Ectopia cordis and acrania

W. Adrian Clavelli, MD,

This fetus presents with the uncommon association of an ectopia cordis and acrania. Two other such cases have been reported but in the context of Pentalogy of Cantrell. The ectopia cordis is well demonstrated in these images,

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...and this is the acrania. Note that the presence of acrania in the 3rd trimester is in opposition to the commonly suggested evolution of acrania into anencephaly. Acrania results from a failure of mesoderm migration while anencephaly is a neural tube defect

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And the confirmation with prenatal MRI.

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Reference:

Bognoni V, Quartuccio A, Quartuccio A.

First-trimester sonographic diagnosis of Cantrell"s pentalogy with exencephaly.J Clin UltrasoundĀ  1999 Jun;27(5):276-8

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