Oakland Tribune article on Craniosynostosis

Oakland Tribune
November 19, 2007
Sandy Kleffman, STAFF WRITER

OAKLAND — A tiny cry pierces the recovery room at Children's Hospital Oakland.Little Kacin Rogne, his head wrapped in gauze and a light blue pacifier in his mouth, is waking after 21/2 hours of skull surgery.

Just 11 weeks old, Kacin looks lost in the big white crib. A nurse gives him pain medication and rushes him into his mother's arms, tubes and all.

"Hey, sweetheart," she says softly.

Kacin has just undergone a novel, minimally invasive surgery to correct his prematurely fused skull.

The members of the operating team — neurosurgeon Peter Sun and plastic surgeon Bryant Toth — are among a handful of people in Northern California who do the procedure.

They and other experts say they believe the technique will gain greater acceptance as an option over the standard surgery as more people become familiar with it and obtain the needed expertise.

"There's less blood loss, less time in the hospital, a shorter recovery time," Toth said.

If left untreated, Kacin's condition, known as craniosynostosis, can permanently misshape his head.

The premature fusion prevents the skull from expanding as it should during a baby's rapid brain growth. That can lead to increased pressure and brain damage. In rare cases, it can be fatal.

Craniosynostosis affects one in 2,000 infants.

The traditional surgery, which has been performed for decades, is a much bigger operation, with a five- to seven-day hospital stay and an increased chance that a blood transfusion will be needed. Parts of the skullare removed, reshaped and placed back on the child's brain.

"We make a big incision," Sun said. "The child is more uncomfortable. There's more swelling. He just needs to be in the hospital a lot longer."

By contrast, the surgeons performed Kacin's entire operation through two 3-centimeter-long incisions in his head.

He will be released from the hospital in two to three days and will wear a custom-made helmet for several months to help reshape his head.

The technique was pioneered eight years ago by a neurosurgeon and plastic surgeon team — David Jimenez and Constance Barone, now at University Hospital in San Antonio.

Sun and Toth perform six to 10 of the minimally invasive surgeries a year and about 40 of the traditional operations. University of California, San Francisco, Children's Hospital also offers both procedures.

Lucile Packard Children's Hospital at Stanford performs only the traditional operation. It remains unclear whether the minimally invasive procedure is better, said Dr. David Kahn, a craniofacial plastic surgeon at Stanford.

Sun and Toth are convinced of its benefits, but they note that it is not for everyone. For one thing, it must be done at an early age. They use the technique only on children who are 3 months or younger because the skull bones are thin enough to cut easily.

That means pediatricians and parents must catch the abnormal head shape early, get a confirming X-ray or CT scan and schedule the surgery, all within the required time frame.

"Three months goes by pretty fast," Sun said.

The more extensive surgery typically is not performed until a child reaches 6 months of age or older.

Most babies are born with six suture lines in their skulls to give the brain room to grow. Several of the sutures intersect in the large soft spot on the top of the head. The bones typically do not fuse until the child reaches age 2.

Pleasanton resident Jill Van Slyke's pediatrician first noticed an abnormal head shape in her son Matthew when he was 2 to 3 weeks old. She suggested his parents position him differently as he slept.

But when they brought him back a week later, his head was becoming too long and skinny.

X-rays revealed that Matthew had a fusion of the sagittal suture, which runs from the front to the back of the head. It is the most common type of skull fusion, preventing a baby's head from growing sideways. So it pushes in the other direction, becoming long and narrow, often with a bulging forehead.

The Van Slykes' pediatrician suggested they talk to Sun. They opted for the minimally invasive surgery and have been happy with the outcome.

"We wanted the surgery to be easier on him," Van Slyke said. "Also, being able to fix the problem sooner seemed better."

The minimally invasive technique is particularly well-suited for babies in which the sagittal suture is fused, Sun and Toth say.

They prefer to use the bigger, traditional surgery for infants with other types of skull fusions.

