Pain & Central Nervous System Week
January 1, 2007
Craniosynostosis genetics; study data from University of Oxford, Weatherall Institute of Molecular Medicine provide new insights into craniosynostosis genetics
Data detailed in “Clinical dividends from the molecular genetic diagnosis of craniosynostosis” have been presented. According to recent research from Oxford, the United Kingdom, “A dozen years have passed since the first genetic lesion was identified in a family with craniosynostosis, the premature fusion of the cranial sutures. Subsequently, mutations in the FGFR2, FGFR3, TWIST1, and EFNB1 genes have been shown to account for approximately 25% of craniosynostosis, whilst several additional genes make minor contributions.”
“Using specific examples, we show how these discoveries have enabled refinement of information on diagnosis, recurrence risk, prognosis for mental development, and surgical planning. However, phenotypic variability can present a significant challenge to the clinical interpretation of molecular genetic tests,” wrote A.O. Wilkie and colleagues, University of Oxford, Weatherall Institute of Molecular Medicine.
The researchers concluded: “In particular, the difficulty of analyzing the complex interaction of genetic background and prenatal environment in determining clinical features, limits the value of identifying low penetrance mutations.”
Wilkie and colleagues published their study in American Journal of Medical Genetics Part A (Clinical dividends from the molecular genetic diagnosis of craniosynostosis. American Journal of Medical Genetics Part A, 2006;140(23):2631-9).
For additional information, contact A.O. Wilkie, John Radcliffe Hospital, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK.
Bury Free Press
January 12, 2007
Hannahs Home after Op Setback
The parents of a toddler who underwent major skull surgery have spoken of their joy at being able to in the New Year at home together, despite setbacks that forced her back into hospital.
Three-year-old Hannah Webster, from Ingham, has a condition called craniosynostosis, where one of the growth points in her skull fused before birth and is now causing pressure on her brain.
Hannah was originally treated by specialists in Oxford at the beginning of November, when she was given a new forehead. But just a few weeks after returning home, mother Liz said Hannahs scar-line opened up and she had to go back for more surgery.
Liz said: “Obviously there was a high risk of infection getting into the bone or even into the brain as a result of this so she had to go back in for repairs. In theatre, she had some of the bone removed from her new forehead, the wound cleaned and restitched.”
Hannah is now recovering at home, but Liz said it was hard to stop her jumping around.
“She didnt really cope well with going back into hospital and is quite down and unsettled at times,” she said. “Shes really missing going to playgroup and doing lots of active things she used to do. Its hard to keep a bouncy three-year-old from bouncing.”
At the end of October, Hannahs playgroup, the Fornham St Martin Mother and Toddler Group, raised £1,200 for her with a dance and raffle.
The cash helped to pay for travel and accommodation while Hannah was in hospital but Liz has now decided to donate the remainder of the cash to charity.
The West Australian (Perth)
April 5, 2007
Babys Surgery Fixes Skull Defect
By Cathy OLeary
Perth surgeon uses stainless-steel springs to help 12-week-old Zayviens skull stretch as his brain begins to grow
A Swedish technique and WA surgeons skill spared baby Zayvien having his skull broken to fix an abnormality
Perth surgeons have for the first time in Australia used metal springs to push apart the skull bones of a 12-week-old boy to correct an abnormality which would have meant his skull had to be broken and rebuilt.
Zayvien Cox was born with craniosynostosis, a condition where some of the bones in his head fused too early, not allowing enough room for normal growth.
He had the particular defect known as sagittal synostosis, which meant the sutures that separated the bones of the fontanelle or “soft spot” on the top of his head had closed prematurely, an abnormality that affects up to 10 WA babies each year.
This meant as Zayviens head started to grow, his skull was being forced to expand only in length and not side to side, leaving him with a long narrow head which would become more abnormal over time. More worrying was the risk his brain would not have enough room to expand.
But using a technique developed by Swedish surgeon Claus Lauritzen, Princess Margaret Hospital plastic surgeon Tim Hewitt was able to avoid the need for the usual three to four-hour surgery to cut the skull from ear to ear, often with blood loss, and the removal of big pieces of bone so the skull could be re-shaped.
Instead, in a 45-minute operation at PMH yesterday, Dr Hewitt was able to implant several stainless-steel springs into Zayviens skull, a far less invasive procedure with a quicker recovery.
“In this procedure, all we need to do is cut from front to back the suture in the skull thats fused, so its a smaller skin incision, and then we place the springs so that they slowly push the skull apart over the next few weeks, ensuring the bones go into their correct position,” he said.
“After about three months, we will do a small procedure with an incision only about 3cm to remove the springs once theyve done their job. Eventually we hope to use springs that dissolve so we dont need to do the second procedure.”
Dr Hewitt, who trained in Sweden for a year, said the main advantage of using cranio-facial springs over conventional surgery was that it was less traumatic and safer for very young children, reducing the time that babies were anaesthetised and the likelihood of them needing a blood transfusion.
He said surgery for craniosynostosis was not just a cosmetic treatment because if left untreated the head tended to grow abnormally and could pose health and social problems.
Zayviens mother, Sarah Cox, who has two older sons, said she was relieved he was able to have the less invasive surgery. The skull abnormality was picked up within days of his birth, after X-rays showed the “soft spot" on his head had started to calcify.
“This new surgery meant he has less time under anaesthetic, a shorter recovery time and should be home on the weekend," she said. "Were very lucky to be able to have it here in Perth.”
PR Newswire US
May 24, 2007
Infant is First to Undergo Minimally Invasive Craniofacial and Neurosurgery Procedure With Viking Systems 3-D Surgical Vision System
“Baby and family are doing just fine,” according to Dr. Steven R. Cohen in an update on the condition of baby Jake Joye. In January, at just 3-1/2 months old, baby Jake became the first patient to undergo a revolutionary surgical procedure at Rady Childrens Hospital in San Diego. Cohen, Radys Chief of Craniofacial and Plastic Surgery, along with Chief of Pediatric Neurosurgery, Hal Meltzer, M.D., used the Viking 3Di Vision System to perform the delicate procedure on the infant to correct a condition known as craniosynostosis. The condition occurs at birth when the open areas that separate the bone plates in the skull become fused. Craniosynostosis can result in abnormal skull growth, changing the shape of the skull and leading to vision problems, developmental delays and brain damage.
