Prenatally diagnosed severe CDH: mortality and morbidity remain high

Megan A Coughlin; Nicole L Werner; Robert Gajarski; Samir Gadepalli; Ronald Hirschl; John Barks; Marjorie C Treadwell; Maria Ladino-Torres; Jeannie Kreutzman; George B Mychaliska

doi:10.1016/j.jpedsurg.2015.10.082

Prenatally diagnosed severe CDH: mortality and morbidity remain high

J Pediatr Surg. 2016 Jul;51(7):1091-5. doi: 10.1016/j.jpedsurg.2015.10.082. Epub 2015 Nov 10.

Affiliations

¹ Section of Pediatric Surgery, Department of Surgery, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI. Electronic address: meganac@med.umich.edu.
² Section of Pediatric Surgery, Department of Surgery, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
³ Division of Pediatric Cardiology, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
⁴ Division of Neonatology, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
⁵ Division of Maternal Fetal Medicine, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.
⁶ Division of Pediatric Radiology, The University of Michigan Medical School, C.S. Mott Children's Hospital, and the Fetal Diagnosis and Treatment Center, Ann Arbor, MI.

PMID: 26655216
DOI: 10.1016/j.jpedsurg.2015.10.082

Abstract

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.

Keywords: Congenital diaphragmatic hernia; Fetal MRI; Lung to head ratio; Postnatal outcome; Prenatal diagnosis.

MeSH terms

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