Expert, Collaborative Care

There are few things new parents find more irresistible than planting a kiss on their infants' soft downy heads. Sometimes, however, as the months go by, it may become apparent that a baby's skull is not the typical round shape; it may be longer or flatter, or appear to bulge in one or more places. In such cases, parents often wonder whether they're doing something to cause the condition.

According to Jeffrey Marcus, MD, chief of pediatric plastic surgery at Duke, it's true that repeatedly putting a baby to sleep in the same position can sometimes cause what's known as "positional molding," which generally can be treated without any surgical intervention. However, an abnormally shaped head can also be caused by a condition known as craniosynostosis.

How Craniosynostosis Occurs

Infants are born with skulls composed of six separate bone plates, separated by areas of soft fibrous tissue known as sutures. (The areas where sutures intersect are the fontanelles, often referred to as the “soft spots.”) Under normal circumstances, sutures expand through early childhood to allow the skull to accommodate the growing brain. The sutures then fuse at specific pre-programmed ages throughout the course of life, resulting in the round head shape we're accustomed to seeing.

If, however, one or more of the sutures closes prematurely, craniosynostosis (abnormal growth and development of the skull) is the result. "The fused suture that occurs in craniosynostosis may feel like a ridge of bone," says Marcus. "In addition to the ridge, the overall shape of the head can be quite unusual as normal areas overcompensate for those that are constrained by the fusion.”

Mysterious Causes, Serious Concerns

The cause of craniosynostosis is still unknown. Children with certain congenital syndromes are at greater risk for the condition. The majority of affected youngsters, however, have “non-syndromic” craniosynostosis, which may be caused by a combination of several factors. "It's important for parents of children with craniosynostosis to understand that they have done nothing wrong to cause this condition," Marcus emphasizes.

Because craniosynostosis can cause increased pressure on the growing brain, its harmful effects can go far beyond cosmetic concerns. Although such cases are extremely rare, craniosynostosis has been associated with developmental delays, mental retardation, and seizures.

Customized Care Crucial

The surgical treatments for craniosynostosis vary according to the suture(s) involved, but generally consist of expansion to relieve possible pressure on the brain and reconstruction to restore normal shape. "However, not every child with craniosynostosis will require surgery," says Marcus. "Each child is evaluated thoroughly before we come to a consensus about how to treat their condition."

Duke is becoming one of the busiest centers for treating craniosynostosis in the country. "The treatment of craniosynostosis is truly a team effort," says Marcus. "Children with craniosynostosis are usually evaluated by my colleagues, pediatric neurosurgeons Herbert Fuchs and Timothy George. The surgical team then works together to deliver the best possible results." For the vast majority of children with craniosynostosis, growth and development proceed normally after treatment, with no further intervention needed.

The Cleft and Craniofacial Program at Duke treats a variety of challenging conditions in young children, from cleft lip and palate to congenital facial paralysis (a rare disorder in which a baby is born without the ability to smile). "We provide care and support to the entire family," Marcus says.

"After all, parents deserve a team that is dedicated to addressing their concerns. And our young patients deserve treatment results that are good enough to last a lifetime."