Craniosynostosis

Craniosynostosis
Q:
Craniosynostosis

Any information concerning craniosynostosis in a newborn. My doctor wants to operate to repair this condition. What can happen if nothing is done?
J.T. Hoy – El Paso, Texas

A:

Dr. Greene`s Answer:

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.

While craniosynostosis can cause misshapening of the head, a far more common reason is positional molding (plagiocephaly). Plagiocephaly is a flattening of the head from lying down on that part of the head. Since the bones of the head are still flexible while growing throughout infancy, head positioning can affect head shape. 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. 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.

Positional plagiocephaly is not a serious problem and will usually correct itself over time. In general, positional molding of the head can be prevented by varying a child’s head position throughout the day. “Tummy time” is a useful strategy to prevent the progression of positional molding. Tummy time involves supervised periods of time when an awake baby is placed on her stomach during the day. This position not only takes pressure off the head, but also helps develop the muscles of the neck and shoulders.

While positional molding of the head (plagiocephaly) is a benign condition, it can sometimes 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 often does indeed require surgery. Still, caution should be taken to insure that the diagnosis of craniosynostosis is correct before pursuing 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 or CT Scan 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 without a confirmed diagnosis of true craniosynostosis, 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.”

Reviewed by: Khanh-Van Le-Bucklin, Liat Simkhay Snyder
Last reviewed: July 19, 2009
Dr. Alan Greene

Article written by

Dr. Greene is the founder of DrGreene.com (cited by the AMA as “the pioneer physician Web site”), a practicing pediatrician, father of four, & author of Raising Baby Green & Feeding Baby Green. He appears frequently in the media including such venues as the The New York Times, the TODAY Show, Good Morning America, & the Dr. Oz Show.

 

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