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Which Treatment for Congenital Diaphragmatic Hernia Diagnosed in Utero?

At this time, fetal surgery cannot be recommended.

Congenital diaphragmatic hernia in fetuses can be diagnosed by ultrasound early in pregnancy. Rates of postnatal mortality are high for fetuses with liver herniation and low lung-to-head ratios, because the lungs aerate poorly after birth. Given that the underlying pathogenesis is pulmonary hypoplasia, various strategies have been used to augment hypoplastic lungs in utero. The current surgical strategy for promoting fetal lung growth is to prevent egress of lung fluid by occluding the fetal trachea.

A team at the University of California at San Francisco (the center with the world's longest experience in fetal surgery) conducted a randomized, controlled trial to determine whether fetal endoscopic tracheal occlusion is more efficacious than expectant treatment followed by surgery immediately after birth (standard care). The trial involved 24 women whose fetuses (mean gestational age, 25 weeks; range, 22-27 weeks) had severe left-sided congenital diaphragmatic hernia (unfavorable lung-to-head ratios and liver herniation) but no other detectable anomalies on ultrasound.

The proportion of fetuses who survived to 90 days of age was similar in the tracheal-occlusion and standard-care groups (73% and 77%, respectively). However, complications, especially premature rupture of the membranes and preterm labor, were much more common in the tracheal-occlusion group. Mean gestational age at delivery was 30.8 weeks with tracheal occlusion and 37.0 weeks with standard care (P<0.001).

Comment: These data fail to support use of a widely touted treatment for a common fetal anomaly. Early experience tantalizingly suggested more favorable outcomes with tracheal occlusion, but perhaps such outcomes merely reflected the state-of-the-art neonatal intensive care available at the tertiary centers where the infants were treated. By contrast, comparison groups unavoidably consisted of untreated fetuses in various medical centers that often had fewer resources.

The UCSF study emphasizes once again the importance of randomized, controlled trials in assessing clinical outcomes. Neonatal survival was poor, and fetal surgery was no more effective than expectant management. Thus, at this time, fetal surgery cannot be recommended for treating diaphragmatic hernia.

— Sandra Ann Carson, MD

Published in Journal Watch Women's Health January 27, 2004

Citation(s):

Harrison MR et al. A randomized trial of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia. N Engl J Med 2003 Nov 13; 349:1916-24.

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