Craniosynostosis

Craniosynostosis 

What is Craniosynostosis?

Craniosynostosis is a condition where the sutures between the bones in an infant's skull close prematurely. 

Craniosynostosis refers to the premature fusion of one or more of the joints (or “sutures”) in an infant's skull and occurs in 1 in 2,000 babies. 

Sutures are fibrous bands of tissue that connect the individual bones of the skull, and their premature fusion can lead to abnormal skull growth and shape. It can also put pressure on the brain, resulting in developmental delay and raised intracranial pressure. 

Types of Craniosynostosis

  • Single suture craniosynostosis: Single suture craniosynostosis is the most common type of craniosynostosis and affects only one of the joins between the bones of the skull. Growth of the skull is restricted as a result of this fusion resulting in a characteristic skull shape depending on which suture is affected. 
  • Syndromic craniosynostosis: Some children have fusion of more than one cranial suture – this is called “Multisuture craniosynostosis”. This may be part of a syndrome, where other parts of the skull and body are affected (eg webbing/fusion of the fingers, hearing loss, cleft palate and underdevelopment of the cheekbones). 

 

Our approach to care

Children with Craniosynostosis need to be managed in a craniofacial unit, as surgery and close follow up is required.  

Craniosynostosis is one of the most common conditions treated by the Craniofacial service of the Sydney Children’s Hospitals Network.  

Our centre strives to ensure a complete and multidisciplinary assessment of your baby when you are referred to us. This includes assessments by Plastic Surgery, Neurosurgery, Ophthalmology and usually a CT scan.  

Single suture craniosynostos

The majority of diagnoses for single suture craniosynostosis are made by a clinical exam and a CT scan is used for surgical planning or when the diagnosis is in question. The protocol for CT scans involves a low-dose bone-only window minimizing radiation while providing excellent information. Plain xrays of the head are not recommended. Pre-operative photography and scanning of the head is done for planning and creation of helmets.  

Surgery is performed in babies with single suture craniosynostosis mainly for appearance and occasionally to deal with elevated pressure inside the head. These are the most common surgical procedures recommended for children with single suture craniosynostosis managed by the Craniofacial service of the Sydney Children’s Hospitals Network. 

Syndromic craniosynostosis 

Syndromic craniosynostosis is complex and rare, but routinely managed by the Craniofacial Service of the Sydney Children’s Hospitals Network. These patients are more likely to present with a number of issues involving brain development, vision, breathing and neurodevelopment. Many require a series of operations throughout their childhood. 

Learn more

For more detailed information on Craniosynostosis conditions including treatment, surgery and therapy, see the Craniosynostosis factsheets. 

Read the Craniosynostosis (single suture) factsheet

Read the Craniosynostosis (syndromic) factsheet

Last updated Wednesday 8th May 2024