Green Bay Press-Gazette
January 31, 2003

Parents seek normal life for daughter
By Thomas Rozwadowski

Vicky Britton wants her 6-month-old daughter, Ashley, to have a normal childhood. A surgery scheduled on Feb. 12 for the toddler, who suffers from bicoronal craniosynostosis, is the first step.

A first-grade teacher at Gillett Elementary School, Britton said she was devastated when doctors discovered Ashley’s condition at the end of last year.

“She is our beautiful daughter, and she will have to go through major surgery with high risk,” Britton said. “It is all of the unknowns that are scary.”

According to a Web site for the National Institute of Neurological Disorders and Stroke, bicoronal craniosynostosis is a congenital anomaly characterized by premature closure of one or more cranial sutures—the fibrous joints between the bones of the skull—before the completion of brain growth. Some cases involve an abnormal head shape, while other symptoms include increased cranial pressure, developmental delay, mental retardation, seizures and blindness.

Britton said most children with the condition require only one surgical procedure. However, Ashley most likely will need multiple surgeries because she has two coronal sutures fused.

Britton said the condition is best treated by an experienced craniofacial team, making it necessary for the family to travel to Johns Hopkins University in Baltimore for the special surgery.

Pamela Williams, Ashley’s aunt, said a fund has been set up to help Vicky and her husband, Jim, with expenses. Even with health insurance coverage, the cost of multiple surgeries, various uncovered medical specialists, blood transfusions and yearly food and lodging will be draining for the family, she said.

Britton, who lives in Cecil, said an outpouring of kindness has already come from the Gillett School District. Donations from families, staff, administration—and even money from student piggy banks and penny jars—have been collected for Ashley’s fund.

Britton said she is grateful for the assistance and is doing her best to stay positive.

“Right now, we are just focusing on the first surgery and are hoping that there will not be more,” she said. “We are hopeful that this surgery will give her a happy and carefree childhood like all children deserve to have.”

Associated Press
February 20, 2003

Posada’s son has surgery again

New York Yankees catcher Jorge Posada rejoined the team Thursday after missing two days because his son had surgery.

Jorge Posada IV, born in November 1999, has craniosynostosis, which occurs when the bones in a baby’s skull fuse before the brain has stopped growing. It can be corrected through surgery.

The younger Posada underwent a 10-hour procedure on Tuesday at a Tampa area hospital and could be released by early next week. It was his third operation.

“This could be his last one,” Posada said. “It all depends on the bones growing. If the bones don’t grow, they’ll have go back in there again when he’s 7 to 8 years old. We’re praying. Hopefully, this is his last one and those bones grow.”

Posada said the procedure included reconstructive work on the forehead, bridge of the nose and around the eye.

“We always tell him what’s going on,” Posada said. “He’s only three years old. You don’t know how much he gets out of it. He said he was going to be strong. It’s tough. Now he wants to go home. As soon as he goes home, he starts acting like a normal kid.”

The Baltimore Sun
August 6, 2003

Opening A Door; When no one would hire their learning impaired son, Mary and Dick Chizmadia bought Kenny a Catonsville candy store to run; Success has rarely been so sweet.
By Gary Dorsey

Fifteen minutes,” he says, unlocking the store’s front door as cars whiz by.

A brisk walk. Perfect timing. Mom’s trip to California means 40-year-old Kenny Chizmadia has The Candy Box to himself this morning. Waffles, chocolate milk and bananas powered him up for the hard ramble to work.

Too sweet?

“Nope!” he says.

He left the house at 8:35 wearing pressed blue shorts, an unwrinkled red T-shirt and white Reeboks, covered the mile stretch, scaled one of the biggest hills in Catonsville and arrived without breaking a sweat.

He checks his watch again—8:50 on the dot.

“Yep!” he says.

He switches on the lights, looks at the thermometer, then checks the clock on the wall. He writes down his arrival time on a calendar in the back room.

At 8:50 a.m., it is 67 degrees in here. Good enough.

The shop can open at 10:00—10:00:00, if he stays on top if it.

Kenny unloads coins from his bellypack into the cash register, then stuffs his bagged lunch—two plain hot dogs, two plain slices of bread, bottled tea—in the cooler. He washes his hands and lifts the cotton cloths covering the old candy cases. Row after row of rich, cool chocolates. He peers down at them through oversized spectacles like a near-sighted jeweler admiring a display of precious stones.

Stainless steel parts from the ice cream machine are laid out on towels like surgical instruments. Kenny will clean the machine and lubricate each part before installing it. He will skim the fat off the chocolate ice cream mix. He will fill the yogurt machine. He will make sugar water ...

“Uh-oh,” he says. People in the apartment upstairs are tromping around. Could be trouble if they start taking showers now. He needs the hot water to make snowball syrup.

When he glances at his watch again it is 9:06.

Eighteen years in the biz, Kenny still runs the morning routine like a precision drill.

A sign on the door says, “Mary’s on twin duty,” which means Kenny’s mom has left for the West Coast to help while her daughter-in-law gives birth to her second set of twins. Kenny may be learning disabled, but he can run the shop all by himself now. He doesn’t need Mom. Did he say he was actually glad she’s away?

“Yep!” he says.

He installs silver blending arms on the ice cream machine, pops open the top and goes to fill it with a silky ice cream mix. The cardboard mouth of the milk carton resists. He tugs; it rips. “This brings back memories,” he says, recalling the same torment back in grade school 30 years ago. “I hate these things with a passion.”

It is 9:35 when the phone rings.

“Kenny Box!” he chirps. (That is, “Candy Box” in Kenny’s fast-paced lingo.) “Hi, Mom!”

Mary Chizmadia is calling from California, where it is going on 6:40 a.m.

“I’m making ice cream as we speak,” he tells her. He does not mention that the supply of ice cream mix for the rest of the week has not arrived yet. Mom does not need to know everything.

It is 9:48 when he stops to clean the yogurt machine, then rushes to the back for a bag of clean towels.

“Here he comes now,” he says, without looking up.

Kenny has heard the squeal of bad brakes. Moments later a short, heavy man comes through the front door rolling a dolly loaded with 12 cases of ice cream mix.

“Here she comes now,” Kenny says, still in the back folding towels.

A moment later, 16-year-old Allison Hedden, his assistant for the day, steps through the front door.

It is 9:58 when he slips past Allison and dodges tall stacks of ice cream mix now deposited around the cash register. He flips the “Open” sign on the front door, and hangs another under the peppermint-striped pole by the road.

Coming back inside, he faces a predicament: He has 72 one-gallon cartons of ice cream mix boxed up and lying on the floor by the cash register—they are waiting to be fit into an already crowded cooler; Allison still hasn’t mixed the snowball flavors; and somebody must stir the sticky pail of marshmallow cream for parfaits. It is 9:59. The signs indicate the Candy Box is open. But Kenny is suddenly a long way from ready now.

“Hmmm,” he says, pausing over the unexpected dilemmas. He hesitates, then heads for the back room to the phone.

“Call Mom,” he mutters, dialing California.

In 1968, when Kenny was 5, a doctor told Dick and Mary Chizmadia that their little boy would be a vegetable by the time he was an adult. He had a brain injury called cranial stenosis. Medical books defined it as an infant disorder of the skull, where one or more of the sutures of the skull fuse too early. Kenny’s brain could not expand normally and his head grew elongated, resulting in serious cognitive problems that the doctor did not know how to fix.

The Chizmadias would not be deterred.

“I tell people not to listen to doctors,” Mary says today. “We just decided it didn’t make any difference what the doctor said, and we’d just go on so Kenny could be the best person he could be.”

Back then, the Chizmadias were told that medical science had little to offer Kenny. The local schools did not have classes for learning-disabled children. They were told to put Kenny in a special school that combined incorrigibles with Down Syndrome children. They were told to put him in a school for retarded children. When the Chizmadias found a brain clinic in Philadelphia that offered a kind of experimental surgery to help children like Kenny, the family doctor in Baltimore scoffed: “Too many kids have too many holes drilled into their heads,” he had said.

But Mary and Dick made their own decisions.

Kenny had brain surgery when he was 9, wore a protective helmet for two years and started all kinds of cognitive and speech therapies. Every three months, the Chizmadias took their son to Philadelphia for testing. Meanwhile, with their other three children in school (Kenny is the second of four), Mary decided to train herself for a challenge that many of the “professionals” in her community simply seemed unprepared to help her with. She went to Towson University for a degree in elementary education, then got a master’s degree in special education at Loyola College. She had decided that she could figure it out, if no one else could. She would help Kenny and, if possible, all the other Kennys in the area, too.

Mary Chizmadia became a special education teacher.

Kenny’s IQ jumped 10 points, his cognitive skills improved, he attended the public schools in Catonsville and he always tried his best. But by the time he graduated from Catonsville High School, at 21, there were still no jobs for people like Kenny Chizmadia.

A high school woodworking teacher told his parents the young man would never be able to do anything too productive with his hands and, by all means, keep him away from machinery. Kenny was trained to shelve books at the local libraries, underwent job evaluations at a nearby cafeteria and an insurance company. He even tested for a low-level job at the National Security Agency. He scored well enough, but his poor eyesight, his parents were told, made him ineligible to work.

No one would hire Kenny. He couldn’t drive. He couldn’t even get an interview.

So Dick and Mary Chizmadia decided to create their own opportunity for Kenny. They decided what their son needed was a sort of college education, something unique, bold and challenging, a matriculation in life that only they could offer.

“He is not going to sit around the house for the rest of his life!” Mary declared. She was angry because she knew the truth. Her son was capable of a lot more than people realized.

The Chizmadias decided to buy Kenny his own business. They looked all over Catonsville and discovered an old feed and grain store on Frederick Road that had been converted into a sandwich shop and then, more recently, a candy store.

“Of course, we don’t know anything about how to run a business,” Mary would tell people.

Then they borrowed $10,000 from the bank, bought the store and on June 22, 1985, opened the doors to the Candy Box.

It was perfect. Mary quit her job. Dick kept his job as an electrical engineer at Westinghouse. Kenny walked to the shop every day. The Chizmadias suddenly had created their own customized, intensive, graduate-level training ground for the Kenny’s new life’s work.

Only one problem: No customers.

By 2 o’clock, Kenny has sold a pound of Australian crystallized ginger to an old lady wearing a pink scarf; $1.17 worth of Fireballs and Bubblicious to a breathless 11-year-old boy with a skateboard and a pocket of change; a strawberry snowball to a ferocious-looking dude wearing a Yankees baseball cap backward; cherry snowballs to a couple of sweaty cyclists; a cone of soft ice cream blended into a chocolate/vanilla twist to a reading teacher from Hillcrest Elementary School; five candy fruit slices, a quarter-pound of almond bark and a bag of bon-bons to a smartly dressed professional woman; a large egg custard snowball to the mailman; and a bag of Gummy Frogs, 10 Cowtales, three Peanut Smoothies, a half-pound of Red Swedish Fish and an orange parfait to a woman who said she suddenly got a “sweet tooth” on her way home from work.

People were also buying paste-on Fourth of July tattoos, candy tins, the latest edition of The Catonsville Times, stacked by the front door in a gift-wrapped cardboard box and many boxes of hand-packed chocolates.

Kenny’s dad is in the back room entering receipts on the computer. Allison is mixing the marshmallow cream. Two more of Kenny’s assistants—high school girls like Allison—are helping customers. Kenny, who managed to get all the ice cream milk packed away and the yogurt machine cleaned before the first customer arrived at 10:17 a.m., barely had time to eat his two hot dogs and check his blood sugar at lunch (he’s diabetic). But finally, around 2, there is a break in the action, and he puts his hands in his pockets and rocks back on his heels.

For a moment, he daydreams about dinner. Mom told him that he and Dad could eat out six times while she was gone. Tonight would be one of those nights. The Country Buffet! Lasagna, Caesar salad, country-style steak!

“Snowballs,” says a voice across the counter.

Four weary, sweat-stained, sun-toasted men are staring at him. They have been in the neighborhood for hours cutting lawns.

“What kind?” Kenny asks.
“Frappe,” the weary man says.
“You want the parfait,” Kenny says.
“What kind—vanilla?”
“Ice cream?”
“What flavor—strawberry?”
“How many—two?”

A woman asks for a gift-wrapped box of chocolates.

Oh, boy, Kenny thinks. He loves gift wrap. Even better than working the credit-card machine.

“How much?” he asks.

“A quarter pound.”
“Creams or nuts?”
“Light or dark?”
“Strawberry? Raspberry?”

When he’s boxed it, Kenny sets it on the scale: 0.27 pounds.

“Shoot,” he mumbles, taking it to wrap. Missed by 0.02.

A man asks for 2 pounds of jawbreakers. Kenny spoons them out and weighs the bag: 2.01.

“Shoot,” he says.

A man with a face like a basset hound asks for 1 1/2 pounds of mixed licorice.

Kenny misses again: 1.52.

A woman in red capri pants wants “anything coconut.” Two pounds.

He boxes it. Weighs it: 2.0, on the dot.

“Yes!” he whispers, clenching his fist in victory.

Sometimes Kenny finds that life at the Candy Box can be true perfection.

Eighteen years have flown by at 1810 Frederick Road.

