The Associated Press
May 25, 1980, AM cycle
Health Officials Baffled By Cluster of Birth Defects
By David Crary
State health officials are at a loss to explain an apparently unprecedented rash of birth defects among babies born last year in Steamboat Springs, Colo.
Five babies, all of them born last spring, had prematurely fused skulls—a condition known as craniosynostosis that can result in brain damage. Three other babies born in the same 12-week period had related defects of a cleft lip and palate, and five others also may have had a form of craniosynostosis.
Dr. Stan Ferguson, director of the Colorado Department of Health’s disease control division, said Routt County Hospital, where the children were born, should have had fewer than one such birth defect during that period, according to statistics.
“We still have no leads as to why there were this many in Steamboat Springs,” Ferguson said after returning from a visit to Routt County last week. “We’ve confirmed eight cases, but we don’t know why they were clustered so.”
The defects can be corrected by surgery.
Ferguson said researchers have begun to sort through the past 100 years or so of medical literature to see if any comparable rashes of the birth defects have been recorded. But thus far, he said, the Steamboat Springs occurrence appears to have no precedent.
“Normally, this would occur in one birth out of 2,000,” he said. “This is the only documented instance of the disorder occurring to such an extent in the same place and time.”
“It’s very difficult to figure out, when there’s so little known,” he said.
Ferguson said the next step in the Health Department’s study will be a detailed evaluation of each case, based on interviews with each infant’s family and other data. So far, however, experts have found no common thread beyond the time and place.
“The only hint we’ve had is a series of papers written in Seattle indicating that babies that drop from the mother’s uterus to her pelvis prematurely prior to birth are more prone to craniosynostosis,” he said. “But that doesn’t answer our basic questions.”
Ferguson said infants born with a prematurely fused skull usually must undergo an operation to separate the skull in order to allow the brain to grow normally.
The Associated Press
August 16, 1981, AM cycle
Birth Defects In Mountain Towns Still Unexplained
Scientists are baffled by a rare skull deformity that has affected newborn infants in two Colorado mountain towns at a frequency perhaps 100 times greater than the national average, the Denver Post says.
A copyright story in the paper’s Sunday editions reports that craniosynostosis, a potentially brain-damaging birth defect found in only about one newborn in 2,000, has been found in a large number of infants in Steamboat Springs and Idaho Springs.
Craniosynostosis is a premature fusing, or growing together, of the bones in a baby’s skull. If not surgically corrected early in life, it may cause the infant’s head to become misshapen and restrict growth of the brain, resulting in mental retardation.
In Steamboat Springs, at least five babies were born with the defect in little more than four months in late 1978 and early 1979. In Idaho Springs, two cases of the defect were reported in December 1979 within three days.
The Post says it also has verified two other cases in or near Idaho Springs during a 4 1/2-year period capped by the 1979 births.
Researchers suspect many environmental causes of the high incidence of the defect, but only remote circumstantial evidence exists to support those theories, the Post says.
One area that has received much attention is the historical existence of impurities in water supplies serving the two communities.
Both Steamboat Springs and Idaho Springs collect their water from basins with mineral mining operations that have been sources of water pollution in the past. Medical authorities, however, emphasize there is nothing conclusive to tie the birth defects to such water contamination.
Robert W. Hendee Jr., chief of neurosurgery at Children’s Hospital in Denver where many of the defects are treated, called the high incidence of craniosynostosis in the two areas “a rather astonishing number.”
The Colorado Department of Health conducted an 18-month investigation of the Steamboat Springs defects, but it was closed last April because of a lack of funding. No conclusions were reached.
However, the health department is now investigating the more recent occurrences of the defect in Idaho Springs.
May 23, 1986
CNN probes Colorado birth defects
CNN Medical Correspondent Gary Schwitzer has investigated what appears to be an epidemic of birth defects in Colorado that has forced hundreds of babies to undergo major skull surgery. It’s a puzzling malady called craniosynostosis in which the child’s skull is formed in a way that inhibits growth of the brain. If not corrected, the results often-times are slow physical and mental development, along with facial deformities. No one knows quite what causes it but what is known is that more than 300 infants in the Denver area have had to undergo skull surgery in the past two years alone. Schwitzer found that resort areas like Steamboat Springs and Vail have had clusters of cases. He also found that not one penny has been appropriated by the state legislature to study the situation, and that no one has been assigned to do the basic research needed to find out way so many Colorado children are affected by craniosynostosis. Schwitzer also spent a day with one couple while their four-month-old child underwent an operation to correct his defect. CNN’s NEWS FROM MEDICINE will air a three-part series on craniosynostosis beginning Monday, May 26 on NEWSWATCH at 5-6:30 p.m. (EDT). It will also run on CNN EVENING NEWS at 10 p.m. (EDT). The entire series can be seen on HEALTHWEEK, Saturday, May 31, at 9:10 a.m. and 2:10 p.m. (EDT)
Los Angeles Times
March 20, 1991, Wednesday, Orange County Edition
Surgery to Save Filipino Baby From Deformity
By Anita M. Cal
To save a Filipino baby from a serious deformity, two Orange County charitable organizations and a local hospital pooled resources to bring the boy to this country for a sophisticated brain surgery procedure not available in the Philippines.
Edison Lawrence Gonzales, 5 1/2 months old, is scheduled to undergo surgery today at Western Medical Center-Santa Ana to correct a rare cranial complication called craniosynostosis. The phenomenon is caused by the premature fusion of the baby’s “soft spot,” which causes the brain to grow in the wrong direction.
The early fusion of these sutures—little bones that form together like thread—atop the baby’s head results in brain damage and cranial deformity if not corrected, doctors said Tuesday. Normally, an infant’s soft spot does not close until about age 18 months, said Michael H. Sukoff, a neurosurgeon who will be performing the delicate operation.
“It’s like Charlie Brown’s head,” Sukoff explained. “Charlie Brown has a big head and a flat face, similar to what Edison would have.”
The operation costs between $40,000 and $60,000, hospital officials said. But the Gonzales family will not have to pay for the travel or medical costs, thanks in large part to a Westminster volunteer group called Pointes of Light.
Nancy Fontaine, director and founder of Pointes of Light, said she learned about six weeks ago about the diagnosis, and that Edison could not receive proper treatment in the Philippines.
Fontaine said she immediately began contacting area hospitals to see which one would perform the surgery free of cost, and Western Medical Center accepted the case. Sukoff said the hospital accepts two or three charity cases a year involving babies in need of complicated and expensive medical care.
Meanwhile, the Make-a-Wish Foundation, a national organization that aids sick and dying children, paid for airline tickets for Edison and his parents, Myrna and Eduardo Gonzales.
The parents are still a little stunned by their sudden good fortune in finding a hospital that will treat their son. “It’s like a dream,” Myrna Gonzales said. “I’m very grateful for all these things. In the Philippines you would never get all this, with everything being free.”
While at the hospital Tuesday, the woman gazed down at her cherubic son as he gnawed on his pacifier and attempted to balance himself on his chubby legs. She said she finds it hard to believe her chortling, seemingly healthy baby must undergo potentially life-threatening surgery.
“He’s so normal,” said Gonzales, who is spending her first week ever outside of the Philippines. “It’s very hard to believe that something is wrong.”
The boy, with his deep dimples and pudgy cheeks, looks like any other baby right now, except for a barely discernible flattening of the front of his head. During the surgery, the doctor will attempt to separate two sutures that have already closed in the front of the boy’s cranium, causing his brain to push back and up.
Two weeks after surgery, Edison’s head should begin to reshape into a normal oval shape, Sukoff said, as the brain begins normal growth. Total recovery should be occur by the time Edison reaches the age of 2, he said. The operation has more than a 90% success rate, he added.
Sukoff said about four children in 10,000 get craniosynostosis. The doctor, who has performed more than 50 similar surgeries, said the deformity develops slowly, but has devastating effects if left untreated.
“Edison would grow up to look abnormal, even ugly” without the surgery, he said. Although his brain is pushing out of shape, Sukoff said the bright-eyed infant is not suffering any pain.
February 8, 1993
By Modena Wilson; Alain Joffe
Q. Our first grandchild was born last summer. His head is so long! No one in our family has a head like that. When I mentioned it to my daughter, she said she thought it was the way he sleeps. Should we do anything?
A. Your description raises several possibilities. First, babies born early often have relatively long, narrow heads. We are assuming, however, that your grandson was not premature. During the first few days of life, the head of any baby may be different from the final shape because of pressure on the head during the birth process.
Another possibility—craniosynostosis—is one that your daughter may wish to discuss with her baby’s doctor. Literally, the word means “union of the bones of the skull.” Let us explain.
When a baby is born, its skull is made up of several separate pieces. It is not yet a complete bony box. This permits continued growth of the inside. As the brain grows, the bones enlarge at their edges. When brain growth is complete, the bones knit together at seams called “sutures.”
Occasionally, one or more of the skull’s suture lines close early causing the growing brain to push in the directions of the still-open sutures. In other words, the head grows where it can-producing an unusual shape. The exact shape will depend on which sutures are open and which are closed. An elongated head can be one of the results.
