Tai Hung-Lin, Mok Tze Yee Diane, Chao An-Shine, Chu Shih-Ming, Lien Reyin
Department of Pediatrics, Saint Paul's Hospital, Taoyuan City, Taiwan.
Division of Neonatalogy, Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan City, Taiwan.
Front Pediatr. 2021 Feb 17;9:633051. doi: 10.3389/fped.2021.633051. eCollection 2021.
Idiopathic congenital chylothorax is a rare but serious disease. Advancement in perinatal care and the renovated treatment modalities have brought about significant improvement in patient outcome. To describe the clinical course of severe forms of idiopathic congenital chylothorax, focusing on the development of recent treatment modalities and their impacts. A retrospective cohort by review of medical records in the NICU of a perinatal referral center in Taiwan. Study period was from January 2006 to June 2017. Neonates with the diagnosis of idiopathic congenital chylothorax with non-immune hydrops fetalis were enrolled. Clinical relevant including demographic data, perinatal interventions, post-natal course, and treatment outcome were described and analyzed. Twenty-eight neonatal patients were included. The median gestational age at birth was 34 (range 27-36) weeks and median birth weight was 2,369 (range 1,430-3,608) g. Prenatal intervention was performed in 39.3% of the patients. Besides, 11 out of the 28 neonates developed tension pneumothorax in the first 24 h and 4 (36.4%) of them died. Sepsis was documented in two patients (7.1%). Overall survival rate was 71.4%. There were five patients enrolled during the last 2 years of the study period. EXIT with intubation was performed in two and octreotide was given to four of these most recent neonates, and all of them survived. Recent advances in the management of these neonates, specifically EXIT with intubation and use of octreotide. Both of them improved patient survival in our cohort, but the evidence of impact has yet to be validated.
特发性先天性乳糜胸是一种罕见但严重的疾病。围产期护理的进步和治疗方式的革新使患者的预后有了显著改善。描述特发性先天性乳糜胸严重形式的临床过程，重点关注近期治疗方式的发展及其影响。通过回顾台湾一家围产期转诊中心新生儿重症监护病房的病历进行回顾性队列研究。研究期间为2006年1月至2017年6月。纳入诊断为特发性先天性乳糜胸并伴有非免疫性胎儿水肿的新生儿。描述并分析临床相关信息，包括人口统计学数据、围产期干预措施、出生后病程及治疗结果。纳入28例新生儿患者。出生时的中位胎龄为34（范围27 - 36）周，中位出生体重为2369（范围1430 - 3608）g。39.3%的患者进行了产前干预。此外，28例新生儿中有11例在出生后24小时内发生张力性气胸，其中4例（36.4%）死亡。2例患者（7.1%）记录有败血症。总体生存率为71.4%。在研究期的最后2年纳入了5例患者。其中2例进行了带气管插管的产时宫外治疗（EXIT），4例最近的新生儿使用了奥曲肽，所有这些患者均存活。这些新生儿管理方面的最新进展，特别是带气管插管的产时宫外治疗和奥曲肽的使用。两者都提高了我们队列中的患者生存率，但这种影响的证据尚未得到验证。
