Abstract：
:We report a rare case of Williams syndrome accompanying moyamoya disease in whom postoperative global cerebral infarction occurred unpredictably. Williams syndrome is an uncommon hereditary disorder associated with the connective tissue abnormalities and cardiovascular disease. To our knowledge, our case report is the second case of Williams syndrome accompanying moyamoya disease. A 9-year-old boy was presented with right hemiparesis after second operation for coarctation of aorta. He was diagnosed as having Williams syndrome at the age of 1 year. Brain MRI showed left cerebral cortical infarction, and angiography showed severe stenosis of bilateral internal carotid arteries and moyamoya vessels. To reduce the risk of furthermore cerebral infarction, we performed indirect anastomosis successfully. Postoperatively, the patient recovered well, but at postoperative third day, without any unusual predictive abnormal findings the patient's pupils were suddenly dilated. Brain CT showed the global cerebral infarction. Despite of vigorous treatment, the patient was not recovered and fell in brain death one week later. We suggest that in this kind of labile patient with Williams syndrome accompanying moyamoya disease, postoperative sedation should be done with more thorough strict patient monitoring than usual moyamoya patients. Also, we should decide the revascularization surgery more cautiously than usual moyamoya disease. The possibility of unpredictable postoperative ischemic complication should be kept in mind.
journal_name
J Korean Neurosurg Socjournal_title
Journal of Korean Neurosurgical Societyauthors
Sim YW,Lee MS,Kim YG,Kim DHdoi
10.3340/jkns.2011.50.3.256subject
Has Abstractpub_date
2011-09-01 00:00:00pages
256-9issue
3eissn
2005-3711issn
1598-7876journal_volume
50pub_type
abstract：:Chronic subdural hematoma (SDH) is a well-known disease entity and is traditionally managed with surgery. However, when associated with spontaneous intracranial hypotension (SIH), the treatment strategy ought to be modified, as classical treatment could lead to unwanted consequences. A 59-year-old man presented with a...
journal_title：Journal of Korean Neurosurgical Society
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journal_title：Journal of Korean Neurosurgical Society
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journal_title：Journal of Korean Neurosurgical Society
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journal_title：Journal of Korean Neurosurgical Society
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journal_title：Journal of Korean Neurosurgical Society
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pub_type： 杂志文章
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journal_title：Journal of Korean Neurosurgical Society
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doi：10.3340/jkns.2014.1112.008
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journal_title：Journal of Korean Neurosurgical Society
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章,评审
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pub_type： 杂志文章
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
doi：10.3340/jkns.2012.51.4.208
更新日期：2012-04-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type：
doi：10.3340/jkns.2010.47.2.158
更新日期：2010-02-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type：
doi：10.3340/jkns.2007.42.5.410
更新日期：2007-11-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
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更新日期：2008-06-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
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更新日期：2015-02-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
doi：10.3340/jkns.2013.54.6.484
更新日期：2013-12-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type：
doi：10.3340/jkns.2011.49.5.302
更新日期：2011-05-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type：
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journal_title：Journal of Korean Neurosurgical Society
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
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更新日期：2010-11-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
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journal_title：Journal of Korean Neurosurgical Society
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journal_title：Journal of Korean Neurosurgical Society
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更新日期：2014-12-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章,评审
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更新日期：2015-06-01 00:00:00
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journal_title：Journal of Korean Neurosurgical Society
pub_type： 杂志文章
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