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Herein, we report a case of microhydranencephaly that was diagnosed and distinguished from anencephaly prenatally.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Case presentation<\/jats:title>\n<jats:p>The patient was an 18-year-old woman, 2 gravida nullipara, who presented at 15 weeks of gestation. Ultrasonography showed a normal biparietal diameter (BPD) and no major anomalies. At 23 weeks of gestation, an ultrasound examination revealed a BPD of 40\u00a0mm (-5.3 standard deviation, SD). At 29 weeks, anencephaly was suspected despite difficulty in visually examining the fetal head above the orbit. At 34 weeks, insertion of a metreurynter made it possible to observe the skull. Three-dimensional computed tomography (CT) and magnetic resonance imaging (MRI) confirmed the presence of the fetal skull, a prominent occipital bone, sloping forehead, marked microcephaly, cerebral loss, and excess cerebrospinal fluid. This allowed differentiation between microhydranencephaly and anencephaly. She delivered vaginally at 37 weeks, and the child had a birth weight of 2342\u00a0g and a head circumference of 24\u00a0cm (-5.4 SD). The baby\u2019s head was flat above the forehead, with a suspected partial head defect. The baby received desmopressin acetate due to central diabetes insipidus 6\u00a0months after birth.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>The use of multiple imaging modalities and physical manipulation of the fetal head are required to accurately differentiate between microhydranencephaly and anencephaly.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s12884-020-03400-1","type":"journal-article","created":{"date-parts":[[2020,11,11]],"date-time":"2020-11-11T14:03:09Z","timestamp":1605103389000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Prenatal diagnosis of fetal microhydranencephaly: a case report and literature review"],"prefix":"10.1186","volume":"20","author":[{"given":"Takahiro","family":"Omoto","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0955-4248","authenticated-orcid":false,"given":"Toshifumi","family":"Takahashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keiya","family":"Fujimori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shogo","family":"Kin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,11,11]]},"reference":[{"issue":"2","key":"3400_CR1","doi-asserted-by":"publisher","first-page":"509","DOI":"10.1002\/ajmg.1320170213","volume":"17","author":"LJ Russell","year":"1984","unstructured":"Russell LJ, Weaver DD, Bull MJ, Weinbaum M. 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