This new version of the procedure performed with Vikings 3Di Vision System requires just a small incision in the patients scalp as opposed to the usual ear-to-ear incision made across the top of the head. The 3Di endoscope is then inserted into the incision, delivering a magnified, high-resolution, 3D image that allows the surgeons to visualize the underlying anatomy. The live images are viewed by the surgeons via Vikings ViView Personal Monitor. The monitor, which resembles the visor worn in virtual reality video games, places the images directly before the surgeons eyes.
Compared to standard 2D visualization tools, the Viking System provides a more natural, clearer view similar to one obtained in conventional open procedures. Dr. Meltzer emphasized the importance of the high- resolution image provided by the Viking System in baby Jakes case. “We needed to visualize the area under the skull bones to be certain there was no leakage of brain fluid or any abnormal bleeding from the brain," he said. “We had to see that area absolutely perfectly.”
“Since the images are delivered within the surgeons natural line of sight, an immersive surgical environment is created,” says Stephen M. Heniges, Viking Systems Senior Vice-President of Global Marketing & Clinical Development. “The ViView Monitor allows the surgeon to focus directly on the surgical field and helps to reduce the fatigue associated with turning or moving to view a standard monitor.”
3D vision also allows spatial relationships to be maintained, so the surgeons hand-to-eye coordination is not sacrificed. This can help surgeons perform more confidently and naturally aiding in the reduction of procedure times.
“There is less bleeding so our need for transfusions is markedly decreased,” noted Dr. Cohen, pointing out the benefits of the procedure in terms of recovery. “These children go to a regular room, they dont go to an intensive care unit, and they are out of the hospital in a day, in most cases.”
For Viking Systems, the case marks both a technical and a human success story. “We are gratified that our technology is playing a role in cases such as this and is helping healthcare professionals positively affect the lives of patients and families,” said Heniges. “We wish baby Jake Joye and his family ongoing health and happiness.” The Viking 3Di Vision System can be used in any laparoscopic MIS procedure to deliver high-quality, live 3D images to the surgical team. Through the 3Di Vision System, the surgical team can also have access to Vikings Infomatix(R) System which provides on-demand access to other clinical images and relevant, real-time patient and procedural information directly into the surgeons field-of-view.
About Viking Systems, Inc.
Viking Systems, Inc. provides high performance 3D Laparoscopic Vision Systems to hospitals for minimally invasive surgery (MIS). Viking is leveraging that position to become a market leader in bringing integrated solutions to the digital surgical environment. Our focus is to deliver integrated information, visualization, and control solutions to the surgical team, enhancing their capability and performance in MIS and complex surgical procedures. Viking Systems is headquartered in La Jolla, CA. For more information, please visit the companys website at http://www.vikingsystems.com/
Obesity, Fitness & Wellness Week
May 26, 2007
Craniosynostosis; Studies from University of Florida Update Current Data on Craniosynostosis
A report, “History of craniosynostosis surgery and the evolution of minimally invasive endoscopic techniques: the University of Florida experience,” is newly published data in Annals of Plastic Surgery. “Craniosynostosis is the premature and abnormal fusion of 1 of the 6 suture lines that form the living skull and can occur as part of a syndrome or as an isolated defect (nonsyndromic). The first reported surgical procedure for correction of craniosynostosis was performed in 1890 by Lannelongue who advocated releasing, but not resecting, the fused suture,” investigators in the United States report.
“Craniofacial surgery has developed its own identity in the last 3 decades, with the Frenchman Tessier seen as the founding father. There have been many new developments such as distraction osteogenesis, biodegradable miniplate fixation, and the development of minimally invasive endoscopic techniques. Through the pioneering work of Jimenez and Barone, minimally invasive approaches to the surgical correction of craniosynostosis are now gaining wider acceptance,” wrote M.A. Clayman and colleagues, University of Florida.
The researchers concluded: “Here the authors review the history of craniosynostosis, the current literature and technique for endoscopic repair of craniosynostosis, as well as their own experience at the University of Florida, with minimally invasive endoscope-assisted techniques.”
Clayman and colleagues published their study in Annals of Plastic Surgery (History of craniosynostosis surgery and the evolution of minimally invasive endoscopic techniques: the University of Florida experience. Annals of Plastic Surgery, 2007;58(3):285-7).
For additional information, contact M.A. Clayman, University of Florida College of Medicine, Division of Plastic and Reconstructive Surgery, the University of Florida Craniofacial Center, Gainesville, FL USA.
The Salt Lake Tribune
June 24, 2007
Surgery Reshapes Disfigured Head of Frankenstein
By Heather May
It wasnt the kindest word to describe his newborn son, but Frankenstein is what came to Kurt Fertigs mind.
Baby Vincents head was a mess. Because a spot where his skull bones fit together had fused prematurely, his brain was forced to grow toward the front of his skull leaving his forehead looking like a bulb. And the odd shape made his October delivery difficult. Smashed against his mothers cervix, Vincent had capillaries in his head burst when doctors tried and failed to remove him using a vacuum extractor.
We just gave birth to Frankenstein, Kurt told his wife, Larissa, who ended up having a Cesarean section.
But seven months later thanks to a new operation being performed in Utah Vincent could fool even his mother. In their Corrine home, she had to hunt through Vincents sandy hair for the scars from the surgery that is rapidly leading to a normal head shape. The boy, who sat on his dads lap eating a cookie or crawled on the front room carpet, also wears a helmet to direct the growth of his brain.
When I take him to the grocery store without his helmet, I dont get any comments about my baby, Larissa Fertig said.
A New Technique
Doctors at Primary Childrens Medical Center in Salt Lake City had been waiting for a child like Vincent. Plastic surgeon Faizi Siddiqi and neurosurgeon John Kestle traveled to San Antonio last September to witness an endoscopic strip craniectomy.
The new technique for separating a prematurely-fused skull has shown better results and fewer complications than the traditional method they were trained to use. The operation corrects a condition called craniosynostosis, which occurs in about 1 out of every 1,000 to 2,000 births. Primary Children surgeons have operated to correct it about 300 times, Kestle said.
