Crouzon Syndrome

 

What is Crouzon Syndrome

Crouzon Syndrome is a rare, craniosynostosis syndromes disorder that affects one in every 60,000 newborns. Babies with Crouzon Syndrome have abnormal head shapes and sizes, large foreheads, beaked noses and bulging eyes. Breathing, vision and hearing problems are common with children who have Crouzon Syndrome. The center of their face may not be proportional and asymmetrical.

Babies with Crouzon Syndrome have premature fusing of certain cranial plates. The premature fusing of the skull bones alters the structure of the face and size of the head. It can, in severe cases limit the growth of the brain or cause pressure on the brain.

There is no cure for Crouzon Syndrome and many mild cases involve non-surgical treatment. Crouzon Syndrome is often detected and diagnosed within a child’s first few years of life. Sometimes, symptoms can occur during infancy or not become apparent until a child is three or four years old.

Crouzon Syndrome is caused by the same genetic defect as Beare-Stevenson Syndrome, Pfeiffer Syndrome, Jackson-Weiss Syndrome and Alpert Syndrome. Crouzon Syndrome can be accompanied by other conditions such as acanthosis nigricans or can cause Saethre-Chotzen Syndrome or other craniofacial issues.

Treating Crouzon’s Syndrome

Not all babies who have Crouzon Syndrome require surgery to treat their condition. Those with one or two prematurely fused sutures and those with the premature fusion of certain skull plates can be treated non-surgically using helmet therapy.

Helmet Therapy

With helmet therapy, a baby will wear a series of custom-fitted helmets for a certain amount of time. The helmets will help the baby’s head size and shape to grow properly. Sometimes helmet therapy will be required after endoscopic surgery is done.

Endoscopic Surgery

If surgery is needed the most common options are endoscopic and open surgery. Endoscopic surgery is recommended for babies six months of age and younger and who have only one prematurely closed suture. It is minimally invasive and often requires only a one-night stay in the hospital.

Open Surgery

For older babies who have multiple premature sutures, open surgery may be recommended. With open surgery, multiple incisions are made in the scalp to correct the shape of the skull. The plates are held in correct position using absorbable screws. A multi-night hospital stay and blood transfusions are required after open surgery.

In very severe cases, multiple open surgeries may be necessary to correct the shape of a baby’s skull.

At the Cleft & Craniofacial Institute of Utah we provide diagnosis for Crouzon’s Syndrome as well as individualized treatment.

Regardless of your child’s age or the severity of his or hers Crouzon Syndrome, we will commit to proving the best possible treatment so he or she can live a healthy and happy life.

Untreated Crouzon Syndrome

The severity of Crouzon’s Syndrome varies from baby to baby. Depending on how many cranial sutures have closed and fused together and what plates of the head have prematurely closed, the risks of non-treatment can vary from mild facial deformities to severe pressure on the brain.

The most common complications that can occur in children with Crouzon’s Syndrome include:

  • Vision and eye disease and infections
  • Difficulty breathing
  • Impaired brain growth
  • Pressure on the brain
  • Hearing loss
  • Impaired speech
  • Chronic headaches
  • Cleft palate
  • Crooked teeth and misaligned jaws (under or over-bite)
  • Difficulty eating
  • Acanthosis nigricans (a skin condition where dark, thick, velvety patches of skin develop)
  • Self-esteem and confidence issues stemming from an abnormal facial appearance
  • Possible intellectual disability

Additional Information

Contact the Cleft & Craniofacial Institute of Utah today to set up a consultation with the doctor and discover your child’s Crouzon Syndrome treatment options.

 

Our Expertise

Cleft Lip/Palate

 Cleft lips and palates are one of the most common birth anomalies that affect about 1/600 newborn babies in the United States and around 1/500 in the state of Utah.  Clefts can range from quite mild to fairly severe.  Most of the time they are an isolated anomaly, but are also associated with a number of gentic syndromes.  Depending on what structures are involved dictates the complexity of the treatment plan.   Our team works together with you to make a customized plan for  your baby.  Even the most severe of cases should be able to be treated with only a few surgical interventions by a well coordinated and efficient team.

 

 

READ MORE

 

Craniosynostosis

The skull is made up of several different bones that are joined together by growth plates called "sutures."  Craniosynostosis is a condition where one or more of these sutures fuse prematurely causing an abnormal head shape, decreased cranial volume, and the possiblilty of raised intracranial pressure.  The treatment of craniosynostosis is surgical and the goals of surgery are to increase the volume of the skul and to correct the abnormal appearance of the head shape.  There are two methods of surgical correction: open vault reconstruction and strip craniectomy.  Dr. Griner's extensive training allows him to perform these complex procedures without the need for blood transfusions and spending only 1-2 nights in the hospital. 

READ MORE

Syndromes

There are many syndromes that can affect the shape of the head and face and include Aperts, Crouzon, Pfieffer, Treacher Collins, and many others.  Most of these syndromes include many other complex anomalies and need to be treated in a coordinated approach in order to reduce the number of operations the child will need.  These syndromes are rare and require specialists that are very familiar with the intricacies of each syndrome.  Dr. Griner's unique training provided him with extensive experience with craniofacial syndromes and makes him the premier choice to guide the management of your child's needs in Utah.

 

 

 

READ MORE

Ear Deformities

Ear deformities range from mild changes in shape to the complete abscence of an ear and deafness.  Mild deformities can be treated in the first few weeks of life while others will require reconstructive surgery and hearing aids.  No matter the severity, our specialists work together to provide normal appearance and improved hearing.

READ MORE

From the Blog

Parenting a Cleft Child: Selecting a Surgeon

You stare at that ultrasound, taking in the news that your baby has a cleft lip and/or palate. You’re referred to a specialist to confirm that diagnosis. You see the specialist, baby’s cleft is confirmed. You don’t know how to feel or what questions to ask. It all...

read more

Staged palate repairs

Staged palate repairs were implemented to try and reduce the chance of stunting the upper jaw growth and needing to have it surgically advanced later in life, while at the same time allowing normal speech to develop.  We know from several studies that the growth of...

read more