The first year, they made $7 selling snowballs; the second year, a $4,000 ice cream machine saved the day.

Dick built new counters. Mary tried new candies. Kenny was joined by his youngest brother and his sister. Then 15-year-old high school girls came to work—first at night, then on weekends and, eventually, for entire summers. Before you knew it, the high school girls graduated and their 15-year-old sisters came to work, too, and soon the Candy Box work force was like an extended family.

Mary also made a practice of hiring learning-disabled children part-time, which led to Kenny’s finding a girlfriend named Melanie, who helped around the shop one summer. “Miss Chatterbox,” he calls her. She’s learning disabled, too. When he turned 40 on June 2, she sang “Happy Birthday” to him. Her mother became Mary’s bookkeeper and close friend.

The kinship patterns at the Candy Box now would challenge a Harvard anthropologist.

Today, the little shop offers licorice from Finland, fancy chocolate truffles for $16.75 a pound, old-fashioned penny candies, Mexican-style party pintildeatas, all kinds of fancy mints, sugar-free chocolate and sugar-free soft-drinks, Gobstoppers and Boston Baked Beans, Turkish paste and pralines, Fudge Meltaways, toasted coconut, Jordan almonds and all kinds of sugary suckers.

It stays open seven days a week from Easter to the last week of September, six days the rest of the year. The Chizmadias work nights, weekends, and holidays, and have only closed the shop twice in 18 years—once, one January, when they painted and wallpapered, and again this past winter during the big snowstorm. Dick and Mary and Kenny take separate vacations so the show will always go on.

The business has endured a ceiling collapse (when someone left the bathwater running upstairs), a couple of armed robberies (no one’s been hurt), and an accident on Frederick Road when a car hit Mary while she was working along the narrow space in front of the building (she walked out of the hospital a few hours later). They have paid off the bank loan, Dick has retired, all their children, except Kenny, have married and produced grandchildren, and in the candy biz, the Catonsville shop is now known as a “destination store”—customers don’t just drop in, like they would at a mall; they will drive for miles to shop exclusively at the Candy Box.

“We don’t owe anybody a dime,” Mary says, proudly. “People tell me, ‘You should really have a business plan.’ I say, ‘What’s that?’ and they say, ‘So you’ll know where you’ll be in five years.’”

She only laughs. She doesn’t know where she’ll be tomorrow, much less in five years. But she does know one thing: Kenny will be here.

He has learned to manage nearly every facet of the business—he runs the cash register, counts the money, orders supplies, wraps the candies, makes snowballs, cleans the machinery, takes out the trash, mixes syrup flavors, answers the phone and remembers everything his parents can’t about their customers—names, favorite candies, even children’s birthdays.

Kenny has become a local celebrity, of sorts, recognized on the streets—“I can’t go anywhere without people knowing who I am,” he says, matter-of-factly—and a model of work ethic devotion that wins admirers in this old-fashioned, middle-class town.

“I’m learning disabled,” he will sometimes say, proudly.

He is also, officially, vice president of the Candy Box, and fully capable of running the store while Mom’s away.

At 4:59 p.m., Kenny straps on his bellypack and leans back on a counter.

“Almost time,” he says.

The high school girls have taken over, handling a late-afternoon crowd that will grow more dense and demanding tonight. Dad’s still balancing accounts on the computer. Mom hasn’t called in a few hours.

At 5, the chime over the front door jingles, and Kenny starts his power-walk home, stopping once to say hello to neighborhood dogs Gizmo and Butch, and waving to a guy named Mike, who has big tattoos on his arms and is starting to water his lawn after arriving home from work, too.

Another successful day?

“Yep!” Kenny says, already dreaming about dinner out—lasagna, chicken-fried steak, a large Caesar salad—and an evening in front of the TV.

There are, in fact, many rewards for a working man, each distinct and fulfilling, every one sweeter than sugar candy.

The Denver Post
December 25, 2003

‘Special needs’ child brings unique gifts of love, courage
By Kevin Simpson

She answers the door wearing a pink sweat suit, a slightly off-kilter smile, eyeglasses perched on full cheeks and a white bow in blond hair that falls in thick waves past her shoulders.

‘I’m Tess,’ she says brightly.

She is 4, a small, festive package set among the holiday decorations in her family’s Parker home. The indentation left by a small hole in her throat winks when she turns her head just so, hinting at a healed-over ordeal.

‘What’s this?’ asks Heather Clark, gently touching the spot where her daughter’s tracheotomy tube used to go.

Tess giggles. ‘My bellybutton!’

In the next year, surgery will smooth the skin, erasing evidence of the breathing apparatus that had been a fixture almost since birth. Until then, Tess has a comic comeback to the inevitable questions that face a kid who looks a little different.

A still-undefined genetic syndrome followed her into the world, a condition that trapped a fully functioning mind in a misshapen skull fused shut against growth. At 10 weeks, she embarked on what Heather calls the ‘surgery track,’ a grueling succession of procedures to disassemble and rebuild her cranium and then reconfigure her face.

Tess has turned a corner in a big year of repair and rehabilitation. Last summer, a pivotal procedure—one of 26 surgeries she has undergone so far—pulled her face’s flattened features into more normal contours.

It also opened her obstructed airway, eliminated the trach tube, and put her on the way toward clearer speech, toward learning to eat solid food.

Toward kindergarten. Toward swimming lessons.

‘I constantly say to God, ‘Thanks for choosing me and Kent to be her parents,’’ says Heather, 35. ‘It’s an honor to watch her grow.’

But the Clarks did not come easily to this place. Not Heather, a self-described perfectionist who considered herself the least-equipped to absorb the demands of her daughter’s condition. Not Kent, who confessed an ingrained aversion to the very term ‘special needs.’

And least of all Tess, whose painful trials have been duly chronicled alongside the snapshots in the family scrapbook celebrating all her small victories.

Kent and Heather look now at Tess, at themselves, and see the incremental changes with greater clarity and broader context. They see a transformation.

On Christmas Eve day of 1998, the Clarks heaved a sigh of relief. The ultrasound test on pregnant Heather, which they feared might reveal fluid on the brain of their second child, showed nothing so dire.

‘Everything looks good,’ they were told.

Little more than a month later, when Tess was born, her parents did a double-take. The back of her head looked unusually flat, and the lack of contour in her face made her eyes seem to bulge. Her lower jaw jutted out noticeably, setting her mouth constantly agape.

Almost immediately, Heather slipped into denial: Maybe this was what her daughter was supposed to look like after a rough trip down the birth canal.

Later, a nurse practitioner delivered a bassinet to her hospital room. Tucked inside was a copy of ‘Smith’s Recognizable Patterns of Human Malformation.’

Kent, a 35-year-old supervisor of mutual fund traders, clung to his innate optimism. He craved information on Tess’s big-picture prognosis, but doctors could supply only pieces.

He focused on the positives and told himself that perhaps the impact on his daughter would be minimal, just a challenge to overcome.

On her third day at home, Tess turned blue and labored to breathe. The Clarks rushed her to Children’s Hospital in Denver, where a small army of specialists examined her.

The most conspicuous element of her diagnosis was craniosynostosis, an abnormal shaping of the bones in her skull. The malformation inhibited her breathing passages, prompting the insertion of the tracheotomy tube.

But the accompanying complications cast Tess as a medical anomaly.

Her intestinal tract was rotated oddly, which would create ongoing issues with feeding. And like only a relative few children born with craniosynostosis, she suffered from abnormal fluid circulation in the brain, which meant installing a shunt to continually drain the fluid into her body cavity.

‘She’s her own unique mutation,’ explains Michael Handler, a pediatric neurosurgeon and part of Tesss care team at Children’s. ‘We know certain syndromes are associated with specific genes. She doesn’t fit any such pattern.’

But Handler did recognize a familiar pattern in the way Tess’s parents received the news.

‘You could almost call it a grieving process,’ he says. ‘They have to give up the notion of a ‘normal’ child. They grieve for the loss of the mental picture they have of this child, which has to be completely recast around an unknown future.’

Heather read everything she could find about Tess’s symptoms to better anticipate the months and years to come. But when she came across a similar case that involved 22 surgeries, she dissolved into tears.

‘I felt like I’d received the largest homework assignment of my life,’ Heather says. ‘I hated eighth-grade science. I had no inclination for it. And now I was realizing I not only would have to understand science, but understand it well enough to participate.’

Meanwhile, Kent struggled not only with his daughter’s medical issues but with the very terminology that clanged against his expectation of a healthy child.

‘It took awhile before I was comfortable with the term ‘special needs,’’ he says. ‘I was in denial, thinking she’ll overcome it.’

Heather laughs now at how clichéd it sounds, but once she sat watching ‘Oprah’ when the guest happened to be the mother of a Down syndrome baby. The woman, who felt emotionally overtaxed by the care demands, was explaining why she had put the child up for adoption.

Heather well understood the mother’s desperation, but the decision to give up the child jolted her. What came shining through the TV screen was a more personal, even spiritual realization about her own circumstances.

‘God made Tess the way he wanted her to be,’ Heather says. ‘And he picked me.’

At a low point during one string of surgeries, Kent and Heather sought out the care director at their church and prayed for something to finally happen predictably, without complication. They emerged feeling that a burden had been lifted.

‘To see what Tess has gone through, the kind of resilience she has, has helped me be stronger,’ Kent says. ‘That’s where my faith kicked in.’

Heather’s chronicle pulls no punches.

She has augmented the cheery pages of a mother’s scrapbook with loose pictures that, by comparison, seem ripped from a photojournalist’s unflinching portfolio.

Here’s Tess with a cupcake celebrating her first birthday. Here she is playing with friends. And here, she looks happily oblivious to her attendant medical apparatus as she plays house in a miniature kitchen.

But over here, she lies in her hospital bed, post-surgery, beneath blood-spattered sheets.

Here’s the close-up of the headgear, surgically attached to her skull, that will gradually stretch her facial bones toward more normal contours.

One page in the scrapbook lists a by-the-numbers account of Tess’ first year:
94 doctor appointments.
58 days in the hospital.
16 doctors and therapists.
7 surgeries.

In a way, Heather says, the family chronicle stands as a metaphor for how they try to live. ‘Before Tess,’ she says, ‘we were concerned with portraying the image of the perfect life. Now, well, sometimes things get a little ugly.’

It got ugly and it got beautiful—all during this past year, when Tess underwent surgery for a ‘mid-face advancement.’ Other operations, like the one to install the shunt or the one that took apart her skull to allow her brain to expand, loomed more critical in the medical sense.

But the mid-face work was the most conspicuous, the most wrenching for Kent and Heather. And it produced the most visible, dramatic results.

The procedure began with the attachment of the headgear to the skull. During that operation, Tess lost more than twice her total blood volume.

‘All of her surgeries, whether they were cranial expansion or whatever, they were not very bloody,’ recalls Kent. ‘And then to walk into the room after the surgery and see her oozing blood from her nose, her ears, her eyes, her mouth. We thought, ‘What have we done?’’

When the surgical trauma healed, leaving her resembling a little girl trapped inside a catcher’s mask, the task of reshaping her face began.

For three weeks, every night before bed either Heather or Kent would locate the two screws on Tess’s apparatus and give them one full turn. The doctors likened it to an orthodontist tightening braces-the process produced pressure, but no pain.

Tess never complained.

The nightly twist incrementally tugged her features forward, while her parents watched a new face take shape before their eyes. And they heard a new voice as Tess’s airway cleared and her nasal tone disappeared.

Once the tightening stopped, the brace remained in place for nine more weeks while the bone grew back.

While her parents once washed her carefully in a shallow bath so water wouldn’t enter her trach tube, they now have enrolled her in swimming lessons. Next year, she may begin kindergarten without the nurse that had to accompany her to preschool.

She still must learn to eat solid food and be weaned off the tube that sends formula directly to her stomach. Hearing aids will need adjusting. Therapy will improve the clarity of her speech and the tone of her muscles.

But the worst of the surgeries has passed. The doctor visits have become fewer and further between.

At Children’s, Tess kicks off her shoes and dives into her session with occupational therapist Patty Kenyon. Her hair has been pulled back in pigtails so it won’t get in the way of an hour-long romp designed to work her trunk muscles and develop greater stability.

For Tess, it’s simply the best kind of play.

Her thick, wavy locks fall generously across the meandering scars on her head, mostly concealing the road map of her medical ordeal. Her bursts of energy riding the tire swings, tossing colored animal figures, pushing a big yellow ball and tumbling about the padded floor make Heather smile with pride.

‘We started out,’ she says, ‘with a baby who just laid there.’

Now, Tess wants to try a more challenging swing.

‘That one’s a little harder,’ Kenyon cautions.

Tess replies in a carefree, singsong voice. “I can do it.”

Llanelli Star
March 18, 2004

Toddler Steffan has a life-changing op
By Katy Williams

A two-year-old Llanelli boy has undergone life-changing surgery. Steffan Burnett has a rare head condition that causes a deformity in the front of his skull.

He has emetopic synostosis—a form of craniosynostosis. The condition is not well-known and went undiagnosed until last year.

Steffan had to have his skull re-shaped at Birmingham Children’s Hospital.

