What is Crouzon Syndrome?
Crouzon Syndrome is a condition resulting from premature fusion of the sutures of the skull and deformity of the skull. Characteristics include:
- Skull is prematurely fused and unable to grow normally (craniosynostosis)
- Bulging wide-set eyes due to shallow eye sockets (ocular proptosis)
- A small underdeveloped upper jaw
- Downward slanting eyelids
- Curved, parrot-like nose
- High, narrow, arched palate
Crouzon Syndrome with Acanthosis Nigricans (AN) is found in an estimated 5-10% of all Crouzon cases. In addition to the facial characteristics, it includes some of the following:
- Darkened, rough patches of skin found in the folds of the body (armpits, neck, groin, elbows, knees, chin/mouth area, eye area, or stomach).
- Signs of the discoloration begin between the ages of 2 and 4.
- AN generally does not advance after the age of 12.
Why did this happen?
There is no link between anything the mother did or did not do while she was pregnant and the occurrence of Crouzon Syndrome. Doctors believe it is caused by changes in the gene (FGFR2) mapped to chromosome 10. Crouzon Syndrome with Acanthosis Nigricans is caused by changes in the gene (FGFR3) mapped to chromosome 4. The cause of the change is not currently known.
Will this happen to children I have in the future?
If you have Crouzon Syndrome, there is a 50% chance that children you have will be born with it. If both parents are unaffected, the risk is very small that it will occur in other children.
What kinds of problems could my child have?
In addition to the physical characteristics common to Crouzon Syndrome, your child may have the following problems:
- Dental problems due to crowded teeth and a narrow palate
- Poor vision
- Ear diseases and hearing loss in about 50% of children
- Difficulty breathing due to small airway
- Possible fluid on the brain (hydrocephalus)
Will my child need surgery?
Depending on the severity of Crouzon Syndrome, your child may have some or all of the following surgeries:
- Frontal orbital advancement to allow the skull to grow properly and to increase the
- Size of the eye sockets
- Jaw surgery
- Orthodontics work
- Surgical advancement of the mid-face
New advances in procedures to treat Crouzon Syndrome are constantly being developed. Be an advocate for your child!
How do I get help for my child?
Your child should be treated by a qualified craniofacial medical team at a craniofacial center. Currently, FACES has information on sixty-four craniofacial teams located in 27 states, the District of Columbia, and Canada. This is by no means a comprehensive list of all the craniofacial teams. Please contact FACES for details.
Am I alone?
No! There are many families and organizations who will be glad to talk with you and help you with information and support. Don't forget books, videos, and websites.
This listing below will get you started.
FACES: The National Craniofacial Association
P. O. Box 11082
Chattanooga, TN 37401
We provide financial support for non-medical expenses to patients traveling to a craniofacial center for treatment. Eligibility is based on financial and medical need. Resources include newsletters, information about craniofacial conditions, and networking opportunities.
International Crouzon Syndrome Support Group
This Facebook site is maintained by a mother born with Crouzon Syndrome, who has two children with Crouzon Syndrome. You will find comprehensive information on Crouzon, as well as informative and helpful guides to dealing with surgeries, schools, teasing, and raising happy and successful children.
Crouzon Support Yahoo Group Network
This link will take you the the active online discussion group where you can meet other families whose members have Crouzon Syndrome.
National Health Law Program
1444 I St., NW, Ste. 1105
Washington, DC 20005
Provides extensive information on health care law affecting families with children who have special health care needs.
Children with Facial Difference: A Parent's Guide
Written by Hope Charkins, MSW. Order from Amazon.com if you cannot find it in your local bookstore. Excellent resource for parents to help them cope with medical, emotional, social, educational, legal, and financial challenges presented by facial differences of their children.
Babyface: A Story of Heart & Bones
Written by Jeanne McDermott. Published in 2000. 1-800-843-7323. Order from Amazon.com.
Jeanne McDermott, the mother of a child born with Apert Syndrome, tells the story of the challenges and triumphs that her family goes through during her son's first year of life. A must read for any family who has a child with Apert, Pfeiffer, or Crouzon Syndrome.
The Craniofacial Center
Dr. Jeffery A. Fearon, MD, FACS, FAAP, Director
7777 Forest Lane, Suite C-700
Dallas, TX 75230
Visit Dr. Jeffrey Fearon's informative website that is very lay-friendly and easy to understand. He has excellent information on Crouzon Syndrome and what surgeries are often necessary.
Plastic & Craniofacial Surgery for Infants & Children
7777 Forest Lane, Suite B-300
Dallas, TX 75230
Children's Hospital & Regional Medical Center
P O Box 5371/4H-5
Seattle, WA 98105
Cleft Palate Foundation Publications