American Surgical Association
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Treatment Evolution in High Risk Congenital Diaphragmatic Hernia: Ten Years Experience
Kevin P. Lally, M.D., The Congenital Diaphragmatic Hernia Study, Group*
University of Texas Houston, Houston, TX

OBJECTIVE(S): Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal mortality. Many novel and advanced therapeutic interventions have been targeted to these infants. The objective of this study was to evaluate the impact of these therapies on the highest risk patients, those with agenesis of the diaphragm.
METHODS: Centers from 10 countries collected data prospectively on all liveborn infants with CDH. We evaluated the treatment and outcomes in patients with diaphragm agenesis in those centers that contributed data for 10 years. Patients were followed until death or hospital discharge.
RESULTS:
1569 patients were seen in 20 centers from January 1995 through December 2004. There were 218 patients with diaphragm agenesis who underwent repair. Overall survival was 68%, but was 54% in the agenesis patients. When patients from the first two years were compared to the last two years; there was significantly less use of ECMO and an increase in the use of inhaled nitric oxide (p<0.05)(Table). There was a clear trend to improved survival; however, the survivors with agenesis still had prolonged hospital stays compared to patients who had primary repair (68 versus 25 days), with many of these patients discharged on tube feedings and oxygen therapy.
CONCLUSIONS:
While overall mortality for infants with CDH remains high, there has been a change in management with less frequent use of ECMO and greater use of nitric oxide in high risk patients with no worsening in survival. However, the hospital length of stay and morbidity in these patients remains significant.
ECMO UseNitric OxideSurvivalHome Tube FeedsHome O2
1995-199627/36 (75%)9/30 (30%)17/36 (47%)8/13 (62%)3/11 (27%)
2003-200433/64 (52%)51/64 (80%)38/64 (59%)12/33 (36%)7/32 (22%)


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