Objective Moyamoya vasculopathy associated with skull base
tumors has been rarely reported except for partially removed
pituitary adenoma and
craniopharyngioma. Only a single case of
meningioma resulted in vast
cerebral infarction. Surgical treatment carries a high risk of damage to the compensatory collateral circulation through leptomeningeal anastomosis leading to a devastating outcome. Case presentation A 46-year-old woman presented to our hospital with progressive visual disturbance. She had a history of medically treated moyamoya vasculopathy. Head magnetic resonance imaging revealed a well-demarcated skull base
tumor extending from the
planum sphenoidale to the diaphragm sellae. The patient had no cerebral ischemic symptoms, but
iodine-123 N-isopropyl-p-iodoamphetamine single-photon emission computed tomography indicated bilateral hemodynamic compromise. Modified extended transsphenoidal surgery was performed, resulting in successful removal without complications including
cerebral ischemia. Conclusion The very low tolerance to
cerebral ischemia associated with
moyamoya disease manipulation in this location carries an extremely high risk of
cerebral infarction. This is the first report of a basal
meningioma associated with
moyamoya syndrome that was successfully removed. The modified extended transsphenoidal approach is the first-line surgery for skull base
meningioma located in the para-midline region.