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一种使用胎儿磁共振成像进行风险分层以预测先天性膈疝结局的新方法:初步回顾性单机构研究。

A new approach to risk stratification using fetal MRI to predict outcomes in congenital diaphragmatic hernia: the preliminary retrospective single institutional study.

作者信息

Yokoi Akiko, Ohfuji Satoko, Yoshimoto Seiji, Sugioka Yusuke, Akasaka Yoshinobu, Funakoshi Toru

机构信息

Department of Pediatric Surgery, Kobe Children's Hospital, Kobe, Japan.

Department of Public Health, Osaka City University Graduate School of Medicine, Osaka, Japan.

出版信息

Transl Pediatr. 2018 Oct;7(4):356-361. doi: 10.21037/tp.2018.09.01.

Abstract

BACKGROUND

Congenital diaphragmatic hernia (CDH) is a condition with a wide range of severity. Prenatal diagnosis is essential to optimize postnatal management, especially for severe cases. The lung to head ratio (LHR) and liver herniation estimated by prenatal ultrasound has been used as prenatal predictors in CDH. However, reliability of these factors remains to be proven and prediction of outcome from prenatal imaging is still challenging. We propose our new stratification system using lung to liver signal intensity ratio (LLSIR) in fetal MRI, which has been shown to be related to pulmonary maturation.

METHODS

Retrospective chart review was conducted on 25 infants with CDH treated from 2009 through 2016 in our hospital. We stratified patients according to fetal T2-weighted MRI as Grade I, detectable ipsilateral lung at the apex; Grade II, undetectable ipsilateral lung at the apex and contralateral LLSR ≥2.0; Grade III, undetectable ipsilateral lung at the apex and contralateral LLSR <2.0. To evaluate this stratification system, we analyzed survival, severity [inhaled nitric oxide (iNO) usage with or without extracorporeal membrane oxygenation (ECMO)], and requirement of patch closure.

RESULTS

All 15 patients survived in Grade I, while 2 out of 6 died in Grade II, and 3 out of 4 died in Grade III (P=0.003). Four were severe in Grade I, and all in Grade II and III who survived (P=0.007). One needed patch in Grade I, and all in Grade II and III (OR: 414,238,332; 95% CI, 0-∞). Liver herniation was noted in five patients, and significantly associated with survival (P=0.04), however, neither with severity (P=1.00) nor with the requirement of patch closure (P=0.52).

CONCLUSIONS

The risk stratification algorithm using contralateral LLSIR in fetal MRI could be useful and more reliable than liver herniation to predict survival, severity, and need of patch closure. Further investigation is warranted.

摘要

背景

先天性膈疝(CDH)病情严重程度差异很大。产前诊断对于优化产后管理至关重要,尤其是对于重症病例。产前超声评估的肺头比(LHR)和肝脏疝出情况已被用作CDH的产前预测指标。然而,这些因素的可靠性仍有待证实,并且通过产前影像学预测预后仍然具有挑战性。我们提出了一种基于胎儿MRI中肺肝信号强度比(LLSIR)的新分层系统,该系统已被证明与肺成熟度相关。

方法

对2009年至2016年在我院接受治疗的25例CDH婴儿进行回顾性病历审查。根据胎儿T2加权MRI将患者分为:I级,肺尖可检测到同侧肺;II级,肺尖未检测到同侧肺且对侧LLSR≥2.0;III级,肺尖未检测到同侧肺且对侧LLSR<2.0。为评估该分层系统,我们分析了生存率、严重程度[使用或未使用体外膜肺氧合(ECMO)时吸入一氧化氮(iNO)的情况]以及补片修补的需求。

结果

I级的15例患者全部存活,而II级的6例中有2例死亡,III级的4例中有3例死亡(P=0.003)。I级中有4例病情严重,II级和III级中所有存活者病情均严重(P=0.007)。I级中有1例需要补片修补,II级和III级中所有患者均需要(比值比:414,238,332;95%置信区间,0至无穷大)。5例患者出现肝脏疝出,且与生存率显著相关(P=0.04),但与严重程度(P=1.00)及补片修补需求均无关(P=0.52)。

结论

基于胎儿MRI中对侧LLSIR的风险分层算法可能有用,并且在预测生存率、严重程度和补片修补需求方面比肝脏疝出更可靠。有必要进行进一步研究。

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