Craniosynostosis

Question 1

What is craniosynostosis?

Answer 1

Craniosynostosis occurs when the skull sutures close prematurely. This results in abnormal head shapes and restriction to the growth of the brain.

If left untreated it will lead to raised intracranial pressure, with resulting symptoms of developmental delay, cognitive impairment, vomiting, irritability, visual impairment, neurological symptoms and seizures.

Question 2

What is the head shape of saggital, coronal, metopic and lambdoid synostosis?

Answer 2

Question 3

What are the clinical features of craniosynostosis?

Answer 3

The main presenting features is an abnormal head shape depending, on the affected cranial suture (see table).

Other presenting features:

• Anterior fontanelle closure before 1 year of age
• Small head in proportion to the body

Question 4

What investigations should be ordered for craniosynostosis?

Answer 4

Where there are suspicions about craniosynostosis the patient should be referred to a specialist for further investigations. The first line investigation is a skull xray.

CT head with bone views is used to confirm the diagnosis or exclude it if there is doubt on the xray.

Question 5

Briefly describe the management of craniosynostosis

Answer 5

Mild cases may be monitored and followed up over time.

More severe cases require surgery for surgical reconstruction of the skull.

Question 6

Briefly describe the prognosis of craniosynostosis

Answer 6

The prognosis is usually good with proper management. They will have a lifelong scar on the scalp where the surgery was performed.