Subtorcular occipital encephaloceles. Anatomical considerations relevant to operative management.

Three cases of occipital encephalocele, one with associated myelomeningocele, are presented. All received preoperative evaluation with magnetic resonance imaging. Such studies provide optimal demonstration of the cerebral and hindbrain anatomy to guide operative treatment and formulate prognosis. Review of available radiographic, operative, and pathological information suggests that most, if not all, occipital encephaloceles are associated with an anomaly of the hindbrain, and the usual anomaly is a rhombic roof encephalocele. In such cases, the site of cranial herniation is caudal to the torcula, regardless of the presence or absence of occipital lobe tissue within the sac. Experimental and clinical analysis suggests that occipital encephaloceles most likely arise from abnormalities in the development of the skull base.