Acrania: an autopsy case and review of the literature

Y. Shinmura, Y. Tsutsui, T. Nishiguchi, Takao Kobayashi
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Abstract

ABSTRACT We report an autopsy case of acrania with a review of the literature. A 31‐year‐old woman was admitted in poor genaral condition and diagnosed as pregnant at the 26th week of gestation. The fetus was suspected to be anencephalic or hydrocephalic by ultrasonography. She delivered a stillbirth male baby at the 28th week of gestation. Autopsy showed the cranium lacked the cranial vault The scalp directly covered the hydrocephalic brain without calvaria. No cerebellum was found macroscopically. On histological examination, the thinned cerebral layer was composed of white matter‐like brain tissue with glial and ependymal cells. In the cerebral layer near the base of the skull, a few NSE‐positive neurons among many GFAP‐positive glial cells and oligodendrocyte‐like cells were observed. The choroid plexuses were observed inside the ependymal layers. The brain stem and the spinal cord were normally preserved with clusters of NSE‐positive neurons. Agnethia and horseshoe kidney were combined as malformations other than the brain. We reviewed 42 cases of acrania from the literature.
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阿克兰尼亚:一例尸检病例及文献回顾
我们报告了一例颅骨畸形的尸检病例,并对相关文献进行了回顾。一名31岁妇女入院,一般情况较差,在妊娠26周时被诊断为怀孕。超声检查怀疑胎儿为无脑或脑积水。她在怀孕28周时产下一个死胎男婴。尸检显示颅骨缺乏颅顶,头皮直接覆盖脑积水,无颅骨。肉眼未见小脑。在组织学检查中,变薄的脑层由白质样脑组织和神经胶质细胞和室管膜细胞组成。在颅底附近的脑层中,在许多GFAP阳性的胶质细胞和少突胶质细胞样细胞中观察到少量NSE阳性神经元。室管膜层内可见脉络膜丛。脑干和脊髓正常保存有NSE阳性神经元簇。阿涅西亚和马蹄肾被合并为除脑外的畸形。我们从文献中回顾了42例颅脑损伤病例。
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