Parietal encephalocele

Parietal encephalocele is a rare congenital neural tube defect characterized by a herniation of brain tissue and/or meninges through a defect in the parietal bone of the skull. Encephaloceles are classified by their anatomical location, and the parietal variant — occurring at the top of the skull — is among the less common subtypes compared to occipital or frontal encephaloceles. The condition arises during early embryonic development when the neural tube fails to close properly, resulting in a sac-like protrusion that may contain meninges alone (cranial meningocele) or meninges along with brain parenchyma (meningoencephalocele). The defect is typically apparent at birth as a visible or palpable mass on the top of the head. The clinical presentation of parietal encephalocele varies depending on the size of the defect and the amount of brain tissue involved. Key symptoms and associated findings may include a visible scalp mass, hydrocephalus, seizures, developmental delay, intellectual disability, and motor impairments. In some cases, the encephalocele may be associated with other central nervous system anomalies or may occur as part of broader syndromic conditions. Microcephaly and visual disturbances may also be present depending on the extent of brain involvement. Treatment is primarily surgical, involving repair of the skull defect and removal or repositioning of herniated tissue. The timing and approach to surgery depend on the size of the encephalocele, the viability of the herniated brain tissue, and the presence of associated complications such as hydrocephalus, which may require separate management with cerebrospinal fluid shunting. Neurodevelopmental outcomes are highly variable and depend on the amount of functional brain tissue within the sac and the presence of additional brain malformations. Long-term multidisciplinary follow-up involving neurosurgery, neurology, developmental pediatrics, and rehabilitation services is typically recommended.

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