Sagittal Craniosynostosis

In brief: Sagittal craniosynostosis is caused by premature closure of the sagittal suture running along the top of the head. The head appears long from front to back and narrow from side to side — a shape known as scaphocephaly or dolichocephaly. Surgical treatment is usually recommended within the first 6–12 months.

• Most common type: Accounts for 40–55% of all craniosynostosis cases.
• Treatment: Endoscopic (3–6 months) or open cranial remodelling (6–12 months).
• Helmet: Post-endoscopic helmet therapy may be required; helmet therapy information.

Sagittal craniosynostosis — also known as scaphocephaly or dolichocephaly — is the most common type of craniosynostosis, occurring when the bones of a baby's skull fuse abnormally.

What Is Sagittal Craniosynostosis?

Craniosynostosis occurs when certain bones in a child's skull fuse together too early. At birth, a baby's skull is made up of several separate bones with growth plates between them.

Because the skull is not yet a single rigid bone, the brain is able to grow and expand in size. Normally, the sutures in the skull close during adulthood.

In some children, the skull bones fuse earlier than they should. Sagittal craniosynostosis refers to premature closure of the suture that runs from front to back along the top of the head. Babies are usually identified as having an abnormal head shape shortly after birth.

When this occurs, the skull's room for expansion is restricted, pressing on the brain and potentially impeding normal brain growth. Scaphocephaly also causes the child's head to appear misshapen. For this reason, we recommend that parents seek treatment early when they notice this condition — preferably before the baby is six months old.

Figure 1. Sagittal Craniosynostosis (1)

Figure 2: Sagittal craniosynostosis (1)

How Is Sagittal Craniosynostosis Treated?

When bones fuse prematurely, surgery is the only way to separate them. As a surgical option, neurosurgeons can perform either endoscopic or open craniosynostosis surgery depending on the patient's condition.

• Endoscopic craniosynostosis repair.  Minimally invasive surgery is not generally recommended for scaphocephaly in suitable patients. Helmet therapy is usually recommended following endoscopic surgery — it is an important support both for protecting the head from trauma and for guiding ongoing shaping in the correct direction.
• Open craniosynostosis repair surgery.  This uses a conventional technique for children who are not candidates for minimally invasive surgery. With this approach, the risk of bleeding and the length of intensive care stay may be slightly longer.

What Causes Sagittal Craniosynostosis?

Sagittal craniosynostosis can occur in a healthy baby with no known cause, but it is also associated with the following conditions:

• Apert syndrome
• Beare-Stevenson syndrome
• Crouzon syndrome
• Jackson-Weiss syndrome
• Muenke syndrome
• Pfeiffer syndrome

How Is Sagittal Craniosynostosis Diagnosed?

Sagittal craniosynostosis can usually be diagnosed by a simple physical examination. A rapid, painless imaging method (X-ray or CT scan) is also requested to confirm the diagnosis.

Reference: R. Seeberger, J. Hoffmann, C. Freudlsperger, M. Berger, J. Bodem, D. Horn, M. Engel,
Intracranial volume (ICV) in isolated sagittal craniosynostosis measured by 3D photocephalometry: A new perspective on a controversial issue, Journal of Cranio-Maxillofacial Surgery, Volume 44, Issue 5, 2016, Pages 626-631, ISSN 1010-5182, https://doi.org/10.1016/j.jcms.2016.01.023.

Frequently asked questions

What is the sagittal suture?

The sagittal suture is the cranial suture that runs along the top of the head, connecting the two parietal bones. In normal development this suture gradually ossifies and closes; however, if it closes too early, sagittal craniosynostosis develops and the lateral growth of the head is restricted.

How is sagittal craniosynostosis surgery performed?

Two main approaches are used: Endoscopic strip craniectomy (at 3–6 months; small incisions, short recovery, followed by helmet therapy) and Open cranial remodelling (typically at 6–12 months; more extensive, may not require a helmet). The choice of method is determined by the neurosurgical team based on age and severity.

Is sagittal craniosynostosis the same as scaphocephaly?

Sagittal craniosynostosis and scaphocephaly share the same head appearance (long and narrow) but have different underlying causes. Craniosynostosis results from premature suture closure, while deformational scaphocephaly arises from positional causes such as intrauterine pressure. The distinction is made by CT scan or careful clinical examination.