Congenital hydrocephalus associated with anomalies of midline telencephalic structures. A case report

Hydrocephalus is classified into communicating, if cerebrospinal fluid (CSF) can flow freely from the ventricle to the subarachnoid space, and into non-communicating, if it cannot. The cause of hydrocephalus is diverse, either congenital or acquired conditions causing obstruction to the flow of CSF. The location of the lesion is more important than the size or nature of the lesion. We describe an unusual case of congenital communicating hydrocephalus associated with multiple malformations of midline telencephalic structures. This was a premature male baby of 30 weeks gestational age. The pregnancy was terminated after revealing a hydrocephalus by ultrasonography. The cerebral hemispheres showed marked thinning of parenchyme with dilated lateral ventricles, communication of lateral and third ventricles with dilated foramina of Monro, absence of the septum pellucidum, and hypoplasia or focal agenesis of posterior portion of corpus callosum with dorsal dilation of the third ventricle. The right fornix appeared as a single thick midline cord in its approximately normal position and the hippocampi were poorly developed, especially in the left side. The left fornix was rudimentary.