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Acrania is a non-NTD congenital disorder related to the CNS. It can transform into anencephaly through the acrania\u2013exencephaly\u2013anencephaly sequence (AEAS). In AEAS, the cerebral tissue is not protected and is gradually destroyed due to exposure to the harmful effect of amniotic fluid and mechanical injuries. These lead to exencephaly and then into anencephaly. In contrast to primary anencephaly (NTDs), this type of anencephaly authors suggests calling secondary anencephaly.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Objective<\/jats:title>\n<jats:p>Analysis of the known prenatal ultrasonography (US) signs associated with AEAS. Simultaneously, the authors propose a new sign in the differentiation of acrania from exencephaly and anencephaly, called the \u201cberet\u201d sign.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>It is a two-centre retrospective observational study. As part of the study, 4060 US scans were analyzed.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>In 10 cases, the absence of calvarium was diagnosed, allowing recognition of either AEAS stages or primary anencephaly. In 5 cases, cerebral structures were enclosed by an inertial rippled thin membrane, with a smooth outer contour. Between the described membrane and the brain structures, a thin anechoic space corresponding to cerebrospinal fluid was observed. This sign was defined as the \u201cberet\u201d sign. In these cases, acrania was diagnosed. In three cases calvarium was missing. The brain structures had an irregular appearance, did not wave and remained motionless. The outer contour was unequal as if divided into lobes. Amniotic fluid was anechoic. Exencephaly was diagnosed in these cases. In two cases calvarium, brain structures, and meninges were missing. The \u201cfrog eyes\u201d sign and slightly echogenic amniotic fluid were visible. In this case, anencephaly was diagnosed.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>The \u201cberet\u201d sign seems to be a promising tool in the diagnosis of acrania. Furthermore, echogenicity of amniotic fluid could be useful during differentiation between primary and secondary anencephaly.<\/jats:p>\n<\/jats:sec>","DOI":"10.1007\/s00404-020-05650-y","type":"journal-article","created":{"date-parts":[[2020,6,17]],"date-time":"2020-06-17T18:04:11Z","timestamp":1592417051000},"page":"619-628","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["The role of the \u201cberet\u201d sign and other markers in ultrasound diagnostic of the acrania\u2013exencephaly\u2013anencephaly sequence stages"],"prefix":"10.1007","volume":"302","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3582-067X","authenticated-orcid":false,"given":"Piotr","family":"Szkodziak","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaros\u0142aw","family":"Krzy\u017canowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arkadiusz","family":"Krzy\u017canowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filip","family":"Szkodziak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u0142awomir","family":"Wo\u017aniak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piotr","family":"Czuczwar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Kwa\u015bniewska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomasz","family":"Paszkowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,6,15]]},"reference":[{"key":"5650_CR1","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1515\/pterid-2013-0028","volume":"24","author":"T Kitova","year":"2013","unstructured":"Kitova T, Milkov D, Kitov B et al (2013) Demographic factors and associated anomalies in fetuses with neural tube defects. 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