Beyond Open Fetal Surgery: A Spina Bifida Success Story

Their story begins in Oregon...

Ester and Samuel met in the Emergency Department at a hospital in Oregon. She was an EMT and he was the Patient Registrar. Ester would bring paperwork to Samuel for the patients she transported via ambulance. Eventually the two began dating, then married.

In May of 2022, the couple welcomed their first child, Rebecca. While the baby was born healthy, Ester needed a cervical cerclage, a procedure to temporarily stitch the cervix closed earlier in the pregnancy, to avoid preterm birth. Eager to grow their young family, Samuel and Ester were expecting their second baby in 2023. Early in the second pregnancy, Ester needed the cervical cerclage again and remained under the care of an OB-specialist.

On their daughter Rebecca’s first birthday, in May 2023, Samuel and Ester had an appointment to find out the sex of their second baby. But the day, which had been full of anticipation, joy and excitement took a dark turn. At their 21-week appointment, Ester and Samuel learned they were expecting a boy…and the baby had spina bifida.

The news shook them both. Especially since Ester’s younger brother was also born with spina bifida and had spent his life in a wheelchair.

“We’re grateful [Connecticut Children’s] was willing to take that risk.”

Ester’s baby had myelomeningocele, the most common and most serious form of spina bifida, in which part of the spinal cord and surrounding nerves protrude from the fetus’s back. Usually, the exposed spinal cord and nerves are contained in a sac that is exposed to amniotic fluid in the womb. Continuous bathing of the fragile developing spinal cord to amniotic fluid, being stretched over the cerebrospinal fluid filled cyst, and direct trauma by contact with uterine wall can result in progressive injury and neurologic deficits. Ester was well-aware of what life for a child with this diagnosis looks like.

The next day, their Maternal Fetal Medicine (MFM) specialist told them about a procedure that could address the spina bifida in utero, which could give their son a better chance at a healthier future. There were inherent risks to the baby and to Ester, and there was no guarantee that the surgery would work. There was no guarantee their child would ever walk. But now they had an option.

The young couple took the weekend to further research the procedure and pray about what to do. On Monday, they regrouped with their MFM and were excited: they wanted to try the procedure. Over the next week, their doctor contacted fetal care centers across the country capable of doing an open fetal surgery to repair myelomeningocele, the type of spina bifida Ester’s baby had.

How can fetal surgery fix spina bifida?

Fetal surgeons have to operate on the mother, open the uterus to expose the baby’s back, then they push the exposed spinal cord back into place and close the opening in the baby's back. The baby is tucked back in the uterus, which is stitched close before surgeons close the mother’s incision, so the baby can continue to gestate as long as possible.

What Ester’s MFM found was devastating: no Fetal Care Center would accept their case because Ester was considered too high risk for giving preterm birth, having already received a cervical cerclage. Centers in California, Colorado, Texas and Pennsylvania all declined to take the case. There was one last chance, but their OB was not optimistic. She had worked with a talented fetal surgeon at Children’s Colorado years ago, who was launching a new fetal care center. She called Dr. Timothy Crombleholme at Connecticut Children’s…and he was willing to consider the case.

After multiple phone conversations and after a series of tests that Ester endured to determine if she was a reasonable candidate for the surgery, the decision was made: she was. Dr. Crombleholme would take her case, on the condition Ester remained in Connecticut after the surgery and until the baby was delivered, so she could be monitored for any complications. “Connecticut Children’s was our last option,” said Samuel. “We’re grateful someone was willing to take that risk. We appreciate the fetal care team’s thoroughness and providing us with all of the risk information to make a decision.”

A cross-country journey from Oregon to Connecticut

Just 24 days after learning of their son’s condition, Ester and Samuel began the long drive from Oregon to Connecticut. Samuel had arranged for FMLA from his job as an assistant manager at the HVAC installation company where he had moved for better pay. They broke the lease on their apartment and put all their belongings in storage. Ester’s family kept 1-year-old Rebecca in Oregon to care for her. They had an air mattress in the car, as they didn’t have a place to live in Connecticut and Ester was searching for apartments online while Samuel drove.

Their church family sprang into action, and through family connections, located a relative of a church member who lives in Springfield, Mass.—about 40 minutes from Connecticut Children’s—with a furnished basement where they could stay indefinitely, free of charge. They received the news somewhere while crossing Ohio. It was a blessing.

