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Craniosynostosis

The normal skull of a newborn baby has several plates of bone that are separated by fibrous joints called sutures. The sutures are found between the various plates of bone that make up an infant’s head. As the infant grows and develops, the sutures close and the bones fuse together, forming a solid piece of bone, called the skull.

Craniosynostosis is a condition in which the sutures close too early, causing problems with normal skull growth. Premature closure of the sutures may also cause the pressure inside of the head to increase, which can affect brain development.

 

What causes craniosynostosis?

Craniosynostosis occurs in one out of 2,000 live births and affects males slightly more often than females. It most often occurs by chance, but sometimes it is inherited genetically.

What are the symptoms of craniosynostosis?

In infants with this condition, changes in the shape of the head and face may be noticeable and are generally the first and only symptom. The appearance of the child’s face may not be the same when compared to the other side. Occasionally, synostosis can increase the pressure within the skull. This is especially true when multiple sutures are fused too early. Symptoms of too much pressure in the skull include:

•    Full or bulging fontanelle (soft spot located on the top of the head)
•    Sleepiness (or less alert than usual)
•    Scalp veins may be very noticeable
•    Increased irritability
•    High-pitched cry
•    Poor feeding
•    Projectile vomiting
•    Increasing head circumference
•    Seizures
•    Bulging eyes and the child’s inability to look upward with the head facing forward
•    Developmental delays

The symptoms of craniosynostosis may resemble other conditions or medical problems. Always consult your child’s doctor for a diagnosis.

How is craniosynostosis treated?

Surgery is typically the recommended treatment. The goal of treatment is to reduce the pressure in the head and correct the deformities of the face and skull bones.

The best time to perform surgery is typically between 6 months and 1 year of age since the bones are still very soft and are easy to work with surgically. Because blood loss can be an issue, surgery is often delayed in the very young child to allow some growth and development as well as a greater blood volume.

Following the operation, it is common for children to have a very swollen face and eyelids.  The eyes may even swell shut.  Most babies go to the intensive care unit (ICU) after the operation for close monitoring.

Problems after surgery may occur suddenly or over a period of time. The child may experience any or all of the following complications:

•    Fever (greater than 101 degrees F) – this is very common
•    Vomiting
•    Headache
•    Irritability
•    Redness and swelling along the incision areas
•    Decreased alertness
•    Fatigue

These complications require prompt evaluation by your child’s surgeon.

What is the long-term outlook for a child with craniosynostosis?

The key to treating craniosynostosis is early detection and treatment. Some forms of craniosynostosis can affect the brain and its development.  The degree of the problems depends on the severity of the craniosynostosis, the number of sutures that are fused, and the presence of brain or other organ system problems that could affect the child.

A child with craniosynostosis requires frequent medical evaluations to ensure that the skull, facial bones, and brain are developing normally. The medical team works with the child’s family to provide education and guidance to improve the health and well being of the child.

Types of Craniosynostosis

plagiocephaly

trigonocephaly

scaphocephaly

There are many types of craniosynostosis. Different names are given to the various types, depending on which suture, or sutures, are involved, including the following:

Plagiocephaly involves flattening of either the right or left side of the head. This can affect the front of the head or the back. When it affects the front, it is due to the coronal suture fusing, which limits the normal forehead and brow growth. Therefore, it creates a flattened forehead and brow on the affected side, with the forehead tending to be overly prominent on the other side. The eye on the affected side may also be misshapen and the back of the head may also be flattened.

Sometimes, a baby’s head will become misshapen because of repeated pressure being applied to the same area, such as when a baby always lies on its back. This is called deformational (or positional) plagiocephaly; it is not true synostosis. Deformational plagiocephaly is treated by altering the child’s usual position of sleep, neck exercises and/or using a specially molded helmet that will reshape the head over time.

Trigonocephaly is a fusion of the metopic suture. This suture runs from the top of the head down the middle of the forehead, toward the nose. Early closure of this suture may result in a prominent ridge running down the forehead. This can progress to make the forehead look pointed, like a triangle, and the eyes look like they are too close together.

Scaphocephaly is an early closure of fusion of the sagittal suture. This is the most common type of synostosis. This suture runs front to back, down the middle of the top of the head. This fusion causes a long, narrow skull. The skull is long from front to back and narrow from ear to ear.

Plagiocephaly Program

Our Plagiocephaly Program treats all deformational conditions of the head, including plagiocephaly, craniosynostosis and torticollis. We provide laser analysis of the head shape and a full range of surgical and non-surgical treatments such as cranial remodeling and custom helmeting.

CHOC Children’s Specialists Neurosurgery
1010 W. La Veta Ave., Suite 710
Orange, CA 92868
Appointments: 714-509-7070
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UC Irvine

CHOC Children's is affiliated with the UC Irvine School of Medicine