By Steve Spencer

The skull base sits at one of the body’s most crowded intersections, where the nose and mouth meet the bottom of the brain. Tumors, birth defects and traumatic injuries here were once only reachable through large craniotomies that pulled the scalp back to work from above. Over the past two decades, endoscopic techniques traveling through the nose have made many of the same lesions accessible with far less trauma to the patient. At UAB and Children’s of Alabama, that shift has taken a particularly unusual form: it is being done routinely in children.

Jessica Grayson, MD, an otolaryngologist and rhinologist, and James Johnson, MD, a pediatric neurosurgeon, have built a skull base program around their two areas of expertise. Grayson opens the corridor through the nasal cavity and sinuses. Johnson takes over once the anatomy shifts to brain tissue, cranial nerves and major blood vessels. Both are on faculty at UAB and operate at Children’s, and their partnership predates the COVID-19 pandemic.

“What’s interesting about this area, and I think that’s one of the reasons why this partnership is so important, is it’s really the interface between the nose and the mouth, and then the base of the skull and the brain,” Johnson said. “We’ve built a powerful partnership based on Dr. Grayson’s expertise with access to this area through the nose, and my expertise with working with these conditions once you get inside the skull.”

The conditions the pair treats fall into three categories. Traumatic fractures at the skull base can cause spinal fluid leaks that lead to meningitis. Congenital defects can leave an opening through which brain tissue descends into the nasal cavity, conditions known as encephaloceles or meningoceles. And a range of tumors, from benign pituitary lesions to malignant growths, can sit in a location traditional neurosurgical approaches struggle to reach.

Grayson describes her portion of the procedure as sinus surgery, performed with a camera and small instruments through the nostrils and no incisions on the face. Once the team reaches the interface between nose and brain, the anatomy changes and so does the risk, with optic nerves, carotid arteries and other structures controlling eye and facial function all running through that narrow corridor.

That planning has become more efficient the longer the two have worked together. “Just like a group of musicians who’ve been playing for a while,” Johnson said of the shorthand they’ve developed. Johnson determines the surgical angle and how much of the brain he needs to visualize. Grayson then decides how much bone can safely be removed and how the space will be closed, a step she said has been the biggest factor in allowing them do more complex cases.

One of the more difficult cases the pair has handled involved a teenager with a transclival chordoma, a rare tumor growing in the clivus, a bone shaped like an ice cream cone between the sinuses and the top of the spine. The mass had compressed a cranial nerve controlling eye movement, leaving the patient with double vision. Because that location sits directly in front of the brainstem and basilar artery, the team could not rely on imaging alone to know what they were dealing with.

“You often need tissue to confirm that,” Grayson said. “Once you open this space, you’ve potentially exposed an artery and the brain fluid, which increases the risk for meningitis. So you have to have a diagnostic and therapeutic plan up front.”

A separate case, involving a two-year-old, shows how easily these conditions can be missed. The child had chronic nasal congestion that appeared to be enlarged adenoids, and a different ENT physician removed adenoid tissue to clear the airway. The obstruction was actually an encephalocele, brain tissue that ha d descended through an opening near the pituitary gland that should have sealed during fetal development. Because the defect connected directly to the spinal fluid surrounding the brain, and the nose is not sterile, the child developed meningitis before the true cause was identified.

“The problem with these encephaloceles is that if there is a connection between the brain and the nose, obviously the nose is not sterile,” Grayson said. “Occasionally we’ll have children with this who present with meningitis, and we treat the meningitis, and then go in and repair it.”

Before transnasal techniques existed, repairing that kind of defect required a craniotomy, opening the skull from above to seal the opening from the top down, a far more difficult recovery for a small child. Grayson’s fellowship training in Australia in advanced rhinology and anterior skull base surgery gave the partnership the tools to avoid that approach, though the case carried its own risk: the pituitary gland was intertwined with the defect, and damage to it in a toddler could mean a lifetime without normal growth hormone production.

“I’m never afraid to ask a question, even if it may sound stupid, because the worst thing you could do is assume that you know and never ask,” Grayson said. “In something like this, where every decision is very important, we do a lot of double checking, even in the middle of a case.”

Johnson agreed the back-and-forth is central to how the team avoids mistakes. “Jessica’s not afraid to tell me if she thinks something’s not a good idea, and it helps me improve my treatment plan,” Johnson said. “Our team approach leads to much better outcomes.”

The program handles roughly 15 to 25 combined cases a year, small compared with the hospital’s broader surgical caseload, but substantial for a pediatric skull base program. Most programs of this kind nationally are adult-focused, with adult neurosurgeons occasionally treating children, rather than physicians trained specifically in pediatric neurosurgery. Johnson trained in both before completing a pediatric fellowship and now practices exclusively in pediatrics. Grayson is currently the only physician in Alabama performing this combined procedure at a children’s hospital, making Children’s of Alabama the only in-state option for many families.

“Some of this was born a little out of curiosity on each of our parts individually, and some of it out of having a heart for the parents and kids of Alabama, and not wanting them to have to go somewhere else,” Grayson said. “So it matters to us for lots of reasons. We’re both parents, and we care very much about how that affects families.”