Craniosynostosis repair

Definition

Craniosynostosis repair is surgery to correct a deformity that causes the bones of a child's skull to grow together (fuse) too early.

Alternative Names

Craniectomy; Synostectomy; Strip craniectomy; Endoscopy-assisted craniectomy; Sagittal craniectomy; Frontal-orbital advancement; FOA

Description

This surgery is performed in the operating room under general anesthesia (your child will be asleep and will not feel pain).

Traditional surgery is called open repair. It includes these steps:

• The most common place for a surgical cut to be made is over the top of the head, from just above one ear to just above the other ear. The cut is usually wavy. The exact placement of the cut may be different for different problems.
• A flap of skin, tissue and muscle below the skin, and the tissue covering the bone are loosened and raised up so the surgeon can see the bone.
• A strip of bone is usually removed where two sutures connect. This is called a strip craniectomy. Sometimes, larger pieces of bone must also be removed. This is called synostectomy. Parts of these bones may be changed or reshaped when they are removed, and then they are put back in. Other times, they are removed and not put back in.
• Sometimes, bones that are left in place need to be shifted or moved.
• Bones are then fastened using a plate with screws that go into the skull.

Surgery usually takes 3 to 7 hours. Your child will probably need to have a blood transfusion during or after surgery to replace blood that is lost during the surgery.

A newer kind of surgery is used for some children. This type is usually done for children younger than 3 to 6 months old.

• The surgeon makes one or two small cuts in the scalp. Most times these cuts are each just 1 inch long. These cuts are made above the area where the bone needs to be removed.
• A tube that allows the doctor to see in (endoscope) is passed through the small cuts. Special medical devices and a camera are passed through the endoscope. Using these devices, the surgeon removes some bone through the cuts.
• This surgery usually takes about 1 hour. There is much less blood loss with this kind of surgery.
• Most children need to wear a special helmet to protect their head for a period of time after surgery.

Children do best when they have this surgery when they are 3 months old. The surgery should be done before the child is 6 months old

Why the Procedure Is Performed

A baby's head, or skull, is made up of many different bones. The connections between these bones are called sutures. When a baby is born, it is normal for these sutures to be open a little. This gives the baby's brain and head room to grow.

Craniosynostosis is a condition that causes one or more of the baby's sutures to close too early. This can cause the shape of your baby's head to be different than normal.

An x-ray or computed tomography (CT) scan can be used to diagnose craniosynostosis. Surgery is usually needed to correct it.

Surgery frees the sutures that are fused. It also reshapes the brow, eye sockets, and skull as needed. The goals of surgery are:

• To relieve any pressure on the child’s brain
• To make sure there is enough room in the skull to allow the brain to properly grow
• To improve the appearance of the child's head

Risks

Risks for any surgery are:

• Breathing problems
• Infection, including in the lungs and urinary tract
• Blood loss (children having an open repair may need a transfusion)
• Reactions to medicines

Possible risks of having this surgery are:

• Infection in the brain
• Brain swelling
• Damage to brain tissue

Before the Procedure

For a planned surgery (not an emergency), you will need to prepare your child.

During the days before the surgery:

• Tell your doctor or nurse what drugs, vitamins, or herbs you are giving your child. This includes anything you bought without a prescription. You may be asked to stop giving your child some of these medications in the days before the surgery.
• Ask your child's doctor which drugs your child should still take on the day of the surgery.

On the day of the surgery:

• Give your child a small sip of water with any drugs your doctor told you to give your child.
• Your child's doctor or nurse will tell you when to arrive for the surgery.

Ask your doctor or nurse about your child's eating and drinking before the surgery. General guidelines are:

• Older children should not eat any food or drink any milk after midnight the night before the operation. They can have clear fluids (juice or water) and breast milk up to 4 hours before the operation.
• Infants younger than 12 months can usually eat formula, cereal, or baby food until about 6 hours before surgery. They may have clear fluids and breast milk until 4 hours before surgery.

Your doctor may ask you to wash your child with a special soap on the morning of the surgery. Rinse your child well afterwards.

After the Procedure

After the open surgery, your child will be taken to an intensive care unit (ICU). After 1 or 2 days, your child will be moved to a regular hospital room. Your child will stay in the hospital for 3 to 7 days.

• Your child will have a large bandage wrapped around the head. There will also be an IV (a tube that goes into the vein). The nurses will watch your child closely.
• Tests will be done to see if your child lost too much blood during surgery. The doctor may give your child blood through a transfusion if needed.
• Your child will have swelling and bruising around the eyes and face. Sometimes, the eyes may be swollen shut. This often gets worse in the first 3 days after surgery, but it will be better by day 7.
• Your child should stay in bed for the first few days. The nurses will keep the head of your child's bed raised to help keep the swelling down.

Talking and singing, playing music and telling stories may help soothe your child. Acetaminophen (Tylenol) is used for pain, but your nurse will have other pain medicines if your child needs them.

Most children who have endoscopic surgery can go home after staying in the hospital 1 night.

Outlook (Prognosis)

Most of the time, the outcome from craniosynostosis repair is good.

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References

Kanev PM. Congenital malformations of the skull and meninges. Otolaryngol Clin North Am. 2007 Feb;40(1):9-26, v.

Baskin JZ. Craniofacial surgery for congenital and acquired deforminities. In: Cummings CW, Flint PW, Haughey BH, et al, eds. Otolaryngology: Head & Neck Surgery. 5th ed. Philadelphia, Pa: Mosby Elsevier; 2010:chap 185.