Parents Clare and Jeff Burnett, of Florence Street, had mixed emotions about the operation on Tuesday (March 16).

Mrs Burnett said: “We were looking forward to it in one sense but were scared in another. The sad thing is I have got used to his bump.

“I’ll miss things like when he goes to give you a kiss he hits you with his bump. He is young enough to forget yet old enough to know pain. But it is for the best.” The Star helped raise awareness of the condition by publishing a series of reports following his development and lead up to surgery.

If left untreated there was a chance Steffan could lose his sight, hearing and develop behavioural problems due to pressure on his brain.

Surgery involved breaking bones in his skull and removing parts of bone that caused the protrusion.

Because the lump is at the front of Steffan’s head, his skin was peeled back. A dissolvable plate was inserted to encourage his skull to refuse normally.

“In five days he will be out of hospital and in four weeks it will all be over,” said Mrs Burnett.

Sun-Sentinel (Fort Lauderdale, FL)
April 28, 2004

Baby Zach Reborn; Surgery Corrects a Potentially Devastating Birth Defect
By C. Ron Allen

It was 9:05 a.m. as a team of doctors at Joe DiMaggio Children’s Hospital in Hollywood sedated Zachary Adt, then scored two V-shaped openings in the dome of his head. They lifted up the scalp and used a state-of-the-art tool to remove a 4-by-10-centimeter strip from his skull.

They replaced the flesh over his skull, smoothed it into place and stitched the incisions. Then, Dr. Drew E. Schnitt, a plastic surgeon, washed Zachary’s tiny pink head with saline. Exactly two hours after the first cut, Schnitt and the two other surgeons put aside their drill and scalpels, the operating room assistants cleaned up the blood and two pairs of hands carefully lifted, turned and repositioned the infant on his back.

Zachary, 3 months and 23 days old, was reborn.

Schnitt, who specializes in pediatric plastic surgery and craniofacial surgery, and his partner, Dr. Eric Stelnicki, as well as Dr. Ian Heger, a pediatric neurosurgeon, had just performed a less-invasive microscopic surgical technique to correct craniosynostosis, a birth defect that causes the soft spot in a baby’s skull to prematurely close.

“The amazing thing is that next week this baby will return playful and happy like nothing happened,” said Jessica Vega, a medical student who observed the operation on March 24. “[But] the parents will be treating him like a piece of glass.”

Two days after the operation, Zachary began to return to his vibrant state.

Doctors aren’t sure what causes craniosynostosis, but Schnitt said about one of every 3,000 newborns is afflicted with the condition.

An infant’s skull is separated into sections by gaps often referred to as the soft spot and several long, thin fault lines called sutures; they usually close in early childhood, Schnitt said.

In Zachary’s case, his sagittal suture, which runs from the forehead to the back of the head, fused, causing his skull to push out at the front and back of his head. This is the most common type of craniosynostosis, called scaphocephaly, or boat-shaped skull. If left untreated, the condition gradually would horribly distort his face, cause physical abnormality of the skull, mental retardation, seizures and blindness, the doctors said.

Zachary’s parents, Matthew and Christina Adt of Sunrise, noticed that his head was an odd shape—long and narrow instead of round—shortly after birth.

“My husband kept saying, ‘You know, there’s something wrong. There might be something wrong with his head,’ and I kept on saying, ‘No, there’s nothing wrong with his head. It’s perfectly fine,’“ Christina Adt said in an interview before the operation. Relatives also noticed the unusual shape, and a pediatrician, filling in for Zachary’s doctor, even hinted at problems.

“She said, ‘His head is too big, he might be brain damaged,’ and then she walked out [of the room],” Christina Adt, 29, recalled.

Zachary’s pediatrician ordered a CAT scan, which confirmed the family’s worst fears. The Adts had never heard of craniosynostosis, so they looked it up on the Internet. They found Web sites showing pictures of children with misshapen heads and lopsided faces. They also found a parent support group.

“If it wasn’t for this Web site, I would still be pulling my hair out going crazy, insane,” said Christina Adt, a database administrator.

They read stories and testimonials of hundreds of families worldwide who experienced similar as well as the other types of craniosynostosis. They even met a Broward County woman, whose son was born five days before Zachary at Joe DiMaggio Children’s Hospital and had a similar operation there a few days earlier.

The new procedure, which can be done at an earlier age than the traditional surgery, results in a smaller incision, less swelling, a shorter hospital stay and less blood loss, said Stelnicki, who designed the specialized set of endoscopic instruments, which look like scissors with rounded blades.

Five years ago, the most common treatment for Zachary’s condition was a surgery known as cranial vault reconstruction, the surgeon said.

“Such an operation could easily have taken eight hours, four to five days in the hospital and blood transfusions,” he said.

Doctors would have had to remove sections of the child’s skull, cut apart, reshape and join the sections back together on the head, Stelnicki said. Today the doctors can perform the same procedure in about 1 1/2 hours, he said.

The surgery requires that the patient wear a molding helmet 24 hours a day for up to six to eight months, Christina Adt said.

“He got his helmet and his head is almost perfectly round,” she said. “The first day after he got it, it made such a huge difference. It was so amazing.”

One month after the operation, Zachary is a dramatically different boy, his parents say. He is giggling and laughing, his deformity buried in his bones now; his head is round and regular.

“He is such a very happy, little baby,” Christina Adt said. “And I’m so happy.”

The Northern Echo
May 14, 2004

Villagers Rally to Help Baby Jake
By David Roberts

Villagers in a small rural community have come to the support of a young couple whose baby son suffers from a rare condition.

The village of Staindrop in County Durham has rallied round to raise money after hearing about the plight of seven-month-old Jake Jones who suffers from a condition called craniosynostosis.

After hearing that Jake needed a major operation to try to correct the effects of the condition, regulars in the Wheatsheaf Inn agreed to do something to help him and his parents, Elise Margetson and Alistair Jones.

Unlike most babies, the plates in Jake’s skull are fused together, which does not allow the brain to grow.

The condition affects one in 2,500 babies and in March, Jake underwent an unsuccessful five-hour operation to try to cure it.

On Tuesday, May 25, Jake will visit James Cook University Hospital for more surgery. Ms Margetson said: “It is a major operation for such a young baby, but Jake’s not really aware of what it is.”

“We were in the pub one night and mentioned Jake needed an extra operation and didn’t think any more of it.

“The next day they asked us to come up to the pub and they had set up a fundraising committee.

“We’ve only lived here a year but they made us really feel like a part of the community.” The money raised by the committee will be donated to the James Cook Hospital.

Fundraising events include a talent show at Staindrop’s Scarth Hall on June 5, from noon to 6pm, football and quoits competitions, sponsored runners in the Raby Fun Run and an auction.

Paul Stephenson, who is one of the dozen members of the fundraising committee, said: “It’s just one of those things where everybody wanted to do something.

“There were grown men holding back tears when they heard about what he had been through and seen photographs of him.”

Gloucestershire Echo
May 18, 2004

What causes craniosynostosis?

In a baby, the skull is made up of several plates of bone which lie side by side but are not actually joined. In normal circumstances these plates expand and grow, allowing the baby’s brain to grow inside the skull. Eventually, when the brain has finished growing in early adulthood, all the plates of bone will join together (fuse) at their seams (sutures) so the skull becomes a fixed box of bone which protects the brain.

In Craniosynostosis, one or more of the seams has joined too early. This happens before birth but is not caused by anything the mother did or did not do during pregnancy.

The baby is born with an unusual shaped head. This is because the skull cannot grow properly in the area that has fused too early.

The baby’s brain needs to grow rapidly during the first year of life, so there may be extra growth in another area of the skull to compensate for the restriction in the affected area. This may accentuate the abnormal skull shape.

The shape of the baby’s head will depend on which suture has fused early.

Some babies may have other abnormalities as well, such as webbing of fingers and toes, (syndactyly) or an inability to breathe through the nose (choanal atresia).

It affects around one in 2,500 babies and can be caused with by genetic or environmental factors.

Gloucestershire Echo
May 18, 2004

It has brought us all closer together

But four weeks before his operation, when George was nine months old, there was another shock in store. At a routine check-up, doctors diagnosed George with craniosynostosis—an unrelated condition which means a baby’s skull plates have fused too early, leading to a misshapen head.

“With George’s heart surgery coming up in less than a month, we were left reeling with shock,” said Jane, 38, from Gloucester.

“We found it hard to take anything in and were thinking if he doesn’t come through the heart surgery, all this will be inconsequential anyway.” Thankfully, George sailed through his operation to correct a large hole in his heart and a narrowing of the pulmonary artery.

But the bad news hadn’t ended. Within a few weeks a specialist team at Oxford’s Radcliffe Infirmary told Jane and Tim that George would need more surgery.

“When we were told the news about George’s condition and the surgery I felt like I was in a scene from Casualty where people are taken into the relatives’ room to be told bad news,” said Jane, who works in advertising.

Doctors gave George six months to recover fully from the heart surgery before they operated again.

“They cut him from ear to ear in a zig-zag and shaved away part of one of the plates of his skull to give his brain more room to grow,” said Jane.

George made a lightening recovery and for 18 months life returned to normal.

But then Jane began to notice problems with George.

“He became quite physically challenging,” she said. “He would pick up a chair and throw it and would act aggressively before returning to normal.” They took George back to Oxford for a check-up and doctors put a wire into his head to measure the pressure on his brain.

“They discovered George’s brain was growing too quickly and the skull bones had started to reknit together,” said Jane.

Three months later they were back at hospital for George’s third major operation. Thankfully, it again went well and since then family life has returned to normal.

“George did have a tough first year at school but the teachers have been very good with him and he’s fine now,” said Jane. “He really is our little miracle.” When George was first diagnosed, Jane and Tim were put in contact with the organisation Headlines, formed to offer support and fundraise for families of children with craniosynostosis.

“We joined the group because it was comforting to know there were other families who were going through the same thing as us,” said Jane.

Now the couple are an integral part of the group and Tim has been voted in as chairman. Headlines has around 700 members, publishes a quarterly magazine and holds conferences and fundraising events. Jane and Tim also run the magazine Headline News. During the period of George’s hospital treatment, Jane and Tim’s older son, Jack, was a pillar of support to his parents.

“He never once got jealous of the attention George had, even when we had to stay with him in hospital during one of his operations while Jack was at home taking his grammar school entrance exams,” said Jane.

“He is tremendously proud of his little brother and he is our hero. He has a natural aptitude with children and would like to train to be a teacher.” The stress of going through what the Prices did could have put a tremendous strain on anything but a rock-solid marriage.

But for Jane and Tim it has had the opposite effect.

“All this has only brought us closer together, if that’s possible,” said Jane.

The Dominion Post (Wellington, New Zealand)
June 15, 2004

What Hope for Keegan?

Keegan Maley, 2, has one of the most infectious grins you’ve ever seen.

Unfortunately, it’s overshadowed by his strange cone-shaped head, the result of a rare and life-threatening medical condition he was born with.

“Baby Keegan—One of a Kind” is the story of his parents’ journey to unravel the mystery behind his condition and find out what can be done to help him.

Keegan was born in Hamilton in April 2002 with multi-sutural cranio-synostosis, the cause of his strangely shaped head. His medical condition also has serious physical implications. His skull is fused and cannot expand to accommodate his growing brain. If he survives, it is unknown how it will affect his development. Although other children have been born with misshapen skulls, Keegan’s condition is extreme and his range of symptoms makes his condition unique in the world.

Every day Keegan’s mother, Michelle, 29, has to face the world knowing her baby is the subject of intense curiosity. Even a trip to the supermarket can be a harrowing experience as Keegan’s odd appearance often makes him the subject of curious stares and cruel comments.

“One of the worst incidents was at the supermarket when I had four young children running around saying, ‘Look at the ugly baby.’ I basically came home, burst into tears and said to my husband ‘I just can’t do it’,” says Michelle.

With three other children to support as well as Keegan, father Kym, 29, works up to 60 hours a week as a diesel mechanic.

Both parents struggle to balance everyday life with trying to discover more about Keegan’s condition.

We meet them as they prepare for major surgery that will reconstruct Keegan’s head so his brain has room to grow. Doctors are also faced with the challenge of trying to give Keegan as normal an appearance as they can.

The surgery will take eight hours and if successful, offers hope that Michelle and Kym’s son may yet have a healthy future.

Derby Evening Telegraph
June 22, 2004

‘Little miracle’ George wins his battle for life

In Jane and Tim Price’s case, they were given this news not once, but twice. George Price was barely 10 days old when doctors told his parents he would need open-heart surgery before his first birthday.

Jane and Tim Price were told that, if he did not have the operation, his chances of surviving beyond the age of 10 would be slim.

Four weeks before his operation, when George was nine months old, there was another shock.

At a routine check-up, doctors diagnosed George with craniosynostosis—an unrelated condition in which a baby’s skull plates fuse too early, leading to a misshapen head.

“With George’s heart surgery coming up in less than a month, we were reeling with shock,” said Jane (38).

“We found it hard to take anything in and were thinking that if he didn’t come through the heart surgery, all this would be inconsequential anyway.” George sailed through his operation to correct a large hole in his heart and a narrowing of the pulmonary artery. But within a few weeks, specialists told Jane and Tim that George would need more surgery.

