J Korean Neurosurg Soc.  2011 Sep;50(3):256-259. 10.3340/jkns.2011.50.3.256.

Unpredictable Postoperative Global Cerebral Infarction in the Patient of Williams Syndrome Accompanying Moyamoya Disease

• Sim YW
• Lee MS
• Kim YG
• Kim DH

• KMID: 1963620
• DOI: http://doi.org/10.3340/jkns.2011.50.3.256

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.

Keyword

Moyamoya disease; Williams syndrome; Postoperative infarction; Ischemic complication

MeSH Terms

Angiography
Aortic Coarctation
Brain
Brain Death
Cardiovascular Diseases
Carotid Artery, Internal
Cerebral Infarction*
Child
Connective Tissue
Constriction, Pathologic
Humans
Infarction
Magnetic Resonance Imaging
Male
Monitoring, Physiologic
Moyamoya Disease*
Paresis
Pupil
Williams Syndrome*