Occipital encephalocele: a pathologic and anatomic analysis.

The brain from an infant with a cystic occipital mass present at birth is examined in serial section. The occipital mass proved to be a rhombic roof ventriculocele. Within the posterior fossa, it was bound to an occipital lobe encephalocele which issued as a diverticulum of the left lateral ventricle through a microgyric cortical defect in the territory of the left posterior cerebral artery. The posterior medial aspects of both cerebral hemispheres were herniated downward into the widened tentorial gap. Craniolacunae were prominent on the inner aspect of the skull. The aqueduct and central canal of the spinal cord were widely dilated, although the lateral ventricles were collapsed. It is suggested that hydrocephalus secondary to obstruction to flow of CSF through the rhombic roof entrained a sequence of events giving rise to the rhombic roof ventriculocele and causing occlusion of the posterior cerebral artery and subsequent diverticulation of the lateral ventricle through an infarcted region of the posterior-medial hemisphere.