The Times
February 22, 1996

I risked our baby’s life for the sake of her looks
By Halina Kierkue

A delicate skull operation has had bad publicity after some children were misdiagnosed. But Halina Kierkue is sure she made the right decision for her daughter.

My twin daughters were about six weeks old when I began to suspect something was wrong. I remember the day, even the second, a couple of weeks later that I knew for sure. An old schoolfriend had dropped in for tea. As she gazed down at them lying side by side, I watched her smile evaporate.

I knew what was coming. “Georgia’s getting a really odd-shaped forehead, isn’t she?” I started cracking feeble jokes about my Eastern European ancestry and our dashing Slavonic browlines. But my stomach turned. For the past few weeks, I had watched silently as my baby’s head had begun to bulge, almost imperceptibly at first, around the temples. At this stage, I might not have noticed at all had it not been for her almost identical twin Clementine, whose head was, by now, an entirely different shape.

Georgia was suffering from a rare skull condition, craniosynostosis. Around 250 children are born each year in the UK with one or more of the network of tiny “expansion” joints which crisscross their heads prematurely fused. It can be hereditary. More often, as in our case, its causes are a complete mystery. When these babies’ brains begin to grow, their skulls are pushed into increasingly odd shapes to compensate for the lack of “give” along the sealed-up seams. As Barry Jones, the director of Great Ormond Street Hospital’s cranio-facial unit, explains: “It is like blowing up a balloon constricted by an elastic band. As the pressure inside increases, the balloon bulges or flattens on either side of the constriction.” At worst, craniosynostosis can cause brain damage. Untreated, it will certainly cause disfigurement, sometimes of both the skull and the face.

Many parents who read last weekend’s reports about the children in Leeds who were misdiagnosed as suffering from this condition, and subsequently underwent painful surgery, may now hesitate to take action if they suspect their children have similar problems. My advice, based on our experience, is that if you have the slightest doubt, you should seek help immediately from one of the three specialist units in the UK which are equipped to deal with the problem. Go anywhere else and you are unlikely to get the treatment your child desperately needs. Mr Jones says: “To treat this condition properly requires a number of specialists from different disciplines working together as a team. Without that very particular combination of expertise, a child is unlikely to be diagnosed or treated appropriately.”

This is what happened to my family. Despite my suspicions, it was many weeks before Georgia’s condition was diagnosed a few months longer and she might have been permanently deformed. At first I kept my fears quiet. The girls had been born two months prematurely at St Thomas’ Hospital in London and had only just come home after five weeks in a special-care baby unit. If something had been wrong, I rationalised, surely the teams of paediatricians at the hospital would have spotted it?

I comforted myself with the thought that no one except me and one friend had sensed anything was wrong neither health visitors, GPs nor my own family had noticed anything amiss and I was only too happy to put my worries down to a new mother’s paranoia. A few weeks later we returned to St Thomas’ outpatients for the twins’ routine check-up. A nurse came to weigh them, and measured Georgia’s head circumference several times, before disappearing abruptly. By the time we got into the paediatrician’s office, I was panicking. He examined her skull immediately, but seemed unperturbed. “She won’t grow out of it,” he said, “but when she’s a teenager she can wear her fringe long.” He reassured us there was nothing seriously wrong.

Over the next month, Georgia’s features began to change. As the top of her head elongated, the whole shape of her face altered. Something was going horribly wrong, though we could hardly bear to think about it. Much less did we know what to do in the face of so much “expert” opinion that suggested there was nothing to worry about.

We were lucky. Georgia was born with a large birthmark on her neck which could be treated. I made an appointment with a plastic surgeon who specialised in laser treatment. The first thing he said when he saw her was: “What are you doing about her head?” I froze. He immediately told me I should forget about her neck and concentrate on her skull. He knew of another plastic surgeon in London who specialised in her sort of “problem.”

Georgia and I saw Barry Jones at Great Ormond Street a few days later. The decision that confronted us was stark. Georgia’s particular form of craniosynostosis was unlikely to cause brain damage, and the chance of developmental problems for her was about 13 per cent. But she would, inevitably, be deformed without surgery.

The operation was a major one, though, and needed to be performed quickly while her skull was still soft. It is an even more difficult procedure for older children. Working in tandem with a neurosurgeon, Mr Jones would have to peel away part of my daughter’s scalp, then remove and remodel the bones of the front of her skull to give her brain the chance to grow into a normal shape. This delicate “carpentry” takes place directly on top of the membranes which cover the brain itself. There were obvious risks. I will never forget the next 24 hours, agonising over whether to risk our baby’s life for the sake of the way she would look. Many of our friends advised against it, but watching her with her twin sister sitting beside her the same, but so perfectly different I knew, for Georgia’s sake, that I had no choice.

At Great Ormond Street, we had been shown the pictures of other children who had come out of the surgery successfully. They were, without exception, so bruised and battered they were barely recognisable. Yet within days they had begun to recover. We asked to wait until after Christmas to have the surgery. On the morning of her six-month “half birthday,” January 21, 1993, she was admitted to hospital. The next day, she was wheeled away for the four-hour operation. My husband and I walked the streets.

When we saw her again she was in intensive care, still asleep, and with a drain protruding from a thin gauze cap on her head. I had steeled myself for the very worst, but miraculously, she was completely unbruised and remained that way. Even more miraculously, her head and face were already a different shape, and continued to improve over the following months as she “grew into” the surgery. Within four days, Georgia was back at home with us, her scalp stapled together with metal clips which were removed in outpatients a week later. Of course, we will never be sure what trauma she suffered or how much pain she felt. But there are no signs, three years later, that she has been affected in the long-term.

Her skull is a normal shape and the only scar that remains is a pencil-thin line stretching from ear to ear beneath her hairline—a source of great fascination for her schoolfriends and slightly ghoulish pride for Georgia.

At her last check-up, over a year ago, I was assured that her head was so strong that she was fit to play rugby for the All Blacks. The threat of developmental problems is gone. And I think she looks like an angel, though she rarely behaves like one.

The last word should go to Barry Jones. “If you have any concerns about the growth or shape of your child’s head, go to a GP or paediatrician who should refer you to one of the three supra-regional centres in London, Birmingham or Oxford. These are expensive units to run, but they are funded centrally, so GPs and health authorities should know that they won’t pay a cent for the referral or treatment.” And to parents his message is simple: “We can treat craniosynostosis very successfully. At Great Ormond Street alone we are operating on 80 children a year. In experienced hands, the risks are very small.” As a parent, I believe the results justify those risks and I think in years to come Georgia will agree.

The Associated Press
May 27, 1996, BC cycle

Surgeons Repair Rare Skull Deformity
By Bill Baskervill

The Guatemalan heat was oppressive, yet the young mother had swaddled her infant in a blanket, covering his head and face.

She stepped into a hotel lobby and looked for the American doctor she hoped could deliver her only child from an anguished life of stares and ostracism and probably retardation and blindness.

Dr. John D. Ward thought the woman had a sack of flour on her shoulder. The 28-year-old mother, smiling and somewhat embarrassed, uncovered her son’s head, revealing an extremely rare deformity of the skull and face.

Jose Daniel Lopez had an unusually severe form of craniosynostosis, the abnormal fusing of the soft membranes that separate bony plates of the skull and allow the brain to grow. In his case, all four of the membranes, or sutures, had closed prematurely. Doctors rarely see more than one or two sutures affected.

The condition had pushed the top of Jose’s head into a cone, flattened the back of the head and spread the sides into a wedge as the brain pushed into an area where the skull had not formed. Jose’s face was flat with no brow; shallow eye sockets caused the eyes to bulge.

Ward, a pediatric neurosurgeon at the Medical College of Virginia Hospitals, the medical center of Virginia Commonwealth University, traveled to Guatemala City in late March to find patients needing craniofacial surgery. He and other surgeons perform the operations free of charge.

A few weeks later, Jose was flown to Richmond.

Jose, 6 months old, cooed and gurgled as Lori Green—who cared for the boy during his stay in Virginia—carried him about the hospital in the days before his surgery. He had suffered no apparent developmental delays, doctors said.

Dr. William G. Loudon, a neurosurgeon, joined Ward and plastic surgeon Dr. Isaac L. Wornom for the April 26 operation.

“It’s a phenomenal head, it’s incredible. The shape of the skull looks like a Darth Vader helmet,” said Loudon. A three-dimensional CT scan on the operating room wall showed numerous protrusions, craters and cavities in Jose’s skull.

The challenge facing Wornom, the primary surgeon, was to remove the thin, delicate skull, piece by piece, then reconstruct it—all without damaging the boy’s brain.

As Wornom removed sections of the skull, the brain fell into a more natural position. Neurosurgeons Ward, Loudon and Dr. John S. Myseros offered suggestions and assisted Wornom.

A slip of the hand “could paralyze this child,” Loudon said.

Wornom had come into the operating room with a mental image of how Jose’s head should look.

“I’m trying to make it (Jose’s head) lower and stick out more in front,” he said, bending to the task under the hot lights.

Seven hours later, Wornom announced, “It’s not a pointy head anymore. We’ve got the top of the head looking really good. I’m very pleased with this.”

So was Ms. Green. “Oh, my goodness, it’s amazing how quickly you forget how he looked before,” she said.

Craniosynostosis may have a genetic origin, but in many cases the cause is unknown.

Surgery to correct the condition recently became controversial in Colorado. State medical authorities there are looking into a hospital where surgeons performed the operations on more than 1,200 children. Some parents contend that the operations were unnecessary.

But the Virginia surgical team said Jose’s case was so severe that there was no question about surgery.

“If we didn’t do this, he could die of intracranial pressure” caused by water on the brain, said Wornom. A week earlier, Ward had implanted a shunt to relieve the persistent hydrocephalus.

While Wornom was able to round out the wedge shape of Jose’s head, the child probably will have an unusually wide face the rest of his life.

Wornom was unable to sculpt the back of the skull because bone spikes close to the brain made the procedure too hazardous.

“Really, I think his hair is going to cover it,” he said as Jose warily eyed the surgeon from his hospital bed several days after the operation.

Wornom left a number of gaps in the skull to eliminate the possibility of future craniosynostosis as Jose’s head grows.

He may perform another operation in a few years to slightly adjust the shape of Jose’s eyes. The bulging was corrected during the skull reconstruction.

Three weeks later, Ward returned Jose to Guatemala. Ms. Green said Jose’s mother, grandmother and other relatives met Ward and the boy May 18 at the airport in Guatemala City.

“They were very happy and they gave Dr. Ward a little gift,” Ms. Green said. “The grandmother started crying.”

The Denver Post
July 28, 1996

Hospital must stand trial in skull-surgery suit Judge won’t dismiss Children’s in case
By Robert Kowalski and Ann Schrader

Children’s Hospital must go to trial in a lawsuit charging that the Denver hospital and a team of its surgeons improperly performed complicated surgery on an infant girl for a rare skull disorder.

Denver District Judge Robert Fullerton on July 18 brushed aside a request by Children’s to be dismissed from the case of Veronica Kerin. The court, he wrote, “finds that material issues of fact exist” concerning alleged negligence by the hospital.

Fullerton’s order supports emerging law in which hospitals increasingly are being asked to be responsible for doctors practicing in their facilities.

In addition, the families of eight more children who underwent skull surgeries for that medical condition—craniosynostosis—at Children’s Hospital in the 1980s also have filed a legal complaint against the hospital and some of its doctors, claiming that the surgeries weren’t warranted.

That brings to 12 the number of families that have taken legal action against the hospital and surgeons concerning the operations for the skull disorder.

CS, as the disorder also is called, is a condition in which the normal openings, or expansion joints, in a child’s skull are closed and the skull can become misshapen as a baby’s brain grows.

During the 1980s, Children’s surgeons performed potentially life-threatening CS surgeries on more than 1,200 children to correct the largely cosmetic condition. About 40 percent of those diagnosed with CS had surgery. Studies indicate the diagnosis rate was two to five times higher than at other hospitals.

The need for the surgeries was questioned by several national organizations, including the Centers for Disease Control and Prevention. In one of its studies, the CDC determined that the “epidemic” was largely because of the number of referrals to Children’s and to the surgeons’ criteria for diagnosis.

Dr. Stan Ferguson, who was the Colorado state epidemiologist during the initial CS wave and peak, recently said it was “very troubling” that the number of surgeries dropped dramatically “after questions were asked regarding diagnostic and operative criteria.”

“One must ask how many cases of craniosynostosis were taken to surgery unnecessarily,” he said.

Now, 10 years after the peak of the surgeries in Colorado, and after a Denver Post investigation last fall reported that the “epidemic” did not exist, a number of lawsuits are being filed against the hospital and its CS surgical team.

Among the first to legally question Children’s and its doctors’ actions in performing craniosynostosis surgeries were the parents of Veronica Kerin, a girl who at 6 months of age underwent CS surgery to correct a skull deformity.