Craniosynostosis probably happens in no more than one in 1,000 babies. Many times it is relatively mild and causes no harm. But if too many sutures close prematurely, the brain may not have enough room to grow. That problem should be detected by the baby’s doctor, who measures the head at each “well baby” visit.
Most cases of craniosynostosis are of a milder type and the brain keeps right on growing. However, if head shape becomes quite odd because of it, surgery to open the sutures can improve appearance.
In the end we want to stress most babies we see whose heads look a bit odd do not have craniosynostosis.
Wilson is director of general pediatrics at the Johns Hopkins Children’s Center; Joffe is director of adolescent medicine.
July 4, 1993
Skull Deformity Found In 22 Babies
Doctors are trying to discover why 22 babies in a small rural community have a rare and potentially fatal skull deformity. The condition, known as cranio-synostosis, has claimed the lives of two babies and been diagnosed in 20 others in an area between York and Selby, North Yorkshire.
The unprecedented number of cases has been found in the past 30 months and experts are looking at possible links with pesticides, pollution or a genetic fault in the closely-knit community. The senior registrar for the North Yorkshire Health Authority, Dr Philip Kirby, is trying to find out whether doctors have experienced similar clusters of cases elsewhere.
A preliminary investigation has not shown any unusual factors linking the children or their mothers but a spokesman for the health authority said: “The cases represent an unusually high rate of more than 1 in 400 births.”
Reported cases from around the world vary widely between one in 1,000 and one in 4,000 births. In Britain, there are usually about 18 cases a year. The condition is noticed at birth or becomes apparent during the first few months of life. It results from two or more bones of the baby’s skull fusing together earlier than would happen in normal growth. Often the problem is mainly cosmetic and is treated by an operation to release the skull bones.
About half the babies in the York cluster have needed surgery for the condition. One of the those died because of complications during surgery and the other from the condition directly. The health authority spokesman added that doctors were continuing to monitor and investigate the situation and were seeking further advice from regional and national experts.
Dr Jon Fear, a consultant to North Yorkshire Public Health Authority, said: “Clearly it’s an unexplained problem which is a concern, but we are still talking about a very small number of cases. They come in the areas surrounding York and Selby but there is not one particular spot which we can pinpoint. They don’t all come from one particular village or anything like that.”
He said about 20% of the cases in the York area were due to genetic family links, but nobody knew the cause of the rest. Dr Kirby and Dr Fear have published a letter in The Lancet because admissions for the condition had gone up recently across the whole of the Yorkshire region.
“We don’t know the reason. It may be that people are willing to recognise it and do some thing about it. But it certainly looks as though there are more babies with this condition than there should be,” Dr Fear said.
July 8, 1993
By Dr Thomas Stuttaford
YORKSHIRE doctors are concerned at the number of babies being born in the York and Selby area who are suffering from craniosynostosis, a comparatively rare condition in which the skull, if left untreated, becomes distorted and the brain damaged.
In the first year of life a baby’s brain doubles in volume. So that the increase in size may be accommodated, the skull is composed of separate bones which are only loosely interlocking and are initially separated by a layer of fibrous tissue. As the brain grows bigger, so the skull shape alters and expands to accommodate it.
In craniosynostosis one or more of these joins in the skull bones, known as sutures, fuses too early and the skull size is prematurely fixed so that the brain becomes compressed, with subsequent damage to it and to the nerves leading to the eye.
In craniosynostosis the skull assumes bizarre shapes. The actual nature of the deformity depends on which of the sutures has closed prematurely, and which have compensated for this by remaining open for too long. The skull can become pointed like a dunce’s cap, a condition known technically as turricephaly, or turret head; in other cases the skull becomes elongated from front to back, the shape being likened to the prow of a ship.
Although the number of cases in Yorkshire is disturbing, particularly as there is no explanation for them, the initial reports which suggested that in a comparatively small area there were as many cases as in the whole of the rest of England misrepresented the situation.
In the York-Selby area, 21 cases have recently been reported, whereas Great Ormond Street alone operates on more than 60 a year. Barry Jones, a consultant plastic surgeon at Great Ormond Street who specialises in correcting skull and facial deformities, said that he thought that at least another 40 were seen either at Oxford or Birmingham, the two other centres which specialise in dealing with craniosynostosis deformity.
Skilled surgery can prevent the bones of the skull joining too early, and by keeping them apart allow the brain to grow without restriction and the skull to assume a normal shape. In the past, silicone was implanted between the skull bones to simulate the natural fibrous tissue, but this has proved to be of little advantage and is being abandoned.
Craniosynostosis is frequently associated with other deformities as part of a more generalised syndrome. In some cases there is also coexistent congenital heart disease and in others limb abnormalities.
In more than 60 per cent of otherwise uncomplicated craniosynostosis cases there are other facial deformities directly related to the abnormal bone structure at the base of the skull, and softer than usual facial bones.
The facial deformities can produce a “dish” face, like that of a King Charles spaniel or pekinese. Surgery when the face is deformed in this way, and breathing thereby restricted, presents special problems for the anaesthetist. As in many cases in which complex surgery is needed, the anaesthetist is as important as the surgeon. Indeed, “In these cases the anaesthetist just has to be brilliant,” Mr Jones says.
Fortunately, this brilliance is still available for the time being and none of the Great Ormond Street babies who were operated on last year died, another example of the importance of patients with rare conditions being treated in specialised units.
The Independent (London)
July 13, 1993
Now Sam looks as nature intended; When their son was born with a misshapen skull, Lucy and Joe Blenkinsopp faced a hard choice. Liz Hunt reports on a rare condition now in the news
ONE OF the hardest decisions that Lucy and Joe Blenkinsopp ever had to make centred on their son, Sam. He was less than a year old, but their decision could determine how he would look for the rest of his life.
Sam was born with an asymmetrical skull which appeared slightly bulbous on the left, and flattened at the back. Doctors could not say with any certainty if it would stay the same or get worse as he got older and, as his brain grew, distort his skull further and twist his features. The remedy was radical surgery in which their baby would be cut from ear to ear, his scalp peeled down, and part of his skull removed by a neurosurgeon, exposing the brain. The skull bones would then be “remodeled” by a craniofacial surgeon and replaced. It was an alarming prospect, but the younger the child the easier and less traumatic the operation.
Sam Blenkinsopp suffered from a relatively rare condition known as cranio-synostosis, in which skull bones fuse prematurely. Until last week few people had heard of it, but then doctors from North Yorkshire Health Authority reported a highly unusual cluster of 22 babies with cranio-synostosis in a small area between Selby and York. A fifth of the cases are genetic in origin, but as yet there is no explanation for the others.
Environmental factors such as lead, pesticides or other chemicals have been suggested. It is not known how many children are born with cranio-synostosis each year, but estimates range from between one in 2,000 and one in 4,000, with about 100 new cases a year.
Babies with oddly shaped heads are not that unusual, and GPs and health visitors are frequently called upon to reassure anxious parents. A baby’s skull is malleable and subject to many influences: how the foetus lay in the womb, and how it was delivered—by forceps or ventouse (vacuum) extraction for example.
“Even postural influences such as sleeping positions may cause distortions of the skull,” according to Norman Waterhouse, a craniofacial surgeon and head of the craniofacial unit at Charing Cross Hospital, London. “In the majority of cases the head will resume a normal shape as the child gets older.”
But occasionally there is a problem; cranio-synostosis is a generic term used to describe a condition in which one or more of the “expansion” joints in the skull, known as sutures, fuse prematurely. A baby has six pliable plates of bone making up the skull that “float” on the surface of the brain, allowing it to grow and expand.
If a suture fuses early, the brain cannot not grow normally, and pushes out another part of the skull, causing an abnormal appearance and sometimes distortions of the eyes and face. Occasionally, cranio-synostosis is only one symptom of very rare syndromes, such as Apert’s and Crouzon’s syndromes, and these babies tend to be more severely affected, and suffer a range of other physical deformities.
The degree of skull abnormality in cranio-synostosis depends on which sutures are fused and how many. Heads can appear bow or keel-shaped, very long, or triangular. In most cases the affected children have normal intelligence, and the problem is cosmetic. However, if more than one suture is fused, and the brain’s growth is restricted in more than one area, there is a risk of mental retardation and surgery becomes a necessity.
In many respects it is easier for the parents of these children to face up to the trauma of surgery than the parents of less severely affected children such as Sam Blenkinsopp, according to Daniela Hearst, principal clinical psychologist in the craniofacial unit at Great Ormond Street Hospital, London.
Do parents say no to surgery and have their son or daughter at 16 blaming them for a life marred by some deformity? Do they wait until the child is older, when she or he has been teased because of their odd appearance, and when surgery will be more difficult? Or do they say yes to major surgery in a young baby? “The easier option is when the surgery is medically dictated,” Ms Hearst said.
In Sam Blenkinsopp’s case one of the sutures in his skull was partially fused, but X-rays and CAT scans failed to establish to what degree—which is why doctors were uncertain about his prognosis. Initially, the Blenkinsopps, who live in Southfields, in south-west London, were at odds with each other over what should be done. Mrs Blenkinsopp believed that if there was the slightest chance that their son would look “strange”, or different from other people in later life, then he should have the operation.
