Craniosynostosis is a birth defect in which one or more of the cranial sutures, or the joints between the bones of the skull, closes before the brain has completed growing. Other areas of the skull continue to grow, forming an abnormal skull shape. If only one of the cranial sutures closes too early, the brain usually develops normally, but the skull will have an abnormal shape. As more sutures close early, the brain is increasingly likely to develop abnormally. Surgery may be performed to relieve pressure on the brain and cranial nerves, as well as give the head an improved symmetry and appearance.

Symptoms of Craniosynostosis

Symptoms of craniosynostosis can vary and get worse or more intense over time, and may include:

  • Abnormally shaped skull
  • Excessive sleepiness or vomiting
  • Developmental delays
  • Mental retardation
  • Seizures and blindness

Causes of Craniosynostosis

The exact cause of craniosynostosis is unknown, but in some cases may be genetic. For other children it may be caused by metabolic diseases such as an overactive thyroid or rickets. Other research indicates that craniosynostosis may be more prevalent in:

  • Babies who are twins
  • Babies that were in a breech position in the uterus
  • Babies born to mothers who smoked during pregnancy

Diagnosis of Craniosynostosis

Craniosynostosis may be suspected if a child has an odd shaped head. Infants' heads may have normal sutures, but be misshapen at birth due to delivery, how they were positioned in the uterus, or caused by external pressure after birth. Tests to help diagnose craniosynostosis include:

  • Physical exam
  • Serial measurements of the head
  • X-Ray
  • CT scan
  • MRI
  • Genetic testing

Treatment of Craniosynostosis

In some cases, treatment may not be necessary for craniosynostosis, especially those cases that are mild and involve only the premature closing of only one cranial suture. Mild cases of craniosynostosis may become less obvious as children grow. In some cases of abnormal head shape, we may recommend a cranial helmet to help improve the shape and symmetry of the head.

In more severe cases of craniosynostosis, especially those causing pressure on the brain and the possibility of developmental delays, surgery is usually necessary. Surgery timing depends on whether there are signs of increased pressure inside the skull, and any other conditions the child may have. This initial surgery may be as early as 1 or 2 months of age if needed so the pressure on the brain can be relieved, and space can be created for the brain to grow. More commonly, surgery occurs at around 1 year of age, when anesthesia and surgery are more safe.

The surgeon makes an incision on the scalp and reshapes the affected part of the skull, commonly using absorbable stitches, plates, and/or screws to hold bones in place. Endoscopic or limited-incision surgery may also be performed to remove the closed sutures and allow the cranium to be molded with a helmet after surgery.

After surgery, children will be monitored and the growth of their head watched closely. Children with any underlying genetic syndromes also need monitoring and treatment for their individual condition.

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