(CNN) A team of Boston doctors has successfully performed new fetal surgery to treat a rare brain condition known as Galen’s vein 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 Thursday in the journal Stroke.
The condition occurs when the blood vessel that carries blood from the brain to the heart, also known as Galen’s vein, fails to develop properly. 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.
“Huge brain damage and immediate heart failure after birth are the two big challenges,” Dr. Darren Orbach, a radiologist at Boston Children’s Hospital and an expert in treating VOGM, told CNN.
Typically, infants are treated after birth using a catheter to insert tiny coils to slow blood flow. However, Orbach said, treatment often comes too late.
Despite advances in care, “50 to 60 percent of all babies with this condition will get very sick immediately. And for those, there seems to be about 40 percent mortality,” Orbach said. About half of infants who survive suffer serious neurological and cognitive problems, he said.
Faced with 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 member to their family.
Kenyatta said nothing seemed particularly notable about the pregnancy. After having three children, Kenyatta considered ultrasounds routine appointments.
“Baby was fine. Anatomy scan came back unremarkable. All of his biophysical profiles were all unremarkable,” Kenyatta, who spoke exclusively to CNN, said.
The Colemans even did genetic testing which characterized Kenyatta’s pregnancy as “low risk”, she said: “We honestly thought we were in the clear.”
But when Kenyatta went for an ultrasound at 30 weeks pregnant, something was different. She remembers her doctor sitting her down and telling her she was worried.
“She told me something was wrong with the baby’s brain and his heart had grown bigger,” Kenyatta recalls.
After more investigation, there was a diagnosis: VOGM.
But the Colemans had heard of a clinical trial being conducted by Brigham and Women’s and Boston Children’s hospitals that could provide treatment before their baby was born.
Kenyatta recalls being told of the possible risks – preterm labor or brain haemorrhage to the fetus – but the Colemans felt there was no other option for them. They wanted to participate in 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 operation, there were two patients: Kenyatta and her baby.
Doctors had to make sure the fetus was in the correct 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 ensure the fetus was not only in the correct position for the procedure, but remained there.
Wilkins-Haug explained that they used a technique borrowed from previous in utero heart surgeries. Once the fetus is in the optimal position, it “gets a little injection of medicine so that it doesn’t move and it also gets a little injection of medicine for pain relief,” Wilkins-Haug said.
From there, doctors inserted a needle through the abdominal wall, carefully threading a catheter through the needle, so the tiny metal coils could fill the vein, slowing blood flow and reducing pressure.
The baby immediately showed signs of improvement, with scans showing lower blood pressure in key areas.
“It was exhilarating at the moment that we were technically able to do the embolization,” Orbach said.
But success wasn’t defined by that single moment, but rather by what was to follow.
“Will she still be able to show progress afterwards? Will she just need some extra support after I’ve had her? Will she still have immediate heart failure?” Kenyatta wondered.
Always a fighter
After the procedure, Kenyatta was slowly losing 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, I – I can’t even put into words how I felt at that moment,” Kenyatta recalled.
“It was just, you know, the most beautiful moment to be able to hold her, watch her and hear her cry.”
Her doctors were also thrilled. “In the immediate newborn period, she was very stable and didn’t need any of the immediate treatment she usually needs, whether it’s placing coils or supporting her heart function with medication. “, said Wilkins-Haug. “Our hope is that she won’t need any more coils placed.”
Derek remembers visiting Denver for the first time in the neonatal intensive care unit and the doctors asked him 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 here.”
Now, nearly two months after Denver was born, she continues to thrive, spending most of her time sleeping and eating. She is not taking any medication for heart failure and her neurological exam is normal. There is no indication that she needs any additional interventions.
“She showed us from the start that she was a fighter,” Kenyatta said, “she demonstrated… ‘Hey, I want to be here. “