Should I be concerned about my baby’s head shape?

CheslerDavid Chesler, MD, PhD
Pediatric Neurosurgeon
Stony Brook Children's Hospital

Craniosynostosis is a condition that affects the head/skull and face. It occurs in one out of 2,500 births. Few pediatric neurosurgeons in our region have the advanced training required to diagnose and treat this condition. David A. Chesler, MD, PhD, a Johns Hopkins fellowship-trained, board-certified pediatric neurosurgeon at Stony Brook Children’s Hospital is an expert in treating craniosynostosis.

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. There are different types of craniosynostosis and some are more common than others.
• Scaphocephaly (sagittal synostosis) is the most common form of craniosynostosis, occurring in 50 to 60 percent of cases. It’s characterized by a skull that’s narrow from ear to ear. It’s also associated with increased pressure on the brain.
• Plagiocephaly (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 isn’t 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’s estimated that 20 to 30 percent of all children born have some degree of positional plagiocephaly.
• Trigonocephaly (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 and how is it diagnosed?
Changes in the shape of an infant’s head and face may be noticeable and are generally the first and only symptom of craniosynostosis. One side of the face may not be the same when compared to the other side. The condition may be congenital (present at birth) or observed later, during a physical exam by your pediatrician. At Stony Brook, we ask if there’s a family history of craniosynostosis or other head or face abnormalities. We also ask about developmental milestones, since craniosynostosis can be associated with other developmental delays. A head measurement is then taken and plotted on a graph to identify normal and abnormal ranges. Diagnostic tests such as head x-rays and computed tomography scans (CT or CAT scan) may also be performed.

Is time a factor in getting treatment?
Yes. Treatment is determined based on your child’s age, overall health and medical history, and the extent of and type of craniosynostosis. So the sooner your child is diagnosed, the better. The best time to perform surgery is before 1 year of age when the bones are still very soft and easy to remove and reassemble. If not surgically treated, there’s a risk of injury to the brain and vision. Traditional open-skull surgery is performed between 6 and 9 months of age. Blood transfusions are common with open surgery and the average hospital stay is five to seven days. Minimally invasive endoscopic surgery, which offers comparable results to open surgery, is generally performed at 3 months of age with an average hospital stay of one night. Postoperative 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. And because there’s rarely a need for blood transfusions with endoscopic surgery, the risk of complications is dramatically lowered.