Veronica’s parents have charged in their lawsuit that doctors performed a 1992 operation on their daughter that unnecessarily removed most of her skull in an attempt to reshape it. During the operation, Veronica’s heart stopped beating. She was resuscitated, the surgery halted and her brain covered with skin.

Veronica had to undergo additional surgeries to repair her skull, including removal of six of her ribs that were used to help cover her brain. Her mother said she believes Veronica suffers development and vision deficits as a result of the initial operation.

Lawyers for Children’s Hospital have argued that the hospital should not be held liable for the injuries Veronica suffered.

“It is well established that a hospital cannot exercise the medical judgments of a physician and cannot supplant its judgments for those of the physician,” they said in court filings.

Lua Blankenship, president of Children’s, said he didn’t have a “particular” reaction to the judge’s dismissal of the hospital’s request for summary judgment.

“This is in midstream. We’re responding to a legal action,” Blankenship said.

James Avery, a lawyer who filed the Kerin lawsuit and who is representing a number of the other families who have taken legal action as a result of craniosynostosis surgeries on their children, welcomed the ruling on Children’s Hospital.

“The court has found that there is a basis for our claim against the hospital, independent from the physicians’ negligence,” he said. “That’s a big hurdle to overcome. Ordinarily, hospitals are not liable for the negligence of physicians.”

Veronica’s mother, Valerie Allen, said last week that she “steered away” for a while from dealing with the legal aspects of her daughter’s situation. But, she added, “I’m really happy that the judge ruled that way.”

Fullerton’s ruling came after lawyers for Veronica filed a statement from an expert.

In that statement, hospital administration consultant John Krismer concluded that Children’s violated numerous local, state and national medical standards in association with Kerin’s surgery.

Krismer wrote that Children’s “failed to provide appropriate medical staff surveillance and improperly allowed unsupervised freedom to perform limitless surgical procedures” by the neurosurgery service run by Dr. Larry McCleary, a neurosurgeon who was one of several doctors involved in the Kerin case.

Hospital administrators have “effectively disregarded” concerns and recommendations about the neurosurgery service from several organizations, including the American Board of Neurological Surgery, the University of Colorado, the Colorado and National Public Health services, including the CDC.

Krismer added that the hospital “has failed to remedy this dangerous situation even subsequent to the Veronica Kerin disaster, which was apparently never properly reviewed by the medical staff in the usually required monthly primary and service meetings.”

The Kerin lawsuit had been scheduled to go to trial in March. But that was postponed after McCleary suffered what his doctor said was heart irregularity. McCleary has since undergone treatment and hasn’t indicated to Children’s officials whether he plans to return to practice.

The trial on McCleary’s involvement in the Kerin surgery now is scheduled next March. Avery said he hopes the case involving Children’s Hospital will be heard as part of that same trial.

Allen, too, wants one trial, saying, “I don’t think I could go through two trials.”

The other lawsuit that the group of eight families sought to join last month is scheduled to go to trial this October. Those families are trying to take part in a pending suit filed late last year by the parents of another child who underwent CS surgery at Children’s.

In their legal filing, the parents of the eight children claim they were misled about the need for the surgeries and that their children have suffered lingering effects.

The legal filing claims the families learned that the skull surgeries may not have been necessary only after reading a series of stories published in The Denver Post in October 1995 titled “The Epidemic That Wasn’t.”

Among those who filed that legal action was Federal Heights resident Rhonda Asklund, whose son Joshua underwent the skull surgery in 1989.

Joshua now is 8 years old and is mentally retarded, having developed to the level of a 2-year-old, said his mother, who added that she doesn’t know if the surgery caused Joshua’s retardation.

Asklund said that before her son underwent the surgery, she had been told he had craniosynostosis and would become mentally retarded if he didn’t have surgery.

But she asked Dr. Ken Winston, who has a pediatric neurosurgery practice at Children’s separate from McCleary’s, to review X-rays that were taken of Joshua’s skull before the CS surgery.

Winston, Asklund said, concluded that Joshua did not have the disorder before his skull was reshaped.

“They did wrong to these children, and they need to learn and understand what they’ve done,” Asklund said.

Business Wire
August 12, 1996

Surgeons use holograms in innovative surgery

Surgeons from the University of California, Irvine, are using holograms in an innovative procedure to treat infants and young children with craniosynostosis, the premature closure of the skull.

Craniosynostosis affects about one in 2,000 births, according to the medical journal “The Lancet” (July 24, 1993). The deformities are usually evident soon after birth and become more obvious as the child grows. Craniosynostosis can have serious consequences, including impaired intelligence.

Traditionally, physicians have treated craniosynostosis by surgically reshaping the skull and holding the bones in place with metal plates, screws and wires. However, the plates and screws tend to migrate inwardly with growth, posing potential problems.

A new surgical procedure eliminates this problem. In the new procedure, the surgeons cut the misshapen part of the skull into segments which they shape into interlocking segments (similar to the concept of a jigsaw puzzle). The segments are secured with absorbable suture material and fuse permanently together as they heal.

To help them plan and carry out the intricate surgery, the surgeons use an innovative, three-dimensional imaging technology, the Voxel Digital Holography System. The digital holograms act as a “transparent twin” of the patient’s skull, allowing the doctors to interact with the life-size images as if they were the patient.

The physicians use the holograms or Voxgrams to help them plan the complex surgeries and to guide them during the difficult procedures.

“Because the Voxgrams are faithful to the original data and are life-size, any measurements I take from the hologram are precise; I can rely on their accuracy,” said Dr. David Furnas, chief of plastic surgery at UC Irvine.

“This is extremely valuable information when planning and performing this type of surgery. With the Voxgrams, we can choose a viewing perspective, examining the hologram from a wide range of angles before beginning surgery,” said Dr. Clarence S. Greene Jr., chief of pediatric neurosurgery.

“With the information present in the holograms, we can plan our surgical approach well in advance of the surgery, potentially minimizing delays and possibly improving the outcome,” he added.

Furnas and Greene are building upon work done by other physicians, including Drs. Jill Hunter and David Goldberg of Children’s Hospital of Philadelphia at the University of Pennsylvania.

The Voxel Digital Holography System was awarded FDA clearance in October 1995. Voxel, based in Laguna Hills is a public company founded to develop, manufacture and market holographically based, volumetric display systems.

Medical Industry Today
August 13, 1996

Holograms Used in Surgery to Treat Craniosynostosis

Surgeons from the University of California, Irvine are using holograms in a new procedure to treat infants and young children with craniosynostosis, the premature closure of the skull. Traditionally, surgeons have treated craniosynostosis by surgically reshaping the skull and holding the bones in place with metal plates, screws, and wires. That procedure could cause potential problems because the plates and screws tend to migrate inwardly with growth. In the new procedure, surgeons cut the misshapen part of the skull into segments which they shape into interlocking segments. They secure the segments with absorbable suture material and the segments fuse permanently together as they heal.

Surgeons use the VOXEL (Laguna Hills, CA) Digital Holography System, three-dimensional imaging technology, to assist them with the surgery. The digital holograms or Voxgrams allow the doctors to interact with the life-size images as if they were the patient, creating a “transparent twin” of the patient’s skull.

“With the Voxgrams, we can choose a viewing perspective, examining the hologram from a wide range of angles before beginning surgery,” said Dr. Clarence Greene Jr., chief of pediatric neurosurgery.

“With the information present in the holograms, we can plan our surgical approach well in advance of the surgery, potentially minimizing delays and possibly improving the outcome,” he added.

Dr. David Furnas, chief of plastic surgery at UC Irvine, said the holograms are an invaluable tool. “Because the Voxgrams are faithful to the original data and are life-size, any measurements I take from the hologram are precise; I can rely on their accuracy,” he said. “This is extremely valuable information when planning and performing this type of surgery.” Craniosynostosis, which affects about one in 2,000 births, according to the Lancet medical journal, can have severe consequences, including impaired intelligence.

The deformities are usually evident soon after birth and become more pronounced as the child grows. Furnas and Greene are building upon work done by other physicians, including Drs. Jill Hunter and David Goldberg of Children’s Hospital of Philadelphia at the University of Pennsylvania. The Voxel Digital Holography System was awarded FDA clearance in October 1995. Voxel, based in Laguna Hills, is a public company founded to develop, manufacture, and market holographically based, volumetric display systems.

CONTACT: Raymond Schulz, VOXEL (714/348-3200)
Web: http://www.voxel.com

The Times
November 9, 1996, Saturday

Prince’s son has been born with craniosynostosis
By Dr Thomas Stuttaford

Prince’s son has been born with craniosynostosis, the premature closure of the sutures in the skull, the jagged joins in the bones which allow the skull to expand to accommodate the growing brain.

