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.
Presentation
The main presenting features is an abnormal head shape depending, on the affected cranial suture:
|
Type of Synostosis |
Affected Suture |
Head Shape |
|
Saggital Synostosis |
Saggital suture |
Long and narrow from front to back |
|
Coronal Synostosis |
Coronal suture |
Bulging on one side of the forehead |
|
Metopic Synostosis |
Metopic suture |
Pointy triangular forehead |
|
Lambdoid Synostosis |
Lambdoid suture |
Flattening on one side of the occiput |
Other presenting features:
- Anterior fontanelle closure before 1 year of age
- Small head in proportion to the body
Investigations
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.
Management
Mild cases may be monitored and followed up over time. More severe cases require surgery for surgical reconstruction of the skull.
The prognosis is usually good with proper management. They will have a lifelong scar on the scalp where the surgery was performed.
Last updated January 2020