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Craniosynostosis

What is craniosynostosis?

The skull of an infant is made up of free-floating bones separated by fibers called sutures. This arrangement allows the infant's head to pass through the birth canal and also enables the skull to grow as the brain grows in early infancy.

These bones eventually fuse and form the developed skull. If fusion occurs prematurely, skull and brain growth are restricted. This is called craniosynostosis. The cause of this premature fusion is unknown. If left untreated, children born with craniosynostosis are at risk for increased pressure on the brain and vision problems.

Long-term studies demonstrate that, in addition to correcting functional problems associated with craniosynostosis, reconstructive surgery has a positive effect on the child's self image.

How is craniosynostosis diagnosed?

Craniosynostosis is often not diagnosed prenatally. In some instances an abnormal head shape is noted on prenatal ultrasound. If suspected prenatally, a fetal MRI may be recommended to evaluate the skull. A targeted ultrasound to rule out the presence of associated anomalies, including those of the hands, feet, and heart, will be performed.

A specialized ultrasound of the fetal heart, called an echocardiogram, and an amniocentesis to look for chromosomal anomalies may be recommended.

Most often, craniosynostosis is diagnosed after birth when the pediatrician or parent notices that the head shape is not normal.  An abnormal head shape at birth may be due to positioning during pregnancy, compression during the birthing process, or from lying in one position during infancy. This is called positional plagiocephaly and does not require surgery.  However if the abnormal head shape is due to premature closure of the sutures (craniosynostosis), the shape will not improve and will result in increased deformity over time and will require surgery.

A diagnosis should be made as early as possible after birth by a craniofacial plastic surgeon or a neurosurgeon. The craniofacial surgeon will order a computerized tomography of the head (CT scan) to confirm the diagnosis if craniosynostosis is suspected.

How is craniosynostosis treated?

Craniosynostosis is surgically corrected after birth.  There is no fetal treatment for this disease. The surgeons are part of the craniofacial team that includes a pediatric neurosurgeon and craniofacial surgeon as well as a specialized anesthesiologist. The neurosurgeon works on the skull and the craniofacial surgeon on the face and head shape. This surgery should be done in a hospital with a pediatric intensive care unit. The Center will develop and coordinate a plan to treat your baby during your consultation. You will be able to meet the entire team of specialists involved in the care of your newborn.

The ideal timing for surgery is prior to 3 months of age when a minimally invasive, microscopic procedure can be used that results in a smaller incision, less blood loss and a shorter hospital stay than the traditional corrective surgery. Early surgical intervention is advised because the bones of the skull are easy to work with at this age, and the covering of the brain, known as the dura, can make bone on its own. Moreover, the growing brain can continue to reshape the skull and face after the surgery. Although surgery is recommended early in life, there is no upper age limit to surgery.

After surgery, the child remains in the pediatric intensive care unit for 1-3 days, depending on the type of surgery. There will be swelling that increases during the first 24-72 hours after surgery that may take a few weeks to completely resolve. The child is usually eating and drinking the day after surgery. After the swelling goes down, the child is fitted for a helmet that protects and helps to reshape the skull following the surgical release of the sutures. The helmet is worn for several weeks to months following surgery.

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Contact the Texas Fetal Center to make an appointment with a high-risk pregnancy doctor.

Note: This information regarding your condition is meant to be a helpful, informative introduction. Because every newborn is different, this may not be applicable to all cases, especially if there are additional defects. Your team of experts at the Center will provide you with information specific to your pregnancy.