Long-term outcome following extracorporeal membrane oxygenation for congenital diaphragmatic hernia: the UK experience

https://doi.org/10.1016/j.jpeds.2003.11.031Get rights and content

Abstract

Objective

We evaluated the long-term outcome of neonates receiving extracorporeal membrane oxygenation (ECMO) for congenital diaphragmatic hernia (CDH).

Study design

A retrospective review of all 73 neonates with CDH supported with ECMO in the United Kingdom between 1991 and 2000, with follow-up to January 2003. Information was from hospital charts and from communication with family doctors and pediatricians. Median follow-up period for survivors was 67 months.

Results

46 infants (63%) were weaned from ECMO, 42 (58%) survived to hospital discharge, and 27 (37%) survived to age 1 year or more. A higher birth weight, higher 5-minute Apgar score, and postnatal diagnosis were “pre-ECMO” predictors of long-term survival. Comorbidity was common in long-term survivors: 13 (48%) had respiratory symptoms, 16(59%) had gastrointestinal problems, and 6 (19%) had severe neurodevelopmental problems. Only 7 children were free of significant neurodevelopmental deficit and required no further medical or surgical intervention.

Conclusion

Using the current referral criteria, ECMO can be used to support the sickest neonates with CDH. However, there is significant mortality in the first year of life, and long-term physical and neurodevelopmental morbidity remains in the majority of survivors.

Section snippets

Patients and methods

Institutional Ethics Committee approval was obtained from each ECMO center to undertake this retrospective review. All neonates who were supported with ECMO between January 1, 1992 and January 1, 2000 in the four UK ECMO centers recognized by the Extracorporeal Life Support Organisation (ELSO) are included in this review.

Results

Seventy-three infants with CDH (43 males, median birthweight 2.9 kg) received ECMO support during this period. The characteristics of the group as a whole are given in Tables Ia and Ib. Fifty (68%) infants were diagnosed with CDH prenatally, and 57 (78%) had left-sided defects. ECMO support was instituted at a median age of 29 hours. Sixty-two infants were cannulated for ECMO before CDH repair, and 11 were cannulated following repair. The median duration of ECMO support was 202 hours (range,

Discussion

ECMO support can be performed in infants with CDH using an OI of 40 as the main referral criterion.5 However, despite seemingly favorable short-term outcomes, with 64% surviving to decannulation and 59% surviving to discharge, the long-term outcomes are less encouraging. Only 37% of our initial cohort survived beyond their first birthday, and only 10% were free of any physical problems. This current review, unique in its duration of follow-up, has revealed an important, previously unquantified

Conclusion

A higher birthweight, postnatal diagnosis, and higher Apgar score at 5 minutes were the only pre-ECMO predictors of long-term survival in this cohort of infants receiving ECMO for CDH. In addition, once on ECMO, the duration of mechanical support was an additional predictor of survival.

We have shown that there remains significant early mortality during the first year of life with major additional physical morbidity in the majority of long-term survivors of ECMO for CDH. Our study would suggest

Acknowledgements

We thank all the general practitioners, ECMO fellows, neonatologists, and general pediatricians who helped us by providing follow-up information for the infants in this review.

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