Groundbreaking Minimally Invasive Brain Surgery
STANFORD -- The name Ari means "lion" in Hebrew, and Ari Ellman's first day back at preschool this September was a roaring success. Ari is small for his three years, but his vocabulary and charm are big enough to more than compensate. He's excited, energetic, curious, and fully engaged with his friends and teachers.
It is hard to grasp how radically different things were for Ari just a few months ago. In August 2018, his parents brought him to the ER for uncontrolled vomiting. It was there that he had his first seizure, shifting doctors' attention from his belly to his head.
An MRI revealed a large growth in the central lower part of his brain, near the base of his skull. Extremely rare for a child Ari's age, the noncancerous but fast-growing tumor, called a craniopharyngioma, was entangling Ari's hypothalamus, pituitary stalk, visual pathways, and critical brain-feeding blood vessels. Unless it was removed, it would endanger all those structures.
The Ellmans's world turned on its head that day. "I barely had time to feel sorry for myself, though," remembers Ari's father, Jonathan. "A friend said, 'There's no time for self-pity, or anything else really … except focused action."
"The night after the diagnosis, my heart was all over the floor," says Ari's mother, Na'ama. "But Jonathan turned his computer toward me and said, 'These are the top hospitals and craniopharyngioma surgeons we need to speak with. Tomorrow!'"
he Ellmans seized the reins of Ari's care and didn't let go. They sent his case to tumor boards at 15 leading medical centers. Some suggested open-brain surgery, a massively invasive procedure that often fails to resect the entire tumor, partly because the roots of such tumors, at the bottom of the brain, are so hard to reach from above. Others suggested radiation, but that can cause lasting side effects in a young child. A third option, transnasal endoscopic skull base surgery, caught the family's attention.
Just over a decade old, endoscopic skull base surgery is minimally invasive; it employs long, thin surgical and imaging tools (endoscopes) inserted through the nose, then through the sinuses and into the skull, where they can access a tumor in the skull base. Because surgeons enter the brain from below, the approach is far less disruptive to other parts of the brain. While thousands of endonasal resections have proven the approach's efficacy, only a small handful of those were on children, and none of them were under 5. Ari was 2! So, while it seemed the best approach, it would be an unprecedented operation requiring special expertise and cutting-edge technology available at only a few centers around the world.
Most surgeons wouldn't even consider the procedure for a child Ari's size. But doctors at Lucile Packard Children's Hospital Stanford, led by Gerald Grant, MD, chief of pediatric neurosurgery, were willing to try. Dr. Grant assembled a team of experts, including Juan Carlos Fernandez-Miranda, MD, a world-renowned skull base surgeon who was recruited to Stanford from the University of Pittsburgh one month before, as well as Peter Hwang, MD, an endoscopic ENT surgeon, who is an expert at endonasal sinus surgery.
When the Ellmans met the three Stanford surgeons who would collaborate on Ari's operation, they knew they had the right team. The group's record of surgical excellence, their special focus on pediatrics, the advanced technology in the new hospital's surgical suites, and the extraordinary attentiveness and warmth of the doctors all confirmed their decision. The trio works so closely together, says Dr. Grant, the pediatric neurosurgeon on the team, that their communication during surgery "feels almost telepathic."
Preparations for Ari began long before the surgery. An MRI-and-CT-scan-derived image of Ari's brain was loaded into a new 3-D virtual reality tool called Surgical Theater, allowing the team to map, rehearse, and perfect an approach that would maximize the amount of tumor removed while protecting critical structures.
"An advantage of the endonasal approach for a tumor like Ari's is that you can remove it from its root instead of its top," says Dr. Fernandez-Miranda. "And clearly visualizing the brain structures surrounding the tumor in advance is key." A resin model of Ari's skull base was also 3-D printed, on which realistic approaches could be tested and further rehearsed.
"A 2-year-old's sinuses are only 15 to 20 mm wide, or narrower. And you're removing a tumor that may be wider than the nasal passage," says Dr. Hwang, the ENT surgeon on the team and an expert in endoscopy. "It's like getting a ship out of a bottle; you have to figure out how to take it apart and bring it out through this very narrow corridor. That's why these additional technologies can play such an important role in pediatric skull base surgery in particular."
By the day of their surgery, the Ellmans had "done everything we could to ensure Ari had the best place, the best doctors, the very best chances of success," says Ari's mother, Na'ama. When they left their Laurel Heights townhouse at 5 a.m. on February 8, they began "by far the hardest drive we've ever had," she says. "At the end of that drive, we knew we'd be handing Ari over and it would be out of our control." But then, she continues, "we were met by Dr. Maass, the anesthesiologist! She was so warm and confident and capable—" says Na'ama, and Jonathan, finishing Na'ama's sentence, adds, "—that we could tell she wasn't going to let anything happen to Ari on her watch."
The sentiment was prescient, Na'ama says. Birgit Maass, MD, would be a tireless protector of Ari's over the 16 hours of surgery… and then over the next six weeks (and subsequent procedures) as well.
From the waiting room, as the Ellmans received reports every few hours of the surgery's progress, Jonathan posted updates to a WhatsApp group of hundreds of friends and family members around the world. 2:45 p.m.: Navigation through the nose and into the skull base complete! 9:41 p.m.: Resection successful!
"When the doctors came out after the [post-surgical] MRI, their faces were beaming, all smiles and red cheeks!" says Na'ama. The final report from Dr. Fernandez-Miranda: "We preserved all structures while completely removing the giant craniopharyngioma."
After the resection, a flap of tissue was placed over the hole between the nasal passage and the brain to keep air and infection out of the brain and cerebrospinal fluid in. Unfortunately, because Ari was so small, the first flap didn't fully seal, which led to a cerebrospinal leak and meningitis. The surgeons then performed a more elaborate repair with a bigger flap from above to get a seal. This procedure had never before been tried on a child Ari's size. Ari began to eat and laugh again. Six weeks after his admission, he was sent home to restart his life as a little boy.
The surgery, the first of its kind in a child so small, was recently featured in the journal Operative Neurosurgery. It has opened a new frontier at Lucile Packard Children's Hospital Stanford that will benefit or save many other children with craniopharyngiomas to come. But at preschool today, Ari isn't thinking about that; he's just fooling around like the playful and miraculous little 3-year-old lion he is.