“When we were told the news about George’s condition and the surgery, I felt like I was in a scene from Casualty, where people are taken into the relatives’ room to be told bad news,” said Jane, who works in advertising.

Doctors gave George six months to recover fully from the heart surgery before they operated again.

“They cut him from ear to ear in a zigzag and shaved away part of one of the plates of his skull to give his brain more room to grow,” said Jane.

George made a rapid recovery and, for 18 months, life returned to normal. Then Jane began to notice problems.

“He became quite physically challenging,” she said.

“He would pick up a chair and throw it and would act aggressively.” They took George for a check-up and doctors put a wire into his head to measure the pressure on his brain.

“They discovered George’s brain was growing too quickly and the skull bones had started to reknit together,” said Jane.

Three months later they were back in hospital for George’s third major operation. It went well and family life has since returned to normal.

“George did have a tough first year at school, but the teachers have been very good with him and he’s fine now,” said Jane.

“He really is our little miracle.” When he was first diagnosed, Jane and Tim were put in contact with the Headlines group, which was formed to offer support and raise money for families of children with craniosynostosis.

“We joined the group because it was comforting to know there were other families going through the same thing as us,” said Jane.

Now the couple are an integral part of the group and Tim has been voted in as chairman. Headlines has about 700 members, publishes a quarterly magazine and holds conferences and fund-raising events. Jane and Tim also run the magazine Headline News.

During the period of George’s hospital treatment, Jane and Tim’s older son, Jack, was a pillar of support to his parents.

“He never once got jealous of the attention George had, even when we had to stay with him in hospital during one of his operations while Jack was at home taking his grammar school entrance exams,” said Jane.

“He’s tremendously proud of his little brother and he’s our hero. He has a natural aptitude with children and would like to train to be a teacher.” The stress of going through what the Prices did could have put a tremendous strain on anything but a rock-solid marriage.

But for Jane and Tim it has had the opposite effect. “All this has only brought us closer together, if that’s possible,” said Jane.

The Arizona Republic (Phoenix, AZ)
August 18, 2004


With dreams of becoming a professional soccer player, Steve Leon, 12 , plays constantly—now that he’s allowed. When Leon was born, he had craniosynostosis, in which the skull’s soft bone hardens too quickly, not allowing proper brain expansion. The condition occurs in one out of 2,000 babies in the United States. The corrective surgery left Leon with a soft spot on his skull, limiting him to sedentary activities, and an S-shaped, bald strip on his scalp. But things are changing for the better. Last year he received a bone transplant, which fixed the soft spot, and in October, he will undergo a hair transplant, follicular unit extraction. The surgery costs approximately $10,000. Because the claim was denied by Leon’s insurance company, and the family does not have the funds to cover the procedure, Leon’s hair transplant surgeon, Dr. Scott F. Alexander, is working with the Craniofacial Foundation of Arizona to cover costs. Biltmore Surgical Hair Restoration will pay for the majority of the remaining balance, according to Alexander.

1. Describe your life now, before hair implant.

Go swimming. Play soccer. When my mom said I could play sports, I started playing sports right away. The first day I played soccer at school I got hit in the face (by a ball). When I played soccer before, I played with friends who I trust. Sometimes when I go to school, people ask me questions like, “What’s on your head?” Sometimes I tell them if they really want to know. Other times I ignore them. Sometimes they make fun of me. It happens a lot every school year. When I’m with my friends, they usually don’t say nothing. I’m with them (friends) a lot.

2. Tell me about when you learned hair transplant surgery was an option for you and how you reacted.

I was happy. I gave my mom and dad a hug. The guy that’s going to do the hair implant, he said it was going to cost a lot of money. I asked him if I was going to have to wear a hat, and I think he said for about three weeks. I’ve been wearing hats for about two years. I have a lot of hats.

3. How did you feel when you still had the soft spot on your head?

I was in the library the whole year (at recess). I did good in school. I got all A’s except for one B. I was kind of mad because I really wanted to play soccer and other sports.

4. How do you expect life to change once you get the hair implant?

I’ll be a lot happier because no one will make fun of me. I’ll still do the same things.

5. Does anything make you nervous about the hair implant surgery?

The pain. My mom said I’ll have to feel the pain, but oh well, I’ll still take it.

6. What comforts you when you think about the pain?

That I’m going to be happy.

Richmond Times Dispatch (Virginia)
August 26, 2004

Surgery Renews a Young Life/ Guyanan Boy Now Talking, Walking After Operation to Correct Skull Deformity
By Tammie Smith

Marcus Semple is taking his first steps. The 13-month-old is holding onto a table at the Ronald McDonald House, where he and his mother, Yonette Joseph, were living temporarily.

Walking is new for Marcus. So is talking.

“He says ‘car’ and ‘no,’” his mother said, laughing.

“He got wiser,” she had said earlier when questioned by Dr. John D. Ward at VCU Medical Center as Ward examined a surgical scar going across Marcus’ scalp from ear to ear.

This month, Ward and plastic surgeon Isaac L. Wornom III led a surgical team that fixed a skull deformity Marcus was born with, culminating months of planning to get the toddler from his home in Guyana in South America to Richmond for the surgery.

If the problem had not been corrected, Marcus could have lost his sight or not developed normally. While there are medical resources in Guyana, Marcus needed surgery that was not available there.

“The good doctor Ward came and he said, ‘Get the child here,’” Joseph said in a thick Caribbean accent. Her son, she added, is definitely more active since the surgery.

“He was asleep a lot” before, she said.

That reason, she believes and doctors confirm, is that the skull deformity was squeezing Marcus’ brain.

The problem was a condition called craniosynostosis. In it, the fibrous joints or sutures of the skull that normally take months to permanently fuse after a child is born instead close much sooner.

When some or all of the sutures fuse prematurely, the head will grow where the sutures are still malleable. In Marcus’ case, his head was long with a protruding forehead.

Besides the odd appearance, there was the danger he could suffer permanent brain damage if the pressure on his brain was not relieved.

In the United States, depending on the number of skull sutures affected, doctors like to correct the condition before a child is 6 months old. Ward said they see about a dozen cases a year at VCU.

At 6 months, Marcus still had not had the surgery.

“We saw a doctor in Trinidad,” Joseph said. Doctors there had the resources to do the surgery, but it would cost $12,000, money she and her husband, Louis Semple, parents to four other children besides Marcus, did not have.

The Guyanan health minister was going to give $5,000, said Joseph, but that left the family to come up with $7,000 plus travel costs.

Marcus’ case made headlines in Guyanan newspapers as the family sought donations, and those stories caught the eye of Dr. Julian Metts, founder of the International Hospital for Children. IHC is a Richmond-based medical mission that focuses its efforts on helping patients with medical needs in Central and South American countries. Metts saw the articles on Marcus in February while in Guyana on one of the organization’s annual medical missions there.

The initial thought was to see if an IHC neurosurgery clinic that Ward was scheduled to do in March in Guyana could accommodate the surgery. That idea was nixed when it was determined Marcus also would need plastic surgery.

Also, because Marcus was older than most children who undergo the surgery, Ward felt the surgery needed to be done in a more controlled environment.

The decision was made to bring Marcus to the United States. IHC’s partner in Guyana, the Georgetown Public Hospital Corp., and Guyana first lady Varshnie Jagdeo helped Joseph get visas, passports and airline tickets, said Rebekah B. Crowther, IHC program director. Crowther worked things from the U.S. end, setting up lodging, arranging a local church sponsor, a youth group at Second Presbyterian Church, and setting up the surgery for Aug. 6 at VCU. IHC negotiates with the hospital on the charges.

In the six-hour procedure, Ward and Wornom removed the toddler’s skull, reconfigured the pieces using absorbable plates, then repositioned the skull and reattached it.

Ward said that over time as Marcus grows, his head will reshape and look more normal. At home, Marcus will be monitored by a newly hired neurosurgeon at the public hospital. Ward will get photos about every six months or so to check the boy’s progress.

“All the fear is gone,” said Joseph, holding her son and looking forward to going home. She has enjoyed her time in the United States but misses her family. Marcus, she said, will be a local celebrity when he gets back.

“This is everybody’s baby,” she said.

San Antonio Express-News
November 21, 2004

Unique Surgery Gives Little Boy New Lease On Life; When their son is diagnosed with craniosynostosis, S.A. parents must decide which type of treatment they should seek.
By Cindy Tumiel

With a blue felt-tipped pen, Dr. David Jimenez drew a series of lines and dashes on the tiny misshapen head of Bishop Cray Uhlrich, propped like a sphinx on the operating room table at University Hospital.

The 12-week-old boy lay unconscious and carefully arranged on beanbags and towels so his shaven, papery scalp pointed toward the operating room ceiling.

Heart monitors beeped and swished as the neurosurgeon sketched several lines—most notably a long dashed one, starting at the fontanel, or soft spot on his skull, crossing the baby’s cowlick and continuing down the backside of his skull.

This, Jimenez said, is a classical case of a fused sagittal suture. The fibrous growth plate beneath the dashed line had hardened too early, and Bishop’s growing brain was distorting his head into a peculiar shape. It was long and skinny, not round. His forehead protruded.

If not corrected, it only would get worse, and the San Antonio infant could suffer a lifetime of headaches, vision problems and the torment of a disfigured face.

Moments earlier, Jimenez had taken a small barber’s razor and gently shaved a patch of hair from where he would cut. Flashing a broad smile, he put the hair into a tiny blue keepsake envelope for the baby’s parents.

“This is going to be the most expensive haircut he’s ever going to have.” Jimenez said.

A Terrifying Diagnosis

Natasha Uhlrich thought her son’s head looked funny even before he was born. Peering at grainy black-and-white ultrasound pictures, the first-time mom saw a boy with a long, narrow skull sharing her womb with a twin sister whose head looked nice and round.

The doctors told her not to worry. Natasha and Brian Uhlrich, practical and low-key professional engineers from Kansas who moved to San Antonio four years ago, did their best to hold their fears in check.

But the outward calm dissolved a few weeks after Bishop and Tatum Uhlrich were born in July. The new parents found themselves in the office of a pediatric neurosurgeon, who put a worrisome new word in their vocabularies. Bishop had something called craniosynostosis.

Newborn babies’ skulls are a set of floating plates, joined at five fibrous seams called sutures—two near the temples, two behind the ears and one along the top of the head. These seams allow the skull to expand in a symmetrical manner as the brain grows rapidly during the first year of life.

Craniosynostosis occurs when one or more of these fibrous seams hardens too early. Then the growing brain takes the path of least resistance, pushing the head into a deformed shape. Depending on which sutures fuse, babies may develop bulging or drooping eyes, protruding foreheads, narrowed or widened skulls. If not corrected, they may suffer vision problems, headaches or cognitive issues as they grow.

The congenital defect is uncommon, but hardly rare. Doctors estimate as many as one in 1,000 babies is born with craniosynostosis.

Bishop’s parents absorbed the news as calmly as they could. But when the doctor began describing the traditional surgery he employed to correct the problem, Natasha Uhlrich’s composure cracked.

First, the scalp is sliced from ear to ear and the skin peeled back. Next, pieces of the skull are cut out and reshaped, then reattached with plates and screws. Babies spend four to six hours in the operating room, often need blood transfusions and spend several days recovering in an intensive care unit.

“Then they showed me some pictures (of the procedure), and that’s when the tissues came out and the tears started,” Natasha Uhlrich said. “I just couldn’t imagine putting him through that.”

There was a surgical team somewhere that was trying a different less-invasive method, the surgeon told her. But he didn’t recommend it; there was danger to major blood vessels beneath the skull during the procedure, and months of helmet therapy would be difficult to implement, the parents were told.

So the Uhlriches went home with their son that day and did what many parents now do when confronted with a medical issue. They turned on their home computer.

A New Approach

Researching her son’s condition, Natasha Uhlrich found the Web site of Jimenez and his wife, plastic surgeon Dr. Constance Barone, who moved to the University of Texas Health Science Center at San Antonio this summer from the University of Missouri–Columbia.

Over the years, the couple had surgically corrected many children with craniosynostosis using the standard operation. But the birth of their own son eight years ago had prompted them to rethink that traumatic approach.

“Being parents, you look at this all totally differently,” Barone said. “You’re a surgeon and a mother. After I had our son, I said there was just no way I would allow somebody to do that drastic a surgery on my kid. I said, ‘David, there just has got to be another answer.’”

What they came up with was a way of using endoscopes—thin tubes fitted with miniature cameras that enabled them to look inside the head without making huge incisions.

They also revived an old surgical concept of removing one larger strip of bone rather cutting and reshaping multiple pieces of the skull. The technique, called a strip craniectomy, largely was abandoned years ago because the skull tended to return to a distorted shape as the bone grew back.

To prevent this, Jimenez and Barone designed a specialized helmet to mold the head into a rounder shape during the months after surgery. They used their own baby son as the model to design the prototype.

Now, parents from around the country and even from Europe find Jimenez and Barone via the Internet.

They’ve done more than 300 such surgeries since then. But their approach is so different that it still hasn’t been widely embraced by other specialists in their field. Cautious and skeptical colleagues say the long-term results remain unproven.

