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双胎胎儿中脊神经与脊髓之间连接缺失:一种非常罕见的畸形,偶尔在尸检时发现。

Absence of connections between spinal nerves and the spinal cord in a twin fetus: a very rare malformation occasionally evidenced at autopsy.

作者信息

Bussani R, Silvestri F

机构信息

Department of Pathological Anatomy, University of Trieste, Italy.

出版信息

Pathol Res Pract. 2001;197(1):47-50. doi: 10.1078/0344-0338-00007.

DOI:10.1078/0344-0338-00007
PMID:11209816
Abstract

We describe a rare case of spinal cord malformation in a dichorionic diamniotic twin fetus aborted at 20 weeks' gestation due to acute chorioamnionitis with placental dysmaturity probably caused by a maternal viral infection. At autopsy, there were no connections between the spinal nerves and the spinal cord. The spinal cord lacked the posterior median fissure and gray matter; only a few neurons were present in the anterior and lateral gray columns. No chromosome anomalies were found. Although we cannot reconstruct the pathogenetic chain of events leading to this malformation, we believe that it is correlated with a maternal viral infection. We hypothesize that this viral infection altered the delicate balance between the factors inducing and those inhibiting fetal spinal neural differentiation.

摘要

我们描述了一例罕见的脊髓畸形病例,该病例发生在一名双绒毛膜双羊膜囊双胎胎儿身上,该胎儿在妊娠20周时因急性绒毛膜羊膜炎伴胎盘发育不成熟而流产,胎盘发育不成熟可能由母体病毒感染引起。尸检时,脊神经与脊髓之间没有连接。脊髓缺乏后正中裂和灰质;仅在前侧和外侧灰质柱中有少数神经元。未发现染色体异常。尽管我们无法重构导致这种畸形的发病事件链,但我们认为它与母体病毒感染有关。我们推测这种病毒感染改变了诱导和抑制胎儿脊髓神经分化的因素之间的微妙平衡。

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