Epidemiology of Nonsyndromic Craniosynostosis in Children

Incidence and Prevalence

• Sagittal synostosis – 1/5000 live births (9,28)
• Coronal synostosis – 1/10,000 births: Coronal craniosynostosis accounts for 20–30% of surgical cases (28).
• Metopic synostosis – 1/7000–1/15,000 births: The incidence of metopic craniosynostosis is increasing (28, 48).
• Lambdoidal synostosis – <1/10,000 births: Lambdoidal craniosynostosis is rarely encountered. It accounts for only 2% of all surgical cases (9,29).

Age Distribution

• All four types present at birth

Sex Predilection

• Sagittal synostosis: There is a slight male preponderance (9, 28).
• Coronal synostosis: There is a slight female preponderance (28).
• Metopic synostosis: There is a slight male preponderance (28, 48).
• Lambdoidal synostosis: There is a slight female preponderance (9, 29).

Geographic Distribution

• No known pockets of increased incidence for any of the four types

Risk Factors

• None known 

Relationships to Other Disease States and Syndromes

• Sagittal synostosis: Sagittal synostosis may be observed in syndromic craniosynostosis. It may be paired with a coronal or lambdoid suture synostosis.
• Coronal synostosis: Coronal synostosis is the most common suture demonstrated in syndromic craniosynostosis. It is more likely to be bilateral.
• Metopic synostosis: Metopic synostosis is becoming more common, along with other midline anomalies including heart defects.
• Lambdoidal synostosis: Lambdoidal synostosis is uncommon in syndromic cases, but may be observed with a sagittal suture synostosis.