Still Experimental

In their most recent published study, Jimenez and Barone looked at 137 endoscopic surgeries they performed on children with the same closed sagittal suture as Bishop.

The study in the Journal of Neurosurgery Pediatrics reported minimal blood loss and few complications, and said none of the children suffered an injury to the large vein that runs just beneath the cut skull.

Two or more years after the surgery, the doctors said, 87 percent of the children had “excellent” head proportions using a standard index.

Still, other doctors say the endoscopic approach is promising but still unproven.

“Additional results are needed before it can be reported to be as safely and effectively done as current treatments of craniosynostosis,” Dr. John Persing of Yale University Medical School said in an accompanying editorial.

His concerns included the $850 cost of each helmet, which must be custom-molded and replaced two or more times as a baby grows after surgery. Persing also noted there’s no long-term data proving the skull is thick enough when the removed portion grows back.

In any case, there has been no shortage of parents willing to take the chance after they compare the two procedures.

“Our family thinks of this experience as a miracle,” said Loretta Rohlinger of Ninety Six, S.C., who brought son Jacob to University Hospital last month for an annual check-up. “I have the son God meant for me to have, and the best doctors to know to call my friends.”

Three years ago, she camped with her husband, parents and infant son in a Missouri motor home park for three weeks while Jacob, then 10 weeks old, underwent surgery and waited for his custom helmet to be built.

Now 3, Jacob has a normal round head, no memory of that early trauma, and a mother who wants the world to know about the surgeons who changed his life.

“We put a sticker on his helmet that said, ‘Just ask,’” Rohlinger said. “We wanted to tell people about it.”

Reshaping A Skull

On the morning of Oct. 21, Bishop Uhlrich took his turn under Jimenez’s knife.

A nurse called the boy’s parents in the waiting room to let them know the surgery was beginning. Then Jimenez called out the time—it was 9:06—and made his first cut.

With an endoscope in his left hand and surgical knife in his right, Jimenez watched on a monitor as he gently separated Bishop’s scalp from the skull beneath.

Relaxed, his gloved hands quick and confident, Jimenez drilled two small holes into the scalp, then picked up his endoscope again and separated the skull bone from the dura, a protective layer of skin between the brain and the skull.

“How are we doing there?” he asked the anesthesia team. Above his mask, his eyes crinkled in a smile.

With the preliminary work done, Barone stepped in to open the fused suture on Bishop’s head. She made a series of quick, crisp cuts with surgical scissors, first snipping a small wedge to enlarge the fontanel, then cutting a larger rectangle, about 2 by 4 inches, from the bony plate behind it. She finished with four narrow wedges that radiated off the newly opened suture, cuts that would help the head mold back into a proper shape.

The doctors finished their work by cauterizing the cut edges of bone tissue to stop them from bleeding.

Arranged on a cloth-draped tray next to the operating table, the skull fragments so resembled the shape of a turtle that the doctors and nurses paused to remark on it.

In 50 minutes, the entire procedure was over.

Outside in the waiting room, Natasha and Brian Uhlrich sat with Natasha’s mother, Caroline Walrafen, engaging in light-hearted banter over which side of the family was endowed with harder heads. They had held up OK, the baby’s parents said.

“The hardest part was waiting for it to start.” Natasha said. “We went to get some coffee, went to the gift shop, came back, and before we knew it, it was over.”

A New Helmet

Six days after his operation, Bishop was back at the University Hospital clinic for his first post-surgery checkup, smiling broadly at the onlookers around him. A small bandage still covered his incision, the only obvious remnant of the surgery.

His molding helmet had been crafted and delivered to San Antonio in three days, after specialists at Hanger Prosthetics scanned the shape of his head into a computer program and e-mailed the information to a fabrication shop in California. The ventilated clear-plastic helmet was snug against Bishop’s forehead and looser at the sides, gently pressuring his long, thin head to grow into a normal shape.

“We’re wondering what we’re going to say to people; maybe that he’s learning how to ride his bike really early,” Brian Uhlrich joked.

The helmet will be a constant bit of attire until Bishop is about a year old. He’ll wear it to church, to play dates, to outings with his twin sister. His happy parents say they won’t mind if people ask questions or stare.

“I think he still might be cute, even with that helmet,” Natasha Uhlrich said.

Pensacola News Journal
January 15, 2005

As ‘angels’ take flight, child soars to good health
By Sheila Ingram

Aaron Mandel scoots around his Pace home in a walker, exploring the world of color and sounds with his mouth and tiny hands.

He says “Da-Da” and pounds a drum with sticks—just like his father, who plays in a local rock band, Black-eyed Blonde.

Aaron performs the normal tasks expected of an 9-month-old despite a rare condition called craniosynostosis. The disorder results from bones surrounding the soft spot on the top of a baby’s skull closing too quickly, literally squeezing out the brain’s chance to grow and develop.

Aaron’s thriving life so far is nothing short of a miracle, mostly because of the diligent efforts of parents Marilyn and Greg Mandel and medical professionals.

But there’s another reason: Angel Flight Southeast, a nonprofit group, based in Leesburg, that uses volunteer pilots for humanitarian missions.

This week, Aaron wrapped up his 52nd flight with Angel Flight, with one of the group’s 850 pilots flying him to Atlanta for his weekly medical appointment and another flying him back. The pilots donate their time, skills and supplies, and there is no charge for those who use the service.

Aaron’s young life has not been easy, with surgeries, medical treatments and the necessity of wearing a custom-made helmet 23 hours a day to reshape his skull so his brain has room to develop.

But his parents say their spirits are lifted by the number of people who have done their best and given freely of their time to help their son get the best treatment.

“I can’t tell you how many angels have come across our path,” said Marilyn, 26.

If it weren’t for Angel Flight, the Mandels say, they would have been forced to move out of the area to be near their baby’s doctors.

“Angel Flight has been a real blessing,” Marilyn said. “Otherwise, I don’t know what we would have done.”

When Aaron was born last April 15 at Baptist Hospital, the Mandels were ecstatic. Aaron was their firstborn after more than three years of marriage—a tiny boy with beautiful blue eyes and a happy disposition.

But the next day, physicians dropped a bombshell on the young couple.

Aaron was diagnosed with the malady that affects one in 3,000 babies and can cause brain damage and deformity.

“It was sheer terror—sheer terror and panic,” recalled Greg, 25, manager at Emerald Coast Growers in Pace.

Aaron’s mother and father were overwhelmed. Then they immersed themselves in finding out anything and everything about craniosynostosis.

“I was petrified,” Marilyn said. “The initial shock was horrible, but we educated ourselves very quickly.”

Family members on both sides helped in the education process. Marilyn’s mother, Lucy Bujnoch of Houston, was familiar with the disorder because she’s an emergency room nurse. Greg’s mother, Jean Mandel of Pensacola, found Miami-area surgeon Eric Stelnicki, an expert in pediatric craniofacial technology.

Aaron first had surgery to open the top of his skull on July 27 at Joe DiMaggio Children’s Hospital in Miami. He then made several more trips for surgery and follow-up treatment.

The Mandels drove to Miami for the initial surgery. But then Stelnicki told them about Angel Flight.

Now Aaron is making weekly trip to Atlanta for continued treatment, with the family availing itself of Angel Flight’s services.

Angels with airplanes

By day, Sonny Cannon, 53, is an executive with the Georgia Department of Education. But just days before Christmas, he was pilot Sonny Cannon of Newnan, Ga., helping Marilyn and Aaron back from yet another trip to Atlanta for treatment to adjust the baby’s helmet and check his progress.

For his part, Cannon says he is happy to help. His experience as a grandfather of three shows in the care he takes with his smallest charges.

Flying through high wind and rain on one flight just before Christmas, Cannon brought the family safely back to Pensacola Aviation in a Cessna 310. Cannon covered the child to protect him from the rain, carried him inside the small terminal and placed him in his mother’s arms.

“I love to help the little ones,” he said.

Angel Flight pilot Stuart Gibson of Cherokee, Ga., flew Aaron from Pensacola to Atlanta earlier in the day.

“It’s easy when you’re flying somebody to get well, like Aaron,” Gibson said. “I’ve been fortunate in my life, and I love to fly.”

Gibson owns a communications engineering company in Cherokee, which is about 30 miles north of Atlanta.

Sheri Hutchinson, director of operations for Angel Flight Southeast, said Aaron is popular with the volunteer pilots.

“A lot of pilots sign up to fly him right away,” she said. “They say: I’d love to fly this little guy. He’s a cutie, and he’s good in a plane.”

Extraordinary efforts

Pensacola pediatrician Quetheline Helvetius-Lanza, Aaron’s local doctor, says Aaron’s mother may be the most heroic of all in a long list of heroes because of the way she has fought for her son’s welfare.

“It’s always refreshing to see someone who says, ‘I’m getting what I feel is best for my child,’” she said.

Helvetius-Lanza said if Aaron hadn’t had the surgery, he likely wouldn’t be sitting up, babbling or holding and transferring objects from one hand to the other.

The Mandels fought with insurance companies about paying for the specialized care and surgery at the hospital in Miami and then for follow-up treatments in Atlanta. The doctor fought alongside the young couple.

“We fight all the time trying to get insurance companies to do the right thing,” she said.

The Mandels also have high praise for the all medical professionals who have helped Aaron.

The care at the hospital in Miami was superb, Marilyn said. Nurses and staff there treated him as if he were their own.

“There are a lot of good people in this world,” she said.

In addition to his job in Pace, Greg has a second job as a drummer in the rock band on Thursday through Saturday nights at Fudpuckers in Destin. This allows Marilyn to stay home and be available to fly with Aaron every week for doctor visits.

Greg said his wife has strengthened him with her courage.

“I think she did better than I did,” he said of the days immediately after Aaron’s surgery in Miami.

The helmet Aaron wears is dotted with little-boy things—green dinosaurs, hammers and saws and also has his name emblazoned across the front. His head appears normal, with or without the helmet.

Aaron’s doctor in the Miami area told the Mandels last week that he should be able to stop wearing the helmet in about 3 1/2 months—around his birthday.

He’ll have to have checkups about once a year after the helmet comes off.

“The doctor just wants to make sure that his head doesn’t become misshapen again,” Marilyn said. “When the helmet comes off, we’ll have a big ‘Aaron’s helmet graduation party.’”

The Halifax Daily News (Nova Scotia)
March 16, 2005

Donor to pay for girl’s trip to Toronto doctor after Jetsgo’s bailout
By Shaune MacKinlay

A day after telling her tale of Jetsgo woe to The Daily News, a Beaver Bank woman and her disabled seven-year-old learned a welcome lesson yesterday in the kindness of strangers.

An airline employee, who asked to remain anonymous, called a metro radio station yesterday to offer Lori Watkins and little Alison Watkins return tickets to Toronto, where Alison has an appointment next month with the surgeon who operated on her face and head.

“I’m just blown away,” said a much-relieved Lori Watkins yesterday. “Isn’t it amazing? I mean, only in the Maritimes.”

The pair had been planning to fly with Jetsgo until the airline’s surprise collapse Friday left them ticketless.

Alison was born with a condition called craniosynostosis, characterized by the early closure of the joints between the bones of her skull. In July 2002, while living in Ontario, she had major surgery at the Toronto Hospital for Sick Children to relieve pressure on her brain and reconstruct her face.

Their story hit the airwaves on the Griff and Donna morning show on KOOL 96.5 FM yesterday morning. Hosts Griff Henderson and Donna Saker suggested someone or some airline should step in to help Alison.

Within seconds, Henderson said, the call came from an airline employee willing to donate a couple of flight passes.

“He said, ‘I’m flying them,’” Henderson said.

Three more people contacted The Daily News with similar offers of assistance.

Alison’s final follow-up appointment with her surgeon is April 20, after which her case will be managed by the IWK Health Centre.

Medical Devices & Surgical Technology Week
September 4, 2005

Cranial Surgery; System for Robot-Assisted Craniotomy Developed

A novel robotic surgical system may improve outcomes for craniotomy patients.

“In the Special Research Centre 414 of the German Research Funding (DFG, Bonn) a system for robot-assisted cranial surgery was developed” for the “accurate and safe execution of craniotomies and repositioning of bone pieces,” scientists in Germany reported.

“The system is intended for use in the surgical therapy of craniosynostosis,” explained G. Eggers and colleagues at Heidelberg University.

“Preoperatively, CT imaging is performed. In a computerized planning system the position and shape of the intended craniotomy is intuitively planned on a virtual model of the patient’s skull,” according to the report. “Intraoperatively, after conventional removal of the covering soft tissue, the robot performs the craniotomy autonomously.”

“Extensive testing in phantom studies and animal tests confirmed the reliability and accuracy of the system,” the researchers concluded.

Eggers and coauthors published their study in Minimally Invasive Neurosurgery (Robot-assisted craniotomy. Minim Invasive Neurosurg, 2005;48(3):154-8).

For more information, contact G. Eggers, Heidelberg University, Dept. of Oral and Craniomaxillofacial Surgery, Heidelberg, Germany.

Publisher contact information for the journal Minimally Invasive Neurosurgery is: Georg Thieme Verlag kg, Rudigerstr. 14, D-70469 Stuttgart, Germany.

