NPR: A prenatal surgery for spina bifida comes of age

Intro to the story at NPR:

When she was 19 weeks pregnant, Sarah White went for a routine ultrasound and got a shock.

"I could tell that something was wrong because the ultrasound tech got real quiet," White says.

White's male fetus had spina bifida — a hole in his lower back that exposed the vulnerable spinal cord.

"When they said, 'Your baby has spina bifida,' I knew it wasn't good," says Joe Hensley, White's husband. "But I didn't have a sense of what was involved."

Often it involves lower-body paralysis requiring a wheelchair or, at best, leg braces. Lack of control over bowel and bladder. Possible brain damage. Surgery at birth and periodically throughout childhood to correct the defect and install shunts, tubes to drain excess fluid that can build up in the brain.

White and Hensley discovered an alternative to standard management of spina bifida. An operation before birth offered a chance to avoid paralysis, brain damage, the need for shunts — in short, the chance for a more normal life.

But the decision was no slam-dunk. "This has been the hardest thing we've been through," Hensley says. The surgery is risky. They could lose the fetus. And White could die — although so far, no mother has died from prenatal surgery for spina bifida.

And, Hensley says, "It's not a guaranteed fix."

So the couple, who live in North Carolina, went to Children's Hospital of Philadelphia, one of just three U.S. hospitals with extensive experience in fetal surgery for spina bifida.

Lee Sutton, a pediatric neurosurgeon on the staff there, says doctors now realize that most of the damage from spina bifida occurs late in pregnancy.

"We were seeing all these kids that were coming in here unborn — you know, little fetuses 18 to19 weeks — and they were moving their legs beautifully on the ultrasounds," Sutton says. "And then when they would get delivered at 38 weeks ... they had club feet and were paralyzed."

Ultrasound tests showed that White's fetus was still kicking. So there was a chance that if they decided to operate during pregnancy, their son wouldn't be paralyzed.

The couple decided they'd never forgive themselves if they didn't give their child the chance to walk. "We would probably look back and say, 'Wow, why didn't we do it? Why didn't we try?' " Hensley says.

So after a whirlwind of tests and consultations to determine her eligibility, White found herself on an operating room table in Philadelphia one Friday morning this summer. She was feeling apprehensive.

"I've never had surgery before, so it's pretty terrifying," she said.

The surgical team was led by Scott Adzick, chief of surgery at Children's Hospital of Philadelphia, known as CHOP. He began the operation with Mark Johnson and Michael Bebbington, who are specialists in obstetrics and maternal-fetal medicine.

Through skin, fat and muscle, the surgeons cut through Sarah's abdomen until they got to her uterus. At nearly six months of pregnancy, it was the size of a child's soccer ball.

"We're actually going to tip the uterus out of the abdomen," Adzick said. That's possible because the organ, shaped like an upside-down Grecian urn, is attached only at the bottom, at the neck of the "urn."

Once White's uterus was almost entirely outside her body, Adzick took a marker and outlined where the placenta was — the thick, pancake-shaped organ that exchanges oxygen and nutrients between mother and fetus. If the surgeons were to cut into the placenta by mistake, the fetus could have died and White could have bled to death.

Next, one of the surgeons took a special stapling device that cuts through the uterine wall, which is especially rich in blood vessels. The device pinches off blood vessels as it cuts and tacks the membrane enclosing the fetus to the uterine wall.

A few more staples and they had opened a small window, about 3 inches across, in the uterus.

"And here's the fetal bottom," Adzick said.

It was an amazing sight — the 6-inch-long fetus, its back facing up into the glare of the surgical lights.