改善先天性膈疝的治疗效果——一家无体外膜肺氧合（ECMO）的三级中心的经验

Pimenta J, Vaz Silva P, Pinto C, Dinis A, Carvalho L, de Castro O, Neves F

Department of Pediatrics, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, EPE, Portugal.

Department of Cardiology, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, EPE, Portugal.

J Neonatal Perinatal Med. 2018;11(1):37-43. doi: 10.3233/NPM-181710.

BACKGROUND

Congenital diaphragmatic hernia (CDH) has a high mortality rate, representing a therapeutic challenge. Prenatal diagnosis (PND) is essential in defining optimal perinatal strategy, particularly delivery planning. Hospital Pediátrico de Coimbra is the referral centre for all neonatal surgery, particularly CDH, for the central region of Portugal. The aim was to evaluate clinical management and outcome of newborns with CDH.

METHODS

An exploratory retrospective study made up of newborns admitted to PICU with CDH was undertaken between January 1995 and December 2014. Two groups were formed based on their year of admission: group A (1995- 2004) and group B (2005- 2014) and were compared.

RESULTS

The mean birth weight of the 69 newborns admitted was 2.762 ± 696 g; the median of the gestational age was 38 weeks. Associated malformations were observed in 28 (40.5%) and 15 (21.7%) had a right-sided diaphragm defect. The global mortality was 13.0%; in group A was significantly higher than in group B (22.2 vs 3.0%; p = 0.029). A reduction in mortality throughout the years was confirmed after adjusting for POS score (OR = 0.77; 95% CI: 0.62- 0.96, p = 0.021). PND was made in 30.6% of cases in the group A and 66.7% in the group B (p = 0.03). Tertiary perinatal hospital birth was achieved in 60% of newborns in the group A versus 84.8% in group B (p = 0.022). Maximum fraction of inspired oxygen showed a statistically significant difference between the two study groups (60% vs 40%; p = 0.009).

CONCLUSIONS

A significant decrease in mortality was observed throughout the study. The authors highlight the increase in prenatal diagnosis and an improvement in perinatal care with planning delivery as important contributors to these results.

背景

先天性膈疝（CDH）死亡率高，是一项治疗挑战。产前诊断（PND）对于确定最佳围产期策略至关重要，尤其是分娩计划。科英布拉儿科医院是葡萄牙中部地区所有新生儿外科手术，特别是CDH手术的转诊中心。目的是评估CDH新生儿的临床管理和结局。

方法

对1995年1月至2014年12月间入住儿科重症监护病房（PICU）的CDH新生儿进行探索性回顾性研究。根据入院年份分为两组：A组（1995 - 2004年）和B组（2005 - 2014年）并进行比较。

结果

69例入院新生儿的平均出生体重为2.762±696g；胎龄中位数为38周。观察到28例（40.5%）有相关畸形，15例（21.7%）有右侧膈肌缺损。总体死亡率为13.0%；A组显著高于B组（22.2%对3.0%；p = 0.029）。校正POS评分后证实，多年来死亡率有所降低（OR = 0.77；95%CI：0.62 - 0.96，p = 0.021）。A组30.6%的病例进行了产前诊断，B组为66.7%（p = 0.03）。A组60%的新生儿在三级围产期医院出生，B组为84.8%（p = 0.022）。两组研究对象吸入氧最大分数有统计学显著差异（60%对40%；p = 0.009）。