What is Craniosynostosis

(Crane -- ee--oh--sin--oh---stow--sis)

When I first began my own research just the sight of that big long word scared the hell out of me ~ how could my child have something I couldn't even pronounce? Its great you found my website...I will try to teach you what you don't already know.

Craniosynostosis happens one in every 2000 births. Many pediatricians and even parenting magazines quickly try to dismiss Craniosynostosis as a rare condition. I can, however break it down for you in a way that makes it appear more common.

Approximately 216000 babies are born every day in the world

There are 24 hours in a day

= 9000 babies born hourly

Now take into account one in 2000 born are affected with Craniosynostosis

In a 24 hour period 108 babies could be born with Craniosynostosis

= approximately 5 babies born hourly with craniosynostosis

So, if five babies born every hour of every day could have been born with Craniosynostosis, why is this condition not more actively warned about and talked about within the medical community?  This is the same question many parents, such as myself, have asked over and over again. There are still too many stories like my own where more than one doctor ignored a parent's concerns on their infant's head shape. It still seems as though not enough attention is paid to this condition on the professional side. I have seen some improvements over the last few years and much more knowledge is available to you parents on the web then what was available to us nine years ago. We have taken a few huge steps for the sake of awareness and knowledge but we are still miles away from the finishing line.

Definition of Craniosynostosis:

Craniosynostosis is the premature closing(fusing) of one or more of the boney gaps in an infant's skull. These fibrous boney gaps are known as sutures.
In a normal infant who is not affected by Craniosynostosis these sutures remain open throughout the first two years of life to allow proper brain growth. As 80% of the brain growth occurs rapidly in the first 18 months of life.

Is Craniosynostosis Cosmetic?

No! You might hear this word mentioned by others that have not researched craniosynostosis and might not understand why an abnormal head shape is such a big concern. To understand why Craniosynostosis is worrisome you need to know possible lifetime consequences to not correcting craniosynostosis.

• Learning delays can result from intracranial pressure(ICP) i
• Eyesight problems
• Hearing delays,
• Mouth, speech, and feeding issues
• Hydrocephalous(an abnormal collection of fluid on the brain which can lead to pressure if untreated.)
• SeizuresChiari Malformation
• Profound psychological effects in childhood and through adulthood due to looking different
• Death(rare but in cases of severe ICP it is a possibility)

If a doctor or insurance company tries to throw the term cosmetic your way rember one thing: According to the American Medical Association the definition of a cosmetic procedure is one that changes a normal structure of the body in order to improve appearance. While a reconstructive procedure is performed on an abnormal structure of the body to improve function, or return it to normal.

What are the warning signs of Craniosynostosis:

The most common sign of craniosynostosis is an irregularly shaped head. Although, many infants are born with an abnormal head shape, due to the trip through the narrow birth canal, most will correct themselves within six weeks following the birth. When an abnormal head shape persists or is not noticed until after six weeks, it is important to determine the cause.  Another sign of  craniosynostosis is a palpable ridge along the skull where the suture has closed.(Ridging and/or an abnormal skull could be positional plagiocephly or simply a natural ridging.) If a single suture is fused your only symptom might be the irregularly shaped head.

However, Craniosynostosis is usually more severe when more than one suture closes too early. This significantly restricts the skull's ability to expand as the brain grows. Intracranial pressure becomes worrisome with multi-suture closure. Warning signs of pressure can include Vomiting, becoming sluggish, sleeping more, and playing less. Irritability because of head pain. Developing swollen eyes or problems moving the eyes or following objects. Problems hearing. Breathing noisily or have periods of not breathing (apnea). When the pressure is very severe, it may cause brain damage and other problems, including seizures, blindness, and developmental delays. Untreated craniosynostosis may cause lasting disabilities.

"I think My Child has Craniosynostosis, what should I do?" 

Many professionals don't give enough credit to mother's for their intuition. If your gut is telling you something just isn't right then more than likely something isn't.  First step is to discuss with your child's physician your concerns. With most insurances you will need a referral to see the proper specialist. However, your child's physician is not trained specifically to diagnose either craniosynostosis nor positional plagiocephaly. 

How Is Craniosynostosis Diagnosed?

An appointment with an Experienced Craniofacial Specialist needs to be made. More than likely CT Scans & MRIs need to be ordered. However, as more is becoming understood about Craniosynostosis some Craniofacial specialist can tell upon physical assessment if your child's condition is in fact craniosynostosis.

"Wait MRIs? CT scans? I discussed my concerns with my child's physician and they want to send for an x-ray."

Although X-rays will show if sutures are present they will not show the suture in enough clarity to catch if fusion has begun or not. Only a craniofacial specialist should be allowed to diagnose or dismiss craniosynostosis.