“I am of the opinion that this world is hard enough without your appearance making it even harder. But my husband found it incredibly difficult to come to terms with. He felt there was so much that could go wrong,” she said. “We discussed it endlessly, but I didn’t want to influence him. He had to work it out himself.”
It was two weeks before Joe Blenkinsopp came to the same conclusion as his wife, and Mr Waterhouse operated on Sam, then aged 13 months, last October. When he returned from theatre he looked “quite horrendous”, his mother said. “His whole head was very swollen, his eyes were swollen and bruised and he was black and blue. Bella, my daughter, who was two, burst into tears when she saw him.” But within a day or two Sam was driving in a toy car up and down the hospital corridor, and the swelling started to subside. In fact, he took his first steps on the day that he was discharged, less than a week after his operation. There have been no psychological or developmental problems; all that remains is a scar on his scalp, running from ear to ear He will, however, continue to see Mr Waterhouse until he is a teenager.
Sam Blenkinsopp was lucky on two counts; when his skull was opened up the degree of fusion was much greater than suspected and would have distorted his face, causing the “drifting” of one eye as he got older. But he was also lucky because his condition had been properly diagnosed in the first place. It was a chance remark by a consultant who was treating Sam for a chest infection when he was three months old that alerted the Blenkinsopps to the problem. Initially, his skull deformity was blamed on a twisted muscle in his neck and he was given physiotherapy.
Mr Waterhouse believes that many children with more severe problems are being missed. They receive inappropriate or no treatment because many GPs and some paediatricians are not fully informed about what cranio- synostosis is, and what craniofacial surgery can offer. Following publicity about the Yorkshire cases last week, the Independent was contacted by several parents who were concerned about the shape of their children’s skulls and were not happy with advice they had received. One couple from west London had seen a GP, an orthopaedic surgeon, a physiotherapist, and a cranial-osteopath. No one had suggested assessment by a craniofacial or neurosurgeon.
Any parent who is concerned should seek referral to one of the specialist centres in Oxford, Birmingham or London. The optimal age for treatment is between three and six months; after this it becomes more complicated but is still worthwhile, Mr Waterhouse said. “We can, more or less, make every child we see look better—or more as nature intended.”
The Independent (London)
September 4, 1993
FEARS that a rare skull deformity which has affected more than 20 babies in North Yorkshire is occurring more frequently were dismissed by doctors yesterday, writes Liz Hunt.
A review of the incidence of cranio-synostosis in the York and Selby areas has shown that it is within expected limits.
In July, North Yorkshire Health Authority confirmed 28 cases of the condition, which involves the fusion of joints in the skull and can lead to distortion of the head. Most cases are cosmetic and can be corrected. In a few, pressure on the brain can cause mental retardation.
Experts have now concluded that 21 of the 28 cases fitted the diagnosis and this is in keeping with an expected total of 25 cases for the population in this area.
Daily Mail (London)
June 20, 1995
They said she’d look like Elephant Man, but now she’s a child model
By Clare Campbell
EVERY mother secretly believes her baby is the most beautiful in the world. So how does she feel when she is told instead that her child is going to be hideously disfigured? Gill Whalley, 32, and her husband Alan faced this dreadful news just six weeks after their daughter Olivia was born, apparently healthy and normal. They were told that not only could their daughter could go blind and deaf, but also that she would eventually end up looking like Elephant Man.
Many parents would have felt unable to cope with such a crushing blow, but Gill was determined to remain strong for the sake of her child and the rest of her family, and to do whatever she could to save Olivia from this awful fate. Here she tells of the miracle surgery that not only transformed her daughter’s face but has led her, against all odds, to become a child model. Alan and I always wanted lots of children, and being in the fortunate position of owning two successful nursing homes, money was no problem. After marrying at the age of 22, I gave birth to our son, Simon, less than a year later. Tragically, Georgina was born prematurely and survived for only 24 hours. Losing her was a terrible shock to me and remains the greatest sadness of my life. But both of us still wanted to try again and Hannah was born by Caesarean section in 1989, followed by Olivia, two years later. Both pregnancies were very straightforward and Alan and I were happy to have three such lovely, healthy babies.
Yet from the minute I set eyes on Olivia, I knew instinctively that her face did not look as it should. One eye was small and half-closed while the other appeared far too large and wide open for a child of that age.
I asked my husband, family and friends what they thought, but everyone kept reassuring me that Olivia was perfect and that I was just being an over-fussy mother.
Olivia passed all her hospital health checks and was allowed home with us. But I simply could not get rid of this awful feeling that I was the only one who could see there was something seriously wrong with my child.
It was not until her first check-up at the age of six weeks that I finally shared my anxieties with my GP. He said he would measure the circumference of Olivia’s skull and check it again a fortnight later. From this, he told me, he would be able to judge if her head was developing normally. In the intervening days, our daughter started to hold her head up for the first time. Again, it was only me who noticed that her head kept dropping to one side every time she tried to raise it. When we returned to the doctor two weeks later, I knew without having to be told that something was drastically wrong.
Olivia’s skull had grown more than two centimetres in that time—far more than it should have done for a baby her age. We were immediately referred to a specialist.
Alan was terribly worried by now, but despite my own anxiety, I also felt a sense of relief: at last someone was taking me seriously. All I cared about was finding out what was wrong with my baby and putting it right as soon as possible.
We were given an appointment to see the specialist, Paul May, at Alderhay Hospital very quickly. To me it was another sign that Olivia’s illness must need urgent treatment. Almost as soon as the specialist had examined her, he told us what was wrong.
Our daughter, he said, was suffering from a rare bone disorder known as craniosynostosis, which had left her with one eye larger than the other because of a missing socket.
His face was grim but I felt I had to know the worst. Having already lost one child, I was not afraid to face the prospect of a sick child. As long I knew that Olivia would live, I felt I could cope with anything.
Alan, however, went pale as the consultant described the effects of our daughter’s condition. Not only might she become blind and deaf, but she would end up with a head so deformed that her skull would be permanently curved to one side.
Her only hope of a normal life lay in an operation within the next few months to rebuild her face. In the meantime, we were told, she would have to undergo intensive physiotherapy to straighten the muscles of her neck, which would otherwise be shortened by the imbalance of her head.
I looked down at Olivia and wondered how she could survive such an ordeal. But when Alan and I looked at one another, we knew we had no choice. The alternative—a handicapped child with terrible facial disfigurement—was too awful to contemplate. This was not a cosmetic operation: it was a vital necessity.
Over the next few months our lives became dominated by our daughter’s daily care. Although we tried to act as normal as possible for Simon and Hannah’s sake, both Alan and I were very scared about what was going to happen. Three visits a week to the hospital plus the physiotherapy we had to give Olivia ourselves at home meant that Olivia’s illness ruled our lives. Each morning, afternoon and evening, I had to hold her down on a table while pulling her neck muscles to prevent them from shortening on one side.
Every time I did this, she would scream so much that my son Simon would beg me to stop it. Even Alan was so distressed by the procedure that he was unable to help me and my father had to assist instead.
Looking back now, I wonder how I did it myself. But I was just so determined that Olivia was going to get better that I was prepared to do whatever it took, however painful or upsetting.
During the months that we waited for a hospital bed, I noticed with some terror that Olivia’s head was gradually becoming increasingly oddly-shaped. Something had to be done quickly to prevent our daughter from being deformed for life.
When Olivia was six months old, we were finally told that the hospital had got a bed for her. We lived directly opposite the hospital, so the consultant suggested we go home again and return in about seven hours-the time it took for the operation to be performed. He promised to ring us immediately if there were any problems.
That must have been the longest seven hours in both our lives. I didn’t know what to hope for. I just kept praying that by some miracle the surgeon would be able to make our daughter look normal.
When she was finally wheeled out of intensive care, her eyes were so blackened by bruising that I didn’t, at first, recognise my own child. All I could see was a tiny body, swathed in bandages: I could see nothing of her face at all.
The surgeon, Paul May, was wonderful, walking straight up to me and reassuring me immediately that everything had gone well. But he also warned me that Olivia’s head would at first be very pointed from the pressure of the clamps on her skull, and also that she would not be able to see for a few days, until the bruising around her eyes had had a chance to subside.
Two days later, Oliva was taken off the ventilator she had been put on to assist with her breathing during the operation. Two days after that the bandages were removed. But to my great disappointment, as the consultant had said, extensive bruising meant it was still impossible to tell what the final shape of her skull would be.
At the end of that week we were allowed to bring Olivia home. I did not have to give her any special care, other than to keep bathing her eyes. It was heartbreaking to hear her crying because she couldn’t see.
I wished with all my heart that she had been old enough to understand what I kept telling her over and over again-that not only would she soon be able to see again, but also that she was going to be the most beautiful baby in the world.
I had been under such a strain for so long that I found it hard to believe all our problems could be over that easily. I kept taking Olivia back to the consultant for check-ups, continually asking Alan if he thought our daughter looked normal as we sat in the waiting room together.
