"I always thought something was up with her."
That's what Michele Ackerman said about her daughter, Rebecca.
But what was up with Rebecca exactly? What was it that caused the variety of symptoms Michele noticed in her 5-year-old? What caused the slight abnormalities Rebecca had been born with, almost too minor to be considered birth defects, since she had obviously grown into a happy, healthy, well-developed kid?
Rebecca lives with her family outside Chicago. She was born with a cleft lip, but it was so minor as to have gone unnoticed at first. Surgeons corrected it when Rebecca was 4 months old.
Rebecca also came into the world with what's known as a sacral dimple, a little divot at the base of her spine. Nothing to worry about.
It seemed that no kid had as chronic a runny nose as Rebecca. Every morning it would run for a couple of hours. Is this a problem? Michele wondered. No, she was told. "They thought I was a crazy mother," she said.
It was Rebecca's feet that finally energized her parents to push for answers. Her feet fell asleep all the time.
So her parents took Rebecca to Dr. Robin Bowman, a pediatric neurosurgeon at Children's Memorial Hospital in Chicago. Bowman ordered an MRI of Rebecca's spine and then, almost as an afterthought, she asked Michele if Rebecca had ever had a brain MRI.
"I said no," said Michele. "Why would anyone scan her head?"
One good thing about MRI scans of the head is that they very clearly show all the bits of the brain hidden inside and answer two very important questions: Is there anything there that shouldn't be there? And is everything that should be there in the right place?
In Rebecca's case, the answer to the second question was no. What Michele and her husband, Sam, saw on the MRI was chilling: Rebecca's brain was growing into her nose from a hole in her skull. It wasn't phlegm running out of Rebecca's nose every morning. It was spinal fluid.
Rebecca had been born with what doctors call an encephalocele. It occurs when the skull doesn't form completely and part of the brain protrudes from it. It's what's known as a midline defect, and many of Rebecca's problems -- her cleft lip, her sacral dimple -- were related to the failure of her neural tube to close up while she was in her mother's womb. About a dozen children are born with the condition each year in the United States.
This tiny part of Rebecca's brain included some pretty important stuff: her pituitary gland, her hypothalamus and her optic nerves. Also troubling was the fact that just as spinal fluid could get out, bacteria and viruses could get in. Doctors were amazed that Rebecca had never suffered from meningitis, an infection of the lining around the brain and spinal cord.
Medical science has struggled with how best to treat this condition: how to gently push back the brain tissue and seal the hole. The affected area is at the base of the skull. Surgeons can't go through the back or top of the head because of what's in the way: the brain. For a while the favored technique was to go through the roof of the mouth, but that is difficult and can damage the pituitary gland.
Among the physicians Bowman consulted was Dr. Derek Bruce, an attending neurosurgeon at Children's Hospital here in Washington. Bruce has pioneered a type of surgery ideally suited to treat conditions such as Rebecca's, as well as tumors found in the same area of the brain.
The Ackermans contemplated traveling to Washington to have Bruce perform the operation, but they were comfortable with the team in Chicago, which included Bowman and craniofacial surgeon Dr. Frank Vicari. So Bruce offered to travel at no charge to Chicago and assist with the procedure, which he had performed about 70 times, 10 times in patients with encephaloceles like Rebecca's.
The procedure, called a transfacial extended subfrontal craniotomy, involves gaining access to the brain by carefully peeling away the face and nose and going through the front of the head.
"It sounds horrendous," said Bruce. "You tell parents all you're going to do is go in there and take out the nose and you're going to fix this and everything's going to look fine afterward. [They think] you're crazy: 'What do you mean you're going to take my little baby and take her face apart and put it all back together again and everything's going to look okay?' "
But that's exactly what Bruce promised Rebecca's parents: He would help Rebecca's doctors take her face apart. And everything would be okay.
Tomorrow: Rebecca's surgery.
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