关键词: Congenital diaphragmatic hernia Fetal MRI Lung to head ratio Postnatal outcome Prenatal diagnosis

Mesh : Extracorporeal Membrane Oxygenation Female Fetal Diseases / diagnosis pathology Head / diagnostic imaging embryology pathology Hernia / congenital diagnosis Hernias, Diaphragmatic, Congenital / diagnosis mortality therapy Humans Hypertension, Pulmonary / diagnosis Infant, Newborn Liver Diseases / congenital diagnosis Lung / diagnostic imaging embryology pathology Lung Volume Measurements Magnetic Resonance Imaging Male Predictive Value of Tests Retrospective Studies Severity of Illness Index Survival Rate Ultrasonography, Prenatal

来  源:   DOI:10.1016/j.jpedsurg.2015.10.082

Abstract:
OBJECTIVE: This study sought to evaluate prenatal markers\' ability to predict severe congenital diaphragmatic hernia(CDH) and assess this subgroup\'s morbidity and mortality.
METHODS: A retrospective review was performed between 2006 and 2014. Prenatal criteria for severe CDH included: liver herniation, lung-to-head ratio (LHR) <1 on prenatal ultrasound and/or observed-to-expected LHR (o/eLHR) <25%, and/or observed-to-expected total lung volume (o/eTLV) <25% on fetal MRI. Postnatal characteristics included: mortality, ECMO utilization, patch closure, persistent suprasystemic pulmonary hypertension (PHtn), O2 requirement at discharge, and few ventilator-free days in the first 60. Statistics performed used unpaired t-test, p<0.05 significant.
RESULTS: Overall, 47.5%(29/61) of patients with prenatally diagnosed, isolated CDH met severe criteria. Mean LHR: 1.04±0.35, o/eLHR: 31±10% and o/eTLV: 20±7%. Distribution was 72% LCDH, 24% R-CDH. Overall survival: 38%. ECMO requirement: 92%. Patch rate: 91%. Mean ventilator-free days in 60: 7.1±14. Supplemental oxygen at discharge was required in 27%. In this prenatally diagnosed severe cohort, 58%(15/26) had persistent PHtn post-ECMO requiring inhaled nitric oxide±epoprostenol. Comparing patients with and without PHtn: mean ECMO duration 18±10 days versus 9±7 days (p=0.01) and survival 20% versus 72% (p=0.006).
CONCLUSIONS: A combination of prenatal markers accurately identified severe CDH patients. Outcomes of this group remain poor and persistent PHtn contributes significantly to mortality.
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