What to Know About Craniosynostosis Surgery

Surgical repair of craniosynostosis typically occurs during infancy. The procedure has a high success rate with a very low risk of long-term side effects. Most babies go on to live typical, healthy lives.

Craniosynostosis is a condition in which the bones of a baby’s skull fuse together too early, causing asymmetric head and brain growth.

At birth, your baby’s skull is made up of six bony plates. The bones are connected by soft, flexible tissue called sutures.

Over the first 2 years of life, a baby’s brain grows rapidly. During this time, the cranial sutures allow the skull to expand accordingly. After this rapid head growth, the sutures close and transition into hard, bony skull.

But when a suture closes too early, skull growth ceases in that area. As the rest of the bony skull continues to grow rapidly, this can cause pressure on the developing brain and distortion of the skull and face.

Doctors can often identify craniosynostosis early in infancy by examining your baby and reviewing X-ray or CT imaging of the skull. Although each diagnosis is different, surgery is usually successful, and many babies go on to live typical, healthy lives.

What is the purpose of craniosynostosis surgery?

Doctors usually perform craniosynostosis surgery within the first year of life, when your baby’s brain and skull are growing most rapidly. This is a critical period for your baby’s learning and development.

Craniosynostosis repair allows for healthy brain and skull growth after surgery, along with improved appearance of the skull and facial bones. It reduces the risk of serious complications from severe craniosynostosis.

What is the procedure for craniosynostosis surgery?

Doctors choose one of two main types of craniosynostosis surgery, depending on which they think is right for your baby.

Open cranial vault remodeling

Doctors have long relied upon this surgical approach to treat craniosynostosis. It’s ideal for older infants (ages 6 to 12 months) with less flexible sutures and bones.

Doctors will make a long incision along your baby’s scalp and remove and reshape the affected bone. They then reinsert the bone, sometimes using bone grafts, absorbable stitches, or plates for support.

Endoscopic suturectomy (strip craniectomy)

This procedure is ideal for infants younger than 6 months .

In endoscopic surgery, doctors make small incisions along your baby’s scalp and use a thin, camera-based tool to remove the affected part of the bone.

Sometimes the surgeon places devices like springs or separators to help prevent the abnormal suture from quickly closing again. A second surgery may be required to remove these devices.

Compared with open surgery, this can be a shorter procedure (about 1 to 2 hours ), often with less blood loss and a shorter recovery period. But after surgery, your baby will likely need to wear a helmet for several months.

What are the risks of craniosynostosis surgery?

Like all major pediatric surgeries, craniosynostosis surgery carries some risks.

In some instances, doctors may need to repeat or revise the surgery as your child grows.

Other risks may include:

• fever
• excessive bleeding
• infection
• bone loss
• cerebrospinal fluid leakage
• injury to the brain and surrounding tissues

Craniosynostosis repair requires exposure to general anesthesia at a young age. Your doctor will help you balance the risks of anesthesia with the benefits of early infancy craniosynostosis repair when the skull bones and sutures are more malleable.

Recent research on anesthesia in infancy has been reassuring. A 2019 study evaluated children after 1 hour of general anesthesia in early infancy and found no associated brain deficits at ages 2 and 5 years. A 2018 study indicated no significant IQ deficits in children exposed to anesthesia before age 3 years.

Your doctor can help you understand any further risks specific to your child’s condition.

What is the survival rate for craniosynostosis surgery?

The survival rate after craniosynostosis surgery exceeds 99% .

After initial surgery, some children may need multiple surgical procedures and continual postoperative helmeting for a period of time to help encourage healthy skull growth. Your child’s medical team will let you know how long helmeting is necessary.

Still, most children have an excellent overall outcome, with relatively typical growth and development.

How do I prepare for craniosynostosis surgery?

Your child’s doctor and craniofacial care team will meet with you to help you prepare for surgery. This includes:

• planning for your hospital stay
• planning for at-home care
• coordinating any postsurgery medication, therapy, or equipment needs

Your child’s medical team may ask to order bloodwork for your child, particularly a complete blood count and blood typing and crossmatching to prepare for possible blood transfusion .

Your child will also have skull and brain imaging performed, usually with a CT scan . This detailed 3D imaging helps doctors carefully plan for surgery.

What can I expect during recovery from craniosynostosis surgery?

Although every diagnosis is different, your child will typically remain in the hospital for a brief period after surgery. They may need to spend time in the intensive care unit for close monitoring. Based on your child’s circumstances, your doctor can help you understand the expected hospital stay.

Immediately following surgery, your child may need intravenous (IV) fluids and pain medication. In some instances, they may need a ventilator.

Your doctor may have placed surgical drains through the scalp during surgery. These are tubes used to remove excess fluid or blood. Your doctor will remove them in the hospital and teach you how to care for your child’s scalp incision.

It’s common to have swelling after craniosynostosis surgery. Depending on the type of surgery, your child’s eyes might briefly swell shut, but they will recover.

Your child will be ready to go home to complete recovery once:

• they’re eating and drinking by mouth
• their pain is well-managed with oral medication
• their swelling is improving

Your care team will discuss a home care plan.

Frequently asked questions

How serious is craniosynostosis surgery?

Even when craniosynostosis affects only a single skull suture, it’s still a relatively major operation for a small child. Your child will need care from an experienced team of pediatric neurosurgeons or craniofacial plastic surgeons and will continue to require monitoring after surgery and throughout early childhood.

Can a baby with craniosynostosis live a normal life?

Yes. Most babies with craniosynostosis are otherwise healthy and, with treatment, will go on to lead typical lives.

Some children with craniosynostosis may experience developmental delays or cognitive disabilities.

About 15% to 30% of babies have a type of craniosynostosis called syndromic craniosynostosis. This type occurs as part of a genetic syndrome and has links to additional medical challenges. Common examples include Apert syndrome and Crouzon syndrome .

What happens if you do not fix craniosynostosis?

Without treatment, very mild forms of craniosynostosis may simply result in an asymmetric head appearance. There is still a chance of neurocognitive problems, even in mild cases.

More serious forms of craniosynostosis, particularly when multiple sutures are involved, can produce significant distortion of the skull, face, and brain. Increased pressure on the developing brain may lead to developmental or cognitive disabilities. Serious complications like respiratory problems, seizures, blindness, and brain damage can also occur.

Takeaway

In craniosynostosis, the bones of a baby’s skull fuse too early, causing progressive problems with skull and brain growth and development. Doctors may notice this craniosynostosis during a physical exam as early as infancy and can offer surgical repair during infancy as well.

Early surgery maximizes healthy postoperative brain and skull growth during the period when your child is growing and learning most rapidly. Expert craniofacial surgery teams can treat most types of craniosynostosis using either endoscopic surgery or open cranial vault remodeling.

With treatment advances, many children born with craniosynostosis can now go on to lead healthy lives.