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不对称联体三肢畸形胎儿的非侵入性研究,具有颅荐联体、双头联体和颅多头联体的特征。

Noninvasive investigation of asymmetrically conjoined tripus twins with features of rachipagus, parapagus dicephalus, and cephalopagus.

机构信息

Department of Anatomy and Cell Biology, Queen's University, Kingston, Ontario, Canada.

出版信息

Clin Anat. 2012 Nov;25(8):1023-9. doi: 10.1002/ca.21149. Epub 2011 Mar 23.

Abstract

A hypothetical mechanism for conjoined twinning postulated by Spencer ([2003] Developmental Malformations and Clinical Implications, Baltimore: Johns Hopkins University Press, p 1-476) suggests that, after separation, monovular twins fuse in one of eight predictable homologous sites. The tripus fetal specimen under study embodies characteristics of three types therefore preventing it from classification into a simple variant of any one of the eight twin types described by Spencer. The aim of this study was to reveal internal structural anomalies of the fetal specimen by using magnetic resonance imaging and computerized tomography. Dorsally appended to the primary twin is a secondary head mass (brain tissue and ocular globe) and two spinal columns converging at T4/T5, suggesting rachipagus twinning. The ventral orientation of the secondary twin's (right lateral) lower limb suggests parapagus twinning. The caudal divergence of the spinal columns and the presence of a secondary hemipelvis, separate from the primary pelvis, suggest cephalopagus twinning. Measurements of the long bones indicate a gestational age of ∼20-23 weeks. Secondary malformations of the primary fetal body include anencephaly, cleft palate, renal agenesis, decreased left ventricular outflow, and a prematurely terminating descending aorta. This study demonstrates the possibility of using current imaging techniques to study very old, formalin-preserved human material for documentation and scientific discussion without destroying the specimen, thus keeping it intact for posterity.

摘要

斯宾塞([2003]发育性畸形与临床意义,巴尔的摩:约翰霍普金斯大学出版社,第 1-476 页)提出了一种联体双胎的假设机制,认为在分离后,单卵双胞胎在八个可预测的同源部位中的一个部位融合。本研究中研究的三体胎儿标本具有三种类型的特征,因此无法将其归类为斯宾塞描述的八种双胞胎类型中的任何一种简单变体。本研究旨在通过磁共振成像和计算机断层扫描揭示胎儿标本的内部结构异常。初级双胞胎的背部附有一个次级头部肿块(脑组织和眼球)和两个在 T4/T5 处汇聚的脊柱,提示为颅联胎。次级双胞胎(右侧外侧)下肢的腹侧取向提示为并肢畸形。脊柱的尾部分叉和次级半骨盆的存在,与原发性骨盆分开,提示为头联胎。长骨的测量表明胎龄约为 20-23 周。初级胎儿身体的继发性畸形包括无脑畸形、腭裂、肾发育不全、左心室流出道减少以及过早终止的降主动脉。本研究表明,目前的成像技术有可能用于研究非常古老的、福尔马林保存的人体材料,以便在不破坏标本的情况下进行记录和科学讨论,从而使其完整地留给后代。

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