Figure 2.

Figure 2.

Semilobar HPE

A. MRI showing semilobar HPE. Note fusion of the frontal lobes, but presence of some septation posteriorly with presence of a falx and interhemispheric fissure (red/thin arrow). The splenium of the corpus callosum is present but more anterior portions are usually absent. A small, partially formed third ventricle is seen. More significant fusion of anterior brain structures (cortex, basal ganglia, thalamus) persists in this variant (blue/thick arrows). A dorsal cyst may be seen. In mild cases, lack of frontal horn development distinguishes this from the lobar type.

B. Note microcephaly, closely spaced eyes, depressed nasal ridge with cleft lip.

Gropman & Muenke [2005] Management of Genetic Syndromes. Copyright John Wiley & Sons Limited. Reproduced with permission.

From: Holoprosencephaly Overview

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