MADISON, Wis. — In 2009, UW Health doctors Beverly Aagaard Kienitz and Bermans Iskandar were faced with a heart-breaking situation: A 5-year-old girl named Danielle kept having strokes, and medicine at the time didn’t offer a way to stop them.
“I just looked at her images and thought, 'There has to be something we can do,' ” said Aagaard Kienitz, a neurointerventionalist at UW Health, and professor of radiology and neurological surgery at the University of Wisconsin School of Medicine and Public Health.
In 2003, Danielle Storhoff was born seemingly healthy, just like her older siblings, Charlie and Kirsten, to her parents David and Shelley Storhoff, from Beaver Dam. But, just days after birth, Danielle’s oxygen levels weren’t stabilizing, so Meriter Hospital in Madison sent an ambulance to bring her back safely for examination and three weeks of observation, Shelley said.
She was then transferred to American Family Children’s Hospital, where surgeons repaired a small hole in her heart and widened a narrowed aorta on Valentine’s Day.
“What was supposed to be a quick, ‘basic’ surgery, turned into open heart surgery,” Shelley said.
Danielle’s heart now stable, the Storhoffs continued with life ready for the normal challenges of raising an infant — but Danielle’s life would be upended again.
At age 3, she was admitted to American Family Children’s Hospital for an RSV infection, but while recovering, she couldn’t move her right leg. An MRI showed a series of strokes had occurred. The strokes, in turn, also impacted Danielle’s right arm and right side of her face.
While at American Family Children’s Hospital, Iskandar, a pediatric neurosurgeon, UW Health, and professor of neurological surgery, UW School of Medicine and Public Health, and Aagaard Kienitz began working with the Storhoffs to solve the riddle of Danielle’s strokes.
Aagaard Kienitz specializes in imaging the brain and executing procedures using catheters via large arteries in the body to treat various neurological conditions, while Iskandar’s focus is the more familiar type of brain surgery that requires the skull to be opened. Together they examine all cases like Danielle’s, Aagaard Kienitz said.
After reviewing incredibly detailed images of Danielle’s brain, the pair was able to deduce that Danielle was suffering from narrowing or blocking of arteries at the base of the brain, which prevents blood flow, known as Moyamoya disease. The name means “puff of smoke” in Japanese, and refers to a tangled web of new blood vessels that form to compensate for the blocked vessels, according to the National Institutes of Health.
The disease is most common in children but can occur in adults. It is very rare, however, with only about one in a million people each year suffering from the condition in the United States, though it is more common in Japan and other East Asian countries.
Surgery, followed by more strokes
There are two treatment options for Moyamoya disease, according to Iskandar.
One involves creating a bypass, called direct revascularization, similar to a heart bypass, where a healthy blood vessel from outside the skull is re-routed and attached directly to the brain arteries beyond the area of narrowing, so that blood flow in these arteries is restored and the brain is perfused again.
The second treatment type, called indirect revascularization, involves opening the skull and attaching healthy tissues to the brain itself, with the expectation that these tissues would help create new blood vessels that supply the brain. The most commonly used indirect procedure in the U.S. is the encephalo-duro-arterio-synangiosis, or EDAS, in which the healthy tissue used is an artery outside the skull. The direct bypass treatment is more often done in adults, while the indirect procedure is more commonly performed in children, mainly because of the small size of blood vessels in children. That was the case for Danielle, Iskandar said.
The team performed the EDAS procedure on both sides of Danielle’s brain, one in March and the other in April, and she went home to recover and rehabilitate. But, a year later, more strokes occurred, which were followed by more rehabilitation, then more strokes again at age 5.
“The EDAS did what it was supposed to do according to our imaging studies, but she still had strokes,” Iskandar said. “She had major strokes that required prolonged periods of rehabilitation.”
Iskandar and Aagaard Kienitz suspected that while the EDAS had created appropriate new arteries, the revascularization was limited to the narrow region of the brain where the surgery was done.
“We realized that we needed a way to create new vessels in a much wider portion of the brain,” Iskandar said.
Aagaard Kienitz and Iskandar were determined to help Danielle.
