WASHINGTON — Every morning, Rebecca Ackerman’s nose would run for a few hours, infuriating the 5-year-old. Then a doctor made a startling discovery: Those pesky drips weren’t allergies but spinal fluid — Rebecca had a hole inside her skull.
She was lucky: A dramatic surgical fix healed this rare birth defect that lets brain tissue grow in the back of the nose, before the Chicago girl caught a brain infection. It’s a technique that can help children with certain hard-to-remove tumors, too, and the operation’s developer is on a quest to teach more surgeons, and parents, about the option.
Too often, ‘‘neurosurgeons say nothing can be done,’’ laments Dr. Derek Bruce of the Children’s National Medical Center in Washington, who coached Rebecca’s surgeon in the painstaking operation.
At issue are encephaloceles, openings where the skull didn’t form properly and parts of the brain can slip through. Some are large and fatal either before birth or soon after.
Rebecca’s type, called a sphenoidal encephalocele, is sneaky. Thought to occur in a few dozen births a year, it’s often undiagnosed until babies or young children suffer repeated bouts of meningitis and puzzled doctors order a brain scan.
During fetal development, the pituitary gland — the master gland that controls most hormone production — forms in a little channel between the back of the throat and the brain. With this neural tube defect, bone doesn’t form to close off that channel. The pea-sized pituitary slides from the bottom of the brain into the back of the nose, and bacteria can easily travel the other direction, into the brain.
Once, patching that hard-to-reach hole meant risking serious pituitary damage and loss of the sense of smell, Bruce says. That’s because the traditional fix involved operating through the roof of the mouth, pushing the pituitary upward until surgeons could slide in a bone patch to keep it in place. That’s difficult enough, but cleft palates are common in these children as well, even harder to work through.
Bruce, a pediatric neurosurgeon, wanted a more direct route, through the nose. Working with craniofacial surgeons, he found a way to do so without destroying the nerves that control smell: He makes a cut in a child’s scalp and peels back the facial skin. Then he lifts off the nasal bone, leaving olfactory nerves still partly attached. With open access to the misplaced pituitary, he gently slides it into the brain and patches the hole. The child’s only scar is hidden in hair.
Yet it’s shocking-sounding surgery. The children outwardly ‘‘look fine and some guy’s telling you take off half their face,’’ acknowledges Bruce, who has patched 10 encephalocele patients this way, from babies to a 14-year-old who had suffered an astonishing 12 bouts of meningitis.
But ‘‘it becomes a way to get there without doing new damage,’’ he explains.
That makes the technique also useful for removing certain childhood tumors that form near the skull’s base. Some are cancerous, some not but still cause vision loss or other problems. Regardless, getting all of the growth is crucial to avoid a recurrence. Bruce says 90 percent of the 80 children with tumors he’s operated on remain well years later.
‘‘It gets directly to the root of the problem,’’ agrees Dr. Robin Bowman of Children’s Memorial Hospital in Chicago, who fixed Rebecca’s encephalocele in October after consultations with neurosurgeons around the country convinced her to try Bruce’s technique.
It’s difficult surgery. Bowman practiced on a cadaver first. She not only had to avoid damaging Rebecca’s olfactory nerves, but her optic nerve that controls vision, which also had slipped through the skull opening.
That Rebecca needed the 13-hour operation was a surprise: A minor, unrelated complaint sparked a brain scan that uncovered the encephalocele and prompted Bowman to ask about a chronic runny nose, a classic symptom.
Today, Rebecca’s a normal 5-year-old, with a healthy brain, nose and vision.
‘‘We all look at her and shake our heads in awe,’’ says her mother, Michele Ackerman. ‘‘She’s our little trooper.’’
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EDITOR’S NOTE — Lauran Neergaard covers health and medical issues for The Associated Press in Washington.
