Epidemiology of Nonsyndromic Craniosynostosis in Children - The ISPN Guide to Pediatric Neurosurgery
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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).
- All four types present at birth
- 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).
- No known pockets of increased incidence for any of the four types
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.