Dallas
214-456-2240
Fax: 214-456-8881
Plano
469-497-2501
Fax: 469-497-2507
Request an Appointment with codes: Plastics and Craniofacial Surgery
Children with syndromic craniosynostosis (syn·drom·ic - cra·nio·syn·os·to·sis ) require a team of specialists to best manage their care. It is best for a patient to see all of these specialists in one clinic visit. At Children’s Health℠ the entire team sees the patient in a single two-hour visit.
214-456-2240
Fax: 214-456-8881
469-497-2501
Fax: 469-497-2507
Request an Appointment with codes: Plastics and Craniofacial Surgery
Patients with syndromic craniosynostosis, a congenital (present at birth) condition, have more complicated needs than those with isolated (non-syndromic) craniosynostosis. There are several reasons for this. The first is that these patients have globally impaired growth of the skull and facial bones that makes it harder to achieve a lasting correction of the appearance and maintain adequate space in the skull for the growing brain. A patient may need several operations during childhood to “catch up” to where they should be if their skulls and face were growing normally.
Patients with syndromic craniosynostosis frequently have significant additional problems with breathing, hearing, speech and eating. They may also be born with deformed hands and feet which present additional functional and surgical challenges. Patients with so many complex problems require a team of specialists with expertise in each of the child’s problem areas and experience in caring for children with syndromic craniosynostosis.
These syndromes are usually genetic in nature. The problem may occur when a gene mutates, or may be genetically inherited. At a busy craniofacial center like ours, we see even the most rare syndromes, but on this webpage, we will cover only the more common craniosynostosis syndromes.
Craniosynostosis syndromes are usually named after the person who first described it. There are at least 70 conditions that can cause syndromic craniosynostosis.
The most common of syndromes include:
These may share some common features. For Crouzon, Apert and Pfeiffer syndromes the patient’s eyes may look abnormally large or prominent. The eyes are actually of a normal size, but the eye sockets are more shallow than normal because of abnormal growth of the bones. This causes the eyes to bulge and appear large, and when this is severe it can become difficult for the eyelids to close which can dry the eyes severely.
These patients also have flatness in the middle of the face caused by poor growth of the upper jaw. The hands and feet may also have deformities.
The risk of increased pressure in the skull rises greatly with each closed cranial suture. This is because it makes the open sutures work much harder to make bone. Also, patients with syndromic craniosynostosis may have other problems related to the added pressure in the skull. They have a higher risk of:
Waiting too long for surgery while elevated pressure is present can cause blindness and learning delays. This makes diagnosis and prevention of elevated pressure in the skull a priority in treating patients with craniosynostosis.
Children with syndromic craniosynostosis require a team of specialists to best manage their care. It is best for a patient to see all of these specialists in one clinic visit. At Children’s Health the entire team sees the patient in a single two-hour visit. Our team then sits in a conference where each specialist talks about the patient’s needs and a collective plan created by all providers is mapped out for the upcoming year.
We always try to anticipate as many problems as possible in order to reduce risks presented by anesthesia in our surgeries. We follow our patients until they are fully grown adults.
Team members include:
What kind of surgery and when to do it depends on each patient’s needs. We perform a limited skull surgery to remove closed cranial sutures if skull pressure is high before six months of age. This allows the brain to grow without alleviate pressure and the skull bones to thicken so a definitive open cranial vault expansion can be performed.
Patients with syndromic craniosynostosis have a much greater risk for developing elevated pressure in the skull than non-syndromic craniosynostosis patients. These patients sometimes require an average of three or more skull surgeries. The major risk in young patients is blood loss because of their small size and small circulating blood volume. During most surgeries, these patients need blood transfusions.
At Children’s Health, we typically treat syndromic craniosynostosis patients with their first surgery between six months and one year of age. However, we operate earlier if there are multiple sutures causing elevated pressure on the brain.
Surgeries to treat syndromic craniosynostosis include: