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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.