SummaryWe report a case of hydranencephaly with its clinical and morphological aspects. The origin of hydranencephaly is discussed. There is strong evidence of very early damage to the brain substance in this condition. Therefore this disease should not be regarded as a malformation. The diagnostic procedures are described and their values discussed.Since the first description of a patient by Curveilher 6 "with a normal head and face but without a brain" many authors have described such cases. Curveilher 6 coined the word "hydranencephaty", whereas other authors called it "cerebral agenesis" 34, "aplasia of the cerebral hemispheres" 15, or "a child without a forebrain" 3. Two years ago, we saw a child whose first clinical diagnosis was hydrocephalus on admission. Further clinical investigations and necropsy revealed hydranencephalus.
Classification and IncidenceThe term "hydranencephaly" is agreed to describe an anomaly of the central nervous system consisting of nearly complete absence of the cerebral hemispheres but without anomaly of the covering layers. It has been called a destructive lesion by de Myer 7, and it has to be differentiated from disorders of size (i.e., macrocephalus), disorders of sulcation and migration (i.e., schizencephaly), disorders of diverticulation (i.e., holoprosencephaly), and disorders of closure (i.e., cranioschisis).