Out of 57 reported cases of Chiari III malformation, encephaloceles were in a high cervical/low occipital position in 23, 8 were in a high cervical position, 17 were in low occipital position, and the position in 9 cases was not reported. The pathogenesis of Chiari III malformation remains unclear. The majority of patients have concomitant hydrocephalus. Brain parts occurring in the sac from the most to least common include the following: cerebellum, occipital lobe, and parietal lobe. The severity of symptoms is correlated with the amount of brain structures within the encephalocele. Neurologic functional outcomes have been varied and depend on the neurological status of the patient before surgery.

Arterial variations around the atlas are surgically significant. Awareness of their existence and course may provide better pre-operative planning and surgical intervention, potentially leading to better clinical outcomes. Three-dimensional computed tomography angiography (3D CTA) is an important tool for identifying and diagnosing such abnormalities and should be used when such vascular anomalies are suspected.

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