Craniosynostosis

Craniosynostosis is the premature fusion of one or more of the skull sutures in a baby — before the brain has finished growing. It affects roughly 1 in 2,000 live births and, when it involves a functionally important suture, requires surgery to give the brain the room it needs.

What Is Craniosynostosis?

In craniosynostosis, one or more skull sutures fuse too early, restricting brain growth in one direction and forcing compensatory growth in others. The pattern of skull deformity depends on which suture fuses. Most cases are isolated (no syndrome), but ~15–20% are syndromic — part of Apert, Crouzon, Muenke, or Pfeiffer syndromes.

At a Glance

Craniosynostosis is premature suture fusion in infancy — affects ~1 in 2,000 births
The skull shape reveals which suture is fused: sagittal = long narrow skull; coronal = asymmetric forehead; metopic = triangular forehead; lambdoid = flattened back of skull
Surgery before 12 months gives the best cosmetic and brain-growth outcomes
Two main approaches: open cranial vault remodeling, or minimally invasive spring-assisted craniectomy (for infants under 6 months)
Outcomes are excellent when operated early at a high-volume center

Symptoms

Skull shape abnormalities — the primary finding

Sagittal synostosis: scaphocephaly — long, narrow, 'boat-shaped' head
Unilateral coronal synostosis: plagiocephaly — asymmetric, flattened forehead on the fused side; deviated nose
Metopic synostosis: trigonocephaly — triangular forehead, hypotelorism
Bilateral coronal: brachycephaly — short, wide, 'turricephalic' head (especially in syndromes)

Raised intracranial pressure (less common; more in syndromic)

Irritability, bulging fontanelle, papilledema
Headaches in older children

Diagnosis

Types

Treatment

Open cranial vault remodeling: standard approach for most cases; typically done at 6-12 months
Performed jointly by neurosurgery and craniofacial plastic surgery. Bone is carefully removed, reshaped, and repositioned. Most babies in the hospital 3-5 days.

Spring-assisted craniectomy: for infants under 4-6 months
Minimally invasive strip craniectomy with springs that gradually separate the fused suture. Requires a second brief procedure to remove the springs at ~3-4 months. Less blood loss than open remodeling; must be done at very young age.

Distraction osteogenesis: for older children or syndromic cases with significant bone deficit

Outcomes

Suture Type	Surgical Timing	Expected Cosmetic Outcome	Re-operation Rate
Sagittal (open vault)	6-12 months	Excellent in ~90%	<5% at 5 years
Unilateral coronal (open vault)	6-12 months	Good-excellent; some residual asymmetry	~10%
Metopic (open vault)	6-12 months	Excellent in ~85%	<5% at 5 years
Spring-assisted (sagittal)	<4-6 months	Excellent when performed early	~5-10%

References

Slater BJ, et al. Cranial sutures: a brief review. Plast Reconstr Surg. 2008;121:170e-178e. PMID: 18349578
Jimenez DF, Barone CM. Endoscopic craniectomy for early surgical correction of sagittal craniosynostosis. J Neurosurg. 1998;88:77-81. PMID: 9420076
Yan Y, Bacos JT, DiPatri AJ Jr, Gosain AK. Spring-assisted distraction osteogenesis for treatment of shunt-induced craniosynostosis. Cleft Palate Craniofacial J. 2020. PMID: 32783477

Our Specialists

Arthur DiPatri, MD

Pediatric Neurosurgery

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