Craniosynostosis: Causes, Symptoms, Diagnosis, Treatment, Prognosis, Complications

Craniosynostosis – This is a pathological condition observed in infants at birth in which the bones of the skull tend to close up prematurely and hence the brain remains underdeveloped resulting in a visible head deformity. This condition is at times associated with certain brain abnormalities.

Craniosynostosis as stated is a pathological condition found in infants where the joints of skull close prematurely leading to underdevelopment of the brain and a visible skull deformity. In Craniosynostosis, a solitary joint in the skull or multiple joints may be affected causing visible deformity of the head. The only way to treat Craniosynostosis is to separate the fused bones and if there is no underlying brain abnormality postprocedure the brain starts to grow and develop normally.

The exact cause of Craniosynostosis is still not known. Researchers are of the opinion that this condition may be caused due to defective genes, although this condition is also found in children who have no family history of this disease. Some genetic conditions which may be caused as a result of Craniosynostosis are Crouzon Syndrome, Apert Syndrome, Pfeiffer Syndrome etc. Majority of the children with Craniosynostosis lead an otherwise normal life.

Some of the symptoms of Craniosynostosis are:

• Absence of fontanelle in a newborn baby
• Visible head deformity
• No increase in the size of the head as the baby grows.

Craniosynostosis has been classified into three types:

Sagittal Synostosis: Also known as scaphocephaly, this is by far the most common form of Craniosynostosis. The premature closing makes the head become long and narrow. Babies born with this type of Craniosynostosis tend to have a broad forehead and this is more common in boys.

Frontal Plagiocephaly: This type of Craniosynostosis is also quite common and is found more in girls.

Metopic Synostosis: This is a rare form of Craniosynostosis. In this form, the baby’s head shape may be described as trigonocephaly which may vary from being mild to very severe.

In order to diagnose Craniosynostosis, the physician may perform the following tests which are enough for a confirmatory diagnosis of Craniosynostosis:

• Measurement of the width of the baby’s head.
• Skull x-rays.
• CT of the head.

The main treatment for Craniosynostosis is surgery, which is usually done when the child is still an infant. The main aim of the surgery is the following:

• Relieving any pressure that may be present in the brain
• Creating enough space in the skull for the brain to grow and develop normally
• Improving cosmetic appearance of the head of the child.

The overall prognosis of Craniosynostosis is reasonably good, especially for those children who do have any genetic abnormality.

Some of the possible complications of Craniosynostosis are permanent deformity of the head if left untreated along with increased intracranial pressure, development of a seizure disorder, delays in attaining developmental milestones.

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