Craniosynostosis

by Alan Green, MD, FAAP

In the last few years there has been an alarming increase of craniosynostosis, or other conditions that mimic craniosynostosis. This has resulted in an increase in major surgery on infants, some of it unnecessary.

Six separate bones make up the cranium of the head: the occipital (in the rear), the frontal (in the front), the two temporal (low on either side) and two parietal (most of either side). These bones develop, and are in place, by the 5th month of gestation. They are like pieces of a jigsaw puzzle, separated by crooked lines, called sutures.

These puzzle pieces form an almost complete covering for the brain. The remaining open places are called soft spots, or fontanels. The sutures and fontanels allow the bones of the skull to shift during delivery. This molding of the head makes it possible for the skull to fit through the birth canal.

Continuing after birth, the brain grows very rapidly during the first several years of life. This growth is made possible by corresponding growth of the cranial bones along the suture lines. Premature closing of the suture lines is called craniosynostosis, and results in a misshapen head. The resulting head shape depends on which suture has closed prematurely. For instance, if the sagittal suture fuses (the long one running from front to back in the center of the head), the head becomes long and narrow. Sagittal synostosis is the most common form of craniosynostosis.

Since the bones of the head are still flexible while growing throughout infancy, head positioning can affect head shape. In recent years, as parents have been appropriately not allowing their babies to sleep on their stomachs (to prevent SIDS), there has been a dramatic increase in misshapen heads. If a baby lies on her back awake and asleep, a flat head will often result. If a wedge pillow is used to hold a baby in the same position on her side whenever she sleeps, one side of the skull may become deformed. The pillows are fine, but one should alternate on which side the baby sleeps. In general, positional molding of the head can be prevented by varying a child's head position throughout the day.

Positional molding of the head can look like craniosynostosis. The Academy of Pediatrics (AAP) is concerned that positional molding is being misdiagnosed as craniosynostosis, resulting in unnecessary major surgery in children. This concern was voiced to pediatricians in the April 1996 edition of the official newspaper of the AAP.

Craniosynostosis will not correct itself over time, and does indeed require surgery. Hector James, M.D., a member of the Executive Committee of the AAP Section on Neurology, has said that now 90% of children's misshapen heads are due to external pressure, and that 98% of these can be corrected without surgery, usually by appropriate repositioning.

An accurate diagnosis requires both a skilled physical exam and some type of X-ray of the sutures. Don't trust a diagnosis made by exam alone. Also, neurosurgeons vary widely in their experience treating children. Make sure that the involved neurosurgeon is well-versed in pediatric care (your insurance company may make this difficult, but your pediatrician should be able to help). Any recommendation for surgery should be taken with a grain of salt. A second opinion is often wise, and most insurance firms will support this.

Regarding surgical correction of misshapen heads, I agree with John Brooks, M.D. of the AAP Task Force on Infant Positioning, who said, "(Parents) should be very skeptical of any suggestions that (their children) should have this surgery."

Dr. Alan GreeneDr. Alan Greene, author of Raising Baby Green and Feeding Baby Greene, is the founder of Dr.Greene.com and the WhiteOut Movement. He is a frequent guest on such shows as Good Morning America, The Today Show, and the Dr. Oz Show. He is on the Board of Directors of Healthy Child Healthy World and The Lunchbox Project. Dr. Greene is a practicing pediatrician at Stanford University's Packard Children's Hospital.

Copyright © Greene Ink, Inc., all rights reserved. Permission to republish granted to Pregnancy.org. Reviewed by: Khanh-Van Le-Bucklin M.D. & Liat Simkhay Snyder M.D. July 20, 2009.