But Paul May was always happy with Olivia’s progress. Her eyes were fine and, as she grew, her skull developed normally. The consultant emphasised that I must never hesitate to bring Olivia to see him if I ever felt worried about her. Little could he have guessed how many times in the next two years I would take him up on that offer.
As Olivia learned to walk, I was continually nervous that she would damage her head in some way. All the normal childhood accidents threw me into immediate panic. When she was only ten months old, her sister Hannah accidentally dropped her on to her head; and then, a few months later, Olivia ran so close to a radiator at home that she bruised the part of her skull into which metal wire had been inserted at the time of the operation. On both occasions I rushed straight to the hospital, where Paul May came straight out from the operating theatre to examine Olivia and put my mind to rest. Even now that she is three years old, I still cannot allow her even to comb her own hair for fear that she might damage the scar on her skull.
But the miracle has happened. No one would think to look at Olivia that there had ever been anything wrong with her. Totally spoilt as a result of all the extra attention she has received from the family, she is as cheeky and lively as any other happy, healthy three-year-old.
Alan and I already thought our daughter was beautiful, but it amazed me to find that now other people seemed to think so, too.
I have always loved having lots of photographs taken of our children, and following a recommendation from a friend to a local photographer, I was thrilled, although a little suspicious at first, to receive a call from a child modeling agency who said they had seen recent photos of Olivia and would like to put her on their books.
We were all terribly excited, not quite able to believe that this could really be happening. But after talking to the agency in more detail, I realised with joy that this really was a serious professional offer. They were not asking us for any money but were simply so impressed by Olivia’s looks that they wanted to shoot a whole portfolio of further photographs of her for nothing. It felt like we had won the lottery.
I thought about all we had been through—the horror of imagining our daughter disfigured for life—and compared it to the way I felt now, accepting an offer from a modeling agency on her behalf. It all seemed too wonderful to be true.
Not only was our child now perfectly healthy, but she was also considered exceptionally pretty by people who knew nothing of her previous deformity.
Today, I feel incredibly thankful that Olivia has been so lucky. Even now, I sometimes wake up at night and wonder how I would have coped if things had not worked out so well for her.
We have been told by Paul May that Olivia now only needs check-ups every 12 months until the age of 16, after which she will probably be discharged. He doesn’t expect any further problems, so feel she is in the clear. Because it has been corrected by surgery, the damage caused by the bone disorder she suffered during my pregnancy should not recur.
I am eternally grateful to Paul May for performing this miracle on our daughter. He’s a lovely man who simply counts himself fortunate to have been given the gift of being able to help sick children in this way.
I know it’s something that every mother says, but I look at Olivia now and really do believe that she is one of the most beautiful children in the world.’
The Times (London)
August 4, 1995
Why doctors feel newborn’s head
By Dr Thomas Stuttaford
ABIGAIL WATTS, whose death in the intensive care unit at the Royal Manchester Children’s Hospital is being investigated by police, had an abnormality of the skull, pan-cranio synostosis, which had compressed her brain and had formed her skull in such a manner that when viewed from the front it looked like a clover leaf.
Although the term “clover-leaf skull” does not appear in medical dictionaries, it has the advantage of being descriptive and more easily pronounced than pan-cranio synostosis. In the normal newborn baby, the flat skull bones are separated by a thin layer of fibrous tissue. The gaps between the bones are known as suture lines and allow the bony skull to expand to accommodate the child’s growing brain.
In cranio synostosis, one or more of the sutures closes prematurely with the result that the diameter of the skull is reduced in this area, and there is excess compensatory growth in other parts where the sutures are still open.
In pan-cranio synostosis, from which Abigail is reported to have suffered, all the sutures are prematurely closed, the brain is unable to expand and if surgery is not possible it becomes so squashed in its inadequate container that the child suffers serious brain damage.
Watch doctors when they are introduced to a new baby. Whatever the social occasion, they tend to leave the chat about supposed family resemblances to others and to stretch out their hands to feel the baby’s skull.
The fontanelles, the wide gaps where the suture lines meet, reveal how well the baby is hydrated and give other clues to health. The doctors also feel the sutures themselves. Their condition, together with the overall shape of the skull, can be a guide to what happened during delivery, as well as to any cranio-synostosis.
A baby whose head has been in an awkward position during delivery may mould in such a way that its shape is altered forever. For instance, the child whose head turned late before birth may have a permanently high professorial forehead. Others with abnormal skull shapes may have had a normal delivery but because of cranio synostosis have a skull distorted to an unusual shape. In turricephaly tower head the head is dome-shaped; in other children in which different suture lines have closed prematurely, the head may have a flat back or an unusually prominent forehead.
When cranio synostosis has occurred there is always a possibility of damage to the brain or eyes by increased intracranial pressure. These cases need to be referred as soon as possible for assessment at a specialised neurosurgical unit, where a combined team of neuro and plastic surgeons can work to correct the problem, if necessary by reconstituting the gaps between the skull bones.
The most common cause of an asymmetrical skull in which one side of the head appears to have grown more than the other, giving the baby’s head and face a twisted appearance is the baby’s posture.
This deformity, plagiocephaly, usually has a cause no more sinister than that the baby always lies on the same side when in the cot.
The Straits Times (Singapore)
October 14, 1995
Surgery for month-old baby to save his brain
By Lim Li Hsien
BABY Nakulan was born with a skull that would not grow. So as his brain grew, his skull compressed it. Surgery on his skull when he was just a month old allowed the skull to grow and his brain to develop normally.
His mother, Madam Jayamanee Govindasamy, 34, said she cried when told her baby could grow up retarded: “I knew I had to do something. I could not let him grow up like that.”
She gave her consent for Dr Woffles Wu, a craniofacial plastic surgeon, and Dr Prem Pillay, head of neurosurgery, at the Singapore General Hospital, to operate on her baby.
The operation involves cutting out the parts of the skull that have stopped growing and rejoining the remaining pieces to re-shape the skull.
But to give the skull a chance to heal itself, the best time is in the baby’s first year, said Dr Wu, 35.
“It used to be the convention that it was better to wait until the child is older. But in the first year, the skull has a much better chance of regenerating itself,” he said.
He had gained his experience in craniofacial plastic surgery and written a research paper on skull and suture regeneration when he was attached to the Royal Children’s Hospital in Melbourne.
Nakulan suffers from craniosynostosis, a rare birth defect which fuses the skull’s sutures.
A normal skull is not a solid piece of bone, but made up of plates of bone joined by fibrous tissue called sutures.
Sometimes, some of these sutures fuse and stop growing, preventing the skull from expanding normally.
An Australian study found that this defect occurs in one in 4,000 births, or about 15 cases a year here. Other studies show rates up to four times higher.
But Dr Wu said that as he saw only seven such cases last year at the SGH, with four less than a year old, there could be people here who did not know this deformity could be corrected. A child with this deformity could survive to adulthood but there was a chance the brain would be retarded.
Nakulan had an extreme form of this condition, called clover-leaf skull, which leaves a gap in the centre where the brain tissue protrudes. This gives a “cone-head” appearance. The eyes also bulge, caused by outward pressure of the expanding brain.
Mrs Jayamanee, who works as the manager of the shipping division of an international freight forwarding company, said that she wanted to help other parents with her story.
Her husband, K. Thrumalinggam, 37, is a land surveyor, and they have a five-year-old daughter who is normal. Other members of both families are also normal.
On Thursday, The Straits Times visited baby Nakulan at his parent’s Yishun flat.
His mother looked at him gurgling and paddling about in his stroller and said: “I know how much pain I went through when I found out he had this problem. Now I think I made a very good choice. He is developing well. He can wave good-bye, and gives his father a high-five.”
The cost of the two operations on Nakulan would have been about $30,000 but the family paid only $5,000 as the baby was put into a subsidised ward.
The Straits Times (Singapore)
October 18, 1995
Reshaping a newborn’s misformed skull
By Lim Li Hsien
IT IS like taking off the shell of the top of a hard boiled egg, said the surgeon who reshaped baby Nakulan’s head when he was one month old.
“His head was pointy, like the top of an egg,” said Dr Woffles Wu, a craniofacial plastic surgeon at the Singapore General Hospital (SGH).
“We took it apart like Humpty Dumpty, only here, we have put him back together again.”
Nakulan, now nine months old, was born with craniosynostosis, a rare birth defect in which the skull cannot grow, compressing his brain. This put him in danger of growing up mentally retarded.
Last year, SGH saw seven patients with the same deformity.
Dr Wu operated on Nakulan’s skull with head of neurosurgery Dr Prem Pillay, also of SGH, The Straits Times reported on Saturday.
Dr Pillay removed the bone over the dangerous areas in the brain.
Dr Wu removed the bone around the eyes and reconstructed the head skull after removing the bone that could not grow.
Nakulan had to be anaesthetised for the two operations it took to transform his skull. Both took up to eight hours.
It can take four to five operations to correct this defect, said Dr Wu.
After the operation, the head swells like a football. Care of the baby is crucial, as the back of the skull is left without bone, making the head very fragile. It may have to be protected by a helmet.
Infection can be dangerous and cause meningitis, which can be fatal. The baby has to go for frequent checks with his plastic surgeon, neurosurgeon and paediatrician.
