A Crown for Kathrina: The Face of an Infant (Part 2)


They’re often likened to cartoon characters who share many of the same features: enormous eyes, tiny button noses, small mouths.

“That sort of anatomy in an older child doesn’t work. If the eyes are too big and bulging forward, they’re not protected. If the nose isn’t developed, the child can’t breathe,” said Dr. Richard Hopper, Surgical Director for the Craniofacial Center.

Kathrina’s condition has brought her to the CranioFacial Center at Seattle Children’s Hospital, one of the few medical centers in the world that will even attempt the kind of risky surgery she needs.

Given a choice, Dr. Hopper, would prefer to wait.

“The ideal time to do it would be as late as possible because you want to get as much of their growth out of the way before you do an operation like this. We didn’t have that luxury with Kathrina,” he said.

Kathrina’s underdeveloped forehead and cheekbones leave her eyes unprotected. Her tiny nose doesn’t bring her enough air to her brain.

“She just tries to gasp and get enough oxygen when she’s sleeping, but is not able to get enough oxygen for her brain development,” said Dr. Hopper.

Doctors have waited as long as they can. Any longer and the potential damage would be irreversible.

“Her prognosis would be very poor. It wouldn’t take much for her to fall, hit her eyeball, and then she loses her vision,” said Dr. Hopper.

Kathrina’s condition is considered to be the most rare form, the most severe, the most extreme. Fixing it with surgery will be difficult.

The goal is to change the shape of Kathrina’s skull, build up her forehead and cheekbones to protect her exposed eyes and construct a nose that brings her more oxygen.

But even such a complex surgery can only do so much. To accomplish the rest, Dr. Hopper plans to use a new device called the synthes mid-face external distractor Essentially it’s a metal cage attached to the skull that will help pull Kathrina’s face forward, one millimeter a day, to further open her airways.

“The nice thing about distraction is that you can make much bigger movements than you could with any other type surgery so this one surgery will probably replace another 4 or 5 potential smaller operations,” said Dr. Hopper.

Before the surgery, Kathrina braves another doctor’s appointment. She is fitted with a special splint that will eventually help attach the device to her face. Because she’s so young, the fit needs to be exact to protect Kathrina’s developing teeth.

More adjustments are made, and through it all, Kathrina sits patiently, never once complaining as doctors complete the tedious fitting.

It’s during this visit with Dr. Hopper when Kathrina’s parents, Paul and Tamara, are taken through the upcoming surgery step by step. What follows is not an easy thing for any parent to hear, especially since Dr. Hopper isn’t leaving anything out.

“What we’re doing is separating the face from the skull. And to be able to do that we do the incision, the cut on the scalp and everything gets folded forward,” said Dr. Hopper.

“You think about all the risks that are associated with surgery, just any simple surgery, the risks that are involved, it’s scary. Even the risk of anesthesia and all those things. So when you look at a big surgery like this, your risks are even greater. So you think too much,” said Tamara.

“The other thing that’s very different with Kathrina are these soft spots � and right here, you can feel her soft spot right here. I would be touching her brain right there.

“You realized how close it was to the brain and all the other internal organs in the head and it really kind of made you shiver a little bit when you realize the magnitude of the actual surgery. We always knew about it but when you actually see it described it becomes a little frightening,” said Paul.

To ease any fears Kathrina may have, she’s allowed to see, for the first time, the device she’ll soon be wearing.

“You almost feel as if you’re tricking her a little bit. Because you’re getting her to wear it and you’re getting her the feel of it, but you realize she doesn’t understand the magnitude of what she’s getting into,” said Paul.

Kathrina’s surgery will take place in another week. The Goodwins go home now to prepare themselves, their daughter and their two sons for what’s to come.

Even though the surgery is not intended to be cosmetic, it will result in some noticeable physical changes. Tamara is already worried she’ll miss the old Kathrina.

“I like her face the way it is. If I had my way we wouldn’t change anything, just leave it alone,” said Tamara.

“The risks associated with this surgery in terms of death, of loss of vision, of stroke of any sort of brain damage are high enough that it would not be justified in terms making her look better. This is something that’s going to help her brain get enough oxygen. It’s going to protect her vision,” said Dr. Hopper.

« Part 1: A Crown for Kathrina | » Part 3: Preparing for Surgery