Fetal Craniosynostosis

Fetal craniosynostosis is a congenital defect that affects the skull and brain. In a normal skull, the bones are separated by fibrous joints called sutures. When an infant is growing and developing after birth, the sutures close to form one solid piece of bone, the skull. When this happens too early, while the baby is still in the womb, fetal craniosynostosis occurs. Because the sutures close before brain development is complete, the brain compensates by growing into areas of the skull where the sutures have yet to close.

This results in an abnormally shaped head; increased pressure on the brain may also occur. Fetal craniosynostosis occurs in approximately one in every 2000 live births, but it affects males twice as often as females.

What Causes Fetal Craniosynostosis?

The occurrence of this condition can be genetically linked, so family members are advised to obtain genetic counseling.

How Is Fetal Craniosynostosis Diagnosed?

Fetal craniosynostosis can be diagnosed through ultrasound (sonogram) when an asymmetry or bulge in the skull appears. Although it can be diagnosed through ultrasound, it may be difficult to detect. If it is found, it is usually not discovered until the third trimester. Evaluation of the craniofacial structure is part of the routine ultrasound examination done by many obstetricians as part of their prenatal care.

Preparation

If a baby is diagnosed with craniosynostosis before she is born, the surgeons and nurses spend time counseling parents about what to expect when their baby is born.

How Is Fetal Craniosynostosis Treated?

As soon as the baby is born, they are given a detailed physical examination; measurements of the infant's skull are included in the examination. Additionally, other diagnostic tests may be performed to confirm the diagnosis. These may include an X-ray or computerized tomography scan (CT scan), which produces cross-sectional images of the baby's head.

Surgery to correct this defect is typically recommended before the child is one-year-old. However, the timing and extent of the surgery will depend on the severity of the condition. The surgeon will discuss the surgery with the parents beforehand, explaining how it is performed. It is typically very successful in correcting the shape of the child's head and can relieve the pressure on the brain.

What Are the Long-term Effects of Fetal Craniosynostosis?

The long-term outlook for a child born with craniosynostosis is often linked to early detection and treatment. The results of the surgery are usually excellent both aesthetically and functionally, giving the child an outstanding quality of life and a normal life expectancy. However, it is recommended that these children receive follow-up care and evaluations to monitor the development of their skull and brain.