ABSTRACT
Objective:To explore the value of fetal lung volume and mediastinal shift angle (MSA) based on fetal MRI in predicting the prognosis of congenital diaphragmatic hernia (CDH).Methods:Fetuses with left CDH that did fetal MRI in Xinhua Hospital Affiliated with Shanghai Jiao Tong University School of Medicine from September 2016 to January 2022 were retrospectively collected. There were 65 patients, and their gestational age was 29 (26, 35) weeks when they were diagnosed with left CDH by MRI. Observed fetal lung volume and MSA were measured based on fetal MRI, and observed/expected lung volume (o/eFLV) based on gestational age was calculated. The clinical data were collected from birth to discharge, and patients were divided into survival group and death group in case of prognosis at discharge, with 54 cases in the survival group and 11 cases in the death group. The student′s t test was used to compare the difference of o/eFLV and MSA between the survival group and the death group, and the receiver operating characteristic (ROC) curve was used to evaluate the value of o/eFLV and MSA in predicting the prognosis of left CDH. Results:The o/eFLV of the survival group was 51.5%±18.3%, higher than that of the death group (27.8%±4.4%), and the difference was significant ( t=8.29, P<0.001). The MSA of the survival group was 33.1°±1.2°, lower than that of the death group (41.8°±2.7°), and the difference was significant ( t=-11.15, P<0.001). The area under the ROC curve (AUC) of o/eFLV to predict the fetal survival or death was 0.939 (95%CI 0.851-0.983), the cutoff value was 33.8%, the sensitivity was 100%, the specificity was 88.9%. The AUC of MSA was 0.998 (95%CI 0.941-1.000), the cutoff value was 37.2°, the sensitivity was 100%, the specificity was 98.2%. Conclusions:The o/eFLV and MSA that were measured based on fetal MRI can effectively predict the fetus′s prognosis with left CDH.
ABSTRACT
El enfisema lobar congénito es la hiperinsuflación con atrapamiento de aire de uno o más lóbulos pulmonares histológicamente normales, por un mecanismo valvular; produce compresión del parénquima normal y desplazamiento del mediastino. Se presenta el caso de un niño de 45 días con dificultad respiratoria in específica y pectum excavatum. La radiografía de tórax mostró hiperclaridad del pulmón derecho y desplazamiento del mediastino. Se realizó lobectomía, que confirmó el enfisema lobar congénito. Continúa evolución favorable 29 meses después.
Congenital lobar emphysema results from hyperinsufflation and air trapping in an otherwise histologically normal lobe, secondary to a bronchial valvular mechanism. It induces compression of normal lung and mediastinal shift. We present the case of a 45 day old male infant seen due to nonspecific respiratory difficulty and pectus excavatum; the chest X-ray showed a hyperlucent right lung and mediastinal shift. The resected middle lobe showed lobar emphysema. He remains in good condition 29 months later.