In Nakulan’s case, he needed only a bandage, not a helmet, during the recovery phase, and he did not fall prey to infection.
Now, four months after his second operation in June, his skull has grown back. Doctors continue to monitor his progress.
The plastic surgery techniques used in such cases are very new, said Dr Wu, and were pioneered by French plastic surgeon Dr Paul Tessier, now in his 80s.
A third-generation craniofacial surgeon, Dr Wu, 35, had performed this surgery first under Australian surgeon Dr Anthony Holmes, who had learnt his techniques from Nobel prize-winning plastic surgeon Dr Joseph Murray of Boston.
The clover-leaf skull deformity: Baby Nakulan’s skull reconstructed step by step.
1. A normal skull is not a solid piece of bone, but made up of plates of bone joined by fibrous tissue called sutures. But with a clover-leaf skull deformity, shown above, the sutures of the brain fuse and the skull stops growing. The brain is compressed and spills out through the gap at the top of the head where a suture has not fused.
2. The surgeon cuts the skull along the dotted lines. He removes the top of the skull, and bone around the eye in the shape of a spectacle frame. The expansion of the brain had caused the eyes to bulge, making them vulnerable to blindness.
3. The skull is cut and disassembled, exposing the brain. The pieces are trimmed, removing the fused sutures. Only bone which can still grow is reused. A new forehead is reconstructed using the trimmed remnants of the bone, and the “spectacle frame” for the eyes is moved forward to give the brain room to expand.
4. The bone is joined using tiny plates and screws, made of titanium or like material which can stay in the skull indefinitely.
There is not enough bone to cover the entire brain so the back and top of the skull is left bare. But a baby under a year in age can grow his own skull back, a process which takes about three to six months.
The Denver Post
October 29, 1995
The epidemic that wasn’t Scare faded under glare of scrutiny
By Robert Kowalski, and Ann Schrader
Dr. Andrew Rhea didn’t notice anything unusual when he began his first-year residency as a neurosurgeon at Children’s in June 1984. But by his third year, that had changed. There were-to him-too many CS surgeries and too little hard evidence showing they were needed.
“I wasn’t happy about it,” said Rhea. He said he took his concerns to Dr. Glenn Kindt, head of the CU medical school’s neurosurgery program.
And he shared his qualms with “other professors that were there, and I let them know I didn’t think the thing was exactly on the up and up, and it just didn’t seem like the thing to do.”
Rhea, now in private practice in South Carolina, said he probably assisted in “several hundred” of the CS surgeries as a resident. Whether the operations were needed, Rhea said the surgeons themselves were competent.
One of his duties was to take pre-operative medical histories and physicals on the children the night before surgery. He’d ask the parents what abnormalities had they noticed about their child. What he heard from many was disconcerting. A parent would tell him, “I don’t really know,” Rhea said.
Rhea said even parents who could see nothing wrong with their children often felt pressured by the surgical team to go ahead with surgery.
According to Rhea, Hendee would tell parents that he couldn’t say how their children would turn out if they didn’t have the operation.
“Why those kids’ families didn’t get second opinions from somebody else just baffled me,” Rhea said. “If somebody’s going to operate on my kid for something that I didn’t understand, I wouldn’t get just a second opinion. I’d get three or four.”
There was plenty of blame for what happened, Rhea said.
“It’s not just the doctors’ fault. It’s in a lot of ways the parents’ fault, the referring doctors’ fault, the hospital,” Rhea said. “Everybody shares some responsibility for all those cases that were done if they were done unnecessarily.”
Rhea certainly was not the only neurosurgeon who thought something was amiss at Children’s. The team had become controversial around the country as word of the rate of Colorado CS spread.
In December 1986, Dr. Larry McCleary, who had recently become Hendee’s partner, learned just how deeply the feelings ran.
Addressing a conference of pediatric neurosurgeons in Pittsburgh, McCleary tried to explain the team’s latest medical finding:
Some parents were bringing in children with no obvious facial or cranial deformities. But the children were fussy, crabby, had trouble sleeping and were behaving as if they had headaches. And their skulls weren’t growing as quickly as their bodies, McCleary said.
Suspecting these children might be suffering from increased intercranial pressure even though they didn’t have skull deformities associated with severe craniosynostosis, the team drilled a hole into each child’s skull and inserted a pressure-monitoring device. McCleary said they performed the procedure in 10 to 20 such children.
The reaction to his report at the conference was swift and harsh. The neurosurgeon’s questioned McCleary’s presentation.
“How in the world did he justify monitoring these children? Can you imagine what we will be going through if we monitor intracranial pressure in children with behavioral disorders?” said Dr. Joan Venes, a University of Michigan neurosurgery professor.
She was critical of Children’s Hospital doctors for operating to measure the brain pressure of the children who didn’t have the skull deformities of CS. It was-to her-unjustified.
But the discussion at the neurosurgery conference quickly spread to the broader issue of the craniosynostosis situation in Colorado.
The presentation “blew the roof off,” recalls Dr. John Shillito, a retired pediatric neurosurgeon at Boston’s Children’s Hospital. “It provoked as much comment as you could ever imagine at a meeting.”
The neurosurgeons ripped into McCleary’s presentation and then began demanding information about the CS situation in Colorado.
McCleary had been scheduled to give a short presentation. It was not. Even after breaking for lunch, the neurosurgeons could talk of little else. They resumed the debate again in the afternoon.
“I think he was just shot,” Shillito said of McCleary.
Shillito later served as a consultant in a craniosynostosis malpractice lawsuit against some of the doctors performing the surgeries in Denver.
“Nobody agreed with (McCleary),” added Dr. John Laurent, chief of pediatric neurosurgery at the Baylor College of Medicine, who also attended the session.
McCleary agrees that the other neurosurgeons at the conference were really more interested in what was going on with craniosynostosis back in Colorado than in the small study on brain pressure.
“I think that the paper was forgotten as soon as I stopped talking, to tell you the truth. I don’t think people cared about that,” he said.
“They were very straightforward. They said: ‘How many cases of craniosynostosis are you doing in Denver? Or how many cranial surgeries have been done in Denver?” McCleary said.
Venes says the debate that began in Pittsburgh continued to rage for months among the nation’s 100-or-so pediatric neurosurgeons.
Dr. Tom Vernon, then executive director of Colorado Department of Health, said, “One physician at Children’s was seeing cases behind every tree as I remember.
“Frankly, this is a fairly classic epidemic curve when the epidemic turns out to be an artifactual one,” he said.
About the time of the Pittsburgh confrontation, Children’s organized a conference at United Bank that was widely advertised to the medical and nursing communities. The conference, titled “Controversies in Craniosynostosis,” revealed that CS diagnosis and the need to correct it with surgery weren’t clear-cut. Some left the conference more in doubt than before.
“As a result of the conference some people began to feel more comfortable (not operating on children) with less-severe degrees of craniosynostosis,” said Dr. David Tubergen, Children’s medical director from 1981 to 1991.
In the mid-1980s, the insurance industry was also undergoing change and was reviewing its policies toward medical coverage, said Carl Miller, a spokesman for Blue Cross and Blue Shield of Colorado.
At Blue Cross, craniosynostosis surgery was one of the medical procedures that was evaluated, Miller said, although he could not say if the coverage for the treatment changed.
“It may be coincidental, it may not be, I can’t answer that,” Miller said, referring to the situation with craniosynostosis in Colorado. “But I can just tell you that certainly that was the same time that our company—and we presume other insurance companies—started to do more critical review of medical procedures.”
Looking back, Children’s President Lua Blankenship says the fact that the craniosynostosis surgeries were being questioned shows the medical system worked.
“The thing that I thought was good about that is that it was a very open process,” Blankenship said. “There was this collaborative questioning and investigation, when you identify what you believe to be a trend in medicine you begin to, you begin to ask questions like what’s the cause here? What’s going on? Am I going to try to second-guess a surgeon on whether they should use surgery A or B? No, but I’m certainly going to ask the medical staff to be sure they have tuned into the process,” Blankenship said.
Three months after the 1986 Pittsburgh neurosurgery conference, the American Association of Neurological Surgeons contacted Dr. Kindt at the neurosurgery program at the CU School of Medicine.
“I am sure you are familiar with the craniosynostosis ‘problem’ in Colorado. As you know, it has caused a lot of discussion and some concern on the part of many,” the association wrote. Ominously, the letter said the association’s board was “considering whether it should take any investigative action or study of this problem.”
For Kindt, this was big trouble. McCleary and Hendee were on the CU faculty. And that made CU vulnerable, Kindt thought.
“They could come and investigate, and if they thought it was inappropriate, for what was being done, the University of Colorado could be involved in losing their training program,” Kindt said. “From my viewpoint, the best thing to do was to drop the training immediately. So we stopped training with the neurosurgeons at Children’s at that time, and we’ve never had any association with them since.”
Kindt rescinded McCleary’s and Hendee’s faculty status. No longer would CU neurosurgery residents rotate through Children’s Hospital under these surgeons.
