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

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.

Congenital diaphragmatic hernia presenting with symptoms within the first day of life; outcomes from a non-ECMO centre in Denmark

“…Lung-to-head ratio evaluates lung volume prenatally in CDH infants, and is used as a prognostic marker for outcome [16]. Magnetic resonance imaging is also used to prenatally evaluate the degree of lung hypoplasia, however, this modality has only recently been taken up at our institution and was therefore not evaluated here [32]. Unfortunately, data for "liver-up", lung-to-head ratio, and other possible risk factors were not registered in a consistent and structured manner throughout the study period.…”

The Role of the Fetal MRI to Predict the Postnatal Survival in Fetuses with Congenital Diaphragmatic Hernia

“…Twenty-five patients were included in this study. The mean gestational week at the fetal MRI was 25.08± 1.1 (18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36). Among 25 fetuses, 6 were alive and 19 were dead within 30 days after birth.…”

“…We thought that this finding may help to predict the survival along with other specific parameters. Yokoi et al (22) proposed a risk stratification using both the detectable lung parenchyma at the apex and the contralateral LLSIR with a cut-off value of 2 on MRI. They reported that all fetuses with detectable lung parenchyma had survived.…”

Mediastinal Shift Angle in Fetal MRI Is Associated With Prognosis, Severity, and Cardiac Underdevelopment in Left Congenital Diaphragmatic Hernia

“…Some MRI predictors, such as observed/expected total fetal lung volume (O/E TFLV) ( 18 ), could accurately measure the volume of lungs. However, most of them focused on the three-dimensional volume, which requires higher measurement techniques and greater time investment, so they were not widely used.…”

“…However, most of them focused on the three-dimensional volume, which requires higher measurement techniques and greater time investment, so they were not widely used. Some scholars suggested using the lung to liver signal intensity ratio (LLSIR) ( 18 , 19 ) to facilitate measurement. Still, the signal intensity may be affected by amniotic fluid, pulmonary interstitial effusion, etc., decreasing measurement accuracy.…”