ROANOKE TIMES

                         Roanoke Times
                 Copyright (c) 1995, Landmark Communications, Inc.

DATE: THURSDAY, May 31, 1990                   TAG: 9005310220
SECTION: NATIONAL/INTERNATIONAL                    PAGE: A-1   EDITION: METRO 
SOURCE: Associated Press
DATELINE: BOSTON                                LENGTH: Medium


SURGERY FIXES LETHAL DEFECT BEFORE BIRTH

In a dramatic but controversial attack on a lethal birth defect, doctors said they partially removed a fetus, rearranged its internal organs and returned it to the womb to develop into a normal, healthy baby.

Experts agreed that the operation is the most ambitious and invasive example yet in the new field of fetal surgery. However, some also questioned whether it is worth the risk to the mother and the fetus.

The surgery was pioneered at the University of California, San Francisco, to correct fetal diaphragmatic hernia, a severe birth defect that occurs in about one of every 2,000 babies. The surgery was reported in today's New England Journal of Medicine.

During development, a hole in the fetus's 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 breathe.

About three-quarters of all babies with this defect die.

"This problem is immensely frustrating for pediatric surgeons, because the babies are otherwise perfectly nice, but they die," said Dr. Michael R. Harrison, who developed the surgery. "It seemed as though this was one of those situations in medicine where fixing it earlier would be the only way to have an impact."

Harrison has performed the highly experimental repair surgery eight times since 1984. The first six fetuses died, but the last two - one boy and one girl - went on to become healthy children. The journal report is on the first success.

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 Gore-Tex patch. Finally they tucked the fetus's arm back into the womb and closed the incision.

In the first successful case, a Michigan woman carried the 24 1/2-week fetus for seven more weeks until giving birth in August. The baby boy was premature and had to spend a month on a respirator, but is healthy at 9 months.

The second successful operation was performed March 3 on Devona Anderson, of Tacoma, Wash., who is now 6 weeks old. She did better and was able to breathe on her own on her second day of life.

The girl's parents, Lora and Stephen Anderson, met with reporters in San Francisco on Wednesday. Anderson said they were devastated when they were told the fetus had only a 25 percent chance of survival if born with the defect.

"The more we thought, the more we thought, `Let's do it,' " Anderson said.

The operation takes just under an hour and costs about $16,000.

"The disease he is attacking is a very big problem in pediatrics," said Dr. Joseph P. Vacanti of Children's Hospital in Boston. "It is an important approach that ought to continue to be investigated."

At the University of Manitoba, Dr. Frank Manning noted that at least a quarter of babies with the disorder survive without surgery. A major difficulty is separating these fetuses from those that are doomed, since the operation itself is highly dangerous to the fetus.

He also noted that women who undergo the surgery risk having a ruptured uterus if they get pregnant again.

"I don't want to pour on cold water, because this is an innovative thing he has done," said Manning. "But I can't give you unbridled enthusiasm and tell you this will be the panacea for diaphragmatic hernia."

Doctors have experimented for the past 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. To show that it is safe for the mother as well as potentially effective, 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.



 by CNB