SPINA BIFIDA OPERATED IN WOMB

National Post - January 18, 2002
http://www.nationalpost.com/home/story.html?f=/stories/20020118/1168990.html
Success high in fetal surgery, researchers say
Spina bifida: Three U.S. hospitals make repairs on spine inside womb
Heather Sokoloff

A procedure to lessen the effects of spina bifida, a spinal birth defect, by operating on fetuses inside their mothers' wombs, is enjoying a high rate of success, U.S. researchers reported yesterday.

Surgeons at the Children's Hospital of Philadelphia said 40 babies who might otherwise have been severely disabled are surprising everyone by moving their legs and wiggling their toes.

The research team presented the short-term outcomes of its first 40 fetal surgeries at the annual meeting of the Society for Maternal-Fetal Medicine in New Orleans.

First performed in 1998, the open-womb procedure is still highly risky, the doctors caution. Three fetuses died from pre-term labour caused by the surgery.

"We've never had a doubt it was the right thing to do," said Cara Dansie of Utah, whose 20-month-old daughter had the surgery at the Philadelphia hospital.   Born 2 1/2 months premature, Challace Rose now ambles about her house holding on to furniture, occasionally taking a few timid steps on her own.

Doctors do not expect that she will need leg braces. "Just high-top sneakers to support her ankles. To look at her, you would never know she had spina bifida," said Mrs. Dansie, who has five other children.

Spina bifida develops during the first month of pregnancy when the spinal cord fails to close completely. In some cases, it causes a tiny bundle of nerves to protrude from the fetus's back. It is diagnosed through ultrasounds, blood tests and amniocentesis.

Surgery takes place during the second or early third trimester of pregnancy, between 20 and 26 weeks.  After surgery, the mothers are confined to bed rest. Doctors fight off early labour by monitoring the women with weekly ultrasounds until the babies are delivered by Caesarean section at about 35 weeks, some five weeks premature, though a few are born much earlier.

Both mother and fetus are at a high risk for infection and serious complications. If a mother goes into pre-term labour, the incision on her uterus could separate, causing it to rupture.  Within a week of her operation, Mrs. Dansie's water broke. She remained in a hospital bed for the next eight weeks, lying flat on her back.

Despite the risks, the doctors say the good health of the babies is encouraging.  Before the surgery was available, parents faced with a diagnosis of spina bifida had two choices: abortion or surgery to close the lesion after the baby's birth, at which point most of the damage is done. The doctors say it is too early to tell whether the children will be able to walk as they get older, or whether the surgery will have any long-term benefits at all.

Challace Rose, for example, can say "mommy" and "daddy," but not much more. It is also too early for doctors to know whether the children can be potty trained. Some suspect not. Nerves that control bladder and bowel function are low on the spinal column, an area likely to be affected by the lesion.

These problems aside, Challace Rose did not need additional surgery after her birth to insert a permanent tube, called a shunt, to divert spinal fluid from her brain -- a significant advancement, the doctors say. Only 35% of the babies who had the fetal surgery in the Philadelphia study required shunts to treat a complication called hydrocephalus, or "water on the brain," which causes the skull to swell.

Generally, 85% of spina bifida children require shunts, and more than half of them develop subsequent infections.

Another striking effect of the surgery is the reversal of a devastating condition called hindbrain herniation, in which a portion of the brain is squeezed into the spinal column, causing death in 10% to 15% of afflicted babies.

Not surprisingly, demand for the surgery, which is performed at only two other U.S. hospitals, is great. The Philadelphia hospital turned away 80 couples after determining from ultrasound images that their fetuses appeared too damaged to benefit from the surgery.

"What I often hear from parents is they don't want to be asked by their 13-year-old child, if they didn't have the in-utero surgery, 'Gee, mom, why didn't you do that for me?' " said Dr. R. Douglas Wilson, a Vancouver obstetrician who has worked at the Philadelphia children's hospital since June.

As yet, no Canadian hospitals perform the procedure. Three centres -- Philadelphia, the University of California at San Francisco and Vanderbilt University in Tennessee -- have applied to the U.S. government's National Institute of Health to conduct a nationwide trial of 200 pregnant women whose fetuses have spina bifida.