This article was prepared by Medical Devices & Surgical Technology Week editors from staff and other reports. Copyright 2005, Medical Devices & Surgical Technology Week via

The Boston Herald
December 14, 2005

Helping Hand Keeps Young Girl Twirling On Her Toes
By Dawn Witlin

Rachel Montes of Tewksbury, a fifth-grader, lives with a birth defect that doctors at the highest academic level have yet to classify.

“She was born with a cranial facial syndrome. They haven’t classified it, so we always jokingly call it the `Rachel Syndrome,’” explained Rachel’s mother, Gail.

Diagnosed with craniosynostosis, an abnormality caused by improper fusing of the cranial and facial bones in utero, Rachel, 11, has undergone painful surgeries to correct her disorder.

“The skull has various openings, or sutures, in it,” Rachel’s consulting physician, Dr. Murray Feingold, explained. “Some children are born with premature closings of the sutures and the skull grows in the wrong direction; the basic problem was, (Rachel’s) sutures closed too early.”

In 1982, Feingold founded The Genesis Fund in Waltham, a nonprofit that helps coordinate specialized care for Rachel and many other children like her at the National Birth Defects Center.

“They’ve helped us tremendously, being there to make all the appointments for us and guide us as far as what was important medically. Every step of the way they’ve been an invaluable resource,” Gail said.

Rachel has weathered her surgeries so well, Feingold said, because of the support of her family, especially her 17-year-old brother, Jason.

Another healing aid for Rachel, her mother said, is practicing various forms of dance.

“She loves to dance, she’s got some natural rhythm there – unlike her mother. She does ballet – it has helped her with balance issues – and jazz as well.”

Daily Mail (London)
June 20, 2006

Changing Face of a Little Hero
By Tessa Cunningham

WHEN Ollie Cartwright was born with a rare disorder of the face and skull, his mother couldn’t bear to look at him. Here, with searing honesty, Julia Cartwright tells TESSA CUNNINGHAM about the emotional rollercoaster of living with craniosynostosis—and how surgery has transformed his life.

Julia, 41, lives in Northampton with her husband, Mark, 39, an executive chef, and their daughter Ellie, 12. Watching Ollie giggling wickedly as he dive-bombed into the pool of our holiday villa in Spain last month, it was almost impossible to imagine that this was the same baby whose strange appearance I feared meant he would grow up an outcast.

Over the past nine years, doctors have worked miracles on his face. Born with Pfeiffer syndrome, a form of craniosynostosis—a rare disorder which caused the bones in his skull and face to fuse in the womb—Ollie has endured 128 separate procedures under general anaesthetic, including 25 major operations. He’ll need more surgery on his feet and face until his bones stop developing in his late teens.

He has contracted meningitis four times as a side-effect of the surgery, and been in intensive care on at least 12 occasions. He wears hearing aids and is blind in one eye. Yet his spirit is indomitable.

Life will never be easy, and I still run the gauntlet of emotions, from shock to over-protectiveness to denial, as Ollie meets each new hurdle. But he is in mainstream school, and has a cheeky sense of fun and scores of friends. He can also read and write—things we never believed possible.

Trouble began at my routine 22-week scan at Northampton General Hospital. Ollie’s head looked overly large and his eyes were too far apart. They were clues that Ollie had craniosynostosis, but, of course, Mark and I didn’t know that. I don’t think the consultant did either, as he didn’t suggest a second scan. But I demanded a more detailed examination at the Oxford Radcliffe Hospital’s neonatal unit.

At the second scan I was 28 weeks pregnant and the results were devastating. I was told my baby suffered from one of three conditions—craniosynostosis, spina bifida or dwarfism. Mark and I were reeling. It was too late for an abortion, even if we had wanted one. I spent the rest of my pregnancy being physically sick with worry and plagued by nightmares in which I gave birth to a monster.

Ollie arrived four weeks early on October 22, 1997. It was an emergency Caesarean, as he was in the breech position. All I remember is coming around groggily. Ollie—who weighed a hefty 9lb 10oz, again symptomatic of the condition—had been rushed to the special care baby unit. Mark was at my bedside: “It’s a boy,” he said. I didn’t ask any more. Mark told me later that Ollie had stopped breathing while in his arms. It bonded them instantly. But it was many months before I felt the same.

Even the consultant described Ollie as monstrous. He had huge bulging eyes and a beak-like nose. His thumbs were back to front and his abnormally tiny feet looked like bowed bananas.

That night, Ollie was transferred to the Radcliffe Hospital for specialist treatment. We were incredibly lucky to live only 55 miles from the hospital. It’s one of only four centres of craniofacial excellence in Britain.

But I was too frightened to see him. I didn’t receive a single bunch of flowers—just “In Sympathy” cards. It was horrific. Mark and I slept in a room reserved for parents who’ve lost their baby. Pamphlets on funerals were everywhere. My baby was clinging to life, but I was so petrified of the monster to which I had given birth, I couldn’t rejoice.

It was two weeks before Mark persuaded me to see Ollie, who was still on a ventilator. I felt sick. He looked horrific. There wasn’t a flicker of the love I’d felt for our eldest child Ellie—just guilt and denial. “I can never wheel that down the road,” I wailed.

Strangely, Ellie, then three, adored her baby brother from the first. She rushed to his incubator, grabbed his tiny hand and sat there for an hour, eyes shining with love. Looking at this devoted scene, I felt even guiltier and lonelier. By now, doctors had diagnosed Pfeiffer syndrome.

Most babies are born with the bones in their face and skull unjoined, so they can move as the brain develops. The bones fuse during the teenage years. But Pfeiffer syndrome babies have bones that are already fused, with no room for growth. The condition is often hereditary, but in Ollie’s case it was just bad luck. If left untreated, the growing brain will press against the skull, leading to severe brain damage or death. The condition is so rare, affecting about eight births in Britain every year—and every case so different that Ollie’s future was unpredictable.

Some children with the syndrome have gone on to lead normal lives, while others have not survived. All we knew for certain was that he would need endless operations to give his brain room to grow. I remember visiting Radcliffe’s outpatients department. A sea of little faces gazed out from a photo board. These were the “success” stories. Yet they looked horrific.

Ollie underwent his first operation when he was just six weeks old. How could I guess he’d have another 127 procedures, all under general anaesthetic, before his ninth birthday? And on 12 separate occasions he has been rushed to the paediatric intensive care unit. This has added yet more strain. We’ve had to wait up to four hours for a bed—all the while watching as staff battle to keep Ollie alive. Helplessness, panic and distress come in waves.

The first operation was incredibly complex, aimed at releasing the bone at the back of the skull to make space for his brain to grow. The operation didn’t go according to plan—something we’re now used to with Ollie. Doctors had to battle to stop the bleeding and Ollie wasn’t even well enough to come home for Christmas. But I wouldn’t allow myself to worry.

Even when we finally brought him home on December 27, I could barely raise a flicker of interest. Mark couldn’t understand and the gulf between us grew. Now I can’t believe I was so selfish.

That May, Ollie was diagnosed with obstructive sleep apnoea. His condition was hindering his breathing. He spent another three weeks in hospital and came home attached to a machine that would help keep his airways open. When I took him out in his pram once, people stared so rudely I turned around and never went out with him again.

Then came the turning point. It was November 1998 and Ollie, then 13 months old, went into the Radcliffe Hospital to have the front of his head remodelled to protect his eyes and make space for the brain to carry on developing. Ellie had just started school and it was her first Nativity play on the same day as Ollie’s operation. I was determined to be there, but the play overran. Mark and I screeched into the hospital just as Ollie was being wheeled into theatre. Watching his little face as he went, I felt I’d die if I didn’t kiss him goodbye. It had taken a year, but in that instant motherly love kicked in with a vengeance.

We were on tenterhooks. We wandered the streets, every sound seemed muffled. We were cocooned in our own private world of worry. Then, four hours later, Mark’s mobile phone rang. The operation had hit a disaster—the surgeons had cut an artery. Now he was bleeding profusely and they couldn’t wake him.

“I’m afraid he’s very poorly,” the nurse told us. “He is on a ventilator but he is having massive fits.” I remembered how only the day before Ollie had been playing peekaboo with the nurses and holding out his arms to me for a cuddle. How could I lose him now, just when I had fallen in love with him?

It was touch and go, but Ollie pulled through. Finally, four weeks later, he was well enough for surgeons to continue the operation. Once again, he spent Christmas in hospital. But this time we showered him with presents. When he came home on Boxing Day, we spoilt him rotten. By now, life was developing into a routine of operations. The stomach-churning highs and lows that would seem extraordinary to most parents were utterly normal to us.

In 1999, Ollie had a lightweight frame—known as an internal distractor—fitted inside his skull to stretch the bones. Surgeons took his skull apart and left small gaps between the divided parts which his body would fill over time. Three screws either side, drilled into his skull, held the frame. Every day, I held his hand as the surgeon arrived with a screwdriver and gently moved the frame forward by 1mm, to lengthen the bones and allow his face to grow a tiny amount.

Ollie was also fitted with a tracheostomy, as the condition has left his air passages squeezed. This small plastic tube in his neck, allowing him to breathe, stayed for almost a year. That summer the distractor was removed, in preparation for another major operation.

In May 2000, when Ollie was two and a half, he became the youngest child in Britain to have a full titanium frame fitted (at a cost of £10,000). I knew what to expect but even so, seeing my little boy with this contraption bolted into his head was shocking.

Over the next six weeks, consultant plastic surgeon Steve Wall—whom we’ve come to know and love over the years—gradually brought his face forward by gently turning the screws. It was a terribly low point for Ollie, because it was extremely painful. We had hoped that Ollie would bounce back when the frame was removed in August, followed by the tracheostomy in September.

But in October, he became very poorly. Huge, pus-filled sacs appeared all over his head overnight. Terrified, Mark and I bundled him in the car and raced to the Radcliffe. He was rushed into the paediatric intensive care unit where doctors diagnosed a form of meningitis—a side-effect of the complex surgery, which had allowed cerebral spinal fluid to leak down into his nose. Yet again Ollie was back on a ventilator as doctors fought to save him.

He pulled through, but spent another four weeks in hospital. We knew Ollie would need surgery to patch the area where the leak was occurring at the back of his nose. In November 2000, a few days after his third birthday, Ollie went in for a nine-hour operation. By now, operations were commonplace to Ollie. When he came round the first thing he said was: “Can I have a sausage?” Now, after every operation he’s rewarded with a sausage.

Ollie came home on Christmas Eve—his second Christmas at home. And as we toasted the arrival of 2001, we hoped he might be painfree for a while. And that’s the way it went at first. We led a “normal” life—with just one visit to hospital for a minor operation to correct his back-to-front thumbs.

We even took Ollie on his first holiday—two weeks in Greece. But just before his fourth birthday, Ollie developed meningitis again. He was still leaking spinal fluid and needed another operation. This surgery, in January 2002, took 14 hours. At first, things seemed to have gone well. But suddenly Ollie’s lungs collapsed and he had a blinding headache. He pulled through, but spent the next seven weeks in hospital, going into theatre almost 20 times to have fluid drained from his skull. He even contracted meningitis again.

By now we thought we could cope with anything. But then, in July 2002, disaster struck. During one of the complex operations to try to control the flow of spinal fluid, Ollie’s bowel was accidentally cut open. He developed peritonitis and had emergency surgery to remove 30cm of his bowel. But he pulled through and over the next 12 weeks bounced back to his old self.

We were desperately relieved when he finally came home and we could be a family again. We started making plans for him to join Ellie at junior school. On the day he was supposed to start, I took him to hospital to be discharged. As we waited, I noticed he was red hot. When I took off his T-shirt, he collapsed and stopped breathing. He’d suffered an epileptic seizure—yet another side-effect of his condition. It took 24 hours to stop the fit.

Ollie spent the next month in hospital and when he came home, plans for school were abandoned and we decided to take the children to Disneyland Paris. But we didn’t even get inside the gates. Ollie fell over on a puddle of Coke and banged his head. He suffered another fit.

We got home after spending four days in a hospital outside Paris and Ollie was again admitted to the Radcliffe Hospital, leaving a week before Christmas. After suffering a year of absolute hell we were braced for more drama in 2003, but Ollie’s health recovered.

That’s mostly the way it’s been since. He hasn’t had an operation or been ill for more than a year. We know surgery is inevitable as he reaches puberty, but the longer we can lead a normal life, the better.

Ollie’s condition has made him fearless and strong-willed. His spirit leaves me awestruck. Occasionally, he says, “I don’t want a nasty head any more” and I tell him one day he will have the head he wants. Surgeons have already worked miracles, and I know they will carry on doing so.

Bury Free Press
July 14, 2006

Brave Hannah Set for Head Op

A two-year-old girl is to undergo major skull surgery to correct a complex brain condition that went undetected for months.

When Hannah Webster was born, she had a squashed head, was unable to close her right eye—even when she slept—and could hardly open her left eye. Her parents, David and Liz, of Ingham, were told Hannah had an asymmetrical face and were given appointments every three months to check her progress. Although she was soon able to open both eyes, the rest of her features began to twist—her cheekbone has been pressed forward and her eyes are uneven.

