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Holoprosencephaly (HPE) is a severe and complex congenital brain malformation caused by a defect in the midline cleavage of the prosencephalon during early embryonic development. It is the most common prosencephalic malformation in humans and is categorized into three classical forms based on the severity of this cleavage defect: alobar, semilobar, and lobar HPE. A milder interhemispheric variant, called syntelencephaly, is also considered a form of HPE.

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Holoprosencephaly (HPE) is a developmental defect that affects the brain structure due to failure or incomplete division of the prosencephalon at the third week to the fourth week of gestation into cerebral hemispheres. Although the exact cause of HPE remains unclear, it is suspected to result from a combination of genetic and environmental factors. We report a case of alobar HPE.

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Article Synopsis
  • * Three main types of HPE are identified based on severity: lobar, semilobar, and alobar, with additional variants like syntelencephaly and septopreoptic.
  • * The review emphasizes a practical imaging method to diagnose HPE disorders, aiming to improve early detection and offer better support and counseling for families during prenatal care.
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Key Clinical Message: This case of alobar holoprosencephaly and cyclopia emphasizes the value of prenatal check-ups, particularly in low-income countries. Early ultrasound diagnosis leads to early gestational termination, preventing psychological trauma for the parents.

Abstract: Alobar holoprosencephaly is a rare-occurrence malformation with a bad prognosis linked to cyclopia, the most severe cranial feature.

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