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腭前转位作为无脑儿畸形序列的一部分。

Palatal anteversion as part of the iniencephaly malformation sequence.

作者信息

Wilson W G, Randall M E, Babler W J

出版信息

J Craniofac Genet Dev Biol. 1985;5(1):5-10.

PMID:3988890
Abstract

We describe a fetus with hydrocephalus and the cranial and cervical findings of iniencephaly (enlarged foramen magnum, fusion of the upper cervical vertebrae, and a retroflexed, webbed neck) who had an unusual palatal abnormality ("palatal anteversion"). The posterio-lateral border of the secondary palate arose at the oral commissures, giving the palate an appearance of being folded so that the uvula was directed anteriorly. There were no clefts. Histologic sections of the junction of the secondary palate with the inner aspect of the oral commissures revealed continuity of the epithelial basement membranes and no unusual disarray of collagen fibers. This indicates that the unusual palatal orientation occurred during palatal formation and was not due to adhesion formation later in development. Failure of rotation of the palatal shelves prior to fusion and merging could account for the observed findings.

摘要

我们描述了一名患有脑积水以及无脑畸形的颅骨和颈椎表现(枕骨大孔扩大、上颈椎融合、颈部后屈且有蹼)的胎儿,其存在一种不寻常的腭部异常(“腭前翻”)。继发腭的后外侧边界起始于口角,使腭部呈现出折叠的外观,以至于悬雍垂朝前。没有腭裂。继发腭与口角内侧连接处的组织学切片显示上皮基底膜连续,胶原纤维无异常紊乱。这表明这种不寻常的腭部方向是在腭部形成过程中出现的,并非发育后期粘连形成所致。腭板在融合和合并之前未能旋转可能解释了所观察到的结果。

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