What Is Craniosynostosis?
Craniosynostosis is a condition in which one or more of the fibrous joints (sutures) between the bones of a baby's skull close prematurely, before the brain has fully grown. This premature fusion prevents the skull from growing normally and can result in an abnormal head shape.
A baby's skull is made up of several separate bones connected by sutures. These sutures allow the skull to expand as the brain grows during the first few years of life. When a suture fuses too early, the skull cannot expand in that direction and compensates by growing in other directions, leading to a characteristic head shape depending on which suture is affected.
Craniosynostosis affects approximately 1 in 2,000 to 2,500 live births. It can occur as an isolated condition (non-syndromic) or as part of a genetic syndrome such as Apert, Crouzon, or Pfeiffer syndrome. Early diagnosis and surgical treatment are essential to allow normal brain growth and prevent complications such as increased intracranial pressure.
Types of Craniosynostosis
The type of craniosynostosis is determined by which suture is prematurely fused. Each type produces a distinct head shape.
Sagittal Synostosis
Head shape: Scaphocephaly
The most common type, accounting for about 40–55% of cases. The sagittal suture runs along the top of the head from front to back. When it fuses early, the head becomes long and narrow.
Metopic Synostosis
Head shape: Trigonocephaly
The metopic suture runs from the top of the head down the middle of the forehead. Early fusion creates a triangular-shaped forehead with a visible ridge and closely-spaced eyes (hypotelorism).
Unilateral Coronal Synostosis
Head shape: Anterior Plagiocephaly
One of the two coronal sutures (which run from ear to ear across the top of the head) fuses early. This causes flattening of the forehead on the affected side, with the eye socket appearing raised.
Bilateral Coronal Synostosis
Head shape: Brachycephaly
Both coronal sutures fuse early, resulting in a wide, short skull with a tall, flat forehead. This type is more commonly associated with genetic syndromes such as Apert or Crouzon syndrome.
Lambdoid Synostosis
Head shape: Posterior Plagiocephaly
The lambdoid suture at the back of the skull fuses early, causing flattening on one side of the back of the head. This is rare and must be distinguished from positional plagiocephaly (flat head from sleeping position).
Multi-Suture Synostosis
Head shape: Variable
Two or more sutures fuse prematurely. This is the most complex form and carries a higher risk of increased intracranial pressure. It is more commonly associated with genetic syndromes and often requires multiple surgeries.
Signs & Symptoms
Craniosynostosis is usually noticed within the first few months of life. The primary sign is an abnormal head shape that becomes more apparent as the baby grows.
Abnormal head shape that does not improve with repositioning
A hard, raised ridge along the affected suture line
Soft spot (fontanelle) that is absent or closes early
Head circumference that is not growing as expected
Asymmetry of the face, forehead, or eye sockets
In severe cases: irritability, vomiting, bulging eyes, or developmental delays (signs of increased intracranial pressure)
Diagnosis
Physical Examination
The doctor examines the baby's head shape, feels for ridges along the sutures, and measures head circumference. The shape of the skull and the pattern of asymmetry often suggest which suture is involved.
CT Scan with 3D Reconstruction
A CT scan provides detailed images of the skull bones and sutures. 3D reconstructions allow Dr. Chaiyasate to precisely identify which sutures are fused and plan the surgical approach.
Genetic Testing
When a genetic syndrome is suspected (especially with multi-suture involvement or associated anomalies), genetic testing can identify specific mutations such as FGFR2 or FGFR3 that are associated with syndromic craniosynostosis.
Ophthalmologic Evaluation
An eye exam checks for papilledema (swelling of the optic nerve), which can indicate increased intracranial pressure. Regular eye exams are important for ongoing monitoring.
Surgical Treatment Options
Surgery is the primary treatment for craniosynostosis. Dr. Chaiyasate offers multiple surgical approaches tailored to each child's specific needs.
Endoscopic Strip Craniectomy
Best for infants under 4–6 monthsMinimally invasive approach using small incisions and an endoscope
The fused suture is removed through a narrow strip craniectomy
Significantly less blood loss and shorter hospital stay (typically 1 night)
Followed by post-operative helmet therapy for 6–12 months to guide skull reshaping
Shorter operative time (approximately 1 hour) compared to open surgery
Excellent cosmetic outcomes with minimal scarring
Open Cranial Vault Remodeling
Typically for infants 6–12 monthsTraditional open approach for more complex cases or older infants
The skull bones are removed, reshaped, and repositioned to create a normal contour
Provides immediate correction of the skull shape without need for helmet therapy
Hospital stay of 3–5 days; recovery period of several weeks
Fronto-orbital advancement is used for coronal and metopic synostosis
Posterior vault distraction may be used for multi-suture or syndromic cases
Spring-Assisted Cranioplasty
Select cases, typically 3–6 monthsSprings are placed across the craniectomy site to gradually expand the skull
The springs are removed in a second minor procedure after 3–6 months
Less invasive than full cranial vault remodeling
Particularly useful for sagittal synostosis
Allows gradual, controlled expansion of the skull
Posterior Skull Distraction
Complex and syndromic casesUsed for multi-suture or syndromic craniosynostosis with restricted posterior skull growth
Distraction devices are placed to gradually move the back of the skull outward
Increases intracranial volume to relieve pressure on the brain
Particularly important for children with Crouzon, Apert, or Pfeiffer syndrome
May be combined with other procedures as part of a staged treatment plan
Recovery & Long-Term Outcomes
With timely surgical intervention, the prognosis for children with craniosynostosis is excellent. Most children go on to develop normally with a natural-appearing head shape.
After Endoscopic Surgery
1-night hospital stay in most cases
Helmet therapy begins 1–2 weeks after surgery
Helmet worn 23 hours/day for 6–12 months
Regular follow-up visits to monitor skull growth
Most children return to normal activities quickly
After Open Cranial Vault Remodeling
3–5 day hospital stay
Swelling and bruising around the eyes (resolves in 1–2 weeks)
No helmet therapy required
Activity restrictions for 6–8 weeks
Excellent long-term cosmetic results
Long-term monitoring: Children who have had craniosynostosis surgery are followed regularly to monitor skull growth, neurological development, and vision. Most children achieve normal developmental milestones and lead healthy, active lives.
Schedule a Consultation
Dr. Chaiyasate and his team are here to answer your questions and discuss the best treatment options for you or your child.