Miriam’s Story: Unexpected Brain Surgery for a Rare Malformation

Miriam Spak smiling in the forest

When Jason and Megan Spak of Pittsburgh and their healthy daughter Miriam accepted an invitation to participate in the national Adolescent Brain Cognitive Development (ABCD) Study®, they never imagined how it would change their lives. Miriam was 10 years old when she joined the study in 2016. The National Institutes of Health’s ABCD Study is the largest long-term study on brain development and child health in the United States. The University of Pittsburgh was among only 21 institutions in the country chosen to recruit volunteer families for the study.

“We thought it was interesting research and were willing to be a part of it,” says Jason. It looks at how various childhood experiences and changing biology affect brain development and social, behavioral, academic, health, and other outcomes.

An Unexpected Outcome

“It sounded pretty easy. We would answer survey questions several times a year and they would do one low-resolution MRI of Miriam’s brain each year,“ explains Jason. But Miriam’s first MRI revealed what appeared to be a cerebral cavernous malformation (CCM) in her brain. When the ABCD research team told the Spaks about Miriam’s possible CCM, in 2018, the Spaks immediately began their own research.

They learned that a CCM is an abnormal cluster of small blood vessels in the brain. These mulberry-shaped bundles can burst and bleed into the brain. That can lead to seizures, headaches, back pain, hearing or vision changes, and even strokes or paralysis.

“After reading about cavernous malformations and about surgery to remove them, we scheduled an appointment with Dr. Stephanie Greene at Children’s,” says Jason.

Stephanie Greene, MD is director of vascular neurosurgery and perinatal neurosurgery at UPMC Children’s Hospital of Pittsburgh. She is a nationally recognized expert in pediatric vascular neurosurgery. She also serves as co-director of the Neurovascular Center of Excellence at UPMC Children’s. After giving Miriam a thorough examination — including a second, higher resolution MRI, which confirmed that Miriam had a CCM — Dr. Greene gave the Spaks two treatment options. They could wait and see if the CCM got bigger or go ahead with surgery.

She explained that CCMs often enlarge when they bleed and can be life threatening. Miriam’s high-resolution MRI showed a previous bleed. That made the chances significantly higher for another hemorrhage in the next five years.

“After talking with Dr. Greene, we understood that brain surgery would have risks, but that doing nothing — and waiting to see if Miriam had another hemorrhage — would also have risks,” said Jason. After taking a few months to ponder both options, the Spaks decided Miriam would have the surgery. “Dr. Greene reassured us that she does this surgery often and has had good outcomes,” he says.

About the Neurovascular Center of Excellence

The Neurovascular Center of Excellence at UPMC Children’s offers world-class care for young people up to age 23 with a blood vessel disease of the brain or spine. Our experts work together to design the best treatment plan for each patient. We explain their disorder, treatment options, and what to expect from our caring, multidisciplinary team.

In addition to specializing in rare blood vessel conditions, the doctors and staff in the Neurovascular Center of Excellence — and throughout UPMC Children’s — are experts in the unique anatomy and needs of children. Doctors across the U.S. send patients to the Neurovascular Center for its expertise in diagnosing and treating rare conditions.

Miriam’s Experience

Dr. Greene removed a piece of Miriam’s skull behind her left ear (a craniotomy) and used ultrasound to guide her to the malformation. Using an operating microscope, Dr. Greene carefully separated the CCM from the Miriam’s normal brain tissue. She removed the malformation in one piece. Surgery took 6 hours, and Miriam stayed overnight in the Pediatric Intensive Care Unit. She recovered quickly, going home the second day after surgery.

There are risks with this kind of surgery, but they vary depending upon the location of the malformation. According to Dr. Greene, Miriam’s was in a part of the brain that was easy to reach. She was able to remove it without damaging healthy brain tissue.

“It wasn’t an easy decision to make,” says Jason. “But Dr. Greene gave us all the information we needed to make that choice for our daughter.”

“Miriam will have annual MRIs at UPMC Children’s for the next five years to check for new CCMs, but she recovered completely from the surgery,” says Jason. “She’s been pretty good-natured about this whole experience. 

“Dr. Greene is very good about not overstating or understating the risks and realities she sees,” he adds. “By communicating facts, she gives parents the power to make their own decision. We’re very grateful to have a doctor with her expertise in Pittsburgh.”

To learn more about the Neurovascular Center of Excellence at UPMC Children’s, contact us by phone at 412-692-5137, by email at neurovascularcenter@chp.edu, or by submitting our form.