Presentation
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Patient Data
Almost complete absence of cerebral hemisphere. Hemicranium is filled with CSF.
Residual tissues of frontal, anteromedial temporal lobes, brainstem and cerebellum are preserved.
Note the presence of falx.
Midline subgaleal haematoma.
Case Discussion
Differential diagnosis of hydranencephaly includes severe open lip schizencephaly, alobar holoprosencephaly and severe hydrocephalus. In case of severe open lip schizencephaly, the focal cortical defect is outlined by polymicrogyric cortex. Alobar holoprosencephaly is characterised by monoventricle, absence of midline structure (falx, septum pellucidum, corpus callosum) and residual cortical tissue has pancake like morphology fused across midline anteriorly. While in case of severe hydrocephalus, thin cortical mantle is notable.
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