Chris and Nicole Rogne noticed shortly after Kacin was born that he had a bump on the back of his head, but they assumed it was because he had to be suctioned out at birth.

At his two-month checkup, Kacin's pediatrician ordered a CT scan, which revealed the problem — fusion of the sagittal suture.

Because their pediatrician was familiar with Sun, the Nevada couple decided to bring Kacin to Children's Hospital Oakland for the operation.

On the morning of the surgery, they waited nervously in the hospital cafeteria with other relatives.

Both the minimally invasive and more extensive procedure are considered relatively safe, but that does not make it easier on parents.

"This is our little guy," Nicole Rogne said.

The surgery was delayed as the anesthesiologist struggled to find a vein in Kacin's tiny body.

Once the anesthesia took effect, the operating team placed the infant sphinxlike on a beanbag to hold him in place.

When Toth shaved his head in what amounted to his first haircut — a milestone in a baby's life — pediatric nurse practitioner Sue Ditmyer collected the black tufts in a bag for the parents. That has become a tradition at the hospital.

Sun and Toth began the operation by making two small incisions in Kacin's head. With the aid of a lighted tube known as an endoscope, they separated the dura mater, a brain-covering membrane, from the skull to avoid damaging it.

Then they cut the prematurely fused skull with curved scissors and removed portions of it, enabling it to "open like a clam shell," as Sun described it. Everything was done through the two small incisions.

After about 21/2 hours, Sun and Toth sewed up the cuts and wrapped Kacin's head in gauze.

Members of the operating team rolled him into the recovery room, where his mother took him into her arms.

Once the swelling is gone in a couple of weeks, he will be fitted for a custom-made helmet. It applies light pressure on the front and back of the skull, causing it to grow sideways into a normal head shape.

Most children wear the helmet for three to four months, Sun said.

Matthew Van Slyke is recovering in his Pleasanton home after his Aug. 22 surgery, colorful stickers dotting his helmet.

His wears it constantly except for the hour a day when it is removed for cleaning. He struggles a bit when they put it on, but then he seems to forget about it, his mother said.

The Van Slykes have been pleased with their decision to go with the minimally invasive procedure.

They initially took Matthew to Stanford but then sought out Sun.

"His head is already a normal head shape," Jill Van Slyke said, removing the helmet to reveal the rounded noggin topped with strawberry-blond hair.

Lucile Packard Children's Hospital does not perform the minimally invasive procedure, in part because its physicians are not convinced that it is a better technique, Kahn said.

He noted that the results depend on how conscientious a family is at keeping the helmet on the baby's head properly. With the traditional surgery, the correction is made immediately and there is no need for a helmet.

The minimally invasive surgery is "certainly another way of doing it, and it gives good results," Kahn said. "The question is, are those results better than the traditional way of doing it?"

UC San Francisco Children's Hospital offers both surgeries but tends to favor the more traditional procedure, in part because its long-term results are well-known, said Dr. Nalin Gupta, chief of the division of pediatric neurosurgery.

"We're conservative in that we have very good results with it," he said. "We've done several hundred patients over the last several years and we have long-term follow-up. The complication rate is very low, and the outcome is very good."

Among the 40 to 50 surgeries UC San Francisco does each year for prematurely fused skulls, about five are the minimally invasive type.

Gupta said physicians there are monitoring the results of the newer procedure.

"I think in the future there's going to be more and more of those done because there are some very compelling advantages to it," he said. "But the one disadvantage is that the long-term results aren't really well understood.

"You gain a sense of the long-term outcomes over time. That's the one caveat that I always tell families. It's a new thing."

Sun and Toth are convinced of the benefits and hope to get the word out to pediatricians and parents to catch the problem as soon as possible to preserve the minimally invasive option.

"If it's a little bit earlier," Sun said, "a smaller operation can be done to get it resolved."

Reach Sandy Kleffman at 925-943-8249 or skleffman@bayareanewsgroup.com.

(c) 2007 The Oakland Tribune. All rights reserved. Reproduced with the permission of Media NewsGroup, Inc. by NewsBank, Inc.