Cerebral retroflexion in holoprosencephaly developed following ventriculo-peritoneal shunting: a case report

Retroflexion of the hypoplastic fused cerebrum is a rare condition in holoprosencephaly. We describe a new case of this entity in a 7-year-old boy with alobar holoprosencephaly whi developed retroflexion of the holosphere 7 years after a ventriculo-peritoneal shunt was placed. The radiological findings revealed retroflexion of the cerebrum anchored to the cranial vault between the precerebral space and the dorsal sac, which were open to each other. In patients with holoprosencephaly, the possibility of retroflexion of the holosphere should be considered after placement of a ventriculo-peritoneal shunt for associated hydrocephalus.