Craniosynostosis

As a parent, you know your baby better than anyone. You’re the first to notice if something doesn’t look quite right. Slight imperfections in your baby’s head are normal, especially in the first month after birth. But as your baby grows, a misshapen head could be a sign of something else. The earlier you can get a diagnosis—ideally, before the age of 6 months—the more effective treatment can be.

What is craniosynostosis?

Craniosynostosis is a condition in which the sutures in a child’s skull close too early, causing problems with head 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?

The cause of craniosynostosis is often unknown but can be related to genetic disorders or inherited genetically. It occurs in one out of 2,000 live births and affects males slightly more often than females.

What are the symptoms of craniosynostosis?

The most common symptom of craniosynostosis in infants is changes in the shape of the head and face. This is often the most noticeable or only symptom of this condition in infants. These changes cause an asymmetry in a child’s head and face that cause it to appear different from side to side. Occasionally, synostosis can increase pressure within the skull. This is especially true when multiple sutures are fused too early. Symptoms of craniosynostosis with increased 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.

What are the different types of craniosynostosis?

There are 3 different types of craniosynostosis: Sagittal, Coronal and Metopic. Each type is named for the suture that has fused prematurely in the infant’s skull, resulting in craniosynostosis.

Sagittal synostosis

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.

Coronal synostosis

A fusion of the coronal suture, which limits the normal forehead and brow growth. 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.

Metopic synostosis

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 cause a prominent ridge running down the forehead. This can make the forehead look pointed, like a triangle, and the eyes look like they are too close together.

How is craniosynostosis treated?

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

For babies up to 6 months of age, CHOC is the only hospital in the region to offer a minimally invasive, endoscopic procedure. Two small incisions are made and a small strip of bone is removed, to allow the head to grow normally. Babies typically go home from the hospital the next day and will wear a temporary helmet.

For older children with craniosynostosis, CHOC neurosurgeons are the regional leaders in performing an open procedure to correct your child’s head shape, with immediate results. Following the operation, it is common for children to have a very swollen face and eyelids.  The eyes may even swell shut. Most children 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.

Craniosynostosis vs Plagiocephaly (flat head syndrome)

Most of the time, the head deformity is simply positional plagiocephaly, a benign condition that does not require surgical intervention. However, some deformities are caused by craniosynostosis. Similar to craniosynostosis, plagiocephaly can cause flat spots and bulging areas on the head and forehead, but it is not craniosynostosis because no fusion of the cranial sutures has happened. It is important to get your child properly diagnosed to avoid getting these two conditions confused.

Similar to craniosynostosis, plagiocephaly can cause flat spots and bulging areas on the head and forehead, but it is not craniosynostosis.

Contact Us

We treat all 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. Contact us today to schedule an evaluation of your baby’s head.