“There is nothing, I think, more prized than your residency program,” said Dr. Richard Krugman, CU medical school dean for the past five years. “If an outside group looks at you and says, ‘We’re concerned about something,’ and if you want to keep your residency, most people respond.”
Although Hendee and McCleary obviously knew of the residents being pulled, they say they weren’t told about the end of their clinical faculty designation.
“I am really furious about that,” Hendee told The Post. “I may sue Dr. Kindt.”
McCleary said he didn’t learn of loss of faculty status until six years later. And he dismisses Kindt’s decision to pull residents as part of a power play between the two institutions.
McCleary noted that Children’s and the CU academic center were then hammering out an affiliation arrangement and said the medical residents were being used as pawns.
“There was a lot of negotiating at that time,” he said. “Around that time, residents were a bargaining tool.”
At first, Dr. Stan Ferguson, then Colorado’s state epidemiologist, was a true believer. At the height of the craniosynostosis panic, he was quoted as saying the situation was “a full-scale epidemic.”
But Ferguson did something at the beginning of the “epidemic” that would eventually cast the deeper doubt about its existence. He contacted the federal Centers for Disease Control and Prevention in Atlanta to help him find out why there was a sudden outbreak of CS in Colorado.
Two months after the neurosurgery association contacted Kindt, the CDC completed the first part of its study. The results supported the suspicions being voiced around the nation.
Between 1978 and 1982, Colorado’s CS diagnosis rate was 7.4 per 10,000 live births, more than twice Atlanta’s rate. (The CDC used Atlanta as a “control city” since it was conveniently located, had a long history of tracking birth defects and had a similar population size).
The second part of the CDC study surveyed the craniosynostosis incidence in Colorado and Atlanta again in 1986. It concluded that between 1983 and 1985, Colorado had nearly five times as many children diagnosed with craniosynostosis as did Atlanta.
Differences in the criteria the Colorado surgeons used for diagnosing a child with CS, and the way patients were referred to the Denver surgeons, “apparently account for a large portion” of the disparity between Colorado and Atlanta, the CDC noted.
The agency also compared Colorado and Atlanta in another way. Neurosurgeons in Atlanta and at Children’s Hospital were asked to submit X-rays of 60 of their most-recently referred patients.
Dr. Sandra Fernbach, a pediatric radiologist at Children’s Memorial Hospital in Chicago, read the X-rays as an independent consultant. The origins of the films weren’t revealed to her.
About two-thirds of the Colorado cases and a third of the Atlanta cases were classified by Fernbach as not having craniosynostosis.
Though the CDC concluded diagnosis and referral patterns largely explained the Colorado situation, university students in Colorado studied other possibilities. In 1987, water, mine tailings, and even Rocky Flats were reviewed and found not to be likely causes of craniosynostosis, several studies said.
One graduate student found that being male, having a white or an older mother, being born with low birth weight or labor complications and exposure to high altitude may be possible risk factors.
Research by Dr. Beth Alderman, a former assistant CU professor who studied CS, indicates that pregnant women who smoke or exercise at high altitude may increase a child’s chance of developing CS. She believes panic and perception led to heightened awareness about the disease.
And Dr. Jose Cordero, who headed up the CDC investigations, told The Denver Post that there is no remaining puzzle:
“An epidemic means that there is a definitely confirmed disease,” he said. “What we confirmed in this is that there were differences in diagnosis. ... I don’t think we can call that an epidemic.”
Dr. Albin said that over the years he’s learned there is actually little agreement about any aspect of craniosynostosis. That became particularly clear, he said, when he and other surgeons met at the CDC to discuss the situation in Denver.
“It’s a gray zone. And that’s the problem that we have with craniosynostosis,” he said. “And when we sat down there in Atlanta and showed X-rays to each other, that was a big surprise to me.”
Albin, however, insists the Denver team of surgeons operated always in good faith, taking greater care in diagnosing and operating on craniosynostosis than similar teams around the country.
For example, he said, the Denver surgeons took samples of bone removed from skulls during some of the CS surgeries and had them examined by a pathologist to help verify that the disease was actually present.
“I felt confident at that point that we were doing something that no other group was doing,” Albin said.
Hendee said the reaction McCleary received at the Pittsburgh neurosurgery conference didn’t sway what he and his team of surgeons were doing in Denver.
“I have always approached things honestly and with the understanding, my God, I would never do anything that would put a child in jeopardy unnecessarily. Never, ever,” Hendee said.
“The children who came to surgery were, in my humble opinion and with referring physicians, and radiologists and so forth concurring, were in need of surgery.”
But the situation has, in fact, changed. Following the fiery conference in Pittsburgh, the CDC report and the academic actions against the neurosurgeons on the team, the rate of craniosynostosis in Colorado suddenly plunged. From about 225 operations in 1986, Children’s surgeries for CS fell to 43 in 1994. Through September of this year, Children’s has performed only 16 such operations.
The so-called epidemic has gone away.
Though surgeries are down, the current Children’s Hospital team—Hendee retired in 1989-still stirs controversy, not over the number of surgeries but over technique.
Two and a half years after Hendee’s exit, on Feb. 15, 1992, Valerie Kerin gave birth to identical twin girls—Veronica and Chelsea. They were “perfectly normal” at birth, according to her mother.
But in June 1992, the Kerins’ family pediatrician suggested they take Veronica to see McCleary at Children’s Hospital. McCleary concluded Veronica had sagittal synostosis—the growth opening over the top of her skull from the front to the back of her head closed prematurely.
Known among Denver surgeons as a “total calvarial reconstruction,” the technique involves removing almost the entire skull, breaking it into pieces, connecting them back together with wire or some other material and replacing the bone over the child’s brain.
The Children’s Hospital doctors say they end up with a better-looking child when they use a more extensive surgical procedure on the skull—though other surgeons are highly critical of that type of operation.
On Aug. 26, 1992, Veronica Kerin entered the operating room at Children’s. As Albin was performing his portion of the surgery, Veronica’s heart stopped. The doctors resuscitated her, reattached a portion of her skull and ended the surgery earlier than planned.
Veronica now has difficulty seeing, is behind her twin sister in learning and is physically scarred from the procedure. The Kerins filed a medical malpractice lawsuit against Children’s and the surgeons. The case is scheduled to go to trial next March.
Medical experts contacted in the case have given a wide range of opinions about the type of operation the surgical team was performing.
Dr. Ian Munro, a plastic surgeon in Dallas who is world-renowned in the field of craniofacial surgery, said the procedure was excessive.
“An eight-hour operation for this problem in a 6-month-old child is unacceptably long, and this is what the surgeons were anticipating,” Munro wrote in a letter to Veronica’s lawyer, James Avery. “I can see no evidence of adequate preoperative investigations, no evidence for the enormity of the surgery that was planned when a lesser operation could have sufficed.”
Munro is a witness for Veronica.
Dr. Benjamin Carson, director of pediatric neurosurgery at the Johns Hopkins University Hospital, said he no longer performs the extensive surgery. “I’ve never had any fatalities in doing it, but I just think the risks are too high, that the risks outweigh the benefits in that situation,” he said.
Carson added, however, that he isn’t critical of those surgeons who do perform the more extensive surgery.
Other experts have said the extensive skull surgery approach is appropriate in some cases. Dr. Venes of the University of Michigan said there are different ways to do a “total calvarial.” Many surgical teams do it in two surgeries instead of one due to the potential for significant blood loss, she said.
Further, Venes said even when it is clear a baby has CS, there is not universal agreement that any surgery is medically necessary.
Albin insists the outcome of Veronica’s operation was an anomaly, and the only time his team experienced a cardiac arrest during a CS surgery. “I will put our track record of safety up against any craniofacial center in the world,” he said.
And aside from the extensive surgery the doctors performed on Veronica, Albin said what the surgical team did with craniosynostosis in Colorado in the 1980s should be viewed in perspective.
“If you stand back and look at the big picture, I don’t think you’ll come to the conclusion that there was an epidemic necessarily, and I don’t think you’ll come to the conclusion that we’re evil scoundrels who were operating willy-nilly on the patients that we just kind of created out of thin air.”
The Denver Post
October 29, 1995
Illness splits twins’ lives
By Ann Schrader and Robert Kowalski
To know what could have been, Valerie Allen can just look at her 3 1/2-year-old identical twin daughters.
Despite their blond hair and similarly compact bodies, their identities can’t be confused. Chelsea is shy around strangers. Veronica quickly hops into laps and tells just about everyone that she loves them.
Chelsea has a sunny smile; Veronica’s face has a slightly skewed appearance. Chelsea can see; Veronica’s vision is limited. Chelsea didn’t have her skull surgically removed; Veronica did.
On Aug. 26, 1992, when she was 6 months old, Veronica Kerin underwent an operation at The Children’s Hospital to correct a form of craniosynostosis—the “expansion joint” running the length of her skull wasn’t open as it should have been.
Surgeons were removing her skull from ear to ear and from her eye orbits to the back of her head. While she was on the table, her heart stopped. Doctors halted the operation while they resuscitated her. Her heart began beating. The doctors quickly closed her incisions.
