April 15th, 2013 | The Heads Up!!! Foundation recently donated $3,000 to the Cleft and Craniofacial Program at Riley Hospital for Children at IU Health. The gift will pay for the purchase of the “Craniofacial Family Notebook” which is given to parents of newborns at their first clinic visit. The notebook contains valuable educational information about… Continue Reading
Syndromic craniosynostosis is even more complex and rare than craniosynostosis [link to page]. In syndromic craniosynostosis, multiple sutures in the skull fuse too early and result in a variety of syndromes affecting normal growth of the head and face. Our physicians at Riley Hospital for Children at Indiana University Health are the most experienced in the Midwest with expertise in the most advanced treatments for syndromic craniosynostosis, including LeFort III Distraction and Facial Bipartion.
What is Syndromic Craniosynostosis?
In syndromic craniosynostosis, multiple sutures in the skull fuse too early and result in a variety of syndromes affecting normal growth of the head and face. Affected children have abnormally shaped heads as well as significant changes to the appearance of the eyes, cheeks, jaw and sometimes the hands. Other organ systems can also be affected, including the brain and the heart.
- Apert Syndrome—This involves the abnormal shape and placement of the eyes and development of the upper jaw. Children with Apert Syndrome may also have a cleft palate and webbed fingers and toes.
- Crouzon Syndrome—This also involves abnormal placement of the eyes and the development of both the upper and lower jaws.
- Pfeiffer Syndrome—This involves abnormal shape of the forehead and nose, as well as the position of the eyes and development of the upper jaw. Children with this syndrome may also have dental problems, such as crowded teeth, and their thumbs and toes may appear wider than average.
- Saethre-Chotzen Syndrome—This involves the abnormal shape and position of the eyes and nose, along with the development of the upper jaw. Children with this syndrome also typically have low hairlines and short or webbed fingers.
- Craniofrontonasal Dysplasia—This is a very rare inherited form that involves abnormalities of the head and face, hands, feet and sometimes other skeletal bones. The most common of these abnormalities includes wide-set eyes, a cleft, wide mouth and under-developed facial features.
What causes Syndromic Craniosynostosis?
Syndromic craniosynostosis is a rare birth defect. Most cases of syndromic craniosynostosis are caused by genetic mutations. Children who present with syndromic craniosynostosis typically also have other genetic disorders, particularly Crouzon, Apert, Chotzen, and Pfeiffer syndromes.
How is Syndromic Craniosynostosis diagnosed?
- Physical exam
- CT Scan
How is Syndromic Craniosynostosis treated?
Riley Hospital for Children at IU Health has extensive experience treating syndromic craniosynostosis and other rare craniofacial disorders. Because syndromic craniosynostosis is so rare, it is difficult to find physicians with the expertise to apply the latest treatments. We are the only center in Indiana offering these advanced treatments:
- LeFort III Distraction—This is a highly technical surgery that involves making a cut in the bones of the face at specific points in order to loosen the midface from the rest of the skull and advance it to a more correct position. For children affected by syndromic craniosynostosis, this operation can bring significant improvement with breathing, sleeping, chewing and eyesight. The advantage of this procedure is that it does not require bone grafts from other parts of the child’s body in order to hold the facial bones in place. In addition, greater advances of the mid-face are possible with LeFort III distraction, compared to more traditional techniques. Patients receiving this treatment see improvement in facial profile, dental occlusion and a decrease in respiratory problems.
- Hypertelorism—Hypertelorism surgery aims to reduce the distance between wide-set eyes by surgically removing part of a child’s enlarged nose. And then the orbits are able to be brought closer together.
- Cranial Vault Remodeling—This procedure aims to correct any skull abnormalities through restructuring the skeletal structure of the head and sometimes extracting parts of the fused skull which will allow the skull to grow properly.
Our team of pediatric neurosurgeons, plastic surgeons and dedicated pediatric anesthesiologists take the utmost care when performing rare surgical procedures on your child. Surgery is generally necessary to separate the bones that have fused together and relieve the pressure of the skull on your child’s brain to facilitate normal growth. In some cases, multiple surgeries may be required to correct the problem as the child continues to grow and develop.