When their son is diagnosed with craniosynostosis, S.A. parents must decide which type of treatment they should seek
by Cindy Tumiel
San Antonio Express-News
(San Antonio - November 21, 2004) 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 UT 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.
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Unique surgery gives little boy new lease on life
