Abnormal CSP

Holoprosencephaly derives from failure of separation of the cerebral hemispheres. In the most severe forms there is one undivided cerebral mass that contains a crescent shaped rudimentary ventricular cavity. In these cases, severe cranio-facial anomalies (cyclopia, hypotelorism, median cleft face) are associated.

Absence of the septum pellucidum is found with many cerebral malformations, including holoprosencephaly, agenesis of corpus callosum, ventriculomegaly, open spina bifida, cortical malformations. It may be an isolated abnormality and in this case the cerebral anatomy is unremarkable but for fusion of the frontal horns.

Agenesis of the corpus callosum may be either complete or partial. Intracranial anatomy is variable. Other intracranial anomalies are frequently encountered including ventriculomegaly, cysts, lipomas. It may be a part of genetic and genetic syndromes. The prognosis is uncertain.

Septo-optic dysplasia (SOD), also known as De Morsier syndrome, is a rare heterogenous condition that affects 1 in 10,000 live births.