Craniosynostosis (patient information)

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Overview
Craniosynostosis is a congenital (present at birth) defect that causes one or more sutures on a baby's head to close earlier than normal. Sutures are connections that separate each individual skull bones. The early closing of a suture leads to an abnormally shaped head.

What are the symptoms of Craniosynostosis?

 * Absence of the normal feeling of a "soft spot" (fontanelle) on the newborn's skull
 * Disappearance of the fontanelle early
 * 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

What causes Craniosynostosis?
The cause of craniosynostosis is unknown. Which suture is involved determines the abnormal shape of the head.

A person's genes may play a role in craniosynostosis. The hereditary form often occurs with other defects that can cause seizures, diminished intellectual capacity, and blindness. Genetic disorders commonly associated with craniosynostosis include Crouzon syndrome, Apert syndrome, Carpenter syndrome, Chotzen syndrome, and Pfeiffer syndrome.

However, most cases of craniosynostosis occur in a family with no history of the condition, and children with craniosynostosis are otherwise healthy and have normal intelligence.

There are different types of craniosynostosis. Sagittal synostosis (scaphocephaly) is the most common type. It affects the main (sagittal) 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 of craniosynostosis tend to have a broad forehead. It is more common in boys than girls.

Frontal plagiocephaly is the next most common form. It is the closure of one side of 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 of craniosynostosis that affects the suture close to the forehead. The child's head shape may be described as trigonocephaly, and varies in severity.

Who is at highest risk?
Craniosynostosis occurs more often in males than in females.

Children affected by certain genetic disorders may also be affected by craniosynostosis.

When to seek urgent medical care?
Call your health care provider if you think your child's head has an unusual shape. A referral to a pediatric neurologist or neurosurgeon should follow.

Diagnosis
The doctor will feel the infant's head and perform a physical exam. A neurological exam would also help diagnose the condition. The following tests may be performed:


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

Treatment options
The main treatment for craniosynostosis is surgery. Surgery is done while the baby is still an infant. The goals of surgery are to:
 * 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

Where to find medical care for Craniosynostosis?
Directions to Hospitals Treating Craniosynostosis

What to expect (Outlook/Prognosis)?
How well a person does depends on how many sutures are involved and whether other defects are present. Patients who have surgery usually do well, especially those whose condition is not associated with a genetic syndrome.

Possible 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.

It is important to get treatment for this condition.