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.

Perfusion-based SPECT staging correlates well with clinical symptoms and may be a reliable alternative to the Suzuki staging by CA.

The sinking skin flap syndrome (SSFS) is a rare complication after a large craniectomy. Hemorrhage infarction after a cranioplasty is a very rare complication with only 4 cases to date. We report a case of the patient who underwent an autologous cranioplasty to treat SSFS that developed intracerebral hemorrhage infarction. A 20-year-old male was admitted to our emergency department with stuporous mentality. Emergent decompressive craniectomy (DC) have done. He had suffered from SSFS and fever of unknown origin (FUO) since DC. After 7 months of craniectomy, cranioplasty was done. After 1 day of surgery, acute infarction with hemorrhagic transformation involved left cerebral hemisphere. We controlled increased intracranial pressure by using osmotic diuretics, steroid and antiepileptic drugs. After 14 day of surgery, he improved neurological symptoms and he had not any more hyperthermia. Among several complication of large cranioplasty only 4 cases of intracerebral hemorrhagic infarction due to reperfusion injury has been reported. In this case, unstable autoregulation system made brain hypoxic damage and then reperfusion and recanalization of cerebral vessels resulted in intracerebral hemorrhagic infarction. 7 month long FUO was resolved by cranioplasty.