Normally, a babys skull is made up of five pieces of bone that are held together with fibrous joints called sutures, which expand as the babys brain rapidly grows. In the first year, a babys brain triples in volume. But if the sutures close too early, in the womb or after birth, the brain has to grow somewhere else. A deformed head is the result.
The condition can also cause seizures, blindness, or mental retardation if it not fixed in time. Surgery is the only way to correct the problem. But proponents of the new method say the traditional operation is brutal. First, surgeons cut a zigzag pattern from ear to ear across the top of the babys scalp. They expose the skull, remove the bones, reconfigure and re-attach them. The surgery can take several hours, usually requires blood transfusions and several-days stay at the hospital. Swelling and pain are the norm.
Subsequent surgeries arent unusual and the skull may never look quite normal, with lumps, indentations and scars due to metal plates, wires, and screws.
Thinking as Parents
About a decade ago, Constance Barone and David Jimenez, two married surgeons who practice at the University Health System in Texas, found a better way. And craniosynostosis patients have their son, David, to thank.
Eleven years ago we were expecting our first baby and its like we looked at each other one night and it hit us at the same time: What if our kid has craniosynostosis? Jimenez said, adding that David didnt have the condition. We began to look at [the operation] as parents instead of as surgeons.
By using endoscopes, instruments with cameras attached, they make just two incisions in the scalp and cut out the strip of fused suture. The babies are then outfitted with helmets to mold their skull growth. The method eliminates the need for most transfusions and cuts surgery times to under an hour and hospital stays to one day, Jimenez said. He estimated up to 90 percent of his 400 or so patients look excellent meaning nobody would suspect anything had been wrong.
The long-term outcomes are turning out to be just fantastic, Jimenez said. The results are so good that now surgeons can look at them and go, This is something that works.
Siddiqi and Kestle had read about the duos success and both wanted to try the technique. With several other doctors from around the country, they watched Barone and Jimenez perform a live surgery last year. We looked at their results critically, Siddiqi said. That convinced us we should try to treat these patients in this manner. They returned to Utah and waited.
Vincent Fertig was born in October at the University of Utah Hospital. Larissa Fertig called him her little dinosaur because he had ridges on the back of his head and his eyes were pinched. It was like, Whoa, that baby came out of me?
The Fertigs, both 32, soon after met with Siddiqi, who outlined the options. Kurt Fertig, a Brigham City police officer, was skeptical about the new method, but Larissa wanted to try. She was impressed by the techniques apparent fewer complications. And she believed Vincent would be in good hands. I just thought, these surgeons dont want to screw up the first time around, she said.
Siddiqi and Kestle operated Dec. 13, when Vincent was almost 2 months old. The Fertigs said it took two hours. After a one-night stay at the hospital, Vincent went home with two bandages on his skull, a slightly swollen head, and prescription for Tylenol and ibuprofen for the pain. Vincent was given a helmet soon after and will probably wear one until he is 1.
Capitalizing on the brains growth spurt, the helmet prevents his brain from expanding further forward and instead leaves space on the sides. When theyre that young, their head fills that helmet like liquid, Kurt Fertig said.
Larissa Fertig, who homeschools her three other children, hasnt noticed any developmental delays in Vincent because doctors repaired the condition so quickly.
Siddiqi and Kestle have carried out endoscopic strip craniectomies on about a half dozen other patients since Vincent. But they also use the traditional surgery since patients often come to them too late for the less-invasive one, which works best on patients 3 months old and younger.
Kestle said its too early to say which surgery he prefers. He wants to wait to see how the six patients fare with the helmets. And he noted the traditional surgery isnt as traumatic as it sounds: Few patients at Primarys have needed follow-up operations. Im optimistic, he said, but added, We need more experience.
He and Siddiqi are urging pediatricians send them craniosynostosis patients earlier to give them both surgical options. And the Fertigs are trying to help by sharing their story. Were true believers, Larissa Fertig said.
Q and A
What is craniosynostosis?
The condition occurs when the bones in a babys skull fuse too soon. Traditional surgery involves cutting the scalp open ear to ear, then removing and reconfiguring bone. It can take around six hours.
How does the new surgery work?
Surgeons make two incisions in the scalp and cut out a strip of skull in about an hour. Babies then wear helmets to mold skull growth.
Daily News (South Africa)
August 10, 2007
Fresh Start in Life for Rejoice
By Troy Martens
Lightness and hope has filled the life of little Rejoice, the baby abandoned in Durban on Christmas Day with a life-threatening birth defect that could have left her brain-damaged.
Seven-month-old Rejoice has been adopted by a Canadian family, whose name may not be released at this stage while paperwork for the international adoption is taking place. It is not yet certain when she will be allowed to leave the country with her new parents.
Rejoice underwent an emergency operation a month ago to treat a severe condition called craniosynostosis, whereby the skull grows in an abnormal way, causing massive disfigurement to the face and head.
Untreated, the skull would have ultimately crushed the brain, leaving the child severely brain-damaged.
During the five- to six-hour procedure at Nkosi Albert Luthuli Hospital, doctors remodelled the vault of her skull, leaving Rejoice with a new profile and the prospect of a normal life.
Doctors and the surgical team caring for the baby declared the operation a huge success.
After her recovery, Rejoice was put into the care of the Shepherds Keep on the Bluff, a home for abandoned babies.
More good news is that according to Cheryl Pratley, spokeswoman and founder of Shepherds Keep, Rejoice went for her check-up last week.
Doctors monitoring her progress said she was recovering very well.
Pratley recalls how she brought Rejoice home from the hospital after her operation:
She will forever hold a special place in my heart. I have lived every minute of what she has been through what an amazing baby she is, so much wisdom and resilience for someone so little. When I picked her up she actually took my face in her little hands softly, as if to say, Well, this is it were on our way home.
Pratley admits that letting her babies go is the hardest task of all.
I cannot bear to think of the day I will have to say goodbye. But then there is the joy of knowing they are going to loving, happy homes where they will experience the joy and closeness of family life.
Hospital Business Week
September 30, 2007
Investigators at Childrens Hospital Release New Data on Craniosynostosis
According to a study from the United States, Inhibition of bone formation after surgery to correct craniosynostosis would alleviate the need for secondary surgeries and decrease morbidity and mortality. This study used a single dose of Noggin protein to prevent resynostosis and improve postoperative outcomes in a rabbit model of craniosynostosis. Craniosynostosis is defined as the premature fusion of one or more of the cranial sutures, which causes secondary deformations of the cranial vault, cranial base, and brain.
