麻豆果冻传媒

The name Ari means 鈥渓ion鈥� in Hebrew, and Ari Ellman鈥檚 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鈥檚 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鈥� 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鈥檚 age, the noncancerous but fast-growing tumor, called a craniopharyngioma, was entangling Ari鈥檚 hypothalamus, pituitary stalk, visual pathways, and critical brain-feeding blood vessels. Unless it was removed, it would endanger all those structures.

The Ellmans鈥檚 world turned on its head that day. 鈥淚 barely had time to feel sorry for myself, though,鈥� remembers Ari鈥檚 father, Jonathan. 鈥淎 friend said, 鈥楾here鈥檚 no time for self-pity, or anything else really 鈥� except focused action.鈥�

鈥淭he night after the diagnosis, my heart was all over the floor,鈥� says Ari鈥檚 mother, Na鈥檃ma. 鈥淏ut Jonathan turned his computer toward me and said, 鈥楾hese are the top hospitals and craniopharyngioma surgeons we need to speak with. Tomorrow!鈥欌赌�

The Ellmans seized the reins of Ari鈥檚 care and didn鈥檛 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鈥檚 attention.

Endoscopic skull base surgery

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鈥檚 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.

Our team

Most surgeons wouldn鈥檛 even consider the procedure for a child Ari鈥檚 size. But doctors at Lucile Packard Children鈥檚 Hospital Stanford, led by Gerald Grant, MD, chief of pediatric neurosurgery, were willing to try. Dr. Grant assembled a team of experts, including , 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鈥檚 operation, they knew they had the right team. The group鈥檚 record of surgical excellence, their special focus on pediatrics, the advanced technology in the new hospital鈥檚 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 鈥渇eels almost telepathic.鈥�

Preparations for Ari began long before the surgery. An MRI-and-CT-scan-derived image of Ari鈥檚 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.

鈥淎n advantage of the endonasal approach for a tumor like Ari鈥檚 is that you can remove it from its root instead of its top,鈥� says Dr. Fernandez-Miranda. 鈥淎nd clearly visualizing the brain structures surrounding the tumor in advance is key.鈥� A resin model of Ari鈥檚 skull base was also 3-D printed, on which realistic approaches could be tested and further rehearsed.

鈥淎 2-year-old鈥檚 sinuses are only 15 to 20 mm wide, or narrower. And you鈥檙e removing a tumor that may be wider than the nasal passage,鈥� says Dr. Hwang, the ENT surgeon on the team and an expert in endoscopy. 鈥淚t鈥檚 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鈥檚 why these additional technologies can play such an important role in pediatric skull base surgery in particular.鈥�

By the day of their surgery, the Ellmans had 鈥渄one everything we could to ensure Ari had the best place, the best doctors, the very best chances of success,鈥� says Ari鈥檚 mother, Na鈥檃ma. When they left their Laurel Heights townhouse at 5 a.m. on February 8, they began 鈥渂y far the hardest drive we鈥檝e ever had,鈥� she says. 鈥淎t the end of that drive, we knew we鈥檇 be handing Ari over and it would be out of our control.鈥� But then, she continues, 鈥渨e were met by Dr. Maass, the anesthesiologist! She was so warm and confident and capable鈥斺�� says Na鈥檃ma, and Jonathan, finishing Na鈥檃ma鈥檚 sentence, adds, 鈥溾�攖hat we could tell she wasn鈥檛 going to let anything happen to Ari on her watch.鈥�

The sentiment was prescient, Na鈥檃ma says. Birgit Maass, MD, would be a tireless protector of Ari鈥檚 over the 16 hours of surgery鈥� 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鈥檚 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!

鈥淲hen the doctors came out after the [post-surgical] MRI, their faces were beaming, all smiles and red cheeks!鈥� says Na鈥檃ma. The final report from Dr. Fernandez-Miranda: 鈥淲e preserved all structures while completely removing the giant craniopharyngioma.鈥�

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鈥檛 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鈥檚 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鈥檚 Hospital Stanford that will benefit or save many other children with craniopharyngiomas to come. But at preschool today, Ari isn鈥檛 thinking about that; he鈥檚 just fooling around like the playful and miraculous little 3-year-old lion he is.