How this revolutionary surgery tackles spina bifida before birth

A minimally invasive fetoscopic surgery is allowing Intermountain Health Primary Children’s Hospital to treat spina bifida in the womb, reducing the risk of complications for moms and improving the outcomes for babies who have the congenital condition.

Monday morning, maternal-fetal medicine providers introduced two infant girls, both from West Jordan, who each underwent the in utero procedure.

Angela Ibarra Garcia said her daughter Daniela had the surgery at week 25 of the pregnancy. The little girl, now 6 months old, is doing well and was gently moving her feet a little and looking very attentive during the event as she sat on her mom’s lap. She is Ibarra Garcia’s third daughter, with an 11-year-old sister Camila and a middle sister, Natalie, age 6.

Ibarra Garcia was the first patient to undergo the fetoscopic procedure in Utah and during a press briefing she noted the relief she felt from not having to travel out of state, where the family had no support system. She also expressed pleasure that checkups indicate that Daniela is doing well though she will continue to need some physical therapy for her lower body.

Jaycie Vance had the same surgery at 26 weeks into the pregnancy, which allowed her mom, Maddison Vance to carry her two more months before birth. Jaycie will soon be 5 months old. Her mom, dad Grem and big brother Hank, 4, were part of the celebration Monday, during which Maddison noted that Jaycie recently moved her feet a little, which is a very positive sign.

Spina bifida is the most common permanently disabling birth defect in the U.S. The effect varies from child to child. The Spina Bifida Association reports that as many as 166,000 people are living with spina bifida in the U.S.

Spina bifida occurs when the neural tube doesn’t close properly early in the baby’s development, creating a gap in the spine. Depending on the type of spina bifida, the spinal cord and meninges may be exposed. In some cases, children with spina bifida have paralysis of their lower limbs. And while the surgery is not a guarantee of a cure — children still have spina bifida — the medical team said that the procedure improves the chance of avoiding the worst of complications, including hydrocephalus, which is the abnormal accumulation of cerebrospinal fluid in the brain’s ventricles, which can require shunts to remove the fluid and which, left untreated, could result in brain damage or even death.

Reducing that risk also improves the child’s future school-age outcomes and increases the chance the child will be able to walk, according to Dr. Rajiv Iyer, pediatric neurosurgeon.

And with less-invasive surgery, any future pregnancies are lower risk because the uterine incisions are small.

The surgical team emphasized that the surgery does not cure spina bifida. But it can help reduce fetal complications from the condition and provide better physical function. It also makes it more likely a child will have normal brain function.

Tackling the challenge in utero

Bringing the surgery to Utah overcomes one of the barriers to treatment, but was a process that took a couple of years, said Dr. Stephen Fenton, a pediatric surgeon at University of Utah Health and Intermountain Health Primary Children’s Hospital. He directs pediatric surgery at the Grant Scott Bonham Fetal Center.

In 2021, Primary’s team began operating in the womb on babies who had spina bifida, using an open repair that had them lift the uterus and baby partway out of the mother’s abdomen, then make a larger incision in the uterus. The fetoscopic version now being used allows for three small incisions in the uterus, one for a tiny camera and two for the implements to make the repair, Fenton said. He told Deseret News it took about two years to bring the less invasive surgery to Utah.

In the past, families carrying a child with spina bifida might have skipped the procedure if they had to travel out of state to have it done. At the same time, Dr. Janice Byrne, director of fetal obstetrical services at the center, said having the procedure available here has also opened possibilities for moms and babies who might come from other states where it’s not available. They already had one mom-baby pair come from Alaska for treatment, she said.

Dr. Martha Monson, a maternal-fetal medicine practitioner who directs the center’s fetal intervention, said that the older, more invasive fetal repair was the gold standard and provided great benefits for the child, but it was more invasive for moms and made the rest of the pregnancy harder, as well as increasing risk of infection.

With the new operation, she said, moms can carry the baby to term and have the option of a vaginal delivery. The older operation guaranteed a preterm c-section.

Fenton said the surgery is a “three-layer watertight repair” that includes the meninges, muscle and skin. The meninges are three layers of protective tissue surrounding the spinal cord and also the brain.

Without intervention, babies as they grow may have developmental delays and need to use a wheelchair. The advanced fetal surgeries improve the possibility a child will be able to walk with some assistance and have normal brain function.

Fenton said such surgeries have only been available for about a decade, which means that the long-term impact isn’t clear. But he called the possibilities “exciting.”

Fenton praised the medical team for the success they’ve enjoyed with the procedure and noted that it “takes a tremendous team to care for and be able to perform this type of procedure.” It also takes a large team of caregivers to track what’s happening to mom and baby all the way through the complex operation and beyond, he noted.

Byrne said 20 to 30 people might be in the operating suite during such an operation, each with a specific job.

Scary but worth it

It was a scary decision, both moms said.

Ultimately, Ibarra Garcia decided that while the risks were there, the benefits greatly outweighed them, so she opted for the surgery. It is a joy to see baby Daniela kicking and turning her head well, she said.

For the Vances, there was never a question.

Pointing to the medical team seated near the podium, Maddison Vance said, “You are my top five people in the whole world,” while her husband Grem smiled. “I am super grateful to all of you; I love all of you so much,” she said. Once she learned at her 20 week ultrasound that Jaycie had spina bifida, it was “scary news,” but she said she and Grem never questioned that they would have the surgery if they qualified.

It’s too soon, the doctors agree, to know what the outcome will be for Daniela or Jaycie. But in both cases, the scans look good. And Jaycie, like Daniela, is beginning to move her feet on her own.

Both moms said there’s a long journey ahead of them as it remains what Iyer calls a “chronic disorder” that is sometimes predictable and other times not.

The Bonham center at Intermountain Primary Children’s Hospital is a collaboration between the hospital and University of Utah Health. The Intermountain Health’s Primary Promise campaign raised more than $642 million and helped build “the nation’s model health system for children,” per a press release.