Despite these symptoms and Hannah complaining of headaches and being unable to sleep, her condition went unnoticed despite x-rays at West Suffolk Hospital. It was only when the same x-rays were sent to Oxford that she was diagnosed with craniosynostosis - a condition that causes the soft spots in the skull to close prematurely.

“Although they said it was not uncommon for it to go unnoticed, we were told it should have been picked up sooner,” said Mrs Webster. “Had it been, Hannah could have had the operation to correct it when she was seven-months-old—a time when the bones in her head would have been a lot softer. As it was, I was treated like someone who was making a fuss. Yet, if we had known what to look out for, we could have picked it up a lot sooner.”

Hannah, who will be three next month, must undergo a six-hour operation on her skull in eight weeks time to relieve the pressure on her brain and help realign her face. “It’s a major risk, but if we don’t go through with it her face will continue to twist and the inter-cranial pressure would cause all kinds of problems,” added Mrs Webster.

A spokeswoman for the Bury St Edmunds hospital said they could not comment on individual cases, but urged Mr and Mrs Webster to contact them. “When we receive any complaint, we take it very seriously and are anxious that the family get in touch to talk about any complaints they wish to make,” she said.

Family Practice News
August 1, 2006

Maternal Thyroid Disease Possible Risk Factor for Craniosynostosis
By Doug Brunk

Maternal thyroid disease or its treatment may increase the risk of craniosynostosis in offspring by nearly threefold, preliminary results from an ongoing study suggest. The finding is important because thyroid disease is the second most common endocrinopathy, after diabetes, in women of reproductive age, Dr. Sonja A. Rasmussen said at the annual meeting of the Teratology Society.

“Several case reports in the medical literature have linked craniosynostosis to postnatal hyperthyroidism and with maternal Graves’ disease during pregnancy,” said Dr. Rasmussen of the division of birth defects at the Centers for Disease Control and Prevention, Atlanta.

“Congenital hypothyroidism is associated with delayed closure of the fontanelles. In addition, thyroid hormone is known to play a key role in normal bone metabolism, acting on both osteoblasts and osteoclasts. This information suggests that excess thyroid hormone might lead to premature cranial suture fusion,” she said.

To examine the relationship between maternal thyroid disease and craniosynostosis, Dr. Rasmussen and her associates used data from the National Birth Defects Prevention Study, an ongoing population-based case-control study of major birth defects. The data included maternal interviews and clinical information on 4,555 infants born between Oct. 1, 1997, and Dec. 31, 2002, in Arkansas, California, Georgia, Iowa, Massachusetts, New Jersey, New York, and Texas.

Maternal interviews were completed 6-24 months after estimated date of delivery. Infants born with a condition of known etiology, such as a chromosome abnormality or single gene condition, were excluded from the study.Of the 4,555 infants, 433 had craniosynostosis verified by radiographic imaging and 4,122 liveborn infants without major birth defects served as the control group.

Of the mothers of infants with craniosynostosis, 19 (4.4%) had maternal thyroid disease, com pared with 67 (1.6%) of mothers in the control group. Odds ratio analysis revealed that mothers with thyroid disease were 2.8 times more likely to have an infant with craniosynostosis, compared with mothers in the control group.

The association remained the same from a statistical standpoint after the researchers adjusted for several potential confounding factors, including maternal age, education, race, smoking status, use of selective serotonin reuptake inhibitors, body mass index, and preexisting diabetes: infant sex, birth weight, and gestational age; and family history of craniosynostosis. Odd ratios were increased for all types of craniosynostosis excep tfor metopic. The highest odds ratio was for multiple sutures.

“There are several possible mechanisms for the findings we observed,“ said Dr. Rasmussen. First, “mothers with hyperthyroidism may have received inadequate treatment, with passage of excess thyroid hormone across the placenta. Another possible mechanism is that a mother with hypothyroidism received overtreatment with exogenous thyroid hormone. Finally, the mother might have autoimmune thyroid disease that results in production of thyroid-stimulating antibodies that cross the placenta and stimulate the fetal thyroid to make excess thyroid hormone.

“Maternal thyroid disease was based on self-report, so only limited information on the type of thyroid disease was available.”

Dr. Rasmussen also noted that work-ups for genetic causes of craniosynostosis differed among the study sites. Some infants with genetic etiology might have been included.

North Devon Journal
August 24, 2006

Mothers’ Campaign to Raise Awareness of Rare Condition

Two Barnstaple mums are campaigning to raise awareness of a condition which caused skull deformities in their babies.

Ann Marie Morris and Hayley Hedges each have sons with craniosynostosis.When the boys were born, gaps in their skulls which should have closed naturally as they grew had fused prematurely, making their skulls misshapen.

Hayley, 29, said Sam, her first child, was born apparently normally in April 2004. But when he was 10 weeks old she noticed that his head seemed to protrude at the front and back.

A health visitor monitored his head but Hayley became worried. “I noticed his head was a very similar shape to my husband’s, so we thought it could be hereditary,” she said. “I went to the GP and he referred me to Frenchay and they diagnosed sagittal synostosis.” The bone down the middle of Sam’s head had fused too early. His head was growing unevenly, leading to what Hayley called a “boat shape.”

Sam went on the waiting list. Hayley then contacted craniofacial support group Headlines and was put in contact with Ann Marie. By sheer coincidence, Ann Marie, who lives within a mile of Hayley, had also been in touch with the Headlines charity. Headlines was established in 1993 by and for the parents of children with “craniofacial conditions,” or skull deformities.

Sam had a successful operation on his skull at Oxford Radcliffe Hospital—which has craniosynostosis expertise—in July 2005. He seems to have made a full recovery.

The condition was noticed by pure chance in Ann Marie’s son, Jack, who was born in March 2004. Jack was born with exomphalos: his intestines had escaped into his umbilical cord. While at the Bristol Royal Infirmary having that problem solved, a consultant remarked that Jack had “dysmorphic features.” It turned out that Jack had bilateral coronal craniosynostosis—two of the gaps in his skull had fused.

Jack had surgery in Oxford in August 2005. It went well and the next day he was sitting up in bed, asking for a biscuit.

Ann Marie said: “He was fine; he didn’t feel traumatised by the operation.”

Both mothers claimed GPs and consultants were failing to diagnose the condition. They have decided to host their own awareness-raising day.

The condition is rarely life-threatening, but it can have significant consequences. In addition to the social stigma of having a malformed head, craniosynostosis can impair mental development or even cause blindness.

Ann Marie said: “It’s a condition that not many people know about.” The awareness-raising day will be held at Victoria House between 9am and 6pm on Saturday, September 9. There will be a bouncy castle, raffle, and games. For more information call Ann Marie on 01271 328331 or visit

Tampa Tribune (Florida)
August 31, 2006

Second Chances
By Eduardo A. Encina

Devil Rays infielder Kevin Witt doesn’t like to make excuses, but he couldn’t deny that when he first began playing baseball in Japan last season, his mind was somewhere else.

Before he became the International League MVP this season while playing for the Rays’ Triple-A affiliate in Durham, Witt went to Japan to follow his baseball career, but he left his heart in his hometown of Houston.

He was almost half the world away, worried about his ailing young daughter. His only source of comfort: the photos his wife would send him of his daughter in a hospital crib, tubes coming out of her skull, but still smiling.

Just after he had signed to play with the Yokohama BayStars of the Japanese Central League, three weeks before Christmas 2004, Witt found out that his 5-month-old daughter, Skyler, had craniosynostosis, a premature fusing of the bones in the skull of infants that, if untreated, can cause deformity of the growing skull and put dangerous pressure on the developing brain. Craniosynostosis occurs in one out of 2,000 births.

Witt’s only daughter would have to endure a grueling six-hour surgery at Texas Children’s Hospital near the family’s home in Houston. Surgeons would have to cut open her head from ear to ear, reset her skull and place a metal plate and 30 screws in her head.

Making matters worse, the surgery had to be done in March 2005, when Skyler was nearly 9 months old and her bones weren’t so soft. That’s when Kevin was supposed to be starting his season in Yokohama.

“When we first found out, it was like they dropped a bomb on us,” Witt said of the reaction of he and his wife, Lori. “We had never heard of it.”

The Witts decided that Kevin had to honor his contract, because if there’s one thing about this journeyman ballplayer, it is that he doesn’t like the thought of giving up. And he takes pride in his job. He is the same player who - disturbed by the way life in Durham was depicted in a recent USA Today article—wrote a letter to the paper defending the organization.

“I didn’t expect or want him to stay,” Lori said. “But I knew he wasn’t going to back out. He’s a very loyal person.”

Alone and Far Away

Being a 15-hour plane ride away from his family was difficult in itself, let alone being away during a tough time. Witt, who had hit 36 homers the year before with the Cardinals’ Triple-A team in Memphis, was distracted. He felt like an outsider in Japan. He struggled getting used to the deliberate deliveries of the pitchers. He didn’t have many friends because he couldn’t speak Japanese. He caught the Japanese flu on Opening Day and found himself hooked up to an IV and missing the first three days of the season. So he struggled, yet he never told anybody on the team—minus his interpreter—what was going on back home.

“I don’t want to make excuses,” Witt said, “but it’s kind of hard to block that out when you’re so far away. That was the tough part, waiting for the surgery and the thinking about things in all the time up to it.”

Then came March 11, the date of Skyler’s surgery. It was scheduled for 7 a.m. in Houston, but that was 3 a.m. the day before in Japan. After the surgery, Lori rushed to the nearest pay phone in the hospital to call Kevin to tell him the operation was a success. He sounded tired, but relieved.

Lori would e-mail photos of Skyler’s recovery. Some might have been unsettling to any father. Two tubes came out of the side of her head from behind her ears to drain the excess blood and reduce the swelling. But the swelling was still there, so much so that her eyes were nearly swollen shut. Her weight had dropped to 15 pounds.

“I know he was stressed out mentally,” Lori said. “All he had to connect him was the photos and the phone calls. There he is, in another country when his daughter is having her head cut open from ear to ear. I thought I had to be strong for him because he could tell in my voice if I was worried, and that would worry him. I would e-mail him photos of Skyler, but I was careful to send the best ones. People say I had the tough part, but I think he had the toughest part of it all.”

An Adventure, An Inspiration

Regardless, seeing his daughter was an inspiration.

“Two days after surgery, she was standing up in her crib with tubes coming out of her head … with a smile on her face,” Witt said. “It was a rough time, but she came through like a trooper.”

During that time, Witt hit .172 in 64 at-bats at Yokohama before he was sent down to the team’s farm club, the Shanon Searex in nearby Yokosuka. That’s where he began honing his swing, helped by the minor-league batting instructor there, a man who didn’t speak English. Still, the minors were an adjustment. Sometimes they’d play five games a week, other times only three.

The photos kept coming, most of them with young Skyler smiling. Lori would dress her in hats and loose headbands to cover the zig-zag scar across her head. For three months she’d have to wear a white helmet to help keep her healing skull in place. Shortly after, the entire family—Lori and sons Preston and Tanner (the Witts now also have a 6-month-old son, Cullen) joined Witt in Japan. In one photo, Skyler is wearing a T-shirt with the word “adventure” written in Japanese on the front.

That’s exactly what they’d all been through. Slowly, Kevin regained his swing and ended the season hitting .330 with the Searex, prompting the Devil Rays to sign him to a minor-league contract with an invitation to spring training.

Now, he has one more chance at a big-league career. After leading the minor leagues with 36 homers and setting a Durham and organizational record for most homers in a season, the Devil Rays—Witt’s sixth organization—called him up from Durham on Thursday. He made his first big-league start since 2003 on Friday in Baltimore and hit his first home run in the majors in more than three years. In three games with the Rays entering Wednesday, Witt batted 308. And now there are more photos, now with Skyler and her father together. Her blond head of hair has grown back fully, the smile is now even brighter. On June 29 she celebrated her second birthday with a pizza party in Durham.

“She’s his only daughter,“ Lori said. “So the relationship between a father and his daughter is totally different. He’s such a big guy, but he’s such a softy with her. He’ll let her get away with murder.”

For Witt, he’s much happier being close to home, but he hasn’t closed the door on a return to Japan later in his career. He was happy in Durham, but obviously is relishing his late-season call-up to the Devil Rays, and happy to be back with his family. “In order to be successful,” he said, “I think you have to go through the hard times, too.”

The Dominion Post (Wellington, New Zealand)
September 6, 2006

Pressure’s Off Olivia

Wellington surgeons have taken apart the skull of an eight-year-old girl and carefully meshed it back together to relieve pressure on her brain.

Olivia Mayo, of Palmerston North, is now looking forward to a normal life without headaches. As she cuddles her teddybears Squeak and No-Name, the only signs of what she has been through are the bruises around her eyes and a stapled incision across her head, which is mostly hidden by her hair. Those long locks are also what disguised Olivia’s rare medical condition and, if her parents had not acted on their concerns, it could have caused brain damage.

Olivia had developed sagittal craniosynostosis, where the seam that runs from the front to the back of the skull fuses together and cannot expand. Her growing brain was being squeezed so tightly inside her skull that the pressure left hammer-like indentations on the inside of the bone. Plastic surgeon Charles Davis and neurosurgeon Agadha Wickremesekera operated on Olivia last Wednesday.

