Presentation
An infant patient presented with macrocephaly, to rule out hydrocephalus.
Patient Data
Most of the cerebral hemispheres are replaced by fluid signal intensity, with few residual islands of tissue seen at the occipital poles, with normal thalami and posterior fossa.
The falx cerebri is present.
Case Discussion
Hydranencephaly is a rare disorder characterized by bilateral near total absence of both cerebral hemispheres and basal ganglia, sparing the thalami and posterior fossa.
The most common etiology is bilateral occlusion of supra clinoid part of both internal carotid arteries during the fetal life (mainly the second trimester).
Differential diagnosis includes: severe hydrocephalus, alobar holoprosencephaly and severe bilateral open lip schizencephaly.
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