周，出生体重3 160.0（2 755.0~3 645.0）g，早产儿31例，足月儿29例。产前诊断胸腔积液52例，宫内治疗13例，出生时窒息31例，在产房插管27例，复苏时胸腔穿刺8例，双侧胸腔积液30例，胎儿水肿19例，低蛋白血症29例，合并先天性乳糜腹14例，合并先心/染色体异常12例。与足月儿组相比，早产儿组男性比例较低，出生体重较轻，宫内治疗、胎儿水肿、窒息、在产房插管、机械通气、双侧胸腔积液、白蛋白<30 g/L、合并先天性乳糜腹以及死亡比例均较高，差异有统计学意义（P<0.05）。存活组49例，死亡组11例，死亡患儿均于生后1周内死于呼吸衰竭。二分类logistic回归分析发现，胎儿水肿是CC新生儿早期死亡的独立危险因素（P<0.05）。所有病例行保守治疗，随访存活患儿，3例失访，1例复发，余45例情况良好。结论 CC新生儿中，早产儿更容易出现窒息、胎儿水肿、低蛋白血症、双侧胸腔积液、先天性乳糜腹，在产房插管、机械通气及死亡的比例亦高于足月儿；伴胎儿水肿的CC患儿早期死亡风险增大；保守治疗的远期预后良好。

Objective To explore the clinical characteristics of neonatal congenital chylothorax (CC) and the high-risk factors affecting its prognosis. Methods The clinical data of children with CC admitted to the neonatal intensive care unit from January 2012 to May 2022 were retrospectively analyzed. Results Sixty children (36 boys and 24 girls) with CC were included, including 31 premature infants and 29 full-term infants. The gestational age was 36.7 (34.7-38.8) weeks, and the birth weight was 3160.0 (2755.0-3645.0) g. There were 52 cases of prenatal diagnosis of pleural effusion, 13 cases of intrauterine treatment, 31 cases of asphyxia at birth, 27 cases of intubation in the delivery room, 8 cases of pleural puncture during resuscitation, 30 cases of bilateral pleural effusion, 19 cases of fetal edema, 29 cases of hypoproteinemia, 14 cases of congenital chylous ascites, and 12 cases of congenital heart disease/chromosomal abnormalities. Compared with the full-term infant group, the preterm infant group had a lower male proportion, lower birth weight, higher rates of intrauterine treatment, fetal edema, asphyxia, intubation in the delivery room, mechanical ventilation, bilateral pleural effusion, albumin <30g/L, congenital chylous ascites and death, and the differences were significant (P<0.05). There were 49 children in the survival group and 11 in the death group. All the children died of respiratory failure within 1 week after birth. Binary logistic regression analysis showed that fetal edema was an independent risk factor for early neonatal death in children with CC (P<0.05). All children were treated conservatively and followed up. Three children were lost to follow-up, 1 relapsed, and the remaining 45 were in good condition. Conclusions Among neonates with CC, preterm infants are more likely to suffer from sphyxia, fetal edema, hypoproteinemia, bilateral pleural effusion, congenital chylous ascites, and the proportion of intubation in the delivery room, mechanical ventilation and death is higher than that of full-term infants. The CC neonates with fetal edema have an increased risk of early death. The long-term prognosis of children treated conservatively is good.