An Unusual First-Trimester Ultrasound Presentation of the Acrania-Anencephaly Sequence: The "Turkish Turban" Sign

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:
Abortion, Eugenic
Anencephaly - diagnostic imaging
Anencephaly - embryology
Brain - diagnostic imaging
Brain - embryology
Fatal Outcome
Female
Humans
Pregnancy
Pregnancy Trimester, First
Skull - diagnostic imaging
Skull - embryology
Ultrasonography, Prenatal - methods
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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