Her parents, Elton and Kirstie Mayo, are relieved to see their daughter smiling.

“You start thinking about the what-ifs, but we’re on the right side of it now,” Mr Mayo said.

Mrs Mayo had asked a GP about Olivia’s headaches and her unusual head shape but was told it was nothing to worry about. Not convinced, she asked for a CT scan at Palmerston North Hospital.

Olivia said she was a little frightened about having an operation. “I wasn’t scared out of my head, but I was still a bit scared.”

She is now back at home with her family and, apart from having to be careful not to get a knock on the head, she is getting into a normal routine, including returning to school as early as next week.

The Olympian (Olympia, Washington)
September 17, 2006

Saving baby EmmaLee; Surgery to reshape a little girl’s skull proves a rough but rewarding journey for Olympia family
By Lisa Pemberton

A perfectly healthy child—that’s the dream for most expectant parents. But an ultrasound during Katie Guggenmos’s seventh month of pregnancy revealed her unborn daughter had a problem: Her skull wasn’t round—it was shaped more like a clover, caused by a deformity known as craniosynostosis.

“We’re all kind of holding our breath until she’s born,” Guggenmos said in late April, about a month before her due date. “We’re just waiting to meet her.”

About one in every 2,500 children are born with the condition, which occurs when some bones in the skull calcify before the brain is finished growing. EmmaLee’s case was different because it’s extremely rare to have a prenatal diagnosis, according to Dr. Michael Cunningham, head of the Craniofacial Center at Children’s Hospital in Seattle.

A radiologist at Northwest Radiology in Olympia had spotted the deformity during a routine ultrasound. Craniosynostosis usually is diagnosed at birth, or within the first week of life. The early diagnosis allowed Guggenmos to make arrangements for a high-risk delivery at the University of Washington Medical Center, where doctors could be nearby in case there were complications. It also allowed Guggenmos, 20, an Olympia High School graduate, to go online and find out more information about her baby’s condition.

“The things online were not good—there were babies with horribly deformed faces,” she said. Some were deaf and blind, or both. Some had severe mental disabilities. Doctors tried to assure Guggenmos that they could surgically correct the deformity when EmmaLee was a few months old, and that most children with craniosynostosis turn out just fine. But Guggenmos couldn’t help but wonder about those worst-case scenarios.

Finding Support

Meanwhile, grandma-to-be Sandra Kozlowski went into fundraising mode. She knew some of what was ahead because her son, Joshua, Guggenmos’s younger brother, had had open heart surgery at Children’s Hospital. There were so many things that insurance didn’t cover; and it was expensive to stay and eat in Seattle during her son’s hospital stay, Kozlowski said.

Workers at Audrey’s Elements of Style in Olympia learned about EmmaLee's condition, and offered to conduct a Cut-A-Thon on May 4 to help raise money for uncompensated expenses that would go along with the surgery, recovery and possible physical therapy. “We want to get Katie as much cushion as she can so all she has to do is focus on EmmaLee,” Kozlowski said at the time.

Stepping Up

Two days before the event, Guggenmos went to the University of Washington Medical Center for a check-up. The baby was fully developed, but the obstetrician said that EmmaLee hadn’t grown since the last checkup. “She was like, ‘Do you want to have the baby tonight?’” Guggenmos said. “I was like, ‘Yes!’”

EmmaLee’s dad, Aric Wood, 21, of Olympia, drove up for the birth. He and Guggenmos had broken up before she told him she was 10 weeks pregnant. And Wood wasn’t happy to hear news of the pregnancy. “I was like, ‘I can’t do this—there’s no way’” he recalled. “I was being very selfish.”

But learning that his daughter would be born with a birth defect changed him. “I think he saw how much it scared me, and how much she already meant to me, and I think he really got it at that point,” Guggenmos said.

At 3:47 p.m. on May 3, EmmaLee arrived four weeks premature, weighing only 4 pounds, 6 ounces. Kozlowski and Wood were both in the delivery room. As the doctor placed the slippery newborn on Guggenmos's chest, Wood searched for signs of deformity, but he couldn’t see anything wrong.

“He said, ‘That’s a baby—she’s beautiful,’” Kozlowski said. “It was great. We saw a dad being born at that moment.”

Long Days, Loud Screams

Adjusting to life with a newborn can be tough. But EmmaLee’s condition made it even harder. She was sweet, and learned to smile, babble and roll over much earlier than most babies. But she also was in a great amount of pain, her family said. EmmaLee’s screams were 10 times worse than colic, Kozlowski said.

“There are days where she just cries all day long,” Guggenmos said. "Because she’s such a good-natured baby, we know it has to be the cranial pressure.”

Sometimes infant Tylenol helped. But it usually didn’t. By the time she was 2 months old, EmmaLee's head had indentations at each temple. Her forehead protuded. “Her head looks kind of like a peanut,” Guggenmos said. “It is cute. I think it’s cute.”

EmmaLee’s first CAT scan was on July 5. She slept through it. “It was weird, I cried when they took her, but there was no reason for it,” Guggenmos said. “It was just like, ‘Oh, wow, this is really going to happen. She’s really going to get surgery.’”

The surgery was scheduled for Aug. 10, and as that date drew near, the stress level rose in EmmaLee’s household. Travel and hotel arrangements had to be made. Time had to be taken off from work. And EmmaLee was still having what the family began referring to as “fussy days.”

“All of our fuses are really short,” said Wood, a cook at Plenty, a downtown Olympia restaurant, a few days before the surgery.

Preparing for Surgery

By now, the family was extremely attached to EmmaLee and her developing personality. They worried surgery might change more than the shape of her head. But when they arrived at the hospital, they learned that the surgery needed to be rescheduled because it was a busier-than-usual day at the Children’s Hospital intensive care unit.

The family took the news hard. “I was so angry, and it took everything in me not to yell at somebody,” Kozlowski said. “We were already maxed out on stress, we all took time off of work and it was expensive,” Guggenmos said. “It was like a wasted trip.”

A few days later, the surgery was re scheduled for Aug. 21. This time, there was low-cost room for the family at the Ronald McDonald House, which is within walking distance of the hospital. “I just kind of want to get it done because it’s something that needs to be over now,” Guggenmos said. “We want to move on. ... We don’t want to wonder anymore if she’s in pain because of her head. It’s time for it to be over.”

EmmaLee’s hair was partially shaved that morning—it was her first haircut. “They saved the hair and gave it to us,” Guggenmos said. “Of course, we’re going to put it in a baby book.”

The surgery and recovery time took five to six hours. “I’ve never spent that long away from her,” Guggenmos said.

Guggenmos and Wood said they were nervous. They took a few walks. They held each other on the couches in the waiting room.

Shaping the Skull

During the surgery, the doctors made a zig-zag incision in the scalp, from ear to ear. That way, her scar will be better hidden by her hair and won’t be as easy to see when she is older, according to Dr. Richard Hopper, one of the surgeons who performed EmmaLee’s operation.

“The neurosurgery team then did a craniectomy, or removal of the bone of the fused skull suture from the underlying tough coating of the brain,” he said. "Doctors made a series of complex cuts through the skull, and gently bent and curved the bone using special instruments.”

On the sides of EmmaLee’s head, finger-like pieces of bone were opened up like flower petals to widen her head, he said. Next, the back of her head was carefully pulled forward to flatten it.

A craniectomy is a common procedure for youngsters with craniosynostosis, according to Dr. Monica Wehby, with Microneurosurgical Consultants in Portland, who is a spokeswoman for the American Association of Neurological Surgeons. The cuts help make space in the skull for the brain.

“It’s like a water balloon—if you’re squishing the sides up, as soon as you release the pressure, the balloon falls back into its normal shape,” Wehby said.

“She looked like an alien.

Back in intensive care, EmmaLee recovered with an IV in each hand and a tube in her head to drain blood. She was on morphine and slept that night away from her mother, the first time she didn’t have Guggenmos by her side in a bed. During the next two days, her face swelled so much, her eyes couldn’t open—a normal reaction to the surgery.“She looked like an alien,” Guggenmos said. EmmaLee’s recovery went well, and she was sent home in just a few days.

By last week, nearly all 150 or so stitches in EmmaLee's head had dissolved. Her head already had begun to take a new, round shape. She clasped her hands together—something she does when she's excited about something—when she saw her grandma walk in the door.

“She’s such a good baby,” Guggenmos said. “She’s such a blessing.”

What is craniosynostosis?

Craniosynostosis is the early fusion of one or more of the sutures—specialized joints between skull bones that enable expansion of the skull during normal brain growth—between the bony plates of the skull. When the sutures fuse too early, the skull can’t grow normally.

There are different types of craniosynostosis. EmmaLee Wood’s condition is sagittal synostosis. The bones on the back of her skull fused together in utero, resulting in a sharp ridge in the back of her head. What makes her case unusual is that it was identified prenatally, said Dr. Michael Cunningham, head of the Craniofacial Center at Children’s Hospital in Seattle. Babies usually aren’t diagnosed until birth or within the first few weeks of life, but a radiologist in Olympia recognized the condition in EmmaLee during a routine ultrasound, and alerted the family and doctors.

It’s unknown why some children have craniosynostosis. Most children with craniosynostosis have only one suture affected and do not have anything else wrong with them. Sometimes, the deformity occurs as part of a genetic syndrome.

Though an exact rate is unknown, it’s believed craniosynostosis is diagnosed in 1 out of every 2,500 children, Cunningham said. Surgery to reshape the skull is the most common treatment. “It’s not a cosmetic thing. You are correcting a deformity by doing it,” said Dr. Monica Wehby with Microneurosurgical Consultants in Portland, a spokeswoman for the American Association of Neurological Surgeons. “There’s also risk to the brain if you don’t because the brain needs space to grow and if it’s restricted, there could be increased pressure.”

Law & Health Weekly
September 30, 2006

Improved treatment option gives surgeons greater control in reshaping babies’ heads

One child in 2,000 in the United States has a misshapen head resulting from a birth defect in which the bones of the skull prematurely close. The condition, called craniosynostosis, can constrict growth of the child’s brain and lead to vision problems.

An improved treatment option at Morgan Stanley Children’s Hospital of NewYork-Presbyterian gives surgeons greater control in reshaping the skull. Traditionally, craniosynostosis was treated using metal screws and plates. Now, pediatric plastic surgeons use absorbable plates made of a combination of polyglycolic and polylactic acid that are absorbed by the body much like surgical sutures. These plates can be placed wherever is necessary—including the forehead bone. In contrast, surgeons traditionally avoided placing metal plates in the forehead because they would be palpable, and could cause contour irregularities. Another advantage is that absorbable plates do not interfere with x-rays as do metal plates and screws.

Surgery for craniosynostosis is usually performed in the first six months of the child’s life for several reasons, including the fact that the bones of the skull are easier to work with at this age. Moreover, the growing brain can continue to reshape the skull and face after the surgery.

“Deformities like a misshapen head can be very traumatic for parents,” said Dr. Jeffrey Ascherman, director of the Division of Plastic Surgery and director of the Cleft/Craniofacial Center at Morgan Stanley Children’s Hospital of New York-Presbyterian and associate professor of clinical surgery at Columbia University College of Physicians and Surgeons. “It is important for the child’s pediatrician to detect the condition early and for parents to seek the best specialized care for their child.”

Diagnosis of craniosynostosis is done by a pediatric neurosurgeon and plastic surgeon. The causes of craniosynostosis are usually not known. It occurs in many syndromes, including Apert’s, Crouzon’s and Pfeiffer’s syndromes. However, a distorted head at birth does not always result from craniosynostosis and may require other non-surgical interventions.

Bury Free Press
November 30, 2006

Toddler home for Christmas

The parents of a three-year-old who underwent major surgery on her skull have spoken of their relief at bringing her home. Hannah Webster, from Priory Close, Ingham, has been treated by specialists in Oxford to repair damage caused by craniosynostosis.The condition means gaps in an infant’s skull, which should allow the brain to grow, close up before growth is complete.

After spending just over a week recovering in hospital at the Radcliffe Infirmary, the youngster was able to go home last Wednesday. Mum Liz Webster said Hannah was doing well, although it was not yet known whether she would need more surgery in the future.

“She’s got wires inside her head holding everything together at the moment and she may need to have those taken out later," said Mrs Webster. “She’s been amazing. She’s doing really well and she's back to being her bouncy self again,” she said. “Her head does look a bit big, because she still has some swelling, and to begin with she couldn't see because her eyes were swollen—but she looks good now.”

Hannah was taken into the operating theatre at 8am on Monday, November 6, and surgeons worked for nine hours to re-open the gaps in her skull. “It was very worrying and the night before was awful,” Mrs Webster said. “But she had the best surgeons in the country and I trusted them.”

Hannah was diagnosed with the condition in May and will now be followed up regularly until she is 16. And, in the short term, she has to be careful—avoiding slides, swings and riding her bike to safeguard what she has proudly been calling “my new head.”

To help the family, Hannah’s playgroup, the Fornham St Martin Mother and Toddler Group, raised £1,200 with a dance and raffle organised by Rosemary Chenery and Lesley Curry on Saturday, October 28 at Bury Town FC. The cash helped pay for the family's travel and accommodation while Hannah was in hospital and will continue to help in the future.