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Uncharted territory

Thanks to an innovative surgical solution from a University of Minnesota team, 2-year-old Lydia Kohler is not only alive but also right on track with her developmental milestones. (Photo: Scott Streble)

A baby girl’s rare, life-threatening problem is solved by a highly skilled University team—and a strategic use of glue

When Anna and Brandon Kohler headed to University of Minnesota Medical Center, Fairview in September 2011 to have their child, they weren’t expecting anything other than a normal birth—and that’s just what they got. Normal labor, normal birth, normal baby girl.

“Everything was totally fine,” says Anna Kohler, “except she had a tiny bump on the back of her head that the doctors said was likely from the birth and would go away. So we took her home.”

At little Lydia’s two-week checkup, the doctor was slightly concerned that the bump hadn’t gone away, so he ordered an ultrasound, which revealed an abnormality. An MRI followed, and that’s when they discovered that Lydia had a large, blood-filled sac in the back of her head caused by an arteriovenous fistula, or AVF—basically, a network of abnormal blood vessels that continually flowed into the sac. Anna Kohler says that she and Brandon could actually feel a pulse when they touched the spot on Lydia’s head.

The AVF in Lydia’s head was beyond rare—maybe 100 of these malformations have ever been reported in newborns. So the doctors had no roadmap for a course of treatment.

“We knew what it was, but she was still so small, and she was asymptomatic, so we decided to delay treatment,” says Bharathidasan Jagadeesan, M.D., a neurointerventional radiologist at University of Minnesota Amplatz Children’s Hospital and an assistant professor in the University’s Department of Radiology.

“Because it was so rare, we actually reviewed the case with many, many people,” adds Andrew Grande, M.D., a vascular/endovascular neurosurgeon on Lydia’s medical team, which also included vascular/endovascular neurosurgeon Ramachandra Tummala, M.D. “Everybody agreed that the best thing to do was wait.” So they sent the Kohlers home, cautioning them to stay alert to anything unusual.

“Well, we were brand-new parents,” laughs Anna. “We didn’t know anything about what was typical!”

Andrew Grande, M.D.

Emotional ups and downs

When Lydia had a massive seizure at six weeks old—a result of heart failure caused by the strain of pumping huge amounts of blood into the sac in her head—Anna and Brandon Kohler were jolted out of their new normal world into a medical nightmare, which began with a mad race to Amplatz Children’s Hospital.

“We were terrified,” Anna Kohler recalls. “We had no idea what was going on. We just wanted someone to tell us everything would be OK. But the doctors said flat out, ‘We can’t give you a guarantee.’ When she went into the operating room the next day, we weren’t sure we’d ever see her alive again.”

Four grueling surgeries later, each one lasting eight to 10 hours, the Kohlers were numb, going back and forth between grief and hope and despair and faith that everything would be fine. During the fourth surgery, just before Christmas 2011, the extended Kohler clan gathered to wait with Anna and Brandon.

“Our family and friends are so incredibly supportive,” says Anna Kohler. “Our group filled the waiting room that day and, after about 10 hours, Dr. Jagadeesan came out and said, ‘She’s cured.’ Just like that!”

“They gave us a standing ovation,” Jagadeesan says. “This huge group of people on their feet clapping and cheering. It was nice to have such trust and faith from the family.”

“Going through the soft spot was radical, but it was that or nothing,” says neurointerventional radiologist Bharathidasan Jagadeesan, M.D.

A novel approach

The problem from the outset was the tricky combination: the rarity of the condition, the high rate of blood flow into the sac, and Lydia’s young age. While the team knew it had to get inside Lydia’s head and seal off the openings of the abnormal arteries to stop the flow, the approach was risky. The doctors had to thread a catheter through the femoral artery into Lydia’s brain, then inject dye to highlight the network of blood vessels, then inject a glue commonly used to seal off aneurysms.

“During the first surgery, we injected glue into the sac and closed off about 30 percent of the abnormal connections,” explains Jagadeesan. “Then a second surgery got us to about 70 percent. We were chipping away at it, getting closer to shutting them all down.”

Then Lydia experienced seizures and bleeding after the second surgery, and the team knew it had to try a dramatically different approach. Instead of going through the artery to inject glue, the doctors went directly through the soft spot on Lydia’s head—something that had never been done before.

“Going through the soft spot was radical,” Jagadeesan admits, “but it was that or nothing.”

It worked. Using the new technique, the team completely shut down the malformed connections, which caused the sac to shrink and Lydia’s brain to rebound into the space once filled by the sac. Eventually, says Jagadeesan, they expect the sac to go away completely.

“We are so grateful to them. I look at Lydia now, so happy and cheerful, talking like crazy--you'd never know anything was ever wrong. How can you ever say 'thank you' for that?” - Anna Kohler (Photo: Scott Streble)

Triumph of teamwork

Today Lydia is a running, talking, 2-year-old whirlwind who will never remember days spent in lifesaving surgery. That’s the first happy ending.

And what the doctors learned from treating Lydia’s AVF has also given them new tools to use on other patients afflicted with abnormal blood vessel connections; the team has already contributed to heart, brain, and lung procedures using techniques it honed with Lydia. So when, defying the odds, a second baby girl was born this year in the Twin Cities with the same rare AVF, the doctors knew what to do.

Finally, the triumph of teamwork remains a lasting legacy of Lydia’s treatment. Grande sums it up like this: “By working together we were able to understand the problem and gain the confidence to treat it. That’s a unique theme here at the U; we work together on all our cases, arguing, debating, asking—and, as a result, everything we do is better.”

“If we hadn’t thought out of the box,” adds Jagadeesan, “and had this kind of team cooperation, I don’t think either of these girls would have lived.”

And, thankfully, for the Kohlers, today that is so far from their reality.

“I can’t say enough about these doctors,” says Anna Kohler. “We are so grateful to them. I look at Lydia now, so happy and cheerful, talking like crazy—you’d never know anything was ever wrong. How can you ever say ‘thank you’ for that?”

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