PHACE syndrome is a neurocutaneous syndrome characterized by the association of large cutaneous hemangiomas and the cardiac and cerebral vascular anomalies. We report a 6-year-old female with PHACE syndrome presented with left facial hemangiomas, cystic lesion in the cerebral posterior fossa, coarctation of the aorta, aplasia of the left vertebral artery and stenosis of the left internal carotid artery. Surgical repair of the aorta with left heart bypass under beating heart was scheduled. We monitored regional cerebral oxygen saturation (rSO2) with infrared spectroscopy in order to detect cerebral hypoperfusion. A decrease of rSO2 ipsilateral to the cerebrovascular anomalies occurred during anastomosis of the aorta, which was treated by reducing the flow rate of left heart bypass and by increasing the inhalational oxygen concentration. As children with PHACE syndrome are frequently accompanied with cerebrovascular anomalies and at a risk of cerebral hypoperfusion, prevention of cerebral hypoperfusion is crucially important during general anesthesia.
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