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Correction of congenital diaphragmatic hernia in utero, V. Initial clinical experience
M R Harrison1, J C Langer, N S Adzick
1Department of Surgery, University of California, San Francisco 94143-0510.
Insights
Fetal surgery for congenital diaphragmatic hernia (CDH) shows promise, with two successful repairs demonstrating fetal lung growth and improved function. While technically challenging, the procedure appears safe for mothers and their reproductive potential.
Area of Science:
- Perinatal Medicine
- Pediatric Surgery
- Fetal Medicine
Background:
- Congenital diaphragmatic hernia (CDH) has a high mortality rate (77%) despite optimal care.
- Factors like polyhydramnios, early diagnosis, and liver herniation worsen prognosis.
Purpose of the Study:
- To evaluate the safety and feasibility of open fetal surgery for severe congenital diaphragmatic hernia (CDH).
- To assess the impact of in utero repair on fetal lung development and postnatal outcomes.
Main Methods:
- Open fetal surgery was performed on six selected fetuses with severe CDH.
- Surgical techniques included liver reduction and Goretex diaphragm construction.
- Postnatal outcomes and maternal complications were monitored.
Main Results:
- Two fetuses with severe CDH underwent successful in utero repair.
- Repaired fetuses showed rapid in utero lung growth and improved postnatal lung function.
- Maternal complications were limited to amniotic fluid leak and preterm labor; all mothers remained well.
Conclusions:
- Fetal surgery for CDH is feasible in selected cases and appears safe for mothers.
- In utero repair can lead to significant fetal lung development and improved outcomes.
- Fetal CDH repair remains a technically demanding procedure requiring further investigation.
Abstract:
Review of our experience with 45 cases of prenatally diagnosed congenital diaphragmatic hernia (CDH) confirms that most fetuses (77%) will not survive despite optimal pre- and postnatal care. Polyhydramnios, associated anomalies, early diagnosis, and a large volume of herniated viscera (including liver) are associated with a particularly dismal prognosis. After extensive experimental work demonstrated the efficacy, feasibility, and safety of repair in utero, we attempted to salvage six highly selected fetuses with severe CDH by open fetal surgery. Five had liver incarcerated in the chest: three died at operation because attempts to reduce the liver compromised umbilical venous return. In one, a Goretex diaphragm was constructed around the liver, but the baby died after birth. The last two fetuses, one with incarcerated liver, were successfully repaired. Both demonstrated rapid growth of the lung in utero, had surprisingly good lung function after birth despite prematurity, had the abdominal patch removed at 2 weeks, and subsequently died of nonpulmonary problems (an unrelated nursery accident in one and intestinal complications in the other). The only maternal complication was amniotic fluid leak and preterm labor. All six women are well and four have had subsequent normal children. From this phase I experience, we conclude that fetal surgery appears safe for the mother and her reproductive potential, that fetal CDH repair is feasible in selected cases, and that the fetal lung responds quickly after decompression. However, fetal repair remains a formidable technical challenge.