The sequence of acrania–exencephaly–anencephaly (AEAS)- An infrequent case report

H. T. Shenoy, Najah Ismail Kunju, Naseemabeevi Ahmed Khan, Sudhya P Puthanayil, Sangeetha Ambujakshan Ambujakshan
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Abstract

Acrania–exencephaly–anencephaly sequence has an incidence of 3.6–5.4 for 10,000 live births and has been reported in literature. Exencephaly, described here is a defect of the neural tube which occurs due to the absence of closure of the neural fold. The main diagnostic ultrasound features include that are characterized by acrania, decreased size of cranial pole in comparison with the chest, irregular cranial surface, with increased amniotic fluid echogenicity due to the damaged brain tissue. Associated with amniotic band syndrome, Pentalogy of Cantrell, limb anomalies and ventral body wall defects. It is incompatible with life. Conducting programs training the budding neuro-sonographers about the knowledge in detection, diagnosis of NTD according to the Carnegie Classification is crucial to look forward in pathogenesis and application in the clinical scenario.
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无脑儿-无脑畸形(AEAS)的发病顺序--罕见病例报告
无脑畸形-无脑儿-无脑畸形序列的发病率为每万名活产婴儿中 3.6-5.4 例,已有文献报道。这里所说的无脑畸形是一种神经管缺陷,是由于神经皱襞没有闭合而导致的。主要的超声诊断特征包括:尖头畸形、颅骨与胸部相比变小、颅骨表面不规则、因脑组织受损而导致羊水回声增加。与羊膜带综合征、坎特雷尔五联症、四肢畸形和腹壁缺损有关。与生命不相容。根据卡内基分类法,对新晋神经超声技师进行有关检测和诊断 NTD 的知识培训,对于展望发病机理和临床应用至关重要。
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