Uncommon Syndromes of Cerebellar Vermis Aplasia. II: Tecto‐cerebellar Dysraphia with Occipital Encephalocele

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Abstract
New cases are reported of a rare syndrome of malformations consisting of occipital encephalocele, aplasia of the vermis and deformity of the tectum. Less consistent components are aplasia of mammillary bodies, fusion of thalami, anomalies of cerebral gyral patterns, bifid atlas or bifid occipital squame, elevation of torcular, and cervical hydromyelia. Of these cases 1 had survived to the age of 8 yr with a ventricular shunt. This syndrome, of which 5 cases are known, may be a tandem malformation in which either an occipital dysraphia or an encephalocele induces aplasia of the vermis. A classification of aplasias of the cerebellar vermis is proposed.