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丹迪-沃克综合征患儿小脑外观与功能的关系。

Relationship between cerebellar appearance and function in children with Dandy-Walker syndrome.

作者信息

Gerszten P C, Albright A L

机构信息

Department of Neurosurgery, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, PA 15213, USA.

出版信息

Pediatr Neurosurg. 1995;23(2):86-92. doi: 10.1159/000120942.

DOI:10.1159/000120942
PMID:8555101
Abstract

In children with Dandy-Walker syndrome (DWS), the relationship between cerebellar appearance on imaging and either cerebellar function or intellect is unknown. To evaluate those relationships, we retrospectively studied 20 patients with DWS treated in our institution between 1978 and 1994. The patients were treated with ventriculoperitoneal, cystoperitoneal, or ventriculocystoperitoneal shunts. Intellectual and cerebellar function were determined from neurological and developmental testing. Cerebellar development was evaluated by measuring cerebellar and posterior fossa volumes from computed tomography images; the ratio of cerebellar size to posterior fossa size was considered to reflect cerebellar development. The cerebellar function was normal in 50% and the intellectual function in 45% of the patients. There was no correlation between cerebellar size and intellectual development or cerebellar function. There was also no correlation between the type of shunt and the subsequent cerebellar size. We conclude that there is no relationship between the cerebellar development evident on computed tomography scans and the cerebellar or the intellectual function of children with DWS. We, therefore, infer that treatment of Dandy-Walker cysts with posterior fossa shunts is unlikely to enhance the cerebellar function.

摘要

在患有丹迪-沃克综合征(DWS)的儿童中,影像学上小脑外观与小脑功能或智力之间的关系尚不清楚。为了评估这些关系,我们回顾性研究了1978年至1994年间在我们机构接受治疗的20例DWS患者。这些患者接受了脑室腹腔分流术、囊肿腹腔分流术或脑室囊肿腹腔分流术。通过神经学和发育测试来确定智力和小脑功能。通过计算机断层扫描图像测量小脑和后颅窝体积来评估小脑发育;小脑大小与后颅窝大小的比值被认为可反映小脑发育。50%的患者小脑功能正常,45%的患者智力功能正常。小脑大小与智力发育或小脑功能之间无相关性。分流类型与随后的小脑大小之间也无相关性。我们得出结论,计算机断层扫描显示的小脑发育与DWS患儿的小脑或智力功能之间没有关系。因此,我们推断用后颅窝分流术治疗丹迪-沃克囊肿不太可能增强小脑功能。

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