An Unusual First‐Trimester Ultrasound Presentation of the Acrania‐Anencephaly Sequence

A previously unrecognized first‐trimester presentation of the acrania‐anencephaly sequence is described. Ultrasound features included a constriction ring around the external base of the developing skull and an enlarged globular head, resembling a Turkish turban, with large cystic spaces replacing th...

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Bibliographic Details
Published in:Journal of ultrasound in medicine 2020-04, Vol.39 (4), p.829-832
Main Authors: Sepulveda, Waldo, De La Maza, Francisco, Meagher, Simon
Format: Article
Language:English
Subjects:
acrania
acrania‐anencephaly sequence
amniotic band syndrome
first‐trimester ultrasound
open neural tube defect
prenatal diagnosis
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Description
Summary:A previously unrecognized first‐trimester presentation of the acrania‐anencephaly sequence is described. Ultrasound features included a constriction ring around the external base of the developing skull and an enlarged globular head, resembling a Turkish turban, with large cystic spaces replacing the brain. This constellation of findings was noted in 3 first‐trimester fetuses. In 2 of them, it was possible to identify the amniotic membrane attached to the constriction ring. One case presented with anencephaly and fetal demise at 16 weeks. The other 2 women terminated the pregnancies and aborted anencephalic fetuses. This subtype of the acrania‐anencephaly sequence could represent an earlier segmental rupture of the amnion, which subsequently entraps the developing fetal skull.
ISSN:0278-4297
1550-9613
DOI:10.1002/jum.15161