Six-Year-Old Girl Cured of Deadly Brain Malformation
(NEW YORK) -- The operating team gathered behind Dr. Alex Berenstein as 6-year-old Haylie Howe lay unconscious on a table, where seconds before, a special dye had been injected into a catheter to highlight the blood vessels in her brain.
Berenstein, chief of interventional radiology at St. Luke's-Roosevelt Hospital in New York, watched as the negative X-ray image of Haylie's skull transformed to show only her blood vessels.
"We do like that," the Mexico City native said, crossing his fingers in the air and encouraging his colleagues to do the same. "Hopefully, it's all gone."
He paused, searching the screen for a problem.
"Cured," he said, before performing a few more double-checks and giving a few high fives. "Let's get mama here."
Lisa Hribal walked into the room as doctors lowered the blinds on the glass window to the operating room. Still, she had to put on a blue clean room suit, complete with fabric booties and a hair net. She looked around tentatively and took a few steps toward Berenstein.
"Hi," he said. "It's all gone."
Her hands flew to her face as she gasped. She thanked him and hugged him before backing up to tearfully listen to Berenstein use phrases like "normal life" as she clasped her hands and shook her head in shock.
Howe was born with vein of Galen malformation, a rare brain condition in which the brain is missing the blood vessels -- capillaries -- that would normally slow blood flow from high-pressure arteries to low-pressure veins. Without capillaries, blood rushes to those veins, causing them to expand and allowing blood to pool where it's not needed.
But while some areas of Howe's brain drowned in blood, other areas weren't getting the blood they needed, and her heart was forced to work overtime to keep up with the high pressure.
"It's basically a big channel full of blood," said radiologist Dr. Daniel Lefton, pointing to Howe's old MRI scan, which showed a long, thick tube with a bubble at the end in the center of her brain. "With something like this, this kid never would have survived."
He explained that most babies with vein of Galen malformation die of heart failure because the heart is pumping blood with almost no resistance, causing it to pump too hard.
"It's literally always fatal in 98 percent of them," Berenstein said, adding that patients with it often die in the first year of life, if not the first week, of heart failure with seemingly no explanation. Those with a mild enough malformation to survive the first months of life suffer other complications and still die young without (and sometimes with) treatment.
Howe wasn't diagnosed when she was born in a suburb of Chicago in 2006. Her mother noticed Howe wasn't meeting certain milestones and had prominent blue veins on her face, but doctors always told her that Howe would catch up. They pointed out that Howe's skin was pale, so she was more likely to have visible veins anyway.
When Howe still wasn't able to sit up on her own by the time she was a year old, Hribal grew anxious.
"Something is wrong," Hribal recalled saying. "I can't keep hearing she's going to catch up."
At the next pediatrician appointment, a doctor noticed that Howe's head was measuring large and suggested hydrocephalus, which is an abnormal accumulation of cerebrospinal fluid. A specialist touched Howe's head and told Hribal that her baby's skull was actually starting to corrode. Several tests later, doctors reached a diagnosis: vein of Galen malformation.
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