Experts differ about what caused her cardiac arrest. Some, serving as experts for Veronica’s parents in their medical malpractice lawsuit against Children’s and the surgeons, say the type of surgery performed spurred so much blood loss that it caused the girl’s heart to slow at one point and then to stop. Her doctors, however, say the surgery was proper.
They say Veronica went into cardiac arrest because she might have suffered from a venous air embolism—a bubble of air got into her bloodstream during the surgery.
The trial in her parents’ lawsuit is scheduled for March in Denver District Court. But one thing isn’t disputed: Veronica has never been the same.
“They were like two peas in a pod,” said the girls’ mother, Valerie Allen. “Well, they certainly aren’t now.”
Allen said she will never forget what one doctor said after the surgery. “One of them told us that she would be a vegetable for the rest of her life,” she said.
“We took home a 6-month-old newborn, basically,” Allen said. Veronica had to learn how to suck out of a bottle again. She missed the usual childhood development milestones.
Later, Veronica underwent two operations to repair the gap in her skull. New doctors removed six of her ribs, split them in half and used the pieces to replace the missing skull bone.
No one has made a prognosis about what lies ahead for Veronica, Allen said. Several experts say they want to wait until she’s a bit older to be able to understand how far she might develop.
The twins were born Feb. 15, 1992. In their first months, Veronica was “about one step ahead of Chelsea in rolling over and in holding her head up,” Allen recalled.
But when Veronica was about 4 months old, her pediatrician noticed that her head was slightly smaller than Chelsea’s.
Allen had never heard of “craniosynostosis” when her pediatrician raised the term. The pediatrician gave Allen the name of Larry McCleary, a neurosurgeon who practices at Children’s Hospital.
At the time, Allen didn’t realize Veronica’s head shape was abnormal.
McCleary ordered X-rays, which showed craniosynostosis. Using his own head, Allen said McCleary explained the kind of surgery he would do—an entire head reconstruction called a total calvarial remodeling.
Allen said she and her husband were told surgeons would reconstruct Veronica’s skull on another table and place it back on her head. There might be gaps that would grow together after a while.
McCleary, Allen said, told them it was best to do the surgery before Veronica was 6 months old. He said the deformity “was probably causing pressure to her brain. We did ask what happens if we wait. We were told that she could experience very severe deformity and brain damage and possibly die,” Allen said.
McCleary said he didn’t want to discuss the case because the lawsuit hadn’t been decided.
But in a deposition he gave in April in the case, he denied telling the Kerins that Veronica would suffer brain damage or die without surgery.
“I don’t think I have ever seen, read or heard of anyone dying of craniosynostosis, treated or not treated,” he said in the deposition. “Brain damage is not something that is generally listed as being a consequence of untreated craniosynostosis.”
The family also consulted Dr. Richard Albin, a plastic and reconstructive surgeon who would be assisting with the surgery.
Allen said she told McCleary that Veronica had been diagnosed with a minor heart defect. Veronica’s cardiologist cleared her for surgery, which was covered by Medicaid.
The family didn’t think about going somewhere else or getting a second opinion. “To this day, I don’t know why (we didn’t),” Allen said. “I guess because we felt pretty comfortable with it, from what we were told about it” and about the surgeons.
Allen is “aggravated” that she wasn’t given more choices, both in surgical approaches and in alternatives. She’s interested in a group of Arizona doctors that uses medical bands around a child’s head to mold the skull.
Shortly after McCleary emerged from the operating room and Albin began his portion of the surgery, the family felt comfortable enough to lunch downstairs. Then the procedure ended about two hours early. The family was told Veronica was alive, but there had been “some problems.”
The doctors explained Veronica had gone into full cardiac arrest and she had to be resuscitated. Allen began demanding to see her daughter.
About 20 minutes later, Allen walked into the intensive-care unit. “I saw this little tiny baby with a whole bunch of bandages on her head, with tubes and wires hanging out everywhere, machines beeping and wires going all over,” she said, sobbing at the memory.
“Her whole face was swollen-I just wanted to pick her up and take her away.”
A day or two later, Allen noticed Veronica wasn’t seeing. “By the time she could open her eyes, she wasn’t focusing on anything. She was staring into space,” Allen said. Veronica’s eyes were moving, but they “just saw right through you.”
The extent of Veronica’s vision can’t be measured yet. She can see colors and “pick up the tiniest stuff off the floor,” Allen said.
To compensate, Veronica has learned to use her other senses, such as touching walls to walk around a room. In the first two years after her surgery, Veronica devoured the world with her sense of touch.
“She couldn’t get enough from her hands. She licked the walls; she did everything with her mouth,” Allen said.
The first year and a half after the surgery was “awful,” Allen said.
“Back then, Ronnie wasn’t walking. She wasn’t happy to do anything,” Allen said. “We’d have birthday parties and Ronnie was in the background on the floor, crying and cranky. She was a very, very irritable child.”
Chelsea started walking a week before her first birthday. Veronica took her first steps on her second birthday. Without therapy for two years, Allen believes Veronica wouldn’t have been “anywhere.”
Chelsea was toilet-trained at 2 1/2 years, and Veronica still rustles about in diapers. Chelsea is in a preschool group of kids her own age. Veronica is grouped with toddlers.
Veronica doesn’t understand where the safety of yard and sidewalk ends, and the dangers of the street begin.
Allen and the twins’ father, Robert Kerin, divorced in December 1994. Their already-fragile marriage couldn’t withstand the strain of Veronica’s condition. Kerin lives in the metro area and sees the twins every week.
Recently, Veronica was evaluated at the Fletcher Miller-Handicap School in Lakewood. Tests showed her fine motor skills are delayed by eight months, her gross motor skills are about six months behind average, and her cognitive skills are lagging anywhere from 18 to 24 months.
But her speech is at the level it should be. Her words come out with a crisp twist. “Are you my friend?” she asks, though her trusting tone implies she already has decided.
The worst part, Allen said, is having a twin with whom to compare Veronica.
“That’s very hard to deal with, because you see one and then I look at Chelsea and this is the way that Veronica should be.”
The Denver Post
November 19, 1995
Parents claim surgery pushed Doctors deny saying disease was deadly
By Robert Kowalski and Ann Schrader
Faced with surgery on their babies’ skulls, the parents of eight children say a Denver team of doctors told them their children could become blind, brain damaged or die without the complicated operations.
The doctors say the children faced no such perils and deny ever pressuring parents to approve the surgery.
The parents say they were told their children had craniosynostosis, a rare disease in which the normal growth openings of the skull are closed. In this condition, the skull can become misshapen as the child’s brain grows.
Six of the babies underwent the costly surgeries during which parts of their skulls were removed, reshaped and replaced. Two others resisted the option and made the agonizing decision to bypass surgery. Now, years later, they say their kids are bright and their appearances normal.
The doctors who performed the CS surgeries acknowledge in sworn statements and interviews that there is no medical proof that death or mental retardation will result for a child who has the condition but does not receive a skull operation.
The parents, responding to an Oct. 29 Denver Post report that the “epidemic” of craniosynostosis in Colorado didn’t actually exist, say they were told something quite different.
For example, in a sworn affidavit taken Nov. 6 as part of a lawsuit, Sherrie Bean said Dr. Larry McCleary told her and her husband, Ed, in 1988 that unless their 4-month-old son, Kyle, was operated on he would have “brain damage and/or die.” McCleary, a pediatric neurosurgeon, is a member of the team of doctors that performed the majority of the craniosynostosis surgeries at Children’s Hospital.
“At no time did Dr. McCleary indicate to either me or Kyle’s father that his surgery was only cosmetic,” Sherrie Bean stated. “Dr. McCleary made me feel as if I had no options but this surgery and that the surgery had to be performed as soon as possible. I was afraid that Kyle would die without it.” Kyle had the surgery.
During a deposition taken in a lawsuit related to another craniosynostosis surgery, McCleary contradicts Bean’s account of what he told families of children diagnosed with the condition.
He said team members would “go over roughly how the surgery is done, what the risks are and basically say: ‘This is not like having cancer. I mean, your child is not going to die from this condition.’”
He also said he could not recall ever having “seen, read or heard of anyone dying of craniosynostosis, treated or not treated. Brain damage is not something that is generally listed as being a consequence of untreated craniosynostosis.”
McCleary has declined to discuss the case with The Post, citing the pending litigation.
That case involves Veronica Kerin, a 3 1/2-year-old who underwent total skull reconstruction surgery in 1992. Her parents, Bob Kerin and Valerie Allen, said in court documents that the surgery left Veronica with developmental delays and vision damage.
McCleary, they said, told them that CS “was probably causing pressure to her brain” and that Veronica could experience a very severe deformity, brain damage and “possibly die” without surgery.
Among the parents who have contacted The Post with similar comments is Colorado Springs resident Kim Plath. Plath said Dr. Robert Hendee, a CS team member at Children’s, warned her in 1987 that her son, Caleb, would have brain damage or go blind without the surgery.
“You tell a parent that and they feel pressured to have surgery,” Plath said. “He never made us feel, ‘Just wait.’”
Hendee, who retired in 1989 and lives in Austin, Texas, insisted he never told parents their children could die without the surgery and said he did not pressure families into approving the skull operation.
