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Crouzon Syndrome Treatment Options

Skull

Surgery can be done to remove closed sutures and expand and reshape the skull. The timing and recommendations for this surgery (cranial vault expansion and reshaping) is based upon the type and number of closed sutures.

Infants with multiple-suture synostosis resulting in a severe skull deformity may require cranioplasty surgery early in the first year of life.

Ten to twenty percent of children with early cranial vault expansion will require a second cranioplasty.

Eyes

Children with Crouzon syndrome have an abnormal position and shape of their eye sockets.

They are at risk for visual impairment for several reasons including the misalignment of the eyes and increased intracranial pressure.

Children should visit an ophthalmologist on a regular basis for these concerns. Early frontoorbital advancement may be needed for children with extremely shallow orbits resulting in incomplete eyelid closure and risk of keratitis (corneal irritation).

Brain

Because craniosynostosis can occur with normal head shape and symptoms of increased intracranial pressure in older children, evaluation of severe recurrent headaches is warranted.

Some people with Crouzon syndrome do not develop craniosynostosis or symptoms requiring cranial surgery.

Infants and children with Crouzon syndrome should be checked for signs and symptoms of hydrocephalus (excess fluid in the brain). Symptoms in infants include bulging anterior fontanelle (soft spot), irritability (feeling cranky) or lethargy (feeling sluggish) and rapidly increasing head size.

In older children, symptoms include headaches (worse in the morning), vomiting, vision impairment and declining school performance.

Some infants and children with hydrocephalus will require surgery to place a ventriculoperitoneal shunt (tube draining excess fluid from areas surrounding the brain to the abdomen).

Some children with Crouzon syndrome have a Chiari malformation (abnormality in the back of the brain) because of an abnormal shape of the base of the skull.

Sometimes the brain is compressed by the base of the skull, resulting in headaches or other neurological symptoms. Surgery to widen the base of the skull is performed on a subset of children with this disorder.

Hearing

Some children with Crouzon syndrome have conductive (bone) hearing loss or abnormalities of the ear canal. An initial hearing check and periodic follow-up is recommended.

Speech difficulties can occur as a result of hearing difficulties, the abnormally high arched palate or the small nasal cavity. If needed, a team speech pathologist will recommend speech therapy.

Breathing

Infants with Crouzon syndrome may have difficulty breathing through their nose. This can represent a major problem as babies can only breathe through their nose.

Some infants with Crouzon syndrome who have severe breathing difficulty will require placement of a surgical tracheostomy (a breathing tube in the windpipe).

Older children with Crouzon syndrome may develop sleep apnea symptoms or snoring when the combination of the small nose and poor midface growth result in a too-small airway. Sleep apnea can lead to problems with how the heart and brain develop. It needs to be treated early.

A midface advancement (LeFort III midface advancement, or LeFort III osteotomy) to move the middle portion of the face forward and align the upper and lower jaw to create a larger airway is often considered in childhood.

Seattle Children’s specializes in this method. We have one of the largest groups of patients in the country who have had this treatment. It may cure sleep apnea, as it did for the girl in these photos.

LeFort III midface advancement (also called LeFort III osteotomy) before and after pictures:  

crouzon_beforeduringafter 

BEFORE (Above, left): The middle of this girl’s face didn’t grow well because of Crouzon syndrome. The airway between her nose and mouth was small, which caused sleep apnea (severe breathing problems during sleep). Sleep apnea can lead to problems with how the heart and brain develop. It needs to be treated early.

DURING (Above, center): She had surgery to separate her facial bones from her skull and attach a device called a distractor. Starting about four days later, the distractor was turned 1 to 2 millimeters a day. This way, we could bring her upper jaw and cheekbones forward. This procedure is called Le Fort III midface advancement.

AFTER (Above, right): New bone grew to fill in the gaps, and then the distractor was removed. Thanks to our research, we’ve developed a way to remove the distractor within six weeks—which is shorter than other hospitals that use the same method. This girl no longer has sleep apnea. Her teeth fit together better, and her cheekbones protect her eyes better.

SCAR (Below, scar shown on a different patient): At Seattle Children’s, the LeFort III method leaves a single scar hidden in the hair. To connect the upper jaw to the device, we use small incisions and a custom splint that fits in your child’s mouth. We do not make incisions to the mouth or the eyelids, and we do not place posts in their cheeks, as most other hospitals do. Our method helps prevent scars on your child’s face.

scar_beforeafter
The timing of midface advancement is a team decision based on age, degree of jaw misalignment (chewing and speech difficulties) and symptoms of obstructive sleep apnea (based on an overnight sleep study).

Midface advancements may need to be repeated because the lower jaw will continue to grow, eventually passing the upper jaw again.

Midface advancements can be done with a single surgery or over a period of several months through distraction osteogenesis, a technique that uses a device to pull the midface slowly forward after surgery and move it further forward than a single surgery can.

Other surgical options for children with sleep apnea include adenoidectomy, a procedure to remove adenoids or lymphoid tissue in the back of the nose, or tracheostomy.

Teeth

All children with Crouzon syndrome are seen on a regular basis by a team orthodontist due to crowding of the teeth and jaw misalignment.

Because of the small upper jaw, some upper teeth may need to be extracted to relieve crowding as the child develops.

Placement of orthodontic braces to expand the palate and align the teeth is coordinated as needed with jaw surgery. To provide a normal bite, final jaw surgery and braces must often wait until after facial growth is finished.

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Spring 2014: Good Growing Newsletter

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