Craniosynostosis

Congenital Defect; Skull/Brain

Craniosynostosis is a birth defect that involves the skull of a child.  This defect causes the growth sutures on a fetus’ skull to close sooner than they should, resulting in a misshapen head and possibly other issues.

Craniosynostosis Causes, Incidence, and Risk Factors

During the development of a fetus there are a number of loose membranes known as ‘sutures’ located in the skull.  These connect the skull to itself while still allowing it to remain malleable and grow as it should.  They allow not only the proper formation of the head but also the correct growth of the brain.  At the appropriate developmental stage these sutures will fuse closed, keeping the skull from changing further and forming a protective shell over the brain.  In cases of craniosynostosis these sutures fuse closed earlier than normal, resulting in abnormal skull or face shapes as well as possibly causing the brain to encounter difficulty growing.

There are different types of craniosynostosis, and the condition is classified by its causes and which cranial sutures it actually affects.  Some of the most common types include the following.

  • Plagiocephaly
  • Trigonocephaly
  • Scaphocephaly

It is an extremely rare condition that affects an estimated one in every two thousand to one in every two thousand five hundred births around the world.  In most cases only a single cranial suture is affected although five to fifteen percent of craniosynostosis cases affect multiple sutures.  The condition affects male babies twice as often as it does females.

There are several different factors that can contribute to the development of craniosynostosis, including some surprising ones.

  • It seems that genetics play one of the largest roles in craniosynostosis risks, and as many as eleven percent of cases occur in a family where there is a history of the condition.  A genetic screening can help determine if your child will be at higher risk for developing the condition.
  • In some cases an elevated level of the mother’s thyroid hormone can trigger this issue.
  • There is evidence that the way a child lays inside the fetus could cause the condition as well, mainly those that lie in the breech position.
  • Environmental factors could cause the problem as well.  Smoking has been linked to the condition as have some types of amine-containing medications taken during pregnancy.

Craniosynostosis Symptoms

Like many other birth defects, the signs of craniosynostosis will be noticeable immediately.

  • A misshapen head is the main symptom of this condition, although there is no specific deformity that may occur.  One side of the head, the top, back, or front of the head could be deformed.
  • Facial deformities, particularly the nose and eyes, may occur as well.

Craniosynostosis Diagnosis

While a misshapen head might be alarming, there is a chance that it is simply a result of odd posture in the womb and might correct itself over time.  To diagnose craniosynostosis for sure, your doctor will likely run a few tests.  These can include:

  • A CT scan of the infant’s head
  • An X-ray of the head
  • An MRI scan of your child’s head
  • Measuring of the head
  • Checking the sutures and fontanels of the newborn’s head

Craniosynostosis Treatment

The only real treatment option for most babies born with this condition is immediate surgery.  Surgery must be performed as soon as it is safe for the baby.  The surgery will involve a combination of removing the suture that has been fused closed and careful reconstruction of the skull in the areas it has been misshapen.  Undergoing this surgery will help with several different complications.

  • Surgery will help return the skull to a more normal appearance
  • Surgery will help the head and skull grow properly throughout childhood
  • Surgery will also help the brain develop and grow properly

Corrective surgery will usually be performed after your child turns six months old.  At this young age the bones in the skull are still somewhat malleable and able to be reshaped with much less risk and better results.  Doing the surgery prior to six months of age is possible but the risk of blood loss is increased tremendously, and must be done after the age of three months whenever possible to make allowances for the anesthesia required.  The surgery will be followed up by several return visits to ensure that your child’s skull has healed properly and is growing correctly.

Craniosynostosis Prognosis

Surgery is the best chance of overcoming this condition, and if the procedure is performed early the chances of a full recovery are actually quite high.  Children with only a single suture affected by their disease will have much higher success ratios than those who have been affected in more than one suture, although surgery will still help greatly.  Those whose condition is not caused by a genetic defect will have an even higher chance of recovery as well.

Craniosynostosis Complications

The major problems with craniosynostosis come when it is left unchecked.  Lacking corrective surgery, a number of problems could develop from the condition, especially as a child grows older.

  • Vision impairment and blindness are not uncommon
  • Developmental delays and disabilities occur as well since the brain can’t adequately grow
  • Seizures can occur due to the pressure on the brain as well
  • Finally, brain damage of varying severity might occur if the condition is not corrected quickly

These complications might occur early in life, but so long as corrective surgery is undertaken they will likely be averted.

Calling Your Health Provider

It’s likely that your baby’s condition will be noticed right after birth.  If not and you notice that he or she has a misshapen head, you should contact your health care provider for an exam.  If your child has been diagnosed with this condition and you are awaiting corrective surgery there are several symptoms you should keep an eye out for.  If any of these occur in your child you should contact your doctor immediately

  • Sluggish behavior
  • Hearing Troubles
  • Irritability
  • Noisy Breathing
  • Swollen Eyes or Vision Trouble

Craniosynostosis Prevention

Regular visits to your child’s health care provider can help diagnose craniosynostosis before it becomes a major problem.  Genetic screening can help determine if this condition can or will be a problem for your newborn.

Craniosynostosis References

Ridgway EB. Skull deformities. Pediatr Clin North Am. 2004;51(2):359-387. [PubMed]

http://en.wikipedia.org/wiki/Craniosynostosis

Link Resources

Overview

Craniosynostosis Treatment

Craniosynostosis Testing

Craniosynostosis Specific Conditions

Craniosynostosis Organizations

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