His form of the condition, Pfieffer’s Syndrome, is a particularly difficult one to treat and requires very complex surgery. The problem is genetic in origin and affects boys much more often than girls. As well as the changes in the skull, which gives it a clover-leaf appearance, the children tend to have shallow, flattened eye-sockets so that the eyes are unusually prominent. Often, as in this case, the baby has upper-limb deformities and is of small stature.

Many of the children have respiratory troubles which compromise their general condition. The drainage of the blood from the brain may also be disturbed and this may make it difficult to perform the surgery which is essential if the skull is to be able to expand.

The Times
November 14, 1996

Problems of a Prince’s son
By Dr Thomas Stuttaford

The artist formerly known as Prince may have dominated the pop scene in the mid-80s and had a sizzling personality which helped to change fashion as well as music, but he is little taller than Napoleon. His wedged shoes are now legendary. In addition, the pop star’s head is slightly out of proportion to his body, although neither his height nor his unusually shaped head would have excited comment were it not that his newborn son suffers from craniosynostosis.

Craniosynostosis is the premature closure of the sutures of the skull. These sutures have nothing to do with stitches, but refer to the jagged lines which joined the various skull bones. If these joints between the skull bones become obliterated too soon there is inadequate room for the skull to accommodate the brain as it grows, and consequently it may suffer damage from being compressed.

As not all the sutures usually suffer from premature closure, the skull may be distorted into bizarre shapes because those parts of the skull that can expand compensate by doing so to a greater extent than is usual.

Occasionally, minor degrees of craniosynostosis can be spotted in adults. The final shape of the head will depend on which sutures fused early; some heads have a very high pointed forehead known as the tower head, or more precisely, turricephalic. Premature closure of different cranial joints may, for instance, produce a skull that comes to a point between the ears.

The amount of deformity varies greatly. The type of craniosynostosis from which the singer’s son is suffering causes very considerable problems and is one of the more distressing abnormalities of this group of congenital diseases.

In appearance, the baby’s skull is like a clover leaf and this type of deformity is often referred to as the Clover Leaf Syndrome. The changes in the skull in craniosynostosis are often associated with other birth defects. Ocular symptoms are common. In this baby’s case, the orbits are likely to be very flat so that the eyes are unusually prominent. The shallow orbits are part of a general failure of the centre of the skull to develop. The affected child is often very short in stature, and frequently there are other bone and joint changes. This baby has abnormal elbows, and in some types of craniosynostosis there are additional fingers or toes.

Craniosynostosis is an inherited condition and boys are three times more likely to be affected by it than girls. As in any inherited condition, the degree of penetration, that is to say the number of different features of any syndrome which the patient may show, varies, hence the possible interest in the American pop singer’s own abnormally short stature.

In this baby’s case, the type of craniosynostosis he is suffering from is associated with recurrent chest infections and changes in the circulation to the head and neck.

Abnormalities in the system of veins, which are draining blood away from the head, can hinder the already complex surgery which is needed to relieve the symptoms caused by the condition.

Barry Jones, the consultant plastic surgeon at London’s Great Ormond Street Hospital, who has made a specialty of treating cranio-facial deformities in children, says that craniosynostosis is an uncommon condition and it is therefore very important that it should be treated in centres which are equipped for the necessary surgery and aftercare, and which are staffed with people who have relevant experience.

Mr Jones and Richard Hayward, a consultant neurosurgeon, have recently published the results of eight cases similar to that of the singer’s baby who have been treated at Great Ormond Street.

Surgery is necessary not only to give the child a skull that is as normally shaped as possible, but also to relieve any infracranial pressure before there is brain damage.

The Denver Post
July 8, 1997

Infant skull surgery linked to adult woes, attorney says
By Ann Schrader

An 18-year-old Thornton woman who as a toddler had surgery to correct a congenital skull defect still suffers physical and psychological problems, her attorney contended in opening statements Monday in Denver District Court.

China Freyer was 16 months old when she underwent an operation to repair a deformity known as craniosynostosis, in which sutures or expansion joints in the skull fuse too early or are absent.

In Freyer’s case, the growth area on the top of her skull—known as a sagittal suture—was closed. The closure caused her skull to begin resembling a “boat shape” to accommodate her growing brain.

