29. Encephalocele
Definition
Encephalocele is a herniation of a portion of the brain and/or meninges through a defect of the bony skull table. The condition may be congenital or result from trauma or surgery.
Incidence
In the United States the incidence is 1 to 4:10,000 live births; internationally there is no estimate available. Females are affected more often than males.
Etiology
Congenital encephalocele is produced by a defect in the closure of the embryonic neural tube. The closure defect results in an abnormality of the skull and meninges. Encephalocele may also occur as the result of failed basilar ossification. This defect can potentially occur at several skull locations.
Anomalies Associated with Encephalocele
• Arnold-Chiari II malformation
• Brain migrational anomalies
• Chemke syndrome∗
∗See Appendix G: Rare Syndromes.
• Corpus callosum agenesis
• Cryptophthalmos syndrome
• Dandy-Walker malformation∗
• Knobloch syndrome
• Meckel-Gruber syndrome∗
• Roberts syndrome∗
• Spina bifida
• Trisomy 18
• von Voss syndrome∗
| Bony Defect | Occurrence Percentage |
|---|---|
| Occipital | 75% |
| Frontoethmoidal | 13% to 15% |
| Parietal | 10% to 12% |
| Sphenoidal | ∼2% |
 |
| Encephalocele. |
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