Only days before the COVID-19 pandemic came crashing down on the American health care system, specialists at Yale New Haven Hospital performed Connecticut’s first fetal surgery to repair spina bifida –– a birth defect that often occurs before a woman knows she is pregnant, characterized by incomplete formation of the baby’s spine and spinal cord.

The surgery, known for its high level of complexity, was executed by a team of over 20 medical professionals led by David Stitelman, surgical director of the Yale Fetal Care Center; Mert Ozan Bahtiyar, director of the Yale Fetal Care Center; and Michael DiLuna, chief of pediatric neurosurgery. Over the course of two years, members of the team underwent training in different centers where this procedure has been performed, either watching from the sidelines or participating in the surgery themselves. On March 21, after conducting a series of Zoom run-throughs and a logistical simulation in the operating room, the team operated on the fetus, who had been diagnosed with myelomeningocele — a form of spina bifida in which nerves and surrounding tissues protrude out of the spine. The surgery was completed successfully in roughly three hours.

“There are a lot of people who are having surgeries canceled because of COVID-19 … but this was a procedure that had to happen that day, or it wouldn’t have been able to happen at all,” Stitelman said.

With surgical intervention for spina bifida, time is of the essence. Stitelman emphasized that the longer the spinal cord remains exposed to amniotic fluid inside the mother’s womb, the greater the extent of nerve damage incurred by the fetus. Depending on the severity of the defect, there can be direct injury to the spinal cord, which can culminate in bowel and bladder dysfunction, infections, permanent paralysis, brain herniation and, in some cases, death.

According to DiLuna, myelomeningocele is the most common, yet most severe, kind of spina bifida. “In myelomeningocele, the spine is open, the skin is open and then all of the layers that cover the spinal cord are also open,” he said, “so whatever signals that the spinal cord needs to get while in utero to … form its nerves properly are not received.”

Speaking to the delicate nature of the case, Bahtiyar said he was glad to see his team receive “complete support” from the hospital administration and the medical school, especially in the midst of the pandemic. “This [surgery] was for the benefit of the patient,” he added, “and I am proud that we had this support because I know other centers did not.”

The surgery is typically performed without removing the fetus from the mother’s uterus. Such an elaborate procedure demands collaboration across multiple medical areas ranging from obstetric anesthesiology to pediatric cardiology.

“Any person missing from the team would have hampered the surgery,” Bahityar said. “Every person had an essential role and added value. Everything worked perfectly.” 

The fetus and the mother are both doing well, DiLuna said.

In the United States, it is estimated that half of all cases of spina bifida that emerge are terminated, Stitelman said. According to him, there are usually 10 cases of the disease in Connecticut per year, half of which are viable candidates for operation.

With this surgery being the first of its kind to have been performed in the state, YNHH is likely to see increased demand for the procedure in the future. Bahtiyar said he hopes YNHH will perform more surgical interventions of this sort for patients in the New England area.

“I would like us to be a place that could provide this reliably and securely for patients who need this,” he said.

According to DiLuna, YNHH has “all the pieces” to receive more cases for spina bifida treatment, with access to experienced and specialized surgical teams. “Our goal is to make Yale one of the regional centers,” he said.

However, as DiLuna explained, while surgery is important to optimize neurological outcomes in the long term, the procedure in itself is not a cure for spina bifida. The treatment process is long and extends beyond birth.

Stitelman estimates that only about 15 to 20 percent of patients with spina bifida receive fetal surgery, but he suggested that if intervention were less invasive, the figures might be higher. To that end, he leads a lab that is currently researching less invasive therapeutic approaches to treat the condition.

Even though YNHH is currently down to 80 percent capacity and has its own COVID-19 ward, DiLuna said that the atmosphere of the pandemic did not interfere with the procedure, adding that this “speaks to how doctors, surgeons, nurses and technicians function on a day-to-day basis, taking care of patients no matter what.” The biggest difference, Stitelman said, was that “there were no handshakes, there were no high-fives.”

“Behind the doors of the operating rooms everyone is still utilizing all of the skills they’ve been training their entire lives to help people every day, and the pandemic hasn’t changed that,” DiLuna said.

According to the Centers for Disease Control and Prevention, approximately 1,645 babies are born with spina bifida in the United States each year.