Encephaloceles are rare embryological mesenchymal developmental anomalies resulting from inappropriate ossification in skull through with herniation of intracranial contents of the sac. Encephaloceles are classified based on location of the osseous defect and contents of sac. Convexity encephalocele with osseous defect in occipital bone is called occipital encephalocele. Giant occipital encephaloceles can be sometimes larger than the size of baby skull itself and they pose a great surgical challenge. Occipital encephaloceles (OE) are further classified as high OE when defect is only in occipital bone above the foramen magnum, low OE when involving occipital bone and foramen magnum and occipito-cervical when there involvement of occipital bone, foramen magnum and posterior upper neural arches. Chiari III malformation can be associated with high or low occipital encephaloceles. Pre-operatively, it is essential to know the size of the sac, contents of the sac, relation to the adjacent structures, presence or absence of venous sinuses/vascular structures and osseous defect size. Sometimes it becomes imperative to perform both CT and MRI for the necessary information. Volume rendered CT images can depict the relation of osseous defect to foramen magnum and provide information about upper neural arches which is necessary in classifying these lesions.
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