“No, no, I never told any parent that,” Hendee told The Post. “I made it an absolute point never to tell any parent that the child was going to have brain damage or anything like that. I said we do not know.
“I never, ever, to my knowledge, ever-and I made a specific point of this-never, ever tried to frighten a parent, saying their child was going to be brain damaged or anything like that. Never, ever.”
Terry and Sue Hollens of Englewood disagree with Hendee’s recollection. They said they decided on surgery for their son, Matt, after Hendee told them that pressure inside his head could increase, causing mental retardation or death.
Matt had two surgeries by the time he was 13 months old. Today, he is a typical 10-year-old boy and has suffered no ill effects.
Another parent who disagrees with Hendee’s account is William Kershner of Englewood.
“Me, not being a medical-type person, I was given the impression that, that I was putting my son at risk if we do not do the surgery,” said Kershner, whose son, Brandon, was operated on at Children’s for craniosynostosis in the mid-1980s.
Kershner said Hendee “told us that he didn’t know for sure what, what would happen if we didn’t do the surgery, but (Brandon) could go blind.”
In a medical malpractice lawsuit they filed against Hendee and other Children’s Hospital doctors, the parents of Jeremy Brassington said they were told their son needed skull surgery immediately for craniosynostosis.
Without the surgery, the Brassingtons said they were told that their son “would suffer severe, serious and debilitating permanent injuries, including brain damage,” according to court papers.
The parents approved the 1985 surgery, but later two other neurosurgeons told them they could not detect the CS on X-rays.
Earlier this year, the Brassingtons settled the suit and agreed as part of a confidentiality agreement not to discuss terms of the settlement.
Karen Cook and Suzanne Heiser also said Hendee told them their children would suffer brain damage or death without surgery. Both families decided against it.
Cook, who lives in Summit County with her husband, Paul, said Hendee “discouraged us from getting a second opinion” on their daughter Keelyn’s condition. “He said he was the expert, that no one else in the surrounding states could do a second opinion, and that he had never been wrong about doing the surgery.”
Cook said Hendee told her in December 1985 to call the next day and set an appointment for surgery the following week.
“It was so scary to me,” Cook said. She was ready to go ahead with surgery, but her husband insisted on a second opinion. They flew to Atlanta, where a neurosurgeon told them that, in his opinion, Keelyn didn’t have CS.
Keelyn is now 11 1/2 years old, “looks just fine, and is very bright,” Cook said.
In 1986 Heiser, of Greeley, was concerned about the shape of her son Cody’s head. At first, a pediatrician told her not to worry. But she did.
When Cody was 7 to 9 months old, the situation became more urgent when suddenly the pediatrician, saying he had heard about CS, told Heiser to take Cody to Children’s to have her child checked.
Hendee told Cody’s parents the situation was “life-threatening” and surgery was necessary, Heiser said.
“We were basically told we had no other options. There was no other place for us to go, that nobody else had as much experience with this type of surgery,” Heiser told The Post.
Doctors wanted to do a total reconstruction of Cody’s skull, which would involve moving his eye sockets, realigning his ears and reshaping his forehead, Heiser said.
A day before the surgery, Heiser decided to postpone it. “The thought of it all was just too much,” recalled Heiser, who was pregnant at the time. “I said I can’t do this until I’ve exhausted all other possibilities.”
On one hand, she feared that without surgery Cody would be a “really gross-looking child” with medical problems. On the other hand, she worried about the stresses of surgery and the resulting scars “on this adorable little boy.”
She decided to get a second opinion.
The neurosurgeon who next examined Cody’s X-rays couldn’t confirm the CS diagnosis. He suggested a CT scan. The Heisers said they sat in a hospital waiting room as Cody had the scan, worried and uncertain.
“We were afraid we would hear that this has got to be done, or we would hear something else. We didn’t know for sure what the something else was,” Heiser said.
They didn’t expect the neurosurgeon to come out and say, “Your son is just fine. Take him home and let him grow up.” But that’s what he said, and that’s what they did.
But the decision against surgery wasn’t without a cost. “We agonized whether we had done the right thing, wondered if maybe the second doctor had missed something, or wasn’t skilled enough. We went through this horrible, horrible pain, agonizing over it, watching everything,” Heiser said.
Cody now is 10 years old, attends the fifth grade, is bright and is not odd-looking, Heiser said.
“He could have had a minor case of it (CS), but certainly not enough to operate,” Heiser said.
The Denver Post
December 9, 1995
Parents probe skull surgeries
By Robert Kowalski and Ann Schrader
The parents of nine children who were operated on for a rare skull disease at Denver’s Children’s Hospital demanded yesterday that the hospital appoint an independent panel to investigate whether the surgeries were appropriate.
An attorney for the parents said the panel should review how a team of surgeons at the hospital operated on children diagnosed with craniosynostosis, a medical condition in which a child’s head is misshapen and one or more of the skull’s “expansion joints” are not open.
Since 1978, doctors at Children’s have performed skull surgery on more than 1,200 children diagnosed with the condition. Though the Children’s team was criticized by fellow neurosurgeons from around the country in 1986 for doing the operation too frequently, hundreds of CS surgeries continued to be performed.
During the mid-1980s, more corrective surgeries for craniosynostosis were carried out in Denver than any other city in the nation.
“I would remind you that behind these names are live human beings who were subjected to major neurological surgery and who are living with the emotional and physical scars and disfigurement,” attorney James Avery wrote in a letter to the vice chairman of Children’s board of directors.
“They will not have peace of mind if this matter is not resolved in an open and objective matter,” he continued. “The only way, in our view, to conduct a reliable review of the situation, is to appoint an independent panel of physicians who are experts in the field of craniosynostosis surgery.”
Children’s chief executive officer, Lua Blankenship, said yesterday through a spokeswoman that he was not going to respond to the request.
“We cannot respond to a third party requesting an investigation, whether that’s from the The Denver Post or from an attorney,” Children’s spokeswoman Jackie Brown quoted Blankenship as saying.
Blankenship said last week that the hospital will not review its handling of craniosynostosis cases unless it is contacted directly by families of the children who underwent the operations.
“He encourages all parents to call in to obtain their medical records to get a third-party review if they would like to or to call their physician, or contact him directly,” Brown said.
The Children’s board of directors held its regular monthly meeting Thursday and discussed craniosynostosis issues raised in a Denver Post investigation, but took no action, Brown said.
A critical issue is exactly what doctors told parents before the surgeries.
Many parents have said doctors at Children’s told them their children could become blind, or retarded or possibly die if they did not have the operation. The doctors adamantly deny they made such comments and agree there is no medical basis for the claims.
Other parents have told The Post they were not pressured into approving the surgery. Avery is already representing one child who underwent craniosynostosis surgery in a medical malpractice suit that is currently pending against Children’s Hospital and doctors on its CS surgical team.
That child, 3 1/2-year-old Veronica Kerin, suffered a heart attack while the doctors were operating on her skull for CS in 1992. Veronica had to have additional surgery to repair holes left in her skull from the first operation.
Today, she has limited vision and developmental delays, according to the lawsuit, which is scheduled to go to trial early next year.
Valerie Allen, Veronica’s mother, said yesterday that she wants to locate the families of other children who underwent craniosynostosis to form a support group.
Allen said she is one of the parents attempting to get a review of how the CS cases were handled over the years at Children’s Hospital.
“Why not? Why don’t they try to figure out what was going on?” she said. “They just kind of let it go. That’s not fair. It’s not fair to the patients, the kids.”
The number of surgeries performed at Children’s for craniosynostosis rose dramatically in the early 1980s, and peaked in 1986 when, according to the hospital, 225 children underwent the operation.
Called in by the state health department, the federal Centers for Disease Control investigated the craniosynostosis situation in Colorado and concluded the large number of CS surgeries in Colorado was largely due to the Denver surgeons’ broader criteria for diagnosis and to the number of referrals to Children’s.
In late 1986, Dr. Larry McCleary, a pediatric neurosurgeon who is a member of the current Children’s CS surgery team, was heavily questioned by colleagues at a national conference of pediatric neurosurgeons in Pittsburgh.
Among those asking questions at the meeting was Dr. Marion Walker, a Salt Lake City pediatric neurosurgeon who currently is the president of the American Society of Pediatric Neurosurgeons.
“I got up at the meeting and stated that I had seen a number of the children in consultation in Salt Lake and that of the ones that I had seen I had not seen one yet that had true synostosis,” Walker said of children that had been diagnosed by the Denver doctors as having CS.
“The discussion got pretty heated regarding what was going on” in Denver, he said. “It’s quite famous actually.”
After the Pittsburgh meeting, McCleary and Dr. Robert Hendee, another neurosurgeon on the Children’s team who has since retired, had their teaching privileges at the University of Colorado School of Medicine removed.
Surgeries for craniosynostosis then dropped at Children’s, but nonetheless continued, with 110 in 1987, 76 in 1988, and 59 in 1989. This year, as of September, there were 16.
Avery said the independent review would be for more than just the families that he is representing. “Parents who have agonized for upwards of 10 years whether the surgery on their normal appearing and acting children was necessary find this inquiry extremely relevant.”