Atretic Parietal Cephalocele With First Trimester Chiari Malformation and Sinus Pericranii Companion Case

Area of Science:

• Neuroscience
• Developmental Biology
• Pediatric Neurosurgery

Background:

• Encephaloceles are congenital skull defects involving meningeal and neural tissue herniation.
• Vertex encephaloceles (VCs) are the least common encephalocele type but a frequent cause of midline scalp masses.
• Atretic parietal encephaloceles (APC) are a subtype of VC characterized by fibrotic degeneration of meningeal and vestigial tissue.

Observation:

• A neonate presented with atretic parietal encephalocele (APC), a rare form of vertex encephalocele.
• The neonate also exhibited corpus callosum agenesis and a cingulate gyrus lesion.
• A companion case of sinus pericranii was included for comparative analysis.

Findings:

• Atretic parietal encephaloceles (APC) represent benign lesions with a potentially better prognosis due to vestigial tissue involvement.
• The case highlights the association of APC with other developmental anomalies like corpus callosum agenesis.
• Complex venous and neural malformations, often termed the 'tip of the iceberg,' are associated with VCs.

Implications:

• This case contributes to understanding the spectrum of encephalocele presentations and associated anomalies.
• Early identification and characterization of APC are crucial for appropriate management and prognosis.
• Further research into the etiology and management of rare encephalocele subtypes is warranted.