Changing the world: Baby Denver leads the way after first-of-its-kind procedure for VOGM

Denver Coleman is less than 2 months old, but she’s already helped blaze a trail for other children and families, a feat she accomplished before she was even born. Denver, who was diagnosed with a vein of Galen malformation — a type of rare blood vessel abnormality inside the brain — in utero, is the first child in North America to successfully undergo a groundbreaking procedure to treat it while still in her mother’s womb.

“She’s a fighter,” says her mother, Kenyatta. “And she’s been one from the beginning.”

From routine to devastating

The Colemans’ journey started with a completely unrelated issue: Kenyatta’s itchy legs. It’s a common symptom during pregnancy, but when blood tests also showed elevated levels of bile — indicating a problem called cholestasis of pregnancy— her doctor recommended regular ultrasounds to ensure that Denver was developing properly. At 28 weeks, everything looked fine. But just two weeks later, Kenyatta and her husband, Derek, received devastating news.

“This ultrasound took longer, and our doctor’s whole demeanor was different,” Kenyatta remembers. The physician explained that there were drastic changes in Denver’s brain and her heart appeared enlarged. “She told us she had never seen anything like this in her practice.”

A rare, life-threatening diagnosis

The couple was referred to a maternal-fetal medicine (MFM) specialist, and then a neurointerventional radiologist, in their home state of Louisiana. While the Colemans waited to see the neurointerventional radiologist, they hit the internet, searching for a term they had noticed in their appointment notes earlier: vein of Galen malformation (VOGM).

In this malformation, multiple arteries in the brain connect directly with a large central vein, instead of connecting with capillaries. This causes a rush of high-pressure blood into the vein that causes it to enlarge like a balloon and the rapid blood return to the heart can lead to pulmonary hypertension, brain injury, and congestive heart failure, often immediately after birth. If not diagnosed and treated early, VOGM can cause severe problems and may even be life threatening.

Even before the diagnosis was confirmed, Derek and Kenyatta joined an online support group and researched their options. One name kept coming up in their searches: Dr. Darren Orbach.

‘Now, we had hope’

Dr. Orbach is chief of Neurointerventional Radiology and co-director of the Cerebrovascular Surgery and Interventions Center at Boston Children’s Hospital. He’s also an internationally recognized expert in VOGM — and, as it turned out, something of a mentor to the Colemans’ neurointerventional radiologist in New Orleans. During their appointment, the local specialist told Kenyatta and Derek he had already consulted with Dr. Orbach about their case. He also told them about a new clinical trial that Dr. Orbach was leading on a pioneering in utero procedure for VOGM (see sidebar).

The couple reached out and were able to speak with Dr. Orbach within just two days. “Now, we had hope,” says Kenyatta. And there was more good news: Denver met the strict criteria for the study.

“Even though we had done our research and knew what to ask, Dr. Orbach was very detailed and transparent about the procedure,” says Derek. “He told us to take some time to think about it, but we already knew the answer. We were willing to do whatever it took to try to save our baby.”

Fetal surgery for VOGM

Until now, babies have not been treated for VOGM until they are born. Dr. Orbach and his colleagues at Boston Children’s and Brigham and Women’s Hospital designed a new procedure aimed at intervening while infants are still in the womb. Because VOGM is so rare — and because the intervention is meant for fetuses with healthy brain tissue but at high risk for poor outcomes like heart failure — the team has waited for several years to find eligible candidates. Denver Coleman is first of what they hope will eventually be 20 patients in this clinical trial. Learn more about the procedure and ongoing trial.

Time to act

On March 12, less than a month after Kenyatta’s 30-week ultrasound, she and Derek arrived at Boston Children’s Maternal Fetal Care Center, which is also affiliated with Brigham and Women’s Hospital. The center provides support to parents of fetuses with life-threatening congenital anomalies. Thanks to their family and friends in Louisiana, they were able to leave their older three children at home while they met with their care team in Boston and attended a slew of appointments.

By the next day, tests showed that the VOGM was getting bigger — but thankfully Denver’s brain tissue wasn’t damaged. Her clinicians and parents knew it was time to act. For Kenyatta and Derek, there was no hesitation.

“We’re grounded in our faith and were so confident in Dr. Orbach and the team,” says Kenyatta. “Denver was already fighting for her life, and now it was time for us all to help save it.”

A true team effort

Soon, Kenyatta was whisked away to nearby Brigham and Women’s, where the procedure would take place. In a true collaboration between both hospitals, maternal-fetal medicine specialist Dr. Louise Wilkins-Haug and her colleagues used external pressure to position Denver in the womb. Then, using ultrasound guidance provided by radiologist Dr. Carol Benson, she first injected an anesthetic and a medication to stop fetal movement into Denver’s thigh. Then she introduced a needle through Kenyatta’s uterus and carefully into the back of Denver’s skull. Assisted by endovascular neurosurgeon Dr. Alfred Pokmeng See, Dr. Orbach next guided a microcatheter through the needle into the center of the malformation and then inserted 23 tiny metal coils through the microcatheter into the VOGM. The coils would help slow the blood flow and reduce the pressure in the affected vein.

Although both Denver and Kenyatta received pain medication and sedation during the procedure, Kenyatta was awake — and could watch on the ultrasound screen as everything unfolded.

“I listened to music to try to relax, but I saw as the coils entered and felt a collective sense of relief in the room,” she says. All told, the procedure took just 20 minutes.

Surviving — and thriving

Hours later, a repeat ultrasound and echocardiogram revealed that Denver was already improving. By the next day, she was doing even better.

“To go from her being at risk for heart failure to such significant improvements in just a day — that was mind blowing,” says Derek.

Because Kenyatta’s amniotic fluid began to leak — a potential side effect of the procedure — Denver was delivered a bit early, at 34 weeks. It happened to be St. Patrick’s Day. While many people were out enjoying the holiday, the Coleman family was reveling in their own celebration: Their daughter was here — and she was thriving.

Denver spent a few weeks in Boston Children’s and Brigham and Women’s neonatal intensive care units (NICUs), meeting developmental milestones and gaining weight. She’s required no medications to support her heart function and none of the neurointerventional procedures that newborns with VOGM usually undergo. She’s had no issues from the VOGM or the fetal surgery, “just typical preemie stuff,” says Kenyatta. And she became a bit of a star at both hospitals. “She’s alert, nosy, loud, and smiles a lot. Staff members would drop by the NICU just to meet her.”

To help the next family

Today, Denver and her parents are back home in Louisiana with the rest of their family. They’ll be back to Boston in six months for a checkup, but all signs point to a positive — and pioneering — outcome. Meanwhile, the Colemans are passionately spreading the word about the procedure that likely saved their daughter’s life and that has the potential to do the same for other families.

“It was never just about Denver,” says Kenyatta. “We also did it to help the next baby, and the next baby after that.”

In a recent interview, Derek introduced Denver to reporters, adding, “She’s about to change the world.”

She already has.

Learn more about theMaternal Fetal Care Center and the Cerebrovascular Surgery and Interventions Center.