X-rays don't always catch the fused suture only a CT scan read by a craniofacial professional can determine if the sutures are fused or open.

"My Child Has Craniosynostosis, What's the Treatment?"

I hated reading this when my own daughter was first diagnosed but I must say it....   Surgery is the only cure for craniosynostosis.
Some mild cases of craniosynostosis are monitored and may never need surgery. However, Craniosynostosis is a progressive deformity(which means the awkward head shape will only get worse as the suture continues to fuse and the brain continues to grow.)
Surgery on your child is a very scary concept but, honestly, these children bounce back fast. Some children only need one initial surgery while other children could require more. It all depends on the severity of the suture fusion and how your child grows.

More Information on Suture Closure

The Skull of an Infant is made up of  bones that are separated by fibrous joints (sutures). In a normal skull not affected by craniosynostosis these fibrous joints remain open and flexible during the child's first years of life to expand and grow with the rapid growth of the infant's brain.

If one or more of these fibrous sutures closes to early the skull cannot expand normally with growth of the brain, and so assumes an abnormal shape:

Metopic Craniosynostosis:

The metopic suture begins at the nose and continues to meet the sagittal suture. Metopic craniosynostosis results in a narrow, triangular forehead with pinching of the temples laterally(trigonocephaly).

The Metopic Suture is the first and only suture which normally fuses early in childhood(normally around 6 to 8 mths but in some cases as early as 3 months.) Metopic Craniosynostosis is one of the least common forms of Craniosynostosis. However, sometimes the early fusion of the Metopic suture will cause a Metopic ridge with no trigonocephaly. In cases of a metopic ridge surgical intervention may not be needed.

In true Metopic Craniosynostosis with the evidence of trigonocephaly (a triangular forehead and narrowing to the skull), whether this narrowing is mild or severe, many experienced surgeons will recommend that the infant's skull should be surgically repaired.

If your child has a Metopic ridging with no trigonocephaly be sure to consult a craniofacial specialist. Even if you are advised to be wait and see always research and ask questions.

Saggital Craniosynostosis:

The sagittal suture runs from the front of the head to the back of the skull. Fusion of the suture results in a long, narrow skull which may or may not include bulging of both the front and back of the head. The shape of a skull affected with Saggital Craniosynostosis is also known as scaphocephaly. Saggital Craniosynostosis is the most Common form of and estimated to happen one in 1000 births.

It is thought that disproportion in the size of the pelvis of the mother and the size of the fetal head can cause Saggital Craniosynostosis. The limited room for growth is thought to bring the the two parietal bones together too early creating the synostosis in the pelvis before birth. This is why Saggital Craniosynostosis is often seen in first pregnancies. Also, boys have larger heads and that is why it is thought more boys than girls are affected with Saggital Synostosis.

Even with these thoughts on Saggital Craniosynostosis there is nothing that an expectant mother can do to prevent craniosynostosis. There is still not enough known about Saggital and any other form of Craniosynostosis. Research is still being discovered for the causes of premature suture closure.

Coronal Synostosis:

The coronal sutures begin at the ear and continue superiorly to the top of the skull to meet the sagittal suture. One or both sutures may be involved.

When one suture is fused that is known as
 Unilateral (left or right) Coronal synostosis. The shape of the head is sometimes referred to as plagiocephaly (which should not be confused with positional plagiocephaly.) The forehead on the effected side is flattened and swept back with the eye in its socket. Unilateral Cranio can give the appearance that one eye is wider open then the other which is caused by the lack of orbit around the affected eye.

When both sutures are fused that is known as bicoronal synostosis. The head is short and wide with the head shape being referred to as brachycephalic. There is a lack of growth with both eye orbits which can cause bulging of the eyes. The back of the head might appear flat with a bulging of the skull around the ears. The skull tends to tower upwards as it continues to grow.

Coronal Craniosynostosis may or may not be caused by a syndrome. One syndrome babies with Coronal Synostosis can commonly have is Muenke's Syndrome.  Along with the coronal suture fusion a few more symptoms of Muenke's may be, flattened cheek bones/shallow mid-face, wide-set eyes, hand or feet abnormalities(my daughter has Muenke's syndrome and the middle bones of her fingers are shorter than normal), possible hearing loss, and possible learning delays.  In the cases of multisuture craniosynostosis your Craniofacial Specialist might recommend meeting with genetics for  follow-up and testing.

Lambdoid Craniosynostosis:

The Lambdoid suture is located on the back of the skull. It has a right and left side and is shaped like an upside down "V.

One key marker for fusion of the lambdoid suture is a low bump behind the ear on the same side as the fused suture. Another good way to determine if the lambdoid suture is closed is to look and see if the ear on the same side as the posterior skull flatness is pulled backwards and sticks out more. If the ear is forward on the flat side, with respect to the opposite ear, then a skull deformation should be suspected instead of a fused suture. When viewed from above, the affected side of the back of the head is flatter than the opposite side.

Lambdoid Craniosynostosis is the rarest form of Craniosynostosis and fusion of both Lambdoid sutures is more rare. The diagnosis of Lambdoid synostosis is the most difficult to make because on plain skull x-rays the lambdoid suture is frequently misdiagnosed as being fused shut. Ideally, the diagnosis of lambdoid synostosis is made by CT scans, read at an experienced center. Children who have lambdoid synostosis and significant flattening.

Cloverleaf Craniosynostosis:

Also known as kleeblattschadel. Premature fusion of the coronal, lambdoid and posterior sagittal sutures  results in a cloverleaf skull. This is the rarest form of Craniosynostosis and evidence of closure will be evident at birth. Due to the severity of multiple sutures being fused children with Cloverleaf should be closely followed by pediatric neurosurgeons, pediatric craniofacial surgeons, and team of doctors  to follow up through childhood and possibly adulthood. 

How is Craniosynostosis Fixed? 

There is no cure for Craniosynostosis. The only treatment is surgery. Parents that suspect something wrong with their child's head shape should consult with a pediatric craniofacial surgeon and/or pediatric neurosurgeon. The main purpose for surgery is to open up the skull to allow proper skull growth as well as to correct the shape of the skull.  As more of our children are affected with Craniosynostosis research is being done on the impact fused sutures might have on brain function. Some of our children might have cognitive delays while others will grow up with no affects from the synostosis and surgery. This is a scary time for parents of children  with craniosynostosis. It opens up a whole roller coaster of emotion. There are groups that will help you face prediagnosis concerns, surgery, and postop care CAPPSKids.org and CranioKids.org as well as the JorgePosada Foundation strive to help families affected with Craniosynostosis. Their links and info are provided on our support page.

Click here for another great site for Craniosynostosis and Positional Plagiocephaly Information is

What Does Craniosynostosis look like on CT scans?

Normocephaly(Skull with no Craniosynostosis)

Metopic Craniosynostosis

Saggital Craniosynostosis

Unilateral Coronal  &  BicoronalCraniosynostosis

      &        

Lambdoid Craniosynostosis

(back of the skull shown)  

Cloverleaf(kleeblattschadel) Craniosynostosis

Many Images on this page borrowed courtesy of eMedicine - Medical Reference

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Types of Surgery

The first surgical treatment of craniosynostosis was undertaken by Lannelongue in 1892, and involved the correction of a sagittal synostosis. Since then, multiple procedures have been used for the treatment of this condition, ranging from simple suturectomies(removal of fused sutures without skull remodeling to extensive cranial vault reconstruction. The type of surgery done for craniosynostosis is unique to the surgeon performing the repair and unique to the which suture is fused. I encourage parents to thoroughly research all their options before deciding which surgery is best for your child. Second and third opinions from different craniofacial surgeons is recommended if you are unsure of the first consult.

Endoscopic Surgery:

In 1998, Dr. Jimenez and Dr. Barone pioneered the endoscopic surgery for Craniosynostosis. This surgery is the least invasive approach. For this surgery to be the most successful it is advised that surgery be performed on an affected infant before four months old and no later than six months old.  Surgeons will utilize an endoscope through two small incisions measuring about 1.5 inches each at the top of the head. A segment of bone is removed near the fused bone and in a few other places which releases the fusion. No plating or reshaping is performed. Instead, in the first week after surgery, a custom molding helmet is made, which the patient wears for up to a year.

The pro's of the endoscopic surgery are:

• Decreased operative time (1.5 hours or less vs. usually  5 or more hours)
• There is usually no need for a blood transfusion, and much decreased blood loss
• No plates or screws are needed
• Shortened hospital stay: patients generally go home on day 1 or day 2
• Smaller incisions which have significantly reduced scars
• Greatly reduced discomfort and swelling.

A couple of Cons to take into consideration:

• Craniosynostosis must be caught at an early age for the best cosmetic results. 
• travel to a qualified surgeon(few surgeons are qualified to perform the endoscopic procedure)
• a year of helmet wearing which will also require possible traveling for adjustments and checkups as the child grows out of the helmet.

Some complications of endoscopic surgery are: 

• less favorable cosmetic results
• a higher possibility of secondary surgery later
• possibility of missed hydrocephelous when no CT scans are performed prior to surgery(it is stated that CT scans are not ordered in Saggital cases)
• Hyperostotic bone(Excessive or abnormal thickening or growth of bone tissue)
• leptomeningeal cyst which can develop if a dural laceration is not seen and repaired during surgery
• if a complication with bleeding should arise during surgery precious time is lost due to needing to open up the skull for repair.

Click Here for info about endoscopic repair from Dr. Jimenez and Dr. Barone.

Cranial Vault Reconstruction:

Cranial Vault Reconstruction also known as CVR or traditional surgery is the most common surgery performed. It is a complex surgery and will require the use of both a pediatric neurosurgeon and pediatric craniofacial surgeon.

Some surgeons prefer to do either just the front of the skull(anterior CVR) or just the back of the skull(posterior CVR). While other surgeons might choose an overall CVR depending on the degree of reconstruction needed. In some cases a front orbit advancement(FOA) is performed to reconstruct the orbits around the eyes. FOA's are hardly ever performed without an accompanying CVR. 

An incision is made in the scalp from ear to ear in either a zig-zag pattern or u-shape. The skull bones are then cut and removed by a pediatric neurosurgeon. A CVR should never be performed without the neurosurgeon who's job is to protect the duram that surrounds and protects the brain. A pediatric craniofacial surgeon then will reconstruct and piece the skull bones back together using plates, screws, and stitches. Most of these foreign objects are absorbable which means as the new bone grows the plates and screws will slowly disappear.

The pro's of CVR Surgery are:

• Immediate and often lasting correction of the deformity without any visible or palpable bony defects
• Drastically reduced occurrences of secondary surgeries
• More qualified surgeons available to perform CVR surgery
• CVR is most successful when performed on children younger than one year of age but is highly successful when performed on even older children
• Any complications with the duram or blood loss can be immediately handled.
• Most successful treatment for multi-suture synostosis and severe cranial deformity.

The Cons of CVR Surgery Are:

• Blood loss with the need of blood transfusions(although this risk can be greatly reduced with the use of procreate shots and blood recycling procedures.)
• Highly invasive surgery that last 5 to 9 hours typically
• Plates and screws most often are needed
• 48 hour stay in PICU required followed by 3 or 4 more days in hospital
• Swelling and bruising

Strip Craniectomy:

Most commonly an incision is made across the scalp from ear to ear in a zig-zag or u-shaped pattern. However in some cases of Saggital the incision has been made from the front of the scalp to the back across the middle. A strip of bone is usually removed where 2 sutures connect and cuts are usually made down the sides of the skull to allow for natural reshaping as the brain grows. Sometimes smaller pieces of bone are removed and the skull is then allowed to be remolded as the brain grows and the open spots allow the natural growth to take place.

Barrell Stave Craniectomy:

This too is an open procedure so an incision is typically made from ear to ear. Strips and pieces of bone are removed and repositioned with some gaps and in places where reconstruction is needed the bones are cut into smaller, flowering shapes that will move and conform with the brain as it grows. Bone sometimes can be stitched back together without need of plates and screws.

Spring-mediated surgery -

One of the newest methods of correction. Like the endoscopic surgery surgeons will utilize an endoscope through two small incisions measuring about 1.5 inches each at the top of the head. A segment of bone is removed near the fused bone and in a few other places which releases the fusion. Springs are then inserted in the open spaces to help ensure that the opening does not re-fuse.  A second procedure will be required to remove the springs.

As with any surgery all these procedures I have talked about have risk. It is important that parents do research, ask plenty of questions, and make sure you are completely comfortable in the surgical team they choose their your child.

Images above courtesy of eMedicine - Medical Reference

Overwhelmed?

Its completely normal to feel overwhelmed and frightened by the over-abundance of information presented to you during this whole experience. Not only are you dealing with a flurry of emotions, you also have huge decisions to make in a short amount of time. How everyone handles this whole process is unique to the individual. Know that you are NOT ALONE. Many other parents have been down the very path you are starting and are willing to help you by giving a shoulder to cry on, a friend to laugh with, and answers to your questions.

A Few of My Favorite Online Communities are:

CAPPSKids

Jorge Posada Foundation

CranioKids

Faces 

CCA Kids

How Do I Choose A Surgical Team?

For many families craniosynostosis post-surgical follow up last through adolescence. It is important to choose the Craniofacial Surgeon you feel is the best choice for your child. No pediatrician or insurance company should be allowed to bully you from getting the best surgical outcome for your child. Insurance denials can be appealed and many organizations can help with travel cost and boarding during surgery if you need to travel out of state. That being said there are plenty of highly qualified craniofacial surgeons in your area and your country.  You will find if you ask opinions many of us have our favorite craniofacial teams and tend to be biased after years of having them be part of our families.

Click here for Link to List of Surgeons compiled by FACES:

Next: Craniofacial Syndromes