Rare Defects in Fetuses Fixed by New Surgery : Medicine: San Francisco doctors partially removed the babies from the uterus, rearranged the internal organs and made repairs.
UC San Francisco doctors announced Wednesday that they have successfully performed surgery to correct a rare, serious birth defect in two fetuses, allowing them to develop into normal, healthy babies.
At a news conference here, a team of UC San Francisco surgeons proudly displayed the two babies--a 9-month old boy and a 6-week-old girl--who had the operation, which involves correcting a hole in the diaphragm.
The UCSF team has performed the highly experimental repair surgery eight times. The first six fetuses died, four during the operation and two others from complications after birth.
UCSF is pioneering the surgical technique, which involves partially removing the fetus from the uterus, rearranging its internal organs and returning it to the mother’s womb. The first of the two operations is reported in today’s New England Journal of Medicine.
The surgery was performed to correct fetal diaphragmatic hernia, a severe birth defect that occurs in about one of every 2,000 babies.
The diaphragm is a key respiratory muscle that separates the lung cavity from the abdomen. In fetuses with this defect, a hole in the diaphragm fails to grow shut, and the stomach, intestines and other organs squeeze into the chest cavity. As a result, the lungs fail to develop, and the baby is born unable to breath.
About 75% of all babies with this defect die either at or before birth.
“If we wait to operate on newborn babies to correct the hole in the diaphragm, they often die anyway,” said Dr. Michael R. Harrison, who developed the surgery as co-director of the UCSF Fetal Treatment Program. “It is heartbreaking. The baby’s lung, compressed by other organs throughout the fetal development, is simply too small to support life outside the womb.”
The two most recent babies to have the operation went on to become healthy children, Harrison said, proudly holding up a rambunctious baby boy, Blake Schultz of Chelsea, Mich. “He’s our hero.”
Harrison and the team’s other doctors described how one year ago they performed the surgery that allowed the baby to breathe normally. During the surgery, doctors cut into the mother’s uterus and pulled the fetus’s arm out through the hole. Wires were attached to the fetus to monitor its heart and oxygen levels.
Through an incision in the fetus, they put the misplaced stomach, intestines and other organs back where they belonged and covered the hole in the diaphragm with a patch made of the synthetic material Gore-Tex. Finally they tucked the fetus’s arm back into the womb and closed the incision.
Blake was premature and had to spend a month on a respirator, but was healthy at 8 months. The second successful operation was performed March 3 on Devona Anderson of Tacoma, Wash. Although operated on at 25 weeks, she was able to breathe on her own on her second day of life.
Both sets of parents discussed their experiences Wednesday, describing their apprehension about the experimental operation.
“I was scared. I had never had surgery for any reason before,” said Beth Schultz. “I didn’t know what to expect.”
Laura Scott, mother of Devona Anderson, agreed. “I was concerned about losing the baby--it was scary.” But her husband, Stephen, gestured to his sleeping daughter and added: “It was the only chance she had.”
The operation takes just under an hour and costs about $18,000, Harrison said. But insurance companies are reluctant to pay for the procedure.
The doctors conceded that a major difficulty is separating those fetuses that are likely to survive from those that are doomed, since the operation itself is highly dangerous to the fetus.
“We had to learn how to select for treatment only the fetuses who really needed it,” Harrison said
“All this is made possible by advances in prenatal diagnosis, imaging and intervention techniques,” added Dr. Mitchell Globus, a UCSF obstetrician and co-director of the team
Doctors have experimented for the last decade with treating and repairing defective fetuses before birth. Most interventions have been relatively minor, however. Among the most common is opening up urinary blockages.
Harrison said he has been working on the latest procedure for about 12 years. His team tested it on 1,000 fetal lambs and 200 fetal monkeys. They also reviewed 200 fetuses in which the disorder was not treated before birth.
