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Craniosynostosis

Craniosynostosis belongs to the category of birth defects known as cranio facial defects.

What it is

Craniosynostosis is a birth defect in which the bones of an infant’s skull fuse together prematurely. A baby’s skull consists of seven bones, which usually do not fuse until the baby is two years old. Until that time, the skull expands evenly in order to house the growing brain.

In babies suffering from craniosynostosis, premature fusing of the bones can occur in one or more of the joints (called cranial sutures) in the baby’s skull. The severity of the defect depends in part upon how many sutures fuse prematurely. In some babies, only one suture will close, causing little more damage than a misshapen head. However, when multiple sutures fuse shut prematurely, the growth of the baby’s brain may become restricted and pressure within the skull increases. If surgical intervention is not made in time, the baby’s brain development may be permanently impaired.

Symptoms

Symptoms may not be visible at birth, but become noticeable within the first few months of a baby’s life. They may include:

  • An abnormally-shaped skull (the shape of the skull will depend on which suture(s) are affected)
  • An atypical feeling or receding “soft spot” on the skull
  • A raised, hard ridge along the sutures that have become fused
  • Little or no growth of the skull as the baby grows
  • Developmental delays or mental deficiencies, brought on by restriction of brain growth
  • Seizures
  • Blindness

Causes

Anti-depressants: A major study of birth defects revealed that maternal use of certain selective serotonin reuptake inhibitor (SSRI) anti-depressants during pregnancy is associated with a 2.5x increased risk of delivering a baby with craniosynostosis. Major brands of SSRIs include:

Depakote: Maternal use of anti-seizure drug Depakote (valproric acid) during pregnancy has been linked with a 7-fold increased risk of delivering a baby with craniosynostosis, according to a recent study published by a major medical journal.

Clomid: A study conducted by the Centers for Disease Control and Prevention (CDC) found that use of the fertility drug Clomid (clomiphene citrate) during the two months prior to conception or in the first month of pregnancy was linked with a nearly doubled risk of delivering a baby with craniosynostosis.

Treatment

In milder cases of craniosynostosis that only involve one suture, surgery may not be necessary. Under these circumstances, the brain may have enough room to develop fully. Concerns about skull abnormality may be addressed with the use of a cranial helmet that helps to reshape the head.

For most infants suffering from craniosynostosis, however, surgery will be necessary. The primary goals of surgery are to relieve any pressure on the brain, to create more room for the brain to grow, and to improve the symmetry and appearance of the misshapen head. If there is no underlying brain abnormality, the prognosis for treatment is good. There are two different types of surgery used to treat craniosynostosis.

Traditional surgery involves cutting into the infant’s scalp and cranium, and correcting the shape of the skull. Plates and screws, made out of a dissolving material, may be used to secure the bones in their new position.

Endoscopic surgery may be another option for some babies. In this less invasive procedure, the surgeon makes small incisions in the skull, and opens the affected sutures using a lighted tube, called an endoscope. This procedure is quicker, causes less blood loss and trauma to the area, and usually has a much shorter recovery time than traditional surgery.

 

 

 

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