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Craniosynostosis

Medically reviewed by Drugs.com. Last updated on Sep 15, 2022.

Overview

Craniosynostosis (kray-nee-o-sin-os-TOE-sis) is a disorder present at birth in which one or more of the fibrous joints between the bones of your baby's skull (cranial sutures) close prematurely (fuse), before your baby's brain is fully formed. Brain growth continues, giving the head a misshapen appearance.

Usually, during infancy the sutures remain flexible, allowing a baby's skull to expand as the brain grows. In the front of the skull, the sutures meet in the large soft spot (fontanel) on top of the head. The anterior fontanel is the soft spot felt just behind a baby's forehead. The next largest fontanel is at the back (posterior). Each side of the skull has a tiny fontanel.

Craniosynostosis usually involves premature fusion of a single cranial suture, but it can involve more than one of the sutures in a baby's skull (multiple suture craniosynostosis). In rare cases, craniosynostosis is caused by certain genetic syndromes (syndromic craniosynostosis).

Treating craniosynostosis involves surgery to correct the shape of the head and allow for brain growth. Early diagnosis and treatment allow your baby's brain adequate space to grow and develop.

Although neurological damage can occur in severe cases, most children develop as expected in their ability to think and reason (cognitive development) and have good cosmetic results after surgery. Early diagnosis and treatment are key.

Cranial sutures and fontanels

Joints made of strong, fibrous tissue (cranial sutures) hold the bones of your baby's skull together. The sutures meet at the fontanels, the soft spots on your baby's head. The sutures remain flexible during infancy, allowing the skull to expand as the brain grows. The largest fontanel is at the front (anterior).

Symptoms

The signs of craniosynostosis are usually noticeable at birth, but they become more apparent during the first few months of your baby's life. Signs and severity depend on how many sutures are fused and when in brain development the fusion occurs. Signs and symptoms can include:

Types of craniosynostosis

There are several types of craniosynostosis. Most involve the fusion of a single cranial suture. Some complex forms of craniosynostosis involve the fusion of multiple sutures. Multiple suture craniosynostosis is usually linked to genetic syndromes and is called syndromic craniosynostosis.

The term given to each type of craniosynostosis depends on what sutures are affected. Types of craniosynostosis include:

Other reasons for a misshapen head

A misshapen head doesn't always indicate craniosynostosis. For example, if the back of your baby's head appears flattened, it could be the result of spending too much time lying on one side of the head. This can be treated with regular position changes, or if significant, with helmet therapy (cranial orthosis) to help reshape the head to a more balanced appearance.

When to see a doctor

Your health care provider will routinely monitor your child's head growth at well-child visits. Talk to your pediatrician if you have concerns about your baby's head growth or shape.

Causes

Often the cause of craniosynostosis is not known, but sometimes it's related to genetic disorders.

Complications

If untreated, craniosynostosis may cause, for example:

The risk of increased pressure inside the skull (intracranial pressure) from simple craniosynostosis is small if the suture and head shape are fixed surgically. But babies with an underlying syndrome may develop increased intracranial pressure if their skulls don't expand enough to make room for their growing brains.

If untreated, increased intracranial pressure can cause:

Diagnosis

Craniosynostosis requires evaluation by specialists, such as a pediatric neurosurgeon or a specialist in plastic and reconstructive surgery. Diagnosis of craniosynostosis may include:

Treatment

Mild cases of craniosynostosis may not need treatment. Your health care provider may recommend a specially molded helmet to help reshape your baby's head if the cranial sutures are open and the head is misshapen. In this situation, the molded helmet can assist your baby's brain growth and correct the shape of the skull.

However, for most babies, surgery is the primary treatment. The type and timing of surgery depends on the type of craniosynostosis and whether there's an underlying genetic syndrome. Sometimes more than one surgery is required.

The purpose of surgery is to correct the head shape, reduce or prevent pressure on the brain, create room for the brain to grow properly, and improve your baby's appearance. This involves a process of planning and surgery.

Surgical planning

Imaging studies can help surgeons develop a surgical procedure plan. Virtual surgical planning for treatment of craniosynostosis uses high-definition 3D CT scans and MRI scans of your baby's skull to construct a computer-simulated, individualized surgical plan. Based on that virtual surgical plan, customized templates are constructed to guide the procedure.

Surgery

A team that includes a specialist in surgery of the head and face (craniofacial surgeon) and a specialist in brain surgery (neurosurgeon) generally performs the procedure. Surgery can be done by endoscopic or open surgery. Both types of procedures generally produce very good cosmetic results with low risk of complications.

Helmet therapy

After minimally invasive surgery, office visits at certain intervals are needed to fit a series of helmets to help shape your baby's skull. The surgeon will determine the length of helmet therapy based on how quickly the shape responds to treatment. If open surgery is done, usually no helmet is needed afterward.

Coping and support

When you learn that your baby has craniosynostosis, you may experience a range of emotions. You may not know what to expect. Information and support can help.

Consider these steps to prepare yourself and to care for your baby:

Preparing for an appointment

In some cases, your baby's pediatrician may suspect craniosynostosis at a routine well-baby visit. In other cases, you may make an appointment because you have concerns about your baby's head growth. Your health care provider can refer you to a specialist for diagnosis and treatment.

Here's some information to help you get ready for your appointment. If possible, bring a family member or friend with you. A trusted companion can help you remember information and provide emotional support.

What you can do

Before the appointment, make a list of:

Questions to ask might include:

Don't hesitate to ask other questions during the appointment.

What to expect from your doctor

Your health care provider is likely to ask you questions, such as:

Your health care provider will ask additional questions based on your responses. Preparing and anticipating questions will help you make the most of your appointment.

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