Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change morbidity outcomes for high-risk congenital diaphragmatic hernia survivors?
OBJECTIVE
In high-risk congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before extracorporeal membrane oxygenation (ECMO) can be initiated. We previously examined ex utero intrapartum treatment (EXIT)-to-ECMO in our most severe CDH patients, but demonstrated no survival advantage. We now report morbidity outcomes in survivors of this high-risk cohort to determine whether EXIT-to-ECMO conferred any benefit.


METHODS
All CDH survivors with <15% predicted lung volume (PPLV) from September 1999 to December 2010 were included. We recorded prenatal imaging, defect size, and pulmonary, nutritional, cardiac, and neurodevelopmental outcomes.


RESULTS
Seventeen survivors (8 EXIT-to-ECMO, 9 non-EXIT) had an average PPLV of 11.7%. Eight of 9 non-EXIT received ECMO within 2days. There were no significant defect size differences between groups, mostly left-sided (13/17) and type D (12/17). Average follow-up was 6.7years (0-13years). There were no statistically significant differences in outcomes, including supplemental oxygen, diuretics, gastrostomy, weight-for-age Z scores, fundoplication, pulmonary hypertension, stroke or intracranial hemorrhage rate, CDH recurrence, and reoperation. No survivor in our cohort was neurologically devastated. All had mild motor and/or speech delay, which improved in most.


CONCLUSIONS
In this pilot series of severe CDH survivors, EXIT-to-ECMO confers neither significant survival nor long-term morbidity benefit.


METHODS
Level III treatment study.
Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Hester F
HF
Shieh
Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA; Advanced Fetal Care Center, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Jay M
JM
Wilson
Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Catherine A
CA
Sheils
Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA; Advanced Fetal Care Center, Boston Children's Hospital, Harvard Medical School, Boston, MA.
C Jason
CJ
Smithers
Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Virginia S
VS
Kharasch
Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Ronald E
RE
Becker
Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Mollie
M
Studley
Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA; Advanced Fetal Care Center, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Donna
D
Morash
Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA; Advanced Fetal Care Center, Boston Children's Hospital, Harvard Medical School, Boston, MA. Electronic address: terry.buchmiller@childrens.harvard.edu.
Terry L
TL
Buchmiller
D001469:Barotrauma / Q000209:etiology; D015199:Extracorporeal Membrane Oxygenation / Q000009:adverse effects; D005260:Female; D046128:Fetal Therapies / Q000009:adverse effects / Q000379:methods*; D065630:Hernias, Diaphragmatic, Congenital / Q000150:complications / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010865:Pilot Projects; D011183:Postoperative Complications; D012189:Retrospective Studies; D012307:Risk Factors; D017741:Survivors; D016896:Treatment Outcome
CDH morbidity; Congenital diaphragmatic hernia; Ex utero intrapartum treatment; Extracorporeal membrane oxygenation