Friday 2 November 2012


Holoprocencephaly with an associated fused metopic suture /single frontal "plate" of bone. 
Formerly called arrhinencephaly. 
Best diagnostic clue is Monoventricle + fused (uncleaved) frontal lobes ~ absent anterior midline falx/fissures and Anteriorly displaced Sylvian fissures. 
Associated with Hypotelorism, fused metopic suture /single frontal "plate" of bone, Azygous / absent anterior cerebral artery (ACA) on MR Angio.

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