Alobar holoprocencephaly

Case contributed by Doaa Khedr Mohamed Khedr
Diagnosis almost certain

Presentation

Abnormal prenatal US with a small head circumference

Patient Data

Age: 5 days
Gender: Female
ct

Non-contrast CT brain demonstrates a single midline mono ventricle with absent lateral and third ventricle. Fused cerebral cortex with cup shape appearance. Absent falx cerebri, corpus callosum and septum pellucidum. A large interhemispheric cyst (dorsal cyst of holoprosencephaly).

Case Discussion

This case shows features of alobar holoprosencephaly.

Alobar holoprosencephaly is the most severe subtype of holoprosencephaly. It may be associated with craniofacial abnormalities like cyclopia, hypertelorism, and mononostril.

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