Juna’s Story: Hemispherotomy Brain Surgery to Treat Hemimegalencephaly
Juna’s Story: Hemispherotomy Brain Surgery to Treat Hemimegalencephaly
When baby Juna was diagnosed with a condition that causes debilitating seizures and threatened her future development, her parents were uncertain about what to do. Then, they found Benjamin Kennedy, MD, a world-renowned CHOP neurosurgeon. He performed brain surgery, allowing Juna a life free from seizures.
When Juna was just 1-month-old, her parents Greg and Carolyn noticed her squeezing and relaxing her abdomen repeatedly, in clusters lasting a minute or so. An online search led them to suspect Juna might be having infantile spasms, a type of seizure that typically begins in the first one or two years of life. They rushed Juna to the local emergency department near their home in California. After a night of observation, they were told the problem was likely reflux, not seizures. They were sent home with reflux medication and reassured their baby would be fine.
But the episodes continued to occur several times a day. Carolyn, desperate for answers, sought out online support groups for parents of children with seizures. There, she learned about the importance of advocating for proper tests. She shared her concerns with her pediatrician and other local physicians, pushing for further evaluation. Thanks to her persistence, more tests were run, revealing the true diagnosis: left-sided hemimegalencephaly.
Left-sided hemimegalencephaly is a rare genetic condition in which one hemisphere of the brain is abnormally larger than the other. It causes seizures. The condition put Juna’s future development at significant risk.
The diagnosis was devastating but also a relief in some ways. Greg and Carolyn finally had an explanation for Juna’s strange episodes. Determined to do what was best for their daughter, they did remote consultations with a few different specialists on the West Coast. Each doctor had a different opinion. Some recommended immediate surgery, while others suggested it wasn’t yet necessary, and to try medication first.
The uncertainty was overwhelming. The thought of brain surgery was terrifying, but so was letting Juna’s seizures go untreated, risking permanent developmental delays.
CHOP offers hope
As the family navigated these different opinions, they learned about Benjamin Kennedy, MD, a world-renowned neurosurgeon at Children’s Hospital of Philadelphia (CHOP) who specializes in surgery to treat hemimegalencephaly and other conditions that cause seizures. After reviewing Juna’s imaging results remotely, Dr. Kennedy called Carolyn and Greg. During a 90-minute phone call on Memorial Day weekend, he explained that while the need wasn’t urgent, Juna would almost certainly have to have surgery at some point.
Hemimegalencephaly is notorious for causing severe, drug-resistant epilepsy. In most cases, medication alone does not control seizures for children with the condition. The first medication Juna had been put on was already failing after just three months. Continuing down the path of medical management (trying an anti-seizure medication until it no longer controls seizures) could interfere with Juna’s brain development and quality of life.
Dr. Kennedy didn’t sugarcoat the situation or over-promise. He talked about the challenges of surgery on such a small baby, and the risks of blood loss and complications. But he also spoke with confidence about the high success rates he has seen with his surgical approach (trans-sylvian hemispherotomy) vs. traditional hemispheric surgery (functional hemispherectomy).
Both are brain surgeries that involve removing a piece of skull to access the brain and then disconnecting the side (hemisphere) of the brain causing seizures. The brain tissue from the unhealthy side of the brain is disconnected from the brainstem and the healthy side by making an intricate series of cuts around the deep structures of the epileptic hemisphere.
The main differences between the two surgical approaches is the amount of tissue removed and the sizes of the incisions and skull openings. While functional hemispherectomy involves completely removing a large portion of the affected hemisphere, in trans-sylvian hemispherotomy, the primary focus is disconnecting the affected hemisphere from the rest of the brain. In short, complete disconnection is achieved with minimal tissue removal. Due to the smaller amount of tissue removed, Dr. Kennedy’s hemispherotomy approach can be performed through a small, linear incision and a small skull opening (2-5 cm), and decreases the risk of complications from surgery.
Dr. Kennedy explained these nuances and, perhaps most importantly, took the time to truly listen to Carolyn and Greg’s concerns and explain everything in terms they could understand.
Dr. Kennedy really respected our intelligence in the way he talked to us. He didn’t try to convince us to do the surgery or not do it. He just gave us all the information, laid out the pros and cons, and let us decide what was best for our family. I appreciated his transparency. He told us everything, including the things we didn’t want to hear. He was honest about the potential for some deficits, but he also told us that the sooner we did the surgery, the better chance Juna had at reaching her full potential. — Carolyn, Juna's mom
Hemispherotomy surgery to help Juna
Six weeks after their first conversation with Dr. Kennedy, the family flew to Philadelphia so Juna could undergo the hemispherotomy surgery. They were apprehensive, but they trusted Dr. Kennedy’s expertise and less invasive approach.
The surgery was long and complicated, especially considering Juna’s young age. Throughout the process, the surgical team made sure Carolyn could stay close to her. They allowed her to remain by Juna’s side until she went under anesthesia, hold her as soon as she was breathing on her own, and continue nursing after the surgery.
Dr. Kennedy personally checked in with Carolyn and Greg as soon as the surgery was complete, even before they saw Juna. His visits continued every day during her recovery, offering a level of reassurance that meant so much to the family. In conversations with other families since then, Carolyn has realized just how rare it is to receive such personal care and attentiveness. She remains deeply grateful to the CHOP team for their unwavering support. Thankfully, the procedure was a success. After a couple of weeks in the hospital, Juna began her journey toward healing.
At first, things were difficult. Juna had no movement on the right side of her body, which was expected since the left hemisphere had been disconnected (the left hemisphere controls the right side of the body, and vice versa). But with the help of physical therapists and other specialists, she began to regain strength.
After a month of outpatient rehabilitation in Philadelphia, the family returned to California, where Juna continued at-home physical therapy and occupational therapy. Slowly but surely, she started to show signs of progress. She began sitting up unassisted, and she started to reach for toys and bring things to her mouth.
Seeing the ordinary as extraordinary
Today, Juna's life is filled with joy. She laughs all the time, particularly at her brothers and the family dog. She’s a happy, social child. As expected, her right hand still lacks function, serving more as a “helper hand,” and she faces challenges with verbal speech. But the seizures that once controlled her life are gone.
She continues to amaze her family. She can stand with support, jump and “butt scoot” to get around, while also working on walking with a gait trainer. Her physical therapist taught her one-handed sign language, and she now uses over 20 signs, often combining them into phrases like “eat apple.” Recent assessments placed her as “advanced” for her age in both language and cognitive skills.
“It’s a miracle,” Carolyn says, her voice full of emotion. “Juna is doing things we weren’t sure she’d ever be able to do. Every little milestone feels like a victory. Her path may look different from other kids her age, and that’s OK. She’s not out of the woods, but she’s thriving. And that’s a gift. We’re just focused on taking things one day at a time and celebrating every step she takes.”