探讨难治性先天性乳糜胸(CC)新生儿的临床特点、治疗方法。

选择2015年6月至2019年12月，在首都儿科研究所附属儿童医院住院治疗的11例难治性CC新生儿为研究对象，回顾性分析其临床表现、辅助检查结果、治疗、转归和随访情况。本研究遵循的程序符合2013年新修订的《世界医学协会赫尔辛基宣言》，并与患儿监护人签署临床研究知情同意书。

①一般临床资料：11例难治性CC患儿的起病日龄<28 d，入院日龄为1~103 d。其中男性患儿为6例，女性为5例；足月儿为8例，早产儿为3例，出生胎龄为31~40周；接受产前诊断为4例，合并胎儿水肿为3例。②临床表现：生后10 min内起病为7例，11例患儿均表现为呼吸急促，患侧肺部呼吸音减弱，胸部X射线摄片、胸部CT或胸部超声检查结果均提示胸腔积液，其中双侧胸腔积液为7例，单侧胸腔积液(左侧)为4例，胸腔积液乳糜试验均呈阳性。③治疗方法：入院后，对11例患儿均进行保守治疗(饮食调节、呼吸支持、胸腔闭式引流、奥曲肽微量泵持续注射治疗、抗感染治疗等) 2~4周，失败后，对10例采用化学胸膜固定术(胸腔内注射红霉素)，其中7例患儿联合奥曲肽微量泵持续注射治疗，单侧胸腔注射次数为1~7次，未同时联合奥曲肽微量泵持续注射治疗者，胸腔内单侧注射红霉素次数为3~7次。胸腔内注射红霉素过程中，4例患儿出现心率加快、烦躁，1例出现明显血糖浓度升高(18.8 mmol/L)。2例患儿行胸腔镜下探查术和乳糜瘘修补术。④转归：11例难治性CC经治疗后，均吸收好转，住院天数为40~73 d，其中1例自动出院后死亡，1例因怀疑气管食管瘘家长放弃治疗后失访。其余9例患儿出院后均无复发。

难治性CC多见于足月儿，对其采取早期饮食调节、静脉营养、胸腔穿刺闭式引流和奥曲肽静脉输注保守治疗2~4周无效的患儿，可联合化学胸膜固定术(胸腔内注射红霉素)治疗。对于上述治疗>4周无效者，可采用胸腔镜下探查术，明确渗漏点后，予以淋巴管瘘修补术，以提高对难治性CC的治愈率。

To explore the clinical characteristics and treatment strategies of refractory congenital chylothorax (CC) in neonates.

Eleven neonates with refractory CC who were hospitalized in Children′s Hospital, Capital Institute of Pediatrics from June 2015 to December 2019 were selected as research subjects. The clinical manifestations, ancillary tests, treatment strategies, regression and follow-up were retrospectively analyzed. The procedures followed in this study were in accordance with the World Medical Association Declaration of Helsinki revised in 2013, and informed consent for clinical study was signed with the guardians of neonates.

①Among the 11 cases of refractory CC, 8 were term infants and 3 were preterm infants, with gestational age ranging from 31 to 40 weeks; 6 were boys and 5 were girls; 4 received prenatal diagnosis and 3 were combined with fetal edema. ② Seven cases developed within 10 min after birth, 11 cases showed tachypnea, weakening of respiratory sound in affected lung, chest X-ray, chest CT or chest ultrasonography all suggested pleural effusion, including 7 cases of bilateral pleural effusion and 4 cases of unilateral pleural effusion (left side), and the pleural effusion test showed celiac fluid. ③ After failure of 2-4 weeks of conservative treatment (dietary modification, respiratory support, thoracic close drainage, octreotide, anti-infective therapy, etc.), 10 cases were treated with chemical pleural fixation (intra-thoracic injection of erythromycin), of which 7 cases were treated with octreotide during the same time, and the number of erythromycin intrathoracic injections was 3-7 for those who did not combine with octreotide. During intrapleural injection of erythromycin, 4 children showed tachycardia and irritability, and 1 patient showed significantly increased blood glucose (18.8 mmol/L). Two cases underwent thoracoscopic exploration and repair of chylous fistula. ④After treatment, 11 cases of refractory CC were absorbed and improved, and the total length of hospital stay was 40-73 days. Among them, 1 case died after automatic discharge, and 1 case was lost to follow-up because of suspected parents of tracheoesophageal fistula. No recurrence occurred after discharge.

Refractory CC is common in full-term children. For children who have failed in early dietary regulation, intravenous nutrition, thoracic close drainage and octreotide intravenous infusion for 2-4 weeks, chemical pleural fixation (intrathoracic injection of erythromycin) can be combined. For patients with ineffective treatment for more than 4 weeks, thoracoscopic exploration can be used to repair lymphatic fistula after identifying the leak in order to improve the cure rate of refractory CC.