The dilemma facing doctors contemplating the case of 5-year-old Rebecca Ackerman was what you might call a problem of access.
Rebecca was born with a hole in her skull -- what's known as a sphenoidal encephalocele. This hole had allowed a tiny part of the Illinois girl's brain to slip down into her nasal cavity, leaving her at risk for injury and infection. What was long thought to be a chronic runny nose was actually spinal fluid leaking out of her head.
In yesterday's column, I described how doctors finally arrived at the correct diagnosis and how Dr. Derek Bruce, a neurosurgeon from Children's Hospital here in Washington, offered to fly to Chicago's Children's Memorial Hospital, where Rebecca was being treated, to assist with a technique he had pioneered.
Rebecca's congenital problem meant that her pituitary gland, hypothalamus and optic and olfactory nerves were hanging down in a funnel-shaped hole at the back of her nose.
"There's no way to get there from the top or the back [of the head], because you'd have to go right through the brain," Bruce said. His technique -- known as a transfacial extended subfrontal craniotomy -- involves literally confronting the access problem head-on.
The technique is sufficiently complex that Rebecca's neurosurgeon, Dr. Robin Bowman, and her craniofacial surgeon, Dr. Frank Vicari, practiced on a cadaver before doing it for real. Rebecca's surgery took place Oct. 6, 2004, with Bruce offering advice and, once, scrubbing in to lend a hand.
After Rebecca was anesthetized, a zigzagging incision was made in her scalp at the top of her head. (The serrated line helps hair cover the scar.) The skin was then peeled down, exposing her skull down to her eyes.
The surgeons removed an oval-shaped section of skull from her eyebrows to her hairline. This piece would go back at the end of the operation, but not before a sliver of bone was cut from inside it to serve as a plug for the hole the doctors were trying to fix.
The next step was the radical one: taking out the roof of the orbits. The roof is the bone that sits above the eyeballs. They also removed the nasal bone where it joins the cartilage of the nose.
Now the surgeons could see what they were dealing with -- without having to muscle the brain out of the way.
"The more you pull on the brain, the greater risk of damaging it," said Bruce. "But by coming straight in the nasal area, you almost can come straight in and hardly retract the brain at all."
What the surgical team saw were "all these bits of brain going down this hole," said Bruce. The total material was the size of the end of a pinkie, from the first knuckle to the tip.
"This is a very little piece of the brain, but it's a very important piece of the brain," said Bruce. "You can't just slice that off and fill the hole."
The surgeons gently lifted the tiny sack of brain, separating it from the nasal mucosa, the soft, moist tissue at the back of the nasal passage. They routed out two tiny dado-like channels into the bone on either side, into which they slipped the "shelf" that had been taken from inside the skull.
"And then [the brain] can't fall back down again," said Bruce.
By the time all the bones had been put back in place and Rebecca's incisions were sutured, the operation had lasted 13 hours.
"Because the face is growing so rapidly in kids, it heals in no time," said Bruce. "The first kiddie we did was from Kuwait. She was 6, and she's now 17 and absolutely beautiful."
Rebecca is doing great, too. Deep-sea scuba diving is out -- the pressure could be bad for her head -- but she can do pretty much anything else. Her brain is no worse for the experience. Her mom, Michele, said Rebecca is doing fine in school.
"Dr. Bruce was amazing," said Michele. "Who whispered in his ear to come here? Did some angel come down and tell him, 'You better get out there'? . . . Because of him, it gave me faith and hope in people. When you think people aren't good, look at what this one man chose to do."
Bowman, who was eight months pregnant at the time of the operation, is appreciative, too. "It just gives you a little sense of comfort having someone there watching over your shoulder who's done this before and who can provide answers," she said.
I asked Derek Bruce why he did it. He paused, then said: "It was the right thing to do. It was going to be much easier for the surgeons and much better for the child to have me there. It's no skin off my nose to go visit Chicago for a day. We're here to look after kids and do the best that's possible."
Use Your Brain and Your Heart
You can help ensure that all kids can have the best possible treatment by donating to our annual campaign to raise money for Children's Hospital.
Our goal by Jan. 20: $600,000.
Our total so far: $27,691.31.
There are three easy ways to donate:
Make a check or money order payable to "Children's Hospital" and mail it to Washington Post Campaign, P.O. Box 17390, Baltimore, Md. 21297-1390.
Go online to www.washingtonpost.com/childrenshospital and click on "Make a Donation."
To contribute by Visa or MasterCard by phone, call 202-334-5100 and follow the instructions on the recorded message.
To see a photo of Rebecca Ackerman, go to www.washingtonpost.com/johnkelly.