But Freyer claims she never should have had the surgery and filed a suit against Children’s Hospital, neurosurgeon Dr. Robert Hendee, and Dr. Richard Albin, a plastic surgeon who worked as part of the team. The hospital and Hendee reached settlements with Freyer, leaving Albin as the sole defendant in the case.

Albin’s attorney, Greg Tiemeier, told the eight-member jury that records show his client counseled Freyer’s parents, Harold and Kerry, at least three times about the surgical risks.

Tiemeier also questioned whether Freyer has physical and psychological problems, pointing to the lack of notations on medical records through the years and her recent report card.

Freyer was one of the more than 1,200 infants and toddlers operated on in the 1980s by surgeons at Children’s Hospital in Denver to correct various forms of craniosynostosis, a condition that generally affects appearance but not the brain.

Several national organizations, including the Centers for Disease Control and Prevention, later began questioning the need for the surgeries. In one study, the CDC said the “epidemic” of craniosynostosis cases in Colorado was largely due to the large number of referrals to the Children’s Hospital team and to the surgeons’ diagnostic criteria.

A Denver Post investigation led to a series of articles in 1995 and 1996 that reported the “epidemic” did not exist. A number of lawsuits, including Freyer’s, have been filed against the hospital and the surgical team. Freyer’s is the first to go to trial.

In Freyer’s case, both attorneys have acknowledged she did in fact have craniosynostosis. But Freyer’s attorney, James Avery, Monday questioned whether Freyer’s surgery was necessary and appropriate.

Avery contended that the surgical method was inappropriate, the risks weren’t adequately explained to her parents and she has a forehead deformity, an ear-to-ear scar and severe headaches.

During the surgery, Freyer lost “so much blood they couldn’t keep up with it,” Avery said Monday.

Her heart stopped, and she was resuscitated. Avery said more surgeries—10 more over the next several years—were needed to finish the initial operation and repair problems. She suffered wound infections and her bone didn’t regenerate well.

Avery said she had to wear a protective helmet until she was in kindergarten.

A main consideration for the jury, Tiemeier said, is to remember that when Freyer’s heart stopped, Hendee was operating, not Albin “who was, in all likelihood, not in the operating room” since his portion of the surgery came later.

A permanent learning disability, Avery said, has “significantly impacted” Freyer’s earning expectancy.”

A vocational expert will testify she will suffer an earning loss of more than $399,000 over her lifetime due to the surgical problems, said Avery.

The Nelson Mail (New Zealand)
August 22, 1997

Parents combat rare skull condition
By Neal Tracy

Nelson parents tired of seeking answers to the rare medical condition affecting the skulls of their children, have decided to band together for mutual support.

Attempts at establishing a support group for children with craniosynostosis, an unusual and disfiguring skull condition, have yielded a surprisingly strong response, said Nelson mother Allison Morris. An advertisement she and Motueka mother Miriam Roozendaal placed in a community paper attracted 17 calls. Ms Morris’s 3 1/2-year-old son Logan was born with the condition, which meant his skull was fused at a particular joint and required radical surgery when he was five months old. Mrs Roozendaal’s son Luke, 3, underwent surgery for the same condition at six months of age.

Ms Morris said she noticed her son’s head had begun to flatten by about three months, and he was diagnosed with the condition, then underwent the surgery in Wellington Hospital which has allowed his head to grow as normally as possible.

Today, the tell-tale signs have recurred and his distressing habit of hitting his head is a huge worry, Ms Morris said. Logan is now waiting to have another scan.

The women feel they have been given conflicting and incomplete information over the condition. They were told it occurred in one in 20,000 children, but believe more cases exist.

However, Nelson paediatrician Peter McIlroy said he was not aware of any increase in craniosynostosis, but there was an increase in recognition of infants with distorted shaped heads, which can occur for a number of reasons.

Dr McIlroy said the worldwide emphasis on placing babies to sleep on their backs (to prevent cot death) can cause misshapen heads.

I’d never suggest from this babies shouldn’t sleep on their backs, but maybe parents should alternate that with turning their babies on their side.

Mrs Roozendaal said they had to constantly push for answers over their children’s condition, and while the level of severity differed in each child who suffered from it, lack of information is a common factor.

The support group is being set up to provide an outlet for parents wanting to share their coping experiences, Ms Morris said.