Encephalocele is usually a congenital type of neural tube defect (NTD), where a sac containing brain/meninges/cerebrospinal fluid (CSF) forms outside the skull through a bone defect. On occasions, acquired encephaloceles may result from trauma, tumors, or iatrogenic injury. If the sac is formed by protrusion of meninges and CSF, it is appropriately called a meningocele, but it is called an encephalocele when it contains brain tissue. However, both are commonly called encephaloceles.
Repair of the encephalocele is not an emergency, and only those with skin ulceration or CSF leaks need rapid surgery to avoid meningitis. Surgery can be performed as early as two months of age but is usually delayed until about four months to several years due to the small total blood volume of infants. The surgical goals are the closure of the skull defect with a watertight dural closure and the bony defect reconstruction.
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