They arrived in Connecticut on a Tuesday. The Fetal Care team met with them and began preparations with Ester over the next four days. They had one free day before the surgery was scheduled, so Ester and Samuel spent a quiet, sunny day at the beach, looking out over the Long Island Sound and feeling far, far away from their home and family in Oregon.

The first patient to undergo open fetal surgery at Connecticut Children’s

On Monday, June 26, Ester became the first patient under the care of the Fetal Care Center at Connecticut Children’s to undergo an open fetal surgery. After making their incisions, the surgical team freed up the exposed spinal cord and closed the defect with a patch and pulled the surrounding skin together to close the hole from which it had emerged providing a watertight seal. Samuel remembers the agony of pacing and waiting during the surgery. When Dr. Crombleholme emerged he delivered great news: “It was almost boring how well it went.”

Ester remained inpatient at Hartford Hospital for four days, then she was discharged to their temporary home, 40 minutes away in Springfield. The plan was for the baby to remain in the womb until he was more developed and ready for birth. Ester was instructed to call or come back if she experienced any unusual bleeding.

Ester woke at 4:30 am bleeding heavily

In mid-July, after several scares with bleeding, Ester was back at Hartford Hospital, where they determined her water had broken, but they wanted to keep the baby inside as long as possible. She spent the next four weeks in a hospital bed. At the same time, Samuel flew to Oregon to retrieve their daughter and Ester’s younger sister, who would provide childcare while the couple waited for their son to be born.

Every day, Samuel would bring Rebecca to visit Ester during those long weeks of bed rest. At least two nights per week, Samuel would return alone and sleep in Ester’s room. During one of these overnight stays, Ester woke at 4:30 am bleeding heavily. A steady stream of doctors came into her room. “They were speaking very calmly and moving very quickly,” said Samuel.

Welcome to the world, baby Thaddeus!

Ester was moved to an operating room and prepped for a Caesarian section as Samuel gowned up to be by his wife’s side. Baby Thaddeus was delivered at 6:47 am, weighing 3 pounds, 4 ounces. He was 32 weeks, 2 days, gestational age.

The baby didn’t cry. But he looked directly at Samuel, who snapped a photo of his newborn son.

The delivery team immediately intubated the baby to start his breathing. Ester saw Thaddeus for a few seconds, then he was whisked away to the Neonatal Intensive Care Unit (NICU).

At first, Thaddeus did not have any movement in his legs, possibly due to the spina bifida or to being delivered prematurely. His tiny legs had hairline fractures, related to the c-section delivery. But a few weeks after his birth, Thaddeus began moving legs. He never needed a catheter and showed good bowel and bladder function. Even better, Thaddeus has not needed a ventriculoperitoneal shunt one of the important benefits of the prenatal spina bifdia repair. And he was an enthusiastic eater, sometimes getting so excited, he would aspirate. But he began to gain weight. In October, nearly five months after learning their baby had spina bifida, Ester and Samuel were finally able to introduce Thaddeus to his sister, Rebecca, and return to their home and their family in Oregon.

Ester and Thaddeus make medical history

Not only was Ester’s surgery historic for Connecticut Children’s, it may have a unique place in medical history. Dr. Crombleholme had performed open fetal surgery three times, where the mother needed a cervical cerclage post-surgery, in order to avoid a premature delivery. But Ester was one of the first patients with a cerclage in place before open fetal surgery to repair myelomeningocele.

Samuel and Ester came to Connecticut Children’s with no guarantees that open fetal surgery would work. But they knew if they didn’t do anything, there was only one outcome for their baby.

While the surgery posed risks to both Ester and Thaddeus, the young couple remains resolute in their decision to seek treatment. “Someday, we will sit with our first son,” said Samuel, “and we will be able to say, ‘This is the length we went to, in order to give you a chance.’”

The future of fetal care at Connecticut Children’s

Mothers who need the services of the Fetal Care Center at Connecticut Children’s have a lot to look forward to, as construction of Connecticut Children’s new eight-story Tower commences. The entire third floor of the new building will house the Fetal Care Center, providing spacious, well-appointed rooms for moms, whether it’s for one night or for an extended period. Each LDRP (labor, delivery, recovery and post-partum) room will have a place for mom’s support person to comfortably remain by her side, as well as in-room toilet and shower facilities. Following delivery, babies can remain in their mom’s room, in a specially designed NICU-level care space. The new Fetal Care Center will also have family consultation rooms, testing and treatment areas, and state-of-the-art fetal surgery operating rooms for complex surgeries and high-risk deliveries, so all of a mother’s care can be provided in a single, spa-like environment.