Journal of pediatric surgery · 2015
Prenatally diagnosed severe CDH: mortality and morbidity remain high.
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Coughlin Megan A, Werner Nicole L, Gajarski Robert, Gadepalli Samir, Hirschl Ronald, Barks John, Treadwell Marjorie C, Ladino-Torres Maria, Kreutzman Jeannie, Mychaliska George B
Аннотация
PURPOSE: 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).
CONCLUSION: A combination of prenatal markers accurately identified severe CDH patients. Outcomes of this group remain poor and persistent PHtn contributes significantly to 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).
CONCLUSION: A combination of prenatal markers accurately identified severe CDH patients. Outcomes of this group remain poor and persistent PHtn contributes significantly to mortality.
Предметные рубрики MeSH
Extracorporeal Membrane OxygenationFemaleFetal DiseasesHeadHerniaHernias, Diaphragmatic, CongenitalHumansHypertension, PulmonaryInfant, NewbornLiver DiseasesLungLung Volume MeasurementsMagnetic Resonance ImagingMalePredictive Value of TestsRetrospective StudiesSeverity of Illness IndexSurvival RateUltrasonography, Prenatal