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When Vein of Galen Attacks

When Vein of Galen Attacks

When Elliot Correa was diagnosed in utero with the rare vein of Galen neurovascular malformation at an outside hospital, his parents Michael and Jennifer Correa started calling around for specialists who could treat the condition. The name of Johns Hopkins interventional neuroradiologist Philippe Gailloud kept popping up, says Michael. "Thankfully he was available and we quickly transferred Jennifer’s care to Johns Hopkins Children’s Center.”

There, the Correas learned that vein of Galen neurovascular malformation is a life-threatening congenital condition inside the brain in which malformed arteries and veins trigger a rush of high-pressure blood flow that can tax the heart and lead to pulmonary hypertension and heart failure, as well as stroke and seizures. They also learned that not only could Gailloud effectively treat the condition by embolizing, or plugging with coils and glue, the malformation in a number of staged procedures, but that Elliot would also be cared for by a team of neurocritical care specialists in the pediatric ICU.

A lot of parents, local pediatricians and ER referring physicians know that we have a strong pediatric neurology and neurosurgery program, but they are not as well aware of our integrated neurocritical care program,” says pediatric intensivist Courtney Robertson. “Elliot’s case illustrates how that program works to achieve best possible outcomes for patients.”

Upon Elliot’s delivery, however, that outcome was questionable. Already he was suffering from pulmonary insufficiency—backflow from the pulmonary artery to the right ventricle of the heart—and an enlarged heart.

“They were worried about his lungs keeping oxygen moving through his body,” says Michael. “For us emotionally it was crazy. We were trying to hold it together and figure out what was going to happen.”

The neurocritical care team, however, already had Elliot’s plan in mind before his birth. Reviewing his labs and imaging at their weekly Thursday afternoon case conference, they had decided that medically supporting Elliot’s heart—in part, by giving him medications to relax his pulmonary vessels—and staging embolizations to lessen the strain on his heart and reduce the risk of brain injury, were critical. Pediatric intensivist Sujatha Kannan explains why the embolizations to reduce blood flow from the malformation have to be planned over a period of time.

“With vein of Galen malformation, you can have as much as a third or half of all cardiac output going to the brain, so you don’t want to change that flow completely at one time,” says Kannan. “You don’t have a good sense of how the flow is going to normalize after that, leaving the patient with a risk of stroke and bleeds in other parts of the brain.”

Now, with newborn Elliot before them, the team decided he should have his first embolization the next day. It was a prudent decision as Elliot, on ventilator support, continued to decline his first night in the pediatric ICU.

“The sooner you can intervene the better it is. The brain dissolves away as you wait,” says Kannan. “Even though he came to us from an outside hospital, he got to us early and we could intervene quickly.”

After his first embolization and two more in as many days, however, Elliot’s heart and lungs were still struggling. Gailloud and the team decided they needed to close as many vessels as they could on the venous side of the neurovascular malformation. Although studies have shown transvenous techniques to be potentially curative in many cases, some investigators have expressed concern that the approach is associated with worse cognitive outcomes than transarterial embolization. Initially, the risky venous approach appeared successful but a month later, over Christmas, Elliot’s breathing grew more labored, his lungs collapsed and his heart rate climbed dramatically.

“His doctors handed us pagers and prepared us for the worst,” says Michael. “It was a dark moment for all of us.”

“It is a life-threatening condition,” affirms Kannan. “These are the difficult conversations we have with the families while we continue to provide supportive care and give their child time for the heart to recover.”

Quietly and quickly, Michael says, Elliot’s heart did recover. On the 53rd day of his life he was removed from his ventilator and placed on nasal non-invasive ventilation. He got off the rollercoaster, says Michael: “Elliot remained vigilant and continued to improve. Technically, he still has pulmonary hypertension but his lungs are clear and his heart is good.”

In all, Elliot would stay six months in the pediatric ICU and undergo seven embolizations. As expected for patients with vein of Galen malformation, which untreated carries almost 100 percent morbidity and mortality, Elliot did suffer some neurologic deficit. At discharge, he was prescribed medication for seizures and follows up every six months with pediatric neurologist Ryan Felling.

Robertson attributes Elliot’s survival and outcome to the depth and breadth of the members of the neurocritical care team, their collaborative approach and early interventions. Having a deep research enterprise to study brain injury and illness and develop novel treatments, she adds, factors into their goal of achieving best possible outcomes for patients facing serious and life-threatening conditions such as vein of Galen malformation.

“This is the future,” says Robertson. “The neurologic complications in some of these events is still an area we need to work on. You can survive but you need meaningful neurocognitive recovery to have a good quality of life.”

The neurocritical care program, located in the Pediatric ICU in the Charlotte R. Bloomberg Children’s Center building, is a 20-bed unit staffed by a multidisciplinary team of pediatric intensivists, neurologists, neurosurgeons, neuroradiologists, neuropsychologists, neurorehabilitation specialists, critical care nurse practitioners and nurses trained in managing brain injury and illness. 

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