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Congenital diaphragmatic hernia: an unsolved problem
M R Harrison1, N S Adzick, A W Flake
1Department of Surgery, University of California, San Francisco 94143-0570.
Insights
Congenital diaphragmatic hernia (CDH) remains a significant threat to newborns, with high mortality rates. Prenatal surgical repair shows promise for improving outcomes in fetuses diagnosed with CDH early in gestation.
Area of Science:
- Perinatal Medicine
- Fetal Surgery
- Neonatal Surgery
Background:
- Congenital diaphragmatic hernia (CDH) is a serious condition with persistently high infant mortality despite advances in postnatal care.
- Current treatments including delayed surgery, advanced respiratory support, and ECMO improve outcomes for some infants, but survival rates remain suboptimal.
- A significant proportion of fetuses diagnosed with isolated CDH before 25 weeks gestation die despite optimal postnatal management.
Purpose of the Study:
- To evaluate the feasibility and potential efficacy of prenatal surgical repair for congenital diaphragmatic hernia.
- To address the challenges associated with fetal CDH repair, particularly when the fetal liver is incarcerated.
Main Methods:
- Review of recent surgical experience with prenatal CDH repair.
- Focus on overcoming intraoperative technical difficulties.
- Consideration for a future clinical trial to assess prenatal repair's impact on outcomes.
Main Results:
- Intraoperative technical challenges in prenatal CDH repair appear to be surmountable based on recent experience.
- Optimal postnatal care alone has not significantly reduced mortality in early-diagnosed fetal CDH cases.
Conclusions:
- Prenatal repair of congenital diaphragmatic hernia presents a physiologically compelling approach.
- Further investigation through a clinical trial is warranted to determine if prenatal surgical intervention can improve survival rates for infants with CDH.
Abstract:
Despite significant improvements in postnatal care of infants with congenital diaphragmatic hernia (CDH), mortality remains high. Delayed surgery, improved respiratory support, and extracorporeal membrane oxygenation (ECMO) have undoubtedly improved outcome for babies diagnosed after birth, but a prospective study of 52 fetuses with isolated, potentially correctable CDH diagnosed prior to 25 weeks gestation showed that 60% died despite optimal care presently available after birth. Repair of CDH before birth, although physiologically compelling, has proven exceedingly difficult especially when the fetal liver is incarcerated in the chest. Our recent experience suggests that intraoperative technical problems are surmountable, but a trial will be necessary to determine if prenatal repair can improve outcome for babies with diaphragmatic hernia.