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Chromosomal and Genetic Conditions

Apert Syndrome

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Because Apert syndrome is a complex condition, the optimal treatment requires a coordinated approach by a team of specialists in various fields.

On average, these children will undergo about 20 operations (craniofacial and hand surgery combined) by the time they are out of their teens.

Apert Syndrome Treatment Options

Head and skull

Most times, the first major craniofacial operation is cranial vault remodeling and orbital rim advancement, done at 9 to 11 months of age. The procedure may be done sooner if there are signs of increased pressure on the brain.

In this procedure, the skull is reshaped to give the brain more room to grow and to normalize the appearance of the skull and the upper part of the orbits (the bony housing of the eyes).

Waiting until 9 to 11 months of age leaves the bone still malleable enough to be reshaped while being solid enough to hold its new shape.

A craniofacial surgeon works together with a neurosurgeon to do this operation.

Later in childhood or during the teen years, the midface must be advanced, or brought forward, in another operation. Your child may have one of these operations:

Segmental subcranial distraction: before and after pictures

apertboy_front_beforeafter

apertboy_side_beforeafter

BEFORE (Left): The middle of this boy’s face didn’t grow well because of Apert syndrome. To correct this, surgeons used a new method to move the bones of his face different amounts and in different directions. That method is called segmental subcranial distraction. First, the boy had surgery to separate his facial bones from his skull and attach a device called a distractor. Then the device was turned 1 to 2 millimeters (mm) a day to move the bones. New bone grew to fill in the gaps, and then the distractor was removed.

AFTER (Right): After the procedure, the boy’s cheekbones were moved forward 5 mm (1/4 inch). His nose, upper jaw and teeth were moved forward and down 25 mm (1 inch). This made his face longer, gave him a more typical look, and made his teeth fit together better. Our Craniofacial Center is the first center to use this method.

Ideally, the procedure is performed when the growth of the face is almost complete to minimize the likelihood that the surgery will need to be repeated. If possible, this is also done after the development of the molar teeth so that these teeth are not damaged.

However, the procedure may need to be performed earlier to address problems such as severe obstructive sleep apnea that results from the retruded position of the midface and irritation of the eyes from inadequate bony coverage.

Further craniofacial procedures are usually required, such as a LeFort I osteotomy to position the upper teeth in the correct relationship to the lower teeth. The upper jaw is moved forward to correct the underbite and anterior open bite.

In most cases, this is done once the body is done growing.

Hands and feet

Hand surgery procedures begin in the first year of life.

If the thumbs are fused, they must be released early on (about 6 months of age), because the thumb is critical for hand function and development.

Multiple procedures are required for the release of the fingers on each hand. Ideally, all digits are separated by about 2 years of age.

The feet require surgical treatment only in the most severe cases, when the fusion of the toes forces the foot into an abnormal position such that it cannot strike the ground flat or fit easily into shoes.

Who Treats This at Seattle Children's?

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