Gastroschisis is a defect of the Umbilical ring: Evidence from Morphological evaluation of stillborn fetuses
Gastroschisis is a defect of the Umbilical ring: Evidence from Morphological evaluation of stillborn fetuses
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DOI：
10.1002/bdra.23130
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发表时间：
2013-04-01
影响因子：
--
通讯作者：
Mazzitelli, Nancy
中科院分区：
文献类型：
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作者：
Rittler, Monica;Vauthay, Liliana;Mazzitelli, Nancy
背景腹裂(GS)通常被描述为一种腹壁缺陷，位于正常插入的脐带的右侧，没有被挤出的器官的膜状覆盖。然而，文献中缺乏精确的解剖学描述。我们的目的是提供证据，允许重新考虑其当前的定义，以及对产前死亡的解释，基于对患有GS的死胎的详细观察和文献回顾。方法描述5例孤立性GS死胎的产前检查、临床检查和组织学表现，并提供影像证据。结果5例中，脐带仅附着在脐环的左侧，右侧未被遮盖，可摘除腹部脏器。组织学证据表明，在环的游离边缘有粘液样组织，表明脊髓最初是在该位置插入的，后来又分离了。脐环的特征、肠管扩张和急性窒息的尸检结果有力地支持脐带血管受压是胎儿死亡的原因。结论基于这些发现，在文献中缺乏证据表明缺陷与脐带之间的全层腹壁分离，以及对支持缺陷与脐环分离假说的机制的批判性综述，我们认为GS代表着脐带与脐环之间正常附着的失败。产前死亡的胎儿的临床病程一致，表明在妊娠晚期进行仔细的靶向监测。出生缺陷研究(A部分)97：198209,2013年。(C)2013年威利期刊公司。
BACKGROUND Gastroschisis (GS) is usually described as an abdominal wall defect, to the right of a normally inserted umbilical cord, without membraneous covering of the extruded organs. However, precise anatomical descriptions are lacking in the literature. Our aims were to provide evidence that allows reconsideration of its current definition, as well as an explanation for prenatal death, based on detailed observation of stillborn fetuses with GS and a review of the literature. METHODS Prenatal studies, clinical examinations, and histological findings of five stillborn fetuses with isolated GS are described and photographic evidence is provided. RESULTS In all five cases, the umbilical cord was only attached to the left side of the umbilical ring, while the right side remained uncovered, allowing evisceration of abdominal organs. Histological evidence of mucoid-like tissue at the free border of the ring suggests that at that site the cord was initially inserted and later detached. Characteristics of the umbilical ring, bowel dilatation, and autopsy findings of acute asphyxia strongly support compression of umbilical vessels as the cause of fetal death. CONCLUSIONS Based on these findings, on the lack of evidence in the literature demonstrating full-thickness abdominal wall separating the defect from the umbilical cord, and on a critical review of the proposed mechanisms favoring the hypothesis of a defect separate from the umbilical ring, we propose that GS represents a failure in the normal attachment between umbilical cord and umbilical ring. The consistent clinical course of fetuses with prenatal demise suggests careful targeted monitoring during late gestation. Birth Defects Research (Part A) 97:198209, 2013. (c) 2013 Wiley Periodicals, Inc.