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

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.

The Efficacy of Single Barrel Superficial Temporal Artery-middle Cerebral Artery Bypass in Treatment of Adult Patients with Ischemic-type Moyamoya Disease

“…Various types of surgery have been tried since surgical treatment for the MMD began in our institution, and large number of postoperative neurological morbidity have been experienced in the early period of treatment for MMD. The indirect procedure alone potentially decreases CBF in the acute period after the surgery 5)11)20). In the combined revascularization procedure, the immediate increase in CBF from direct bypass probably compensates for the detrimental effect induced by such as indirect procedure, but not perfect.…”

The frequency of postoperative stroke in moyamoya disease following combined revascularization: a single-university series and systematic review

“…10 As a result, patients with severe hemodynamic compromise potentially develop global ischemia postoperatively. 56 Postoperative stroke primarily developed in the adult patients in the present study. The cause of ischemic stroke is partly attributed to progressive occlusion in the main cerebral arteries, as described in previous studies.…”

Progress on Complications of Direct Bypass for Moyamoya Disease

“…MMD postoperative ischemic complications are closely related to hemodynamic instability. Many studies have found that several factors, such as hypercapnia, hypotension and inadequate hematocrit, increase the risk of postoperative ischemia 66 , 67 . In 1997, Sato et al have identified 3 specific risk factors, including the presence of a preoperative low-density area, intraoperative urinary output, and reduced hematocrit 68 .…”