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Holoprosencephaly Type 9
HPE9

Presentation

Semilobar holoprosencephaly The basic structure of the cerebral lobes are present but are fused most commonly anteriorly and at the thalami. [radiopaedia.org]

Entire Body System

  • Disability

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Face, Head & Neck

  • Median Cleft Lip

    Most commonly associated with facial abnormalities such as cyclopia, ethmocephaly, cebocephaly, and median cleft lip. For more details see the article on alobar holoprosencephaly. [radiopaedia.org]

Treatment

Treatment and prognosis The prognosis is dependent on the type of HPE with almost all alobar and semilobar forms incompatible with extrauterine life. There may be recurrence risk for ~6% with non-chromosomal sporadic HPE. [radiopaedia.org]

Prognosis

Treatment and prognosis The prognosis is dependent on the type of HPE with almost all alobar and semilobar forms incompatible with extrauterine life. There may be recurrence risk for ~6% with non-chromosomal sporadic HPE. [radiopaedia.org]

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