Craniosynostosis is a rare condition that affects the head/skull and face. It occurs in one out of 2,500 births. David A. Chesler, MD, PhD, a Johns Hopkins fellowship-trained, pediatric neurosurgeon who is an expert in treating craniosynostosis, explains.
How does craniosynostosis occur?
The normal skull consists of eight bones, which are joined/separated by fibrous joints called sutures. As an infant grows and develops, the sutures close to form a solid piece of bone we call the skull. Craniosynostosis occurs when one or more of the sutures close too early. It causes problems with normal skull growth.
Is it genetic?
Craniosynostosis is thought to be caused by a combination of genetic and environmental factors. Some studies suggest that environmental factors such as smoking or taking certain medications during pregnancy may cause the condition to develop in the fetus. However, there is no data to support this.
Are there different types of craniosynostosis?
Yes, and some are more common than others.
• Scaphocephaly (also called sagittal synostosis) is the most common form of craniosynostosis, occurring in 50 to 60 percent of cases. It is characterized by a skull that is narrow from ear to ear. Scaphocephaly is also associated with increased pressure on the brain.
• Plagiocephaly (also known as coronal synostosis) occurs in about 15 to 30 percent of cases. The forehead and the brow stop growing on one side, and appear flattened while the forehead tends to bulge on the opposite side. Another form of plagiocephaly, called positional plagiocephaly, which is not a form of craniosynostosis, is characterized by the flattening of one side of the back of the head. It is simply due to an infant’s preference to sleep in one position and it is estimated that 20 to 30 percent of all children born have some degree of positional plagiocephaly.• Trigonocephaly (also known as metopic synostosis) is characterized by a noticeable ridge running down the forehead. As a result, the forehead may look pointed, like a triangle.
What are the symptoms?
Changes in the shape of the infant’s head and face may be noticeable and are generally the first and only symptom. One side of the face may not be the same when compared to the other side.
How is it diagnosed?
Craniosynostosis may be congenital (present at birth) or observed later, during a physical exam by your pediatrician. At Stony Brook Children’s, you’ll be asked if there’s a family history of craniosynostosis or other head or face abnormalities. You may also be asked about developmental milestones, since craniosynostosis can be associated with other developmental delays. A measurement of your child’s head is taken and plotted on a graph to identify normal and abnormal ranges. Diagnostic tests such as x-rays of the head and computed tomography scans (CT or CAT scan) may also be performed.
How is craniosynostosis treated?
Treatment is determined based on your child’s age, overall health and medical history, and the extent of and type of craniosynostosis. The best time to perform surgery is before the child is 1 year of age when the bones are still very soft and easy to remove and reassemble. Traditional open-skull surgery is performed between 6 and 9 months of age and can take between 2 and 12 hours. Blood transfusions are common with open surgery and the average hospital stay is five to seven days.Minimally invasive endoscopic surgery for craniosynostosis is generally performed at 3 months of age and typically takes between 90 and 180 minutes, with an average hospital stay of one night. Post-operative care for endoscopic craniosynostosis surgery often includes a custom-fit helmet for the infant to wear for up to a year, to help guide and mold the shape of the skull as the child grows. The endoscopic procedure offers comparable results to open surgery. And because there is rarely a need for blood transfusions with endoscopic surgery, the risk of complications is dramatically lowered.
What is the Stony Brook difference?
One of the reasons Stony Brook Children’s hired me was for my advanced training in both the open and minimally invasive methods of treating craniosynostosis. Few pediatric neurosurgeons in the region have this training and I’m often asked to lecture about it. Also, our multispecialty craniosynostosis team of experts includes pediatric neurosurgeons, pediatric plastic surgeons and pediatric anesthesiologists to meet your child’s specific needs and give you peace of mind.
To schedule a consultation with Dr. Chesler, call (631) 444-1213 (select option 1).