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

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.

Ischemic Complication of Ruptured Anterior Choroidal Artery Aneurysm / 대한뇌혈관외과학회지

OBJECTIVE: Vasospasm is known to play the key role in determining the prognosis of aneurysmal subarachnoid hemorrhage (SAH). We have experienced a higher incidence of vasospasm in the cases of SAH caused by rupture of an anterior choroidal artery (AChA) aneurysm than aneurysms of other area. The purpose of this study is to analyze the ischemic complications in patients with a ruptured AChA aneurysm. METHODS: We retrospectively reviewed 13 patients who were treated for ruptured AChA aneurysm from 1994 to 2007 at our hospital. The prognosis and complications were analyzed based on the institution's data, gender, age, the Hunt and Hess grade, the Glasgow coma scale, the Glasgow outcome scale, the symptoms and neurological examinations, the medical treatment, the digital subtraction angiography (DSA), the computed tomography (CT) and the magnetic resonance imaging (MRI). RESULTS: Forty-one (6%) out of 678 SAH patients had ischemic complications. Thirteen cases (2%) were caused by rupture of an anterior choroidal artery (AChA) aneurysm and 3 of these 13 cases (23.1%) had ischemic complications. Two of these 3 cases (15.4%) had neurological abnormality. Twelve out of the 13 AchA aneurysms had the saccular form. CONCLUSION: In cases of SAH from ruptured AchA aneurysm, the probability of ischemic complications was higher than that of general aneurysmal SAH. This seems to be due to the thin AChA and that the vascular territory of AChA is the area where ischemic brain damage can occur even by a mild vasospasm. So, when operating on a AChA aneurysm, maximum effort should be done to preserve the AChA. However, in the exceptional case with multiple AChAs, one AChA can be trapped without a definitive neurological deficit.

Ischemic Complication after Surgery for Posterior Communicating Artery Aneurysm

OBJECTIVE: The purpose of this study is to elucidate the causes of the ischemic complication after surgery for posterior communicating artery aneurysm and to represent the ways of preventing or minimizing this specific complication. METHODS: Between January 2000 and December 2001, fifty one patients were treated for the posterior communicating artery aneurysm. The medical records and neuroimaging studies of the patients were reviewed retrospectively. RESULTS: Among the series of 51 surgically treated patients, the authors experienced 6 cases of ischemic complication involving 3 thalamus, 2 posterior cerebral artery territory and 1 periventircular white matter. Four patients were treated by aneurysmal neck clipping and one patient with fusiform aneurysm and one patient with infundibulum at internal carotid-posterior communicating artery junction by parent artery trapping and parent artery occlusion, respectively. In 4 cases with aneurysmal clipping the authors could not confirm the patency of the parent artery or posterior communicating artery during operation by the obstacle such as internal carotid artery and/or aneurysmal dome. The neurological deficits developed postoperatively were improved or subside during hospital courses and all six patients showed good outcome(Glasgow Outcome Scale score V) at discharge. CONCLUSION: In most cases the posterior communicating artery itself can be separated from the neck of the aneurysm and thereby preserved to avoid a significant infarct in the thalamus and occipital lobe.