Neurocranial morphology and growth in Williams syndrome
Neurocranial morphology and growth in Williams syndrome
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DOI：
10.1093/ejo/cjh065
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发表时间：
2005-02-01
影响因子：
2.6
通讯作者：
Storhaug, K
中科院分区：
文献类型：
--
作者：
Axelsson, S;Kjær, I;Storhaug, K
威廉姆斯综合征（WS）是一种罕见的先天性神经发育障碍，具有独特的面部特征，心血管异常，身材矮小，智力低下，本研究的目的是描述62例WS患者的颅神经形态和生长情况，并对颅神经进行侧位X线片分析，与纵向数据的颅神经标准进行比较来自奥斯陆大学颅面生长档案。WS受试者脑颅的大小和形态与对照组不同。女性作为一个群体比男性表现出更大的差异。WS雄性和雌性动物的后颅底长度较短，WS雌性动物的前颅底长度较短，而WS雄性动物组的前颅底长度接近正常。然而，颅底角与对照组无差异。WS雄性和雌性顶骨的上级方面均观察到变平。在脑颅的后部，枕骨的突起大于对照组，这也反映在脑颅的总长度更大。额骨和枕骨的厚度明显大于对照组。WS受试者脑颅的异常大小和形态在最年轻的年龄组已经建立，并在整个观察期间保持。WS受试者脑颅的生长模式似乎与对照组相似，除了少数个体。
Williams syndrome (WS) is a rare congenital neurodevelopmental disorder with distinctive facial features, cardiovascular abnormalities, short stature, mental retardation, and behaviour and cognitive characteristics.The aim of this study was to describe the neurocranial morphology and growth in a group of 62 individuals with WS. The neurocranium was analysed on lateral cephalograms and comparisons were made with neurocranial standards from longitudinal data derived from the Oslo University Craniofacial Growth Archive.The size and morphology of the neurocranium in WS subjects differed from controls. Females as a group showed greater differences than males. The posterior cranial base length was shorter in both WS males and females, and the anterior cranial base length was shorter in WS females whereas it was close to normal in the WS male group. The cranial base angle was, however, not different from the control groups. A flattening was seen in the superior aspect of the parietal bone in both WS males and females. In the posterior part of the neurocranium, the prominence of the occipital bone was larger than in the control groups, which was also reflected in a larger total length of the neurocranium. The thickness of the frontal and occipital bones was considerably greater than in the control group. The deviant size and morphology of the neurocranium in WS subjects was already established in the youngest age group and maintained throughout the observation period.The growth pattern of the neurocranium in WS subjects seemed to be similar to that of the control groups, except in a few individuals.