Human pathology

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exencephaly

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Exencephaly in an amniotic band syndrome Exencephaly in an amniotic band syndrome Exencephaly in an amniotic band syndrome Exencephaly (Etiology unknown) Exencephaly (Etiology unknown) Exencephaly (Etiology unknown)

Definition: Exencephaly is a rare malformation in which the scalp and calvarium (acrania) are absent ant the cerebral mass is protected by a thick, dura mater-like membrane.

The brain consists of a disorganized, anarchic outgrowth of nervius tissue with polymicrogyria and nodules of heterotopias.

Etiology (examples)

- amniotic band sequence (ABS)

References

- Katsanis N. Ciliary proteins and exencephaly. Nat Genet. 2006 Feb;38(2):135-6. PMID: 16444248