Current surgical intervention involves extirpation of the fused suture to allow unrestricted brain growth. However, resynostosis of the extirpated regions often occurs. Several bone morphogenetic proteins (BMPs), well-described inducers of ossification, are involved in bone healing. This study tested the hypothesis that a postoperative treatment with Noggin, an extracellular BMP inhibitor, can inhibit resynostosis in a rabbit model of human familial nonsyndromic craniosynostosis. Thirty-one New Zealand white rabbits with bilateral coronal suture synostosis were divided into three groups: (1) suturectomy controls (n = 13); (2) suturectomy with BSA in a slow-resorbing collagen vehicle, (n = 8); and (3) suturectomy with Noggin in a slow-resorbing collagen vehicle (n = 10). At 10 days of age, a 3 x 15-mm coronal suturectomy was performed. The sites in groups 2 and 3 were immediately filled with BSA-loaded gel or Noggin-loaded gel, respectively. Serial 3D-CT scan reconstructions of the defects and standard radiographs were obtained at 10, 25, 42, and 84 days of age, and the sutures were harvested for histological analysis. Radiographic analysis revealed that Noggin-treated animals had significantly greater coronal suture marker separation by 25 days and significantly greater craniofacial length at 84 days of age compared with controls. 3D-CT analysis revealed that Noggin treatment led to significantly greater defect areas through 84 days and to increased intracranial volumes at 84 days of age compared with other groups. Histological analysis supported CT data, showing that the untreated and BSA-treated groups had significant healing of the suturectomy site, whereas the Noggin-treated group had incomplete wound healing. These data support our hypothesis that inhibition of BMP activity using Noggin may prevent postoperative resynostosis in this rabbit model, wrote G.M. Cooper and colleagues, Childrens Hospital.
The researchers concluded: These findings also suggest that Noggin therapy may have potential clinical use to prevent postoperative resynostosis in infants with cramosynostosis.
Cooper and colleagues published their study in the Journal of Bone and Mineral Research (Noggin inhibits postoperative resynostosis in craniosynostotic rabbits. Journal of Bone and Mineral Research, 2007;22(7):1046-1054).
Disease Risk Factor Week
October 7, 2007
New Craniosynostosis Study Findings Have Been Reported by S.A. Rasmussen and Colleagues
In this recent report, researchers in the United States conducted a study To study the relationship between maternal thyroid disease and craniosynostosis using data from the National Birth Defects Prevention Study, a multisite, case-control study. Case infants (n=431) were identified through population-based birth defects surveillance systems at eight sites and had craniosynostosis verified by radiographic imaging.
Control infants (n=4,094) consisted of a random sample of live births with no major birth defects from the same population as the case infants. Information on thyroid disease was based on self-report: mothers who reported either a thyroid disorder or use of a medication to treat a thyroid disorder during pregnancy were considered to have thyroid disease. Using an unconditional logistic regression model, we considered potential confounding factors (maternal age, race or ethnicity, smoking, body mass index, preexisting diabetes, plurality, gravidity, family history, infant sex). Among case mothers, 19 (4.4%) were classified as having thyroid disease, compared with 65 (1.6%) of control mothers. Maternal thyroid disease was associated with craniosynostosis after controlling for maternal age (adjusted odds ratio 2.47, 95% confidence interval 1.46-4.18), the only factor that remained significant in the final model. These data provide additional evidence that maternal thyroid disease (most likely Graves disease) or its treatment is associated with craniosynostosis, wrote S.A. Rasmussen and colleagues.
The researchers concluded: Given the frequency of maternal thyroid disease, this association warrants further investigation.
Rasmussen and colleagues published their study in Obstetrics and Gynecology (Maternal thyroid disease as a risk factor for craniosynostosis. Obstetrics and Gynecology, 2007;110(2 Part 1):369-377).
Inside Bay Area (California)
November 18, 2007
Babies Get Easier Time in Surgery
By Sandy Kleffman
OAKLANDA tiny cry pierces the recovery room at Childrens Hospital Oakland.
Little Kacin Rogne, his head wrapped in gauze and a light blue pacifier in his mouth, is waking after 21/2 hours of skull surgery.
Just 11 weeks old, Kacin looks lost in the big white crib. A nurse gives him pain medication and rushes him into his mothers arms, tubes and all.
Hey, sweetheart, she says softly.
Kacin has just undergone a novel, minimally invasive surgery to correct his prematurely fused skull.
The members of the operating teamneurosurgeon Peter Sun and plastic surgeon Bryant Tothare among a handful of people in Northern California who do the procedure.
They and other experts say they believe the technique will gain greater acceptance as an option over the standard surgery as more people become familiar with it and obtain the needed expertise.
Theres less blood loss, less time in the hospital, a shorter recovery time, Toth said.
If left untreated, Kacins condition, known as craniosynostosis, can permanently misshape his head.
The premature fusion prevents the skull from expanding as it should during a babys rapid brain growth. That can lead to increased pressure and brain damage. In rare cases, it can be fatal.
Craniosynostosis affects one in 2,000 infants.
The traditional surgery, which has been performed for decades, is a much bigger operation, with a five- to seven-day hospital stay and an increased chance that a blood transfusion will be needed. Parts of the skull are removed, reshaped and placed back on the childs brain.
We make a big incision, Sun said. The child is more uncomfortable. Theres more swelling. He just needs to be in the hospital a lot longer.
By contrast, the surgeons performed Kacins entire operation through two 3-centimeter-long incisions in his head.
He will be released from the hospital in two to three days and will wear a custom-made helmet for several months to help reshape his head.
The technique was pioneered eight years ago by a neurosurgeon and plastic surgeon teamDavid Jimenez and Constance Barone, now at University Hospital in San Antonio.
Sun and Toth perform six to 10 of the minimally invasive surgeries a year and about 40 of the traditional operations. University of California, San Francisco Childrens Hospital also offers both procedures.
Lucile Packard Childrens Hospital at Stanford performs only the traditional operation. It remains unclear whether the minimally invasive procedure is better, said Dr. David Kahn, a craniofacial plastic surgeon at Stanford.
