新生儿先天性右侧膈疝的临床诊治

Clinical diagnoses and treatments of neonatal right congenital diaphragmatic hernia

目的探讨新生儿先天性右侧膈疝的产前诊断情况,临床特点及预后。方法回顾性分析23例先天性右侧膈疝患儿的临床资料。根据诊断的时间点分为产前诊断组(12例)和出生后诊断组(11例),统计两组出生胎龄、出生体重、生后机械通气率、需体外膜肺氧合(ECMO)支持率、手术例数、手术日龄、补片应用率,胸腔镜应用率、术后并发症发生率、存活率、术后复发率,采用t检验或Fisher精确概率法进行统计分析。结果产前诊断率为52.1%(12/23),生后需要ECMO支持率为21.7%(5/23),胸腔镜应用率为65%(15/23),需补片修补率39%(9/23例)。随访中位数38个月(8~90个月),总体存活率为83%(19/23),术后复发率为4%(1/23)。产前诊断组与生后诊断组中,出生胎龄、出生体重、生后机械通气率、需ECMO支持率、手术例数、手术日龄、胸腔镜应用率、存活率等指标的差异均无统计学意义(P>0.05)。结论右侧先天性膈疝的产前诊断中仍存在漏诊误诊,缺乏有效的肺发育评估指标;其生后胸腔镜手术应用率较高,总体预后良好。

Objective To investigate the prenatal diagnosis, clinical characteristics and prognosis of neonatal right-sided congenital diaphragmatic hernia(RCDH). Methods We reviewed records of infants with RCDH from 2013 to 2019. According to the timing of diagnosis, they were divided into prenatal diagnosis group(12 cases) and postnatal diagnosis group(11 cases). The perinatal management, postnatal treatment and prognosis in different groups were compared with t test or Fisher′s exact test. Results Twelve infants(52.1%) were prenatally diagnosed, and 11 infants(47.8%) were postnatally diagnosed, and 5 infants(21.7%) needed ECMO support. The rate of thoracoscopic repair was 65%(15/23), and 9 infants(39%) needed patch repair. The median follow-up was 38 months(8-90 months). The overall survival rate was 83%(19/23) and the rate of recurrence was 4%(1/23). There was no significant difference in gestational age, birth weight, ECMO therapy, age of operation, thoracoscopic repair or survival rate between prenatal diagnosis group and postnatal diagnosis group. Conclusion The prenatal diagnosis rate of RCDH is still low, and there is a lack of effective parameters for fetal lung development, but the overall prognosis is good.