“There has to be a better way to do this,” Aagaard Kienitz said. “Some patients like Danielle keep stroking even though they have the most advanced EDAS surgery.”
Finding a new solution
Iskandar and Aagaard Kienitz began researching what else could be done by seeking opinions from multiple specialists around the country.
The prevailing recommendation was to place holes in the skull and open the membrane that covers the brain, called the dura, in each of these holes over a wide region, with the hope that this would ignite blood vessel growth in each of the exposed areas. Iskandar and Aagaard Kienitz discussed the idea and felt the procedure was at once more complicated and less precise than they hoped, Iskandar said.
After discussing the situation thoroughly with the Storhoffs, the pair advised a novel procedure that they had designed to try to address the problem. The idea was to create a very detailed map of Danielle’s brain using Aagaard Kienitz’s angiographic images, open the skull widely to expose as much of the brain as possible, and create a series of incisions in the dura between the blood vessels. Once all the cuts are made, the dural slits would resemble tree branches that cover most of the brain. They predicted the procedure would trigger blood vessel growth into the brain. They called the new procedure wide arterial sparing encephalo-duro-synangiosis, or WASEDS. With a desperate need for a solution, the family agreed, and the procedure was performed.
The results astounded the surgical team and gave the family immense relief — Danielle’s strokes stopped.
After recovering from surgery, Danielle and her family went home, and Danielle continued with rehab. But then they noticed something else changed within weeks of surgery, Aagaard Kienitz said.
“Teachers would say, 'Wow this child really switched on,' " she said. “Her parents would say, 'Her whole personality changed, she is so much brighter and she’s running around.' "
These were unsolicited comments they had not heard before from parents or teachers of children who underwent the traditional EDAS procedure, Iskandar said.
“At first, these unexpected comments made us skeptical, as it was too soon to see such dramatic cognitive improvements,” he said.
However, when they performed the procedure again with other patients, especially the younger ones, the results were the same.
“This is when we realized that these kids were getting more blood to their brains than with prior procedures,” Iskandar said.
And it all started with Danielle, Aagaard Kienitz said.
“Just looking at her scan after the procedure, Iskandar said, ‘There were no new strokes!’ That was a really wonderful moment, she was the trailblazer,” Aagaard Kienitz said.
Since then, the pair have performed 14 of these procedures with the same results, and the data were published in the journal Operative Neurosurgery.
“Because we only have a small number of patients, we have to consider the possibility that some of the improvements we’re seeing are coincidental,” Iskandar said. “But we’re greatly encouraged that the patients are doing so well, which in turn inspires us, and hopefully others, to study the procedure further.”
A life-changing procedure
Once the strokes stopped, Danielle and her family could begin the hard work of getting Danielle back to the life they wanted for her, according to David.
“All our kids played sports like volleyball and basketball and we wanted that for her, and she wanted to do that too,” he said.
Before that could happen, Danielle would have to undergo years of rehabilitation to regain fuller function in her body and learn how to walk and talk again.
Danielle returns to see Iskandar and Aagaard Kienitz every five years for check-ups, and there continues to be no sign of stroke. The only symptom over the years has been blurry vision, but nothing that has led to further worries, Shelley said.
“She had repetitive strokes for three years in a row, and now it’s been more than 15 years since she’s had a stroke,” she said.
Over time, most of her mobility and speech returned, and Danielle was able to play sports just like her siblings, and even went on to attend Wisconsin Lutheran College in Milwaukee for two years to become a teacher. She felt leading a classroom wasn’t for her, but educating children was something she wanted to do. So, Danielle returned home to Beaver Dam and is now a teacher’s aide in the Beaver Dam Unified School District.
“Everybody says, ‘Oh, she’s a miracle,’ but I don’t see myself that way,” Danielle said.
Now, at 21 years old, Danielle recounts how much she enjoyed the therapists who helped her re-learn all the skills she needed to re-learn, and, she said, the importance of being the first to have this life-changing procedure that has helped other children is not lost on her.
“I feel blessed to be part of this and to be the first to have it; I’m thankful for it,” she said.
