By Nadia Kounang and Amanda Sealy, CNN
A team of doctors in Boston successfully performed a novel fetal surgery to treat a rare brain condition known as vein of Galen malformation.
Although in utero surgery — performed before a baby is born — has been used for other conditions, this ultrasound-guided procedure was among the first for this condition. Details of the procedure, which took place in March, were published in the journal Stroke on Thursday.
The condition occurs when the blood vessel that carries blood from the brain to the heart, also known as the vein of Galen, doesn’t develop correctly. The malformation, known as VOGM, results in an overwhelming amount of blood stressing the vein and heart and can lead to a cascade of health problems.
“Tremendous brain injuries and immediate heart failure after birth are the two big challenges,” Dr. Darren Orbach, a radiologist at Boston Children’s Hospital and expert in treating VOGM, told CNN.
Typically, infants are treated after they’re born using a catheter to insert tiny coils to slow down blood flow. However, Orbach said, the treatment often happens too late.
Despite advancements in care, “50 to 60 percent of all babies with this condition will get very sick immediately. And for those, it looks like there’s about a 40 percent mortality,” Orbach said. About half of infants that survive experience severe neurological and cognitive issues, he said.
Facing a choice
When they learned of their fourth pregnancy, Derek and Kenyatta Coleman of Baton Rouge, Louisiana, were surprised and excited. Kenyatta, 36, and Derek, 39, have been married for seven years and were ready to welcome a new addition to their family.
Kenyatta said nothing seemed particularly notable about the pregnancy. After having three children, Kenyatta thought of ultrasounds as routine appointments.
“Baby was doing well. The anatomy scan came back unremarkable. All of her biophysical profiles were all unremarkable,” said Kenyatta, who spoke to CNN exclusively.
The Colemans even did genetic testing that characterized Kenyatta’s pregnancy as “low risk,” she said: “We honestly thought that we were in the clear.”
But when Kenyatta went for an ultrasound at 30 weeks into her pregnancy, something was different. She remembers her doctor sitting her down and saying she was worried.
“She shared with me that something wasn’t right in terms of the baby’s brain and also her heart was enlarged,” Kenyatta remembers.
After more investigation, there was a diagnosis: VOGM.
But the Colemans had learned about a clinical trial run by Brigham and Women’s and Boston Children’s hospitals that could provide treatment before their baby was born.
Kenyatta remembers being told about the possible risks — preterm labor, or brain hemorrhage for the fetus — but the Colemans felt there was no other option for them. They wanted to join the trial.
On March 15, exactly one month after the ultrasound that spotted the malformation, Kenyatta underwent surgery.
A procedure with two patients
But for this surgery, there were two patients: Kenyatta and her baby.
Doctors had to make sure the fetus was facing the right position, with its head facing the mother’s abdominal wall. Dr. Louise Wilkins-Haug, division director of Maternal Fetal Medicine and Reproductive Genetics at Brigham and Women’s Hospital, partnered with Orbach to make sure that the fetus was not only in the right position for the procedure, but stayed there.
Wilkins-Haug explained they used a technique borrowed from previous in utero cardiac surgeries. Once the fetus is in the optimal position, it “gets a small injection of medication so that it’s not moving and it is also getting a small injection of medication for pain relief,” Wilkins-Haug said.
From there, the doctors inserted a needle through the abdominal wall, carefully threading a catheter through the needle, so that the tiny metal coils can fill up the vein, slow the blood flow and reduce the pressure.
The baby showed signs of improvement immediately, with scans showing decreased blood pressure in key areas.
“It was exhilarating at the moment that we had technical success at doing the embolization,” said Orbach.
But success wasn’t defined by that moment alone, but rather, what would follow.
“Will she be able to continuously show progress after? Will she need just additional support after I have her? Will she go into immediate heart failure still?” Kenyatta wondered.
Always a fighter
After the procedure, Kenyatta was slowly leaking amniotic fluid. Two days later, she went into labor at 34 weeks.
On March 17, Denver Coleman was born, weighing 4 pounds and 1 ounce.
“I heard her cry for the first time and that just, I — I can’t even put into words how I felt at that moment,” remembers Kenyatta.
“It was just, you know, the most beautiful moment being able to hold her, gaze up on her and then hear her cry.”
Her doctors were also pleased. “In the immediate new newborn period, she was very stable and didn’t need any of the immediate treatments that they typically need, whether it’s placing coils or whether it’s supporting her heart function with medications,” said Wilkins-Haug. “Our hope is that she won’t need any further coils placed.”
Derek remembers visiting Denver for the first time in the neonatal intensive care unit and doctors asked if he wanted to kiss her.
“I gave her a kiss and she was just making little baby noises and stuff,” he said. “That was all I needed right there.”
Now, nearly two months after Denver was born, she continues to thrive, spending most of her time sleeping and eating. She’s not taking any medications for heart failure, and her neurological exam is normal. There’s no indication that she needs any additional interventions.
“She’s shown us from the very beginning that she was a fighter,” Kenyatta said, “she’s demonstrated … “Hey, I wanna be here.’ “
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