Sun and Toth are convinced of its benefits, but they note that it is not for everyone. For one thing, it must be done at an earlier age. They use the technique only on children who are 3 months or younger because the skull bones are thin enough to cut easily.
That means pediatricians and parents must catch the abnormal head shape early, get a confirming X-ray or CT scan and schedule the surgery, all within the required time frame.
Three months goes by pretty fast, Sun said.
The more extensive surgery typically is not performed until a child reaches 6 months of age or older.
Most babies are born with six suture lines in their skulls to give the brain room to grow. Several of the sutures intersect in the large soft spot on the top of the head. The bones typically do not fuse until the child reaches age 2.
Chris and Nicole Rogne noticed shortly after Kacin was born that he had a bump on the back of his head, but they assumed it was because he had to be suctioned out at birth.
At his two-month checkup, Kacins pediatrician ordered a CT scan, which revealed the problemfusion of the sagittal suture.
Because their pediatrician was familiar with Sun, the Nevada couple decided to bring Kacin to Childrens Hospital Oakland for the operation.
Sun and Toth began the operation by making two small incisions in Kacins head. With the aid of a lighted tube known as an endoscope, they separated the dura mater, a brain-covering membrane, from the skull to avoid damaging it.
Then they cut the prematurely fused skull with curved scissors and removed portions of it, enabling it to open like a clam shell, as Sun described it. Everything was done through the two small incisions.
After about 21/2 hours, Sun and Toth sewed up the cuts and wrapped Kacins head in gauze.
Once the swelling is gone in a couple of weeks, he will be fitted for a custom-made helmet. It applies light pressure on the front and back of the skull.
causing it to grow sideways into a normal head shape.
Most children wear the helmet for three to four months, Sun said.
Matthew Van Slyke is recovering in his Pleasanton home after his Aug. 22 surgery, colorful stickers dotting his helmet.
His wears it constantly except for the hour a day when it is removed for cleaning. He struggles a bit when they put it on, but then he seems to forget about it, his mother said.
The Van Slykes have been pleased with their decision to go with the minimally invasive procedure.
They initially took Matthew to Stanford but then sought out Sun.
His head is already a normal head shape, Jill Van Slyke said, removing the helmet to reveal the rounded noggin topped with strawberry-blond hair.
Lucile Packard Childrens Hospital does not perform the minimally invasive procedure, in part because its physicians are not convinced that it is a better technique, Kahn said.
He noted that the results depend on how conscientious a family is at keeping the helmet on the babys head properly. With the traditional surgery, the correction is made immediately and there is no need for a helmet.
The minimally invasive surgery is certainly another way of doing it, and it gives good results, Kahn said. The question is, are those results better than the traditional way of doing it?
UC San Francisco Childrens Hospital offers both surgeries but tends to favor the more traditional procedure, in part because its long-term results are well-known, said Dr. Nalin Gupta, chief of the division of pediatric neurosurgery.
Were conservative in that we have very good results with it, he said. Weve done several hundred patients over the last several years and we have long-term follow-up. The complication rate is very low, and the outcome is very good.
Among the 40 to 50 surgeries UC San Francisco does each year for prematurely fused skulls, about five are the minimally invasive type.
Gupta said physicians there are monitoring the results of the newer procedure.
I think in the future theres going to be more and more of those done because there are some very compelling advantages to it, he said. But the one disadvantage is that the long-term results arent really well understood.
You gain a sense of the long-term outcomes over time. Thats the one caveat that I always tell families. Its a new thing.
Sun and Toth are convinced of the benefits and hope to get the word out to pediatricians and parents to catch the problem as soon as possible to preserve the minimally invasive option.
If its a little bit earlier, Sun said, a smaller operation can be done to get it resolved.
Jackson Citizen Patriot (Michigan)
January 13, 2008
Surgery For Son Was Hard Choice
By Chris Iott
It was a sunny spring day at Ella Sharp Park. Mara, my 6-year-old daughter, sat on my lap while I leaned against a tree. Her younger brother, Nathan, was doing what he always does: Trying to make friends with everyone he sees.
I went hut-hut golfing, he told a random woman, recalling a recent experience at the parks miniature golf course.
Mara commented about how friendly Nathan was.
Hes like a mini-Chad, I said, comparing Nathan to his favorite cousin, now in his 20s, who as a child tried to befriend every stranger he made eye contact with.
Mara thought for a moment.
Dad, she said, I dont mean to make fun, but did Chad have a really big head?
She wasnt making fun. Nathan has a large head. We noticed it the second he was born, which happened later than it should have because, well, Nathan has a large head. The kid got stuck.
Having a big-headed child was no surprise to me. Large heads run in the family. My dad has one. So does my oldest brother. Ive got one. That label on baseball caps that says one size fits allit lies. Trust me.
So even though Nathans head has always been largerand narrower and longerthan normal, my wife, Amanda, and I never thought much of it. Hes a good-looking kid, with bright blue eyes, an infectious smile and a friendly demeanor.
Hes hilarious. Thinks running into closed doors and falling down on the floor is a riot. Once asked a woman who was wearing an eye patch whether she was a pirate. Told a female nurse who was asking him questions that doctors are boys. When the nurse asked him another question, he replied, When the boy comes in, Ill tell him.
Hes a fun kid, so we naturally had fun with his head. Referred to him as E.T., stuff like that.
Its not so funny anymore.
On Wednesday, doctors are going to cut Nathans skull into pieces.
Nathan has craniosynostosis, a birth defect where plates in a childs skull fuse early. It affects about one in 3,000 children.
Nathan has the most common form of craniosynostosis, called sagittal synostosis. It results in an elongated, narrow head because the plates that allow for outward growth become fused, forcing the head to grow only forward and backward.
Children tend to have surgery to correct the defect at 6 to 9 months old. Nathan was not diagnosed until later because everyone thought he simply had a big head.
His forehead is prominent. The back of his head sticks out well over the back of his neck and comes almost to a point. Nathan cannot sleep on the back of his headits shape will not allow it.
When he finally was diagnosed in July at 31/2 years old, we were given two options:
Allow surgeons to deconstruct and then reconstruct his skull through a process called total cranial vault reshaping.
Decide against it and face the possibility Nathan could be ostracized because of his appearance as well as the fear that serious medical problems could develop.
It has been almost six months since he was diagnosed. During that time we weighed our options.
