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Craniosynostosis

Definition

Craniosynostosis is a birth defect that causes one or more sutures on a baby's head to close earlier than normal.

The skull of an infant or young child is made up of bony plates that allow for growth of the skull. The borders at which these plates intersect are called sutures or suture lines. The sutures between these bony plates normally close by the time the child is 2 or 3 years old.

Early closing of a suture causes the baby to have an abnormally shaped head.

Alternative Names

Premature closure of sutures

Causes, incidence, and risk factors

The cause of craniosynostosis is unknown. Genes may play a role. However, there is usually no family history of the condition.

One type that is passed down through families (inherited) can occur with other health problems, such as seizures, decreased intelligence, and blindness. Genetic disorders commonly linked to craniosynostosis include Crouzon, Apert, Carpenter, Chotzen, and Pfeiffer syndromes.

However, most children with craniosynostosis are otherwise healthy and have normal intelligence.

Symptoms

Symptoms depend on the type of craniosynostosis. They may include:
  • No "soft spot" (fontanelle) on the newborn's skull
  • A raised hard ridge along the affected sutures
  • Unusual head shape
  • Slow or no increase in the head size over time as the baby grows

Types of craniosynostosis:

  • Sagittal synostosis (scaphocephaly) is the most common type. It affects the main suture on the very top of the head. The early closing forces the head to grow long and narrow, instead of wide. Babies with this type tend to have a broad forehead. It is more common in boys than girls. 
  • Frontal plagiocephaly is the next most common type. It affects the suture that runs from ear to ear on the top of the head. It is more common in girls.
  • Metopic synostosis is a rare form that affects the suture close to the forehead. The child's head shape may be described as trigonocephaly. It may range from mild to severe.

Signs and tests

The doctor will feel the infant's head and perform a physical exam. 

The following tests may be done:

  • Measuring the width of the infant's head
  • X-rays of the skull
  • CT scan of the head

Well-child visits are an important part of your child's health care. They allow your doctor or nurse to regularly check the growth of your infant's head over time. This will help identify any problems early.

Treatment

Surgery is done while the baby is still an infant. The goals of surgery are:

  • Relieve any pressure on the brain
  • Make sure there is enough room in the skull to allow the brain to properly grow
  • Improve the appearance of the child's head

Expectations (prognosis)

How well  your child does depends on:

  • How many sutures are involved
  • The child's overall health

Children with this condition who have surgery usually do well, especially when the condition is not associated with a genetic syndrome.

Complications

Craniosynostosis results in head deformity that can be severe and permanent if it is not corrected. Increased intracranial pressure, seizures, and developmental delay can occur.

Calling your health care provider

Call your child's health care provider if:

  • You think your child's head shape is unusual
  • Your child is not growing well
  • The child has unusual raised ridges on the scalp

References

Kinsman SL, Johnston MV. Craniosynostosis.In: Kliegman RM,Behrman RE, Jenson HB, Stanton BF, eds.Nelson Textbook of Pediatrics.19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 585.12.


Review Date: 11/7/2011
Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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