The Craniosynostosis Center in the Division of Plastic and Maxillofacial Surgery currently is the only team in the country developed specifically to address the needs of children born with craniosynostosis and their families. The team conveniently allows the baby and parents to see multiple care providers in a single comfortable setting.
When does the team meet?
The craniosynostosis team currently meets every other month on a Monday morning. The parents and child are asked to be at the Division of Plastic and Maxillofacial Surgery at 7 am. The child will see approximately six care providers over a three-hour span. Of course, it will always be the prerogative of the parents to visit with the team members of their choice. The team than convenes and forms a team summary with findings and recommendations that are then mailed to the families.
The Craniosynostosis Team
Craniofacial Surgeons
Mark M. Urata, MD DDS
Jeffrey Hammoudeh MD DDS
In concert with their neurosurgical colleagues, the craniofacial surgeons remove bones of the skull that will require re-shaping. Then, they are responsible for reconstructing the face and skull providing increased room for the brain while correcting the appearance.
Pediatric Neurosurgeons
Gordon McComb, MD
Mark Krieger, MD
The neurosurgeons work side by side with the craniofacial surgeons to remove the bones of the skull and face necessary for reconstruction. They are responsible for protecting the brain during early part of the operation.
Pediatric Ophthamologist
Kristina Tarzcy-Hornoch, MD, DPhil
In some forms of craniosynostosis, the eye socket is misshapen. This may affect the orientation of the muscles that control the movement of the eyeball (globe). Additionally, there is the rare patient with craniosynostosis that will develop pressure on their brain. Since the eye is like a window to the brain, the pediatric ophthalmologist will look for papillaedema, a sign that there is pressure on the brain. Although infrequent, she will also evaluate the muscles of the eyes to determine whether any corrective surgery will need to be done
Pediatrician
Bonnie Gutierrez, MD
The pediatrician will evaluate the overall health of the baby. Of course, this is not a substitute for regular visits to your own pediatrician. However, our pediatrician is experienced in treating babies with synostosis. In babies with syndromic synostosis, the pediatrician will look for other anomalies that may be present.
Social Worker
Laura Garcia, MSW
Psychologist
Alessi Johns, Ph.D.
The psychologist will help to address any questions you may have about development. Moreover, we conduct developmental tests
Craniofacial Nurse Practitioners
Karla Haynes, RN, MPH, CPNP
Helen Lau, RN, MS, CPNP
Patricia A. McKee, RN, MN, CPNP
The craniofacial nurse practitioner will formulate a summary of finding and recommendations for you and your baby, as well as monitor the overall health of your baby.
COMMON QUESTIONS
What is craniosynostosis?
Normally, the skull of an infant has gaps between the developing bones. These gaps provide room for the rapidly growing brain. Brain growth is approximately 70% completed by the age of 18 months old. As you can see on the graph below, the remaining 30% typically occurs at a much slower rate between 18 months and 7 years.
[insert: Brain line graph]
To allow for this brain growth, the sutures or gaps between skull bones normally begin closing as follows:
[insert: Suture bentz closure graph]
Cransynostosis is the premature closure of one or more of the gaps between the developing bones of the skull. Craniosynostosis is typically discovered by the pediatrician or parents within the first few months of life. For some babies, the diagnosis can best be determined by a trained craniofacial surgeon. The following describes the different types of craniosynostosis from the most common to the rarest:
Sagittal synostosis
Sagittal synostosis is the premature closure of the sagittal suture that is located on the top of the head, running from front to back. A ridge on the top of the head can usually be felt through the scalp. When this suture closes early, the baby begins to have an elongation of the head from front to back(scaphocephaly) with narrowing of the temple region(bitemporal narrowing). Some babies will develop a prominent forehead(frontal bossing).
Unilateral coronal synostosis
There are two coronal sutures, each running from the top of the head down the sides in front of the ears. When one of these sutures closes prematurely, the baby begins to develop flatness of the forehead on the affected(ipsilateral )side. A ridge over the affected suture may be felt through the scalp. The eyebrow may appear raised on the affected side. The ear on the affected side will appear more forward when looking from the top (bird’s eye view). The top of the nose or nasal bridge will be deviated toward the affected side while the tip of the nose will point toward the unaffected (contralateral) side. The chin will appear canted toward the ipsilateral side as well.
Bilateral Coronal synostosis
This is when both coronal sutures are affected. A ridge can be felt on both sides of the head running from the top of the skull down the sides in front of the ears. Depending how early this is discovered, the forehead will appear flat and under projected. This will inturn, make the eyes appear as if they are sticking out. The head will appear taller and wider than usual.
Children with syndromes such as Apert, Pfeiffer, and Crouzon typically have bicoronal synostosis, but not all bicoronal synostosis are part of a syndrome. If the baby has an associated syndrome, he or she may also have a underprojected midface as depicted below.
Lambdoidal synostosis
There are two lambdoidal suture which meet in the back of the head in an upside down configuration. This is the rarest form of craniosynostosis and comprises only 1% of all cases of craniosynostosis.
What causes craniosynotosis?
Craniosynostosis is usually an isolated finding in an otherwise normal child. The precise causes are incompletely understood. Most cases of craniosynostosis occur in families with no history of the condition. Mutations in several genes have recently been identified in certain forms of craniosynostosis. Its heredity form has been associated with various genetic disorders, including Crouzon Syndrome and Apert Syndrome.
Several non-genetic factors have also been implicated in the origins of craniosynostosis, including fertility treatments, paternal profession and such environmental exposures as maternal smoking and certain drugs (sodium valproate).
How do you treat craniosynostosis?
As one of the largest craniofacial centers in the country, we have the highest level of experience and comfort with treating babies born with craniosynostosis. In fact, we are the only center that has established a separate craniosynostosis team to focus on the needs of this special group of children. Typically, the baby will be evaluated by the craniofacial surgeon and one of the craniofacial nurse practitioners if the child appears to have an associated syndrome. The craniofacial surgeon will likely order a CT scan to evaluate the diagnosis and plan the reconstruction.
Appointments
To request a consultation for your child, please contact the Craniosynostosis Center at (323) 361-2154.
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