Do we allow doctors to cut our kid open, then cut his skull into pieces, trim the pieces and screw the entire thing back together with the help of a titanium plate or two? Or do we take the other path, and risk health problems and mental trauma later?
Do we choose putting our child through major surgery, or do we pass and freak out every time the kid has a headache for the next 15 years?
All surgery comes with risk. The easy way around that risk is to simply not have it done.
But what if my kid comes home some day despondent, complaining of the constant ridicule he receives at school, then finds out his parents could have saved him from all that?
Worse, what if serious medical problems were to develop? What would I say to him?
A Tough Decision
There are medical reasons to put a child through the surgery. The narrow skull that results from this defect sometimes leads to pressure problems, which can result in learning disabilities and blindness or even brain damage and, in rare cases, death.
And there are valid cosmetic reasons. If you have spent any portion of your life being made fun of for looking or acting different from the crowd, you know what I mean.
In our book, the medical issues far outweighed those involving his appearance, so we focused on those.
Doctors couldnt give us an airtight reason to go either route.
This case is right down the middle, a neurosurgeon at the Cleveland Clinic said. We can certainly justify doing surgery. But we can just as easily justify not doing surgery.
A neurosurgeon at the Mayo Clinic and both the neurosurgeon and the cosmetic surgeon at the University of Michigan Hospital were more in favor of going through with surgery.
Amanda and I dealt with it as best we could. For weeks and weeks, the routine we went through looked similar to the instructions on the back of a shampoo bottle.
Talk. Cry. Pray. Repeat.
In the end, we opted for surgery.
A Turning Point
Mara made her comment about her brothers head months before he was diagnosed. I have looked back on that moment as an important one. Mara is Nathans siblinghis loving, caring, protective big sister. Shes sensitive, too. She once cried after school because a classmate said something meannot about her but about another classmate.
Besides, she sees her brother every day, so youd think she would assume his head is not that unusual.
So if Nathans sister noticed his head, who else has? Who else would? Certainly his future classmates would.
In October, several months after Mara made the comment about her brothers head, Nathan was playing on another playground on another sunny day when another little girla total strangersaid something Ill never forget:
Why is his head so long?
We had spent two months visiting nine doctors in three states. We put more time and tears into making a decision about surgery than we had about any other dilemma we had faced together.
The day the girl made that commentWhy is his head so long?was the day after we had made the final decision.
At first, I was upset with that little girl. Within seconds, I changed my mind. The girl was just making an observation. She was a gift, validationat least in our mindsthat we had made the right decision.
It was the first time a stranger made a comment to us about his head. I am not naive enough to believe it was the first time one had noticed it.
Theyre going to cut my kids skull into pieces. But as agonizing as that will be for Amanda and me, it wont be the toughest thing weve been through.
Weighing the options, choosing between short-term pain and long-term fear, not being able to ask Nathans opinion and knowing that waiting until he is old enough to offer an opinion is no optionall that was tougher than spending a day in the hospital hoping that surgery goes well.
Tougher than spending a day with him in intensive care or a week with him in the hospital.
Tougher than keeping a young, active boy from playing rough for six to eight weeks after surgery.
Making the decision was the toughest part.
At least we hope and pray it was.
Jackson Citizen Patriot (Michigan)
February 17, 2008
The Boy Is Back; Month after Skull-Reshaping Surgery, Nathans
Personality, Smile Return
By Chris Iott
After I first wrote last month about our son, Nathan, my wife, Amanda, and I were inundated with e-mails, cards and phone calls from readersmost of whom we have never met.
They offered prayers and support. Several shared stories about a child or grandchild who had gone through the same surgery Nathan was scheduled to have. A few have checked in with an e-mail or a call to see how Nathan has been doing.
All of them reached out to us in an attempt to help. They were successful.
If I live long enough, I am going to personally thank everyoneCitizen Patriot readers and co-workers, friends and familywho have helped us through this. For now, please accept my gratitude through an informal note in your newspaper.
My son, Nathan, was merely jumping on his bed. But if you had seen my reaction, you would have thought he was simultaneously lighting the cat on fire while fiddling with a loaded gun and not eating his vegetables.
Ignore the fact that jumping on the bed is what 4-year-olds do. That he wasnt jumping very high. That the bed was in fact just a mattress that had been placed on the floor in an attempt to shorten any falls he might have while sleeping.
The boy got chewed out.
Thats the curse of being a kid who just had three-quarters of his skull cut off, cut up and pieced back together. Your parents wont let you do a lot of things that normal boys do, no matter how fun those things are.
Nathan underwent surgery last month for craniosynostosis, a birth defect where plates in a childs skull fuse early. He always had an elongated, narrow head because the plates that allow for outward growth had fused, forcing his head to grow only forward and backward.
My wife, Amanda, and I were forced to decide whether to put him through a surgical procedure called total cranial vault reshaping, where surgeons would cut off most of his skull, cut it into pieces and then reconstruct his skull into a more normal shape.
If we chose not to have the surgery, Nathan would face an uncertain future both physically and emotionally. Left untreated, some people with craniosynostosis develop excessive pressure on the brain, which can lead to blindness or, in rare instances, death.
Without surgery, Nathan would have lived his entire life with an odd-looking head. Kids can be cruel to those who are different. Adults, too.
But if we chose surgery, Nathan would face, well, total cranial vault reshaping. I mean, isnt that enough?
There were plenty of tough times. Making the decision to put him through surgery was excruciating. Sitting in the waiting room for 10 hours while he was in the operating room was arduous. The memory of seeing our 6-year-old daughter, Mara, cry the first time she saw Nathan after surgery still hurts my heart.
Even the ups had downs. About five hours into surgery, a hospital employee in the waiting room called the operating room so she could get us an update on how surgery was progressing.
Everythings going great, she said as she hung up the phone. The bones off.
You know the sound of someone dragging a needle across a record that television and movie producers use to show someone has been thrown for a loop? When I heard the second sentence, I heard that noise.
I think it was made by my teeth.
Everythings going great.
The bones off.
Those two sentences dont belong in the same breath.
This was my kids skull. Not only does a skull protect the brain, it keeps the brain from flopping onto the floor, where someone might step on it or kick it under the couch.
When the skull is on, the brain stays close to home, where it belongs. When the skull is off, well, I would hardly say things are going great.
We sat through another five hours of surgery before getting the news the surgeons were finished and everything had gone well. We spent the next two days watching Nathans head and face swell beyond recognition, then spent the next several days watching the swelling go down ever so slowly.
A month later, its still not all gone.
We returned home five days after surgery, stressed out and sleep-deprived. It was another three or four days before Nathan managed a smile.
But strained smiles turned into wide smiles, which turned into laughs. Nathan is now almost back to his old self. Even a bout with the flu three weeks after surgery didnt keep him down for long, although it did make my already-skinny son a bit too skinny for my taste. I mean, you can count his ribs from 10 paces.
Nathans head is far from perfect. Its a bit lumpy here, a bit soft there, somewhat crooked over here. Thats what you get when you deconstruct and then reconstruct a skull.
Weve been told the rough edges and sharp angles will smooth out somewhat as he grows. We wont know for sure what his head will look like until the swelling goes away entirely, which could take months, and his head stops growing, which should happen about a year from now.
His forehead is wider than its supposed to be, but the cosmetic surgeon did that on purpose, at least in part because Nathans scalp wants to return to its former shape and will pull the sides of his forehead in as he grows.
He looks somewhat strange to us, but thats because we saw him and his odd-shaped head every day for 4 years. Most everyone who sees him says he looks great.
More important, he feels pretty good most of the time. He didnt look or act a bit like himself for a week or so after surgery, and at times it seemed that the old Nathan would never return. But his goofy personality and bright smile have come back. The boy who thinks its a blast to run into closed doors and fall on the floor has returnedwitness the scene on the bed.
Weve allowed him to pick up all types of bad habits to fill the void since we wont let him romp around recklessly. He spends way too much time inside staring at screens of one type or another. Chasing his sister around outside has been replaced by playing Mario Tennis on an old Nintendo 64. Going to the playground has been replaced by watching Thomas the Tank Engine videos.
And who knew Noggin.com would play such a key a role in keeping my kid from activities that could result in an injured noggin?
From here it should be easy, relatively speaking. Saturday marked one full month since the surgery, which means were almost to the midpoint of his no rough play period. He has had a few near-misses but nothing that has had us feeling for new bumps or divots.
Someday, all of this will be a memory. Ill catch Nathan jumping up and down on his bed, and Ill still tell him to stop. Thats what paranoid parents do.
But I wont freak out. My breathing wont stop. My heart wont miss five beats. My hair wont turn instantly more gray.
That day will come soon.
And it will be a very good day indeed.
Nottingham Evening Post
March 25, 2008
Notts mums three-peak challenge
A Mum is climbing three of the highest peaks in Yorkshire to raise money for a charity which helped her son.
Alexandra Stone, 28, from Cropwell Bishop is undertaking the 22-mile challenge on April 19 to raise money for Headlines, which helped her son Jamie when he was born with a rare skull abnormality.
Craniosynostosis is a condition where the bones in a childs skull fuse together too early.
Miss Stone said: Jamies condition is rare which made it difficult to get helpthe consultant we saw at the QMC said hed only seen it once before.
Jamie is doing well now but he has a scar from ear to ear which is very wide and hell have it for the rest of his life.
Jamie, who was diagnosed with a type of the condition which affects one in every 3,000 babies, underwent four hours of surgery at six months old.
Miss Stone now aims to raise £3,000 for the charity which she said supported her and fiancé Paul by putting them in contact with people who had children with the same condition.
So far she has raised £300 and says she wants to raise the profile of the support group because it desperately needs more money.
Donate at www.justgiving.com/alexandrastone.
Herald News (Passaic County, NJ)
April 15, 2008
The crucial science in infants skull formation; Misshapen
heads can cause neurological problems
By Suzanne Travers
Their families noticed something slightly off almost at birth. The right side of Anthony Barretos head was strangely flat. One of Janiah Germans eyes didnt open as wide as the other.
Anthonys pediatrician said hed grow out of it, but later his parents were alarmed by a relatives pronouncement that the babys head was too flat. At seven months, Barreto began wearing a head-shaping helmet for plagiocephaly.
Janiahs doctor recommended that her parents take her to a specialist, who diagnosed craniosynostosis when she was 5 months old and scheduled major surgery to reconstruct her skull.
Plagiocephaly and craniosynostosis are odd, scary-sounding words for parents, but they are words it would be wise to know. Both terms refer to conditions affecting the formation of the still-soft skulls of infants. The conditions are different but can be confused without a medical evaluation. And as Janiahs family discovered, with craniosynostosis especially, early detection is crucial to treatment.
Plagiocephaly, which refers to asymmetry of the skull - usually with one side flattenedranges in severity. For some children, the issue may be cosmetic, with a bulging forehead, one eye that appears larger than the other or ears an uneven distance from the face; in others, the distortion can cause hearing or vision problems or a misalignment of the jaw.
Craniosynostosis is a much more serious condition in which some bones of the skull, which should be separate at birth to allow for further brain growth, have instead fused together prematurely. Uneven or restricted head growth can distort facial features, pushing the eyes, nose and mouth toward one side of the face; at its most severe, the constriction on development results in neurological damage.
Infants heads are often misshapen as a result of childbirth and usually correct gradually within six weeks. But according to the American Academy of Pediatrics, anywhere from 1 in 300 infants to nearly 50 percent of babies younger than 1 year have some form of plagiocephaly, depending on what criteria are used. Craniosynostosis is much rarer, present in an estimated 3 of every 100,000 births, according to the academy.
Plagiocephaly on the Rise
Plagiocephaly diagnoses are on the rise. One major cause: the academys successful Back to Sleep campaign, launched in 1992 to reduce the number of deaths from Sudden Infant Death Syndrome, or SIDS. The campaign urged parents to lay infants on their backs when sleeping to prevent potential suffocation. But an unintended consequence has been an increase in cases of positional plagiocephaly, children whose heads are flattened in the back or on one side from the pressure of sleeping in the supine position without spending enough non-sleeping time on their stomachs.
For that reason, the academy has maintained its stance on back-sleeping, but added the advice to ensure that infants engage in tummy time, which allows them to develop their head, neck and upper body muscles and relieves pressure against the back of the head.
The rise in multiple births has also increased the incidence of plagiocephaly, since it can be caused by restricted space in the womb, as well as the overuse of infant seats, carriers, strollers and swings that add pressure to the back of the head, according to Cranial Technologies, an Arizona-based company that treats the condition with corrective helmets.
The companys Paramus office, open since 2000, currently has about 200 children, ranging in age from 3 to 18 months, in treatment for plagiocephaly, said Purvi Patel, managing clinician and physical therapist. Most are referred by pediatricians or specialists, but some families learn of the treatment through word of mouth. About a third of the children who come for a consultation end up getting a corrective helmet, typically those with the most severe asymmetries, Patel said.
Were definitely seeing more of it because of the Back to Sleep campaign, said Patel.
George and Mary Barreto, of Bergenfield, took Anthony to the Paramus office after the relatives comment about the flatness of his head prompted George, 36, to do some research on the Internet.
I feel upset with our pediatrician that he never told us about plagiocephaly, he said.
Technicians made a plaster cast of Anthonys head to use as the template for a permanent helmet, and took a series of photographs by which to visualize his progress. Mary Barreto, 30, painted the helmetmade of white orthotic plastic with an interior of light foam that is gradually shaved down as the childs head growsthe same blue as Anthonys bedroom, and says her son has grown attached to wearing it.
At a recent check-up, Whitney Young, a clinician with a doctorate in physical therapy, took a measurement of Anthonys head to assess the growth of his skull in the area that had been flattened.
Two more millimeters, bud, good job! Young reported. His foreheads looking really good, she told his parents. Hes filling in nicely.
The treatment isnt cheap: $3,300 for the casting, helmet and weekly or biweekly visits to adjust the helmet. (A child typically wears the helmet for two to four months.) In 5 to 10 percent of cases, patients require a second helmet, for another $3,300. Insurance coverage varies, but many insurers regard the treatment as purely cosmetic; Cranial Technologies offers a line of credit to pay for the cost. George Barreto, a letter carrier, said the familys insurance covered $850 of the total cost, but he considers the expense worth it. Despite Anthonys full head of hair, his parents were concerned for their sons appearance and said that was the main impetus for the treatment.
As they get older, kids tend to pick on kids, he said. I dont want my son growing up walking around with a flat head.
The skulls of some babies with plagiocephaly correct themselves on their own over time. Patel of Cranial Technologies acknowledges that 20 years ago, before the advent of orthotic helmets, plagiocephaly went largely untreated, because to do surgery on a healthy baby is not anything anyone would ever consider doing.
But with craniosynostosis, early detection and intervention are necessary to prevent facial deformity and, in some cases, neurological damage.
Another major advantage of early detection is that treatment is less invasive: a two-hour surgery consisting of three small incisions, versus the major skull reconstruction required when the condition is detected in older babies.
By the time Janiah German got to St. Josephs Regional Medical Center, she was 5 months old. Her forehead was uneven and her nose was already shifting toward the side of her face, her mother said. Their referring pediatrician hoped a corrective helmet for plagiocephaly might be all Janiah needed, but doctors at St. Josephs determined that the natural closures had fused. It was early enough that she had no brain damage and limited facial deformity, but too late to get the endoscopic surgery, her mother, Melissa Guzman, said.
Craniosynostosis can be caused by a number of genetic syndromes, but in some cases the cause is not known. Guzman said doctors speculated that Janiahs position in the womb may have caused the sutures in her skull to fuse, but had no definitive explanation. Guzman, 20, and the babys father, Epi German, 20, both of Newark, learned more about their daughters diagnosis online, even watching videos of craniosynostosis surgery on YouTube.
Janiahs own surgery, in late February, was performed by Dr. Silvio Podda, a craniofacial and pediatric plastic surgeon at St. Josephs, and Dr. Richard Anderson, an assistant professor of neurosurgery at Columbia University in New York. The doctors cut her scalp from ear to ear, then removed the bone of the top of her skull and forehead. They sliced the bone into pieces and reassembled them over the durathe thin but tough membrane protecting Germans brainleaving spaces between them to allow for growth.
Ten years from now, no one will ever dream of doing it this way, said Anderson, who lectures around the state to make pediatricians aware that early detection makes for less invasive surgery. They (pediatricians) tend to say, Watch it, we can wait, and at that point its so much more invasive.
Eight-month old Luca Olivera, of Kearny, was diagnosed with craniosynostosis and had the laparoscopic surgical procedure at three months. Since that surgery, which involves inserting a camera through three short incisions to identify where the skull has fused and then cutting apart those closed sutures, Olivera has had to wear a helmet and return to St. Josephs for regular check-ups that monitor his head growth.
The diagnosis was upsetting, said his mother, Ana Jimenez, 29her husband returned from Lucas doctors visit crying and cryingbut the family is pleased with the outcome.
He could have had brain damage if he didnt have surgery, she said. The brain has to grow.
Janiah has recovered well from her more extensive surgery, her mother said.
Right away, you saw results, Guzman said. Where it was flat, it was not flat anymore. Her eyes look different now because theyre both open the same. Shes doing real good now.
Craniums and Character
Historically speaking, the idea of a normal head shape is a somewhat loaded concept.
Throughout history, different cultures have used pads or binding to shape infants skulls into shapes that indicated ethnicity or high status, according to a 1998 article in the Archives of Family Medicine, published by the American Medical Association.
The articles researchers, anthropologists at the University of Illinois, found infant head-molding was still a common practice among a variety of ethnic groups in the Chicago area, from Serbians, who preferred the back of the head to be flat, to Chinese, black, Honduran, Mexican and Jamaican parents who valued a nice round head. These families used manual pressure, swaddling and tight head caps on their infants in the first year of life to shape their still-soft skulls.
In the 19th century, the now-debunked field of phrenology attempted to determine a persons character and personality based on the bumps and grooves in the skull. A large forehead was thought to indicate intelligence and thoughtfulness.
Such reading into the shape of the human skull took on a more sinister tenor in 19th- and 20th-century theories that attempted to use science to classify different races based on head size and shape. Inspired by these theories, proponents of Nazi anti-Semitism justified notions of Aryan supremacy over Jews and other groups, claiming that Aryans had long, thin heads and, therefore, greater intelligence than races with short, broad heads